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1. Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index

Author

Carine Wouters, Nicolino Ruperto, Maria Trachana, Angelo Ravelli, Veronika Vargová, Troels Herlin, Angela Pistorio, Pavla Dolezalova, Claudia Bracaglia, Alessandro Consolaro, Silvia Rosina, Ana Rebollo-Giménez, Ana Guilaisne Bernard-Medina, and Laura Carenini

Subjects
Medicine
Abstract

Objectives To develop and validate the cut-offs in the Juvenile DermatoMyositis Activity Index (JDMAI) to distinguish the states of inactive disease (ID), low disease activity (LDA), moderate disease activity (MDA) and high disease activity (HDA) in children with juvenile dermatomyositis (JDM).Methods For cut-off definition, data from 139 patients included in a randomised clinical trial were used. Among the six versions of the JDMAI, JDMA1 (score range 0–40) and JDMAI2 (score range 0–39) were selected. Optimal cut-offs were determined against external criteria by calculating different percentiles of score distribution and through receiver operating characteristic curve analysis. External criteria included the modified Pediatric Rheumatology International Trials Organization (PRINTO) criteria for clinically ID in JDM (for ID) and PRINTO levels of improvement in the clinical trial (for LDA and HDA). MDA cut-offs were set at the score interval between LDA and HDA cut-offs. Cut-off validation was conducted by assessing construct and discriminative ability in two cohorts including a total of 488 JDM patients.Results The calculated JDMAI1 cut-offs were ≤2.4 for ID, ≤6.6 for LDA, 6.7–11 for MDA and >11 for HDA. The calculated JDMAI2 cut-offs were ≤5.2 for ID, ≤8.5 for LDA, 8.6–11.3 for MDA and >11.3 for HDA. The cut-offs discriminated strongly among disease activity states defined subjectively by caring physicians and parents, parents’ satisfaction or non-satisfaction with illness outcome, levels of pain, fatigue, physical functional impairment and physical well-being.Conclusions Both JDMAI1 and JDMAI2 cut-offs revealed good metrologic properties in validation analyses and are, therefore, suited for application in clinical practice and research.

Published
2024
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2. Looking for the best strategy to treat children with new onset juvenile idiopathic arthritis: presentation of the 'comparison of STep-up and step-down therapeutic strategies in childhood ARthritiS' (STARS) trial

Author

Marco Burrone, Marta Mazzoni, Roberta Naddei, Angela Pistorio, Maddalena Spelta, Silvia Scala, Elisa Patrone, Marco Garrone, Maria Lombardi, Luca Villa, Giulia Pascale, Roberto Cavanna, Nicolino Ruperto, Angelo Ravelli, Alessandro Consolaro, and for the Paediatric Rheumatology International Trials Organisation (PRINTO)

Subjects
Juvenile idiopathic arthritis, Anti-TNF, Treat-to target, Randomised clinical trial, Methotrexate, Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background Although a satisfactory disease control is nowadays achievable in most patients with JIA, a substantial proportion of them still do not respond adequately or reach long-term drug-free remission. According to current recommendations, treatment should be escalated in subsequent steps. A different approach is based on the assumption that the initial start of an aggressive therapy may take advantage of the “window of opportunity” and could alter the biology of the disease, leading to an improvement of long-term outcomes, including the prevention of cumulative joint damage. Objectives This randomised clinical trial aims to compare the effectiveness of a conventional therapeutic regimen, based on treatment escalation and driven by the treat-to-target approach, with that of an early aggressive intervention based on the initial start of a combination of conventional and biological DMARDs. Methods JIA patients with oligoarthritis or RF negative polyarthritis aged more than 2 years and with less than 4 months of disease course will be included in the study. Children will be randomised into two arms: patients in Step-up arm with less severe oligoarthritis will undergo an intra-articular corticosteroid injection (IACI) in all affected joints; patients with polyarthritis or severe oligoarthritis will receive IACI and methotrexate. Subsequent treatment will follow a standardised protocol based on the patients’ level of disease activity measured with the JADAS, according to a treat-to-target strategy. Patients in Step-down arm will receive a 6-month early combined treatment (methotrexate plus IACI for less severe oligoarthritis, methotrexate plus etanercept for severe oligoarthritis and polyarthritis). The primary endpoint is the frequency of achievement of the status of clinical remission (i.e. persistence of inactive disease for at least 6 months) at the 12-month visit. Safety events, physician-centred measures and parent/patient-reported outcomes will be collected through the Paediatric Rheumatology International Trials Organisation on line database. Expected results The STARS trial aims to provide important evidence supporting the first-line treatment choices in the care of children with oligoarticular and polyarticular JIA. If the superiority of an early aggressive therapy will be demonstrated, this will demand further studies on the biological definition of the window of opportunity for JIA. Trial registration The Trial is registered on the ClinicalTrials.gov registry (NCT03728478) on the 31st October 2018 and EU Clinical Trials Register on the 14th May 2018 (EudraCT Number: 2018–001931-27).

Published
2022
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3. Functional Magnetic Resonance Urography in Ureteropelvic Junction Obstruction: Proposal for a Pediatric Quantitative Score

Author

Maria Beatrice Damasio, Fiammetta Sertorio, Michela Cing Yu Wong, Irene Campo, Marcello Carlucci, Luca Basso, Lorenzo Anfigeno, Monica Bodria, Angela Pistorio, Giorgio Piaggio, Gian Marco Ghiggeri, and Girolamo Mattioli

Subjects
kidney, fMRU, CAKUT, UPJ, score, Pediatrics, RJ1-570
Abstract

Background:Ureteropelvic junction obstruction (UPJO) is one of the most frequent causes of congenital hydronephrosis. It is essential to distinguish UPJO which needs surgical treatment. fMRU combines high quality morphological details of the kidney and excretory pathways with functional data.ObjectiveThis study aims to introduce a new radiological score based on fMRU findings to be able to differentiate surgical from non-surgical kidneys.Materials and MethodsWe retrospectively selected patients with hydronephrosis due to UPJO who underwent fMRU (January 2009–June 2018). A multidisciplinary team identified a list of fMRU morpho-functional predictive variables to be included in the analysis. To evaluate the role of different independent variables in predicting the outcome, a multivariable logistic regression model has been performed; the outcome variable was the surgical intervention. For each predictive variable, Odds Ratio and 95% Confidence Intervals were calculated. The likelihood ratio test was used to assess the significance of the variables. Using the regression model, we assigned a numerical value to each predictive variable, rounding up the beta-coefficients. The cut-off value of the total score was obtained from the ROC curve analysis.ResultsA total of 192 patients were enrolled, corresponding to 200 pathological kidneys. All of them underwent fMRU; 135 were surgically treated, while 65 underwent ultrasound or MRU follow-up. Predictive variables significantly associated with surgery resulted to be the urographic phase, the presence of abnormal vessels, and a baseline anterior-posterior pelvic diameter >23 mm. Beta coefficients of the logistic regression model were then converted in scores. The ROC curve of the score showed high sensitivity (84.3%) and specificity (81.3%) with a cut-off > 2.5.ConclusionsWe propose a new fMRU score able to identify surgical vs. non-surgical kidneys with UPJO.

Published
2022
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4. Management and outcome of benign acute childhood myositis in pediatric emergency department

Author

Giacomo Brisca, Marcello Mariani, Daniela Pirlo, Marta Romanengo, Angela Pistorio, Alberto Gaiero, Chiara Panicucci, Emanuela Piccotti, and Claudio Bruno

Subjects
Children, Creatine kinase, Rhabdomyolysis, Gait abnormalities, Clinical pathway, Pediatrics, RJ1-570
Abstract

Abstract Background Benign acute childhood myositis (BACM) is a self-limited syndrome associated with viral infections characterized by symmetric lower extremity pain typically affecting school-aged children. Evolution in rhabdomyolysis and kidney damage is rarely reported. Despite this, the acute presentation commonly concerns both parents and health care providers, often leading to unnecessary workup. The aim of the study was to determine the features and outcome of a large series of children with BACM identifying a management pathway for pediatricians in Emergency Department (ED). Methods We conducted a retrospective study of patients with BACM managed in 2 Italian pediatric ED during a period of 8 and a half years. Demographic data, clinical, and laboratory results were extracted from electronic medical records. Recurrence, complications, treatments, and outcomes were also recorded. Descriptive statistics were produced for first-episode patients and for those with recurrence of myositis. A comparison between groups was performed. Results One hundred and thirteen patients with BACM were identified. Ninety-two children (65 males) had a single episode, while ten (nine males) had recurrence. The mean age at presentation was 6.0 years (range 2–13,2). All patients had normal neurological examination and no one developed myoglobinuria, or renal failure. At first evaluation median CK level was 1413 UI/l (normal values

Published
2021
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5. Tocilizumab may slow radiographic progression in patients with systemic or polyarticular-course juvenile idiopathic arthritis: post hoc radiographic analysis from two randomized controlled trials

Author

Clara Malattia, Nicolino Ruperto, Silvia Pederzoli, Elena Palmisani, Angela Pistorio, Carine Wouters, Pavla Dolezalova, Berit Flato, Stella Garay, Gabriella Giancane, Chris Wells, Wendy Douglass, Hermine I. Brunner, Fabrizio De Benedetti, Angelo Ravelli, and for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Pediatric Rheumatology Collaborative Study Group (PRCSG)

Subjects
Biologicals, Disease-modifying antirheumatic drugs (DMARDs), Tocilizumab, Systemic juvenile idiopathic arthritis, Polyarticular-course juvenile idiopathic arthritis, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background Few clinical trials have investigated the prevention of radiographic progression in children with juvenile idiopathic arthritis treated with antirheumatic drugs. This study aimed to investigate radiographic progression in patients with systemic juvenile idiopathic arthritis (sJIA) and patients with polyarticular-course juvenile idiopathic arthritis (pcJIA) treated with the anti–interleukin-6 receptor antibody tocilizumab for 2 years in the TENDER and CHERISH randomized controlled trials, respectively. Methods Standard radiographs of both wrists and both hands in the posteroanterior view were obtained within 4 weeks of baseline and were repeated at weeks 52 ± 4 and 104 ± 4 in both trials. All films were scored by two independent readers using the adapted Sharp–van der Heijde (aSH) and Poznanski scoring methods. Although the Poznanski score indicates bone growth limitation or cartilage growth decrease, which are not the same as joint space narrowing in rheumatoid arthritis, its change reflects damage to cartilage. Therefore, impairment in the Poznanski score as well as the aSH score was considered as a measure of structural joint damage. Radiographic progression was defined as worsening of radiographic scores beyond the smallest detectable difference. Results Poznanski and aSH scores were available at baseline and at one or more postbaseline time points for 33 and 47 of 112 sJIA patients and 61 and 87 of 188 pcJIA patients, respectively, providing a representative subset of the study populations. The inter-reader and intra-reader agreement intra-class correlation coefficient was > 0.8. Median baseline Poznanski and aSH scores, respectively, were − 2.4 and 24.6 for sJIA patients and − 1.5 and 8.0 for pcJIA patients. Compared with baseline, aSH scores remained stable for all sJIA patients at week 52, whereas 9.4% of sJIA patients had radiographic progression according to Poznanski scores at week 52; at 104 weeks, radiographic progression according to aSH and Poznanski scores was observed in 5.4% and 11.5%, respectively. In pcJIA patients, radiographic progression from baseline at 52 weeks and at 104 weeks was 12.5% and 2.9%, respectively, using aSH scoring and 6.5% and 4%, respectively, using Poznanski scoring. Conclusion Tocilizumab may delay radiographic progression in children with sJIA and children with pcJIA. Trial registration Trial registration numbers and dates: TENDER, NCT00642460 (March 19, 2008); CHERISH, NCT00988221 (October 1, 2009)

Published
2020
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6. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

Author

Gabriella Giancane, Joost F. Swart, Elio Castagnola, Andreas H. Groll, Gerd Horneff, Hans-Iko Huppertz, Daniel J. Lovell, Tom Wolfs, Troels Herlin, Pavla Dolezalova, Helga Sanner, Gordana Susic, Flavio Sztajnbok, Despoina Maritsi, Tamas Constantin, Veronika Vargova, Sujata Sawhney, Marite Rygg, Sheila K. Oliveira, Marco Cattalini, Francesca Bovis, Francesca Bagnasco, Angela Pistorio, Alberto Martini, Nico Wulffraat, Nicolino Ruperto, and for the Paediatric Rheumatology International Trials Organisation (PRINTO)

Subjects
Infections, Opportunistic, Juvenile idiopathic arthritis, Immunosuppressive therapy, Biologics, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. Trial registration Clinicaltrials.gov NCT 01399281; ENCePP seal: awarded on 25 November 2011.

Published
2020
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7. A prediction rule for lack of achievement of inactive disease with methotrexate as the sole disease-modifying antirheumatic therapy in juvenile idiopathic arthritis

Author

Cecilia Bava, Federica Mongelli, Angela Pistorio, Marta Bertamino, Giulia Bracciolini, Sara Dalprà, Sergio Davì, Stefano Lanni, Valentina Muratore, Silvia Pederzoli, Silvia Rosina, Benedetta Schiappapietra, Chiara Suffia, Giulia Varnier, Sara Verazza, Gabriella Giancane, Alessandro Consolaro, and Angelo Ravelli

Subjects
Juvenile idiopathic arthritis, Pediatric rheumatology, Methotrexate, Predictors, Prediction rule, Biologic therapies, Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background To investigate the frequency of achievement of inactive disease (ID) in children with juvenile idiopathic arthritis (JIA) treated with methotrexate (MTX) as the sole disease-modifyng antirheumatic (DMARD) therapy and to develop a prediction model for lack of attainment of ID. Methods The clinical charts of consecutive patients started with MTX as the sole DMARD between 2000 and 2013 were reviewed. Patient follow-up was censored at first episode of ID or, in case ID was not reached, at last follow-up visit or when a biologic DMARD was prescribed. The characteristic at MTX start of patients who achieved or did not achieve ID were compared with univariate and multivariable analyses. Regression coefficients (β) of variables that entered the best-fitting logistic regression model were converted and summed to obtain a “prediction score” for lack of achievement of ID. Results A total of 375 patients were included in the study. During MTX administration, 8.8% were given systemic corticosteroids and 44.1% intra-articular corticosteroids. After MTX start, 229 (61%) patients achieved ID after a median of 1.7 years, whereas 146 patients (39%) did not reach ID after a median of 1.2 years. On multivariable analysis, independent correlations with lack of achievement of ID were identified for the disease categories of systemic arthritis, enthesitis-related arthritis (ERA) and polyarthritis and C-reactive protein (CRP) > 1.4 mg/dl. The prediction score ranged from 0 to 3 and its cutoff that discriminated best between patients who achieved or did not achieve ID was > 0.5. The categories of systemic arthritis or ERA, both of which had a score greater than 0.5, were sufficient alone to predict a lower likelihood to reach ID. Polyarthritis and increased CRP, whose score was 0.5, assumed a predictive value only when present in association. Conclusion A conventional treatment regimen based on MTX as the sole DMARD led to achievement of ID in a sizeable proportion of children with JIA. Our findings help to outline the characteristics of patients who may deserve a synthetic DMARD other than MTX or the introduction of a biologic DMARD from disease outset.

Published
2019
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8. The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients

Author

Gabriella Giancane, Claudio Lavarello, Angela Pistorio, Sheila K. Oliveira, Francesco Zulian, Ruben Cuttica, Michel Fischbach, Bo Magnusson, Serena Pastore, Roberto Marini, Silvana Martino, Anne Pagnier, Christine Soler, Valda Staņēvicha, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Elena Fueri, Angelo Ravelli, Alberto Martini, Nicolino Ruperto, and for the Paediatric Rheumatology International Trials Organisation (PRINTO)

Subjects
Juvenile dermatomyositis, Prednisone tapering, Glucorticoids, Disease activity, Core set measures, Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background Prednisone (PDN) in juvenile dermatomyositis (JDM), alone or in association with other immunosuppressive drugs, namely methotrexate (MTX) and cyclosporine (CSA), represents the first-line treatment option for new onset JDM patients. No clear evidence based guidelines are actually available to standardize the tapering and discontinuation of glucocorticoids (GC) in JDM. Aim of our study was to provide an evidence-based proposal for GC tapering/discontinuation in new onset juvenile dermatomyositis (JDM), and to identify predictors of clinical remission and GC discontinuation. Methods New onset JDM children were randomized to receive either PDN alone or in combination with methotrexate (MTX) or cyclosporine (CSA). In order to derive steroid tapering indications, PRINTO/ACR/EULAR JDM core set measures (CSM) and their median absolute and relative percent changes over time were compared in 3 groups. Group 1 included those in clinical remission who discontinued PDN, with no major therapeutic changes (MTC) (reference group) and was compared with those who did not achieve clinical remission, without or with MTC (Group 2 and 3, respectively). A logistic regression model identified predictors of clinical remission with PDN discontinuation. Results Based on the median change in the CSM of 30/139 children in Group 1, after 3 pulses of methyl-prednisolone, GC could be tapered from 2 to 1 mg/kg/day in the first two months from onset if any of the CSM decreased by 50–94%, and from 1 to 0.2 mg/kg/day in the following 4 months if any CSM further decreased by 8–68%, followed by discontinuation in the ensuing 18 months. The achievement of PRINTO JDM 50–70-90 response after 2 months of treatment (ORs range 4.5–6.9), an age at onset > 9 years (OR 4.6) and the combination therapy PDN + MTX (OR 3.6) increase the probability of achieving clinical remission (p

Published
2019
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9. Pharmacovigilance in juvenile idiopathic arthritis patients treated with biologic or synthetic drugs: combined data of more than 15,000 patients from Pharmachild and national registries

Author

Joost Swart, Gabriella Giancane, Gerd Horneff, Bo Magnusson, Michael Hofer, Еkaterina Alexeeva, Violeta Panaviene, Brigitte Bader-Meunier, Jordi Anton, Susan Nielsen, Fabrizio De Benedetti, Sylvia Kamphuis, Valda Staņēviča, Maria Tracahana, Laura Marinela Ailioaie, Elena Tsitsami, Ariane Klein, Kirsten Minden, Ivan Foeldvari, Johannes Peter Haas, Jens Klotsche, Anna Carin Horne, Alessandro Consolaro, Francesca Bovis, Francesca Bagnasco, Angela Pistorio, Alberto Martini, Nico Wulffraat, Nicolino Ruperto, and for the Paediatric Rheumatology International Trials Organisation (PRINTO), BiKeR and the board of the Swedish Registry

Subjects
Juvenile idiopathic arthritis, Registry, Safety, Adverse events, Methotrexate, Biologics, Diseases of the musculoskeletal system, RC925-935
Abstract

Abstract Background The availability of methotrexate and the introduction of multiple biological agents have revolutionized the treatment of juvenile idiopathic arthritis (JIA). Several international and national drug registries have been implemented to accurately monitor the long-term safety/efficacy of these agents. This report aims to present the combined data coming from Pharmachild/PRINTO registry and the national registries from Germany (BiKeR) and Sweden. Methods Descriptive statistics was used for demographic, clinical data, drug exposure, adverse events (AEs) and events of special interest (ESIs). For the Swedish register, AE data were not available. Results Data from a total of 15,284 patients were reported: 8274 (54%) from the Pharmachild registry and 3990 (26%) and 3020 (20%) from the German and the Swedish registries, respectively. Pharmachild children showed a younger age (median of 5.4 versus 7.6 years) at JIA onset and shorter disease duration at last available visit (5.3 versus 6.1–6.8) when compared with the other registries. The most frequent JIA category was the rheumatoid factor–negative polyarthritis (range of 24.6–29.9%). Methotrexate (61–84%) and etanercept (24%–61.8%) were the most frequently used synthetic and biologic disease-modifying anti-rheumatic drugs (DMARDs), respectively. There was a wide variability in glucocorticoid use (16.7–42.1%). Serious AEs were present in 572 (6.9%) patients in Pharmachild versus 297 (7.4%) in BiKeR. Infection and infestations were the most frequent AEs (29.4–30.1%) followed by gastrointestinal disorders (11.5–19.6%). The most frequent ESIs were infections (75.3–89%). Conclusions This article is the first attempt to present a very large sample of data on JIA patients from different national and international registries and represents the first proposal for data merging as the most powerful tool for future analysis of safety and effectiveness of immunosuppressive therapies in JIA. Registry registration The Pharmachild registry is registered at ClinicalTrials.gov (NCT01399281) and at the European Network of Centres for Pharmacoepidemiology and Pharmacovigilance (ENCePP) (http://www.encepp.eu/encepp/viewResource.htm?id=19362). The BiKeR registry is registered at ENCePP (http://www.encepp.eu/encepp/viewResource.htm?id=20591).

Published
2018
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10. Comparative Study Between Functional MR Urography and Renal Scintigraphy to Evaluate Drainage Curves and Split Renal Function in Children With Congenital Anomalies of Kidney and Urinary Tract (CAKUT)

Author

Maria Beatrice Damasio, Monica Bodria, Michael Dolores, Emmanuel Durand, Fiammetta Sertorio, Michela C. Y. Wong, Jean-Nicolas Dacher, Adnan Hassani, Angela Pistorio, Girolamo Mattioli, Gianmichele Magnano, and Pierre H. Vivier

Subjects
obstructive uropathy, dynamic renal scintigraphy, functional magnetic resonance urography, congenital anomalies of the kidney and urinary tract, CAKUT, Pediatrics, RJ1-570
Abstract

Background: Obstructive congenital anomalies of the kidney and urinary tract have a high risk of kidney failure if not surgically corrected. Dynamic renal scintigraphy is the gold standard technique to evaluate drainage curves and split renal function (SRF).Objectives: To compare functional magnetic resonance (MR) urography with dynamic renal scintigraphy in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract.Materials and Methods: We retrospectively collected patients with hydroureteronephrosis or pelvicalyceal dilatation at renal ultrasound, who underwent both functional MR urography and dynamic renal scintigraphy (DRS) within 6 months. DRS studies were evaluated by a single nuclear medicine physician with a double reading. Functional MR urography renograms were blind evaluated twice by two radiologists. The functional MR urographyintra- and inter-reading agreements as well as the agreement between the two imaging techniques were calculated. SRF was evaluated by Area Under the Curve and Rutland-Patlak methods. Drainage curves were classified as normal, borderline or accumulation patterns by both the techniques.Results: Fifty-two children were studied, 14 with bilateral involvement. A total of 104 kidney-urinary tracts were considered: 38 normal and 66 dilated. Considering Area Under the Curve and Rutland-Patlak for SRF, the intra- and inter-reader agreements of functional MR urography had excellent and good results, respectively, and the two techniques demonstrated a good concordance (r2: 67% for Area Under the Curve and 72% for Rutland-Patlak). Considering drainage curves, the inter-readers agreement for functional MR urography and the concordance between the two techniques were moderate (Cohen's k, respectively, 55.7 and 56.3%).Conclusions: According to our results, there are no significant differences between functional MR urography and DRS in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract.

Published
2020
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11. Diffusion-Weighted MRI in the Evaluation of Renal Parenchymal Involvement during Febrile Urinary Tract Infections in Children: Preliminary Data

Author

Lorenzo Anfigeno, Fiammetta Sertorio, Luca Basso, Andrea Fontana, Monica Bodria, Angela Pistorio, Gian Marco Ghiggeri, and Maria Beatrice Damasio

Subjects
pyelonephritis, DWI, diffusion MRI, ultrasound, children, pediatric, Medicine
Abstract

Background: Urinary tract infection (UTI) is the most common infection in pediatric-age patients. Acute pyelonephritis (PNA) represents a worrying situation in pediatric patients due to the risk of sepsis and long-term cicatricial consequences. The purpose of this retrospective study is to evaluate the diagnostic role of DW-MRI in relation to clinical data, to understand if there are any clinical parameters useful in identifying which patients should undergo it. Methods: According to inclusion and exclusion criteria, we enrolled 51 patients ≤15 years old admitted to our Institute between September 2012 and April 2020 with a febrile UTI who underwent DW-MRI evaluation. Clinical, laboratory and imaging data were collected. Statistical analysis was performed. Results: 34 of 51 patients with an fUTI (66.7%) showed signs of acute parenchymal involvement at DW-MRI evaluation. In 27 of these 34 (79.4%), DW-MRI showed multiple areas of pyelonephritis. A statistically significant relationship (p = 0.0004) between older age at admission and pyelonephritis was demonstrated. No statistically significant relationship was found between the other clinical, anamnestic and laboratory parameters and the outcome of DWI. Only two ultrasound examinations allowed the identification of pathological areas on the renal parenchyma. Conclusions: From these preliminary investigations, we can say that selecting the patients with fUTI on whom to perform a DW-MRI is difficult. Nevertheless, thanks to the low cost, the very rare need for sedation and the accuracy in identifying pyelonephritic areas, the use of DW-MRI in patients with febrile UTI seems recommendable.

Published
2021
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12. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

Author

F. De Benedetti, J. Anton, M. Gattorno, H. Lachmann, I. Kone-Paut, S. Ozen, J. Frenkel, A. Simon, A. Zeft, E. Ben-Chetrit, H. M. Hoffman, Y. Joubert, K. Lheritier, A. Speziale, J. Guido, Roberta Caorsi, Federica Penco, Alice Grossi, Antonella Insalaco, Maria Alessio, Giovanni Conti, Federico Marchetti, Alberto Tommasini, Silvana Martino, Romina Gallizzi, Annalisa Salis, Francesca Schena, Francesco Caroli, Alberto Martini, Gianluca Damonte, Isabella Ceccherini, Marco Gattorno, Marie-Louise Frémond, Carolina Uggenti, Lien Van Eyck, Isabelle Melki, Darragh Duffy, Vincent Bondet, Yoann Rose, Bénédicte Neven, Yanick Crow, Mathieu P. Rodero, Yvonne Kusche, Johannes Roth, Katarzyna Barczyk-Kahlert, Giovanna Ferrara, Annalisa Chiocchetti, Silvio Polizzi, Josef Vuch, Diego Vozzi, Anna Mondino, Erica Valencic, Serena Pastore, Andrea Taddio, Flavio Faletra, Umberto Dianzani, Ugo Ramenghi, Qing Zhou, Xiaomin Yu, Erkan Demirkaya, Natalie Deuitch, Deborah Stone, Wanxia Tsai, Amanda Ombrello, Tina Romeo, Elaine F. Remmers, JaeJin Chae, Massimo Gadina, Steven Welch, Seza Ozen, Rezan Topaloglu, Mario Abinun, Daniel L. Kastner, Ivona Aksentijevich, Donatella Vairo, Rosalba Monica Ferraro, Giulia Zani, Jessica Galli, Micaela De Simone, Marco Cattalini, Elisa Fazzi, Silvia Giliani, Ebun Omoyinmi, Ariane Standing, Dorota Rowczenio, Annette Keylock, Sonia Melo Gomes, Fiona Price-Kuehne, Sira Nanthapisal, Claire Murphy, Thomas Cullup, Lucy Jenkins, Kimberly Gilmour, Despina Eleftheriou, Helen Lachmann, Philip Hawkins, Nigel Klein, Paul Brogan, Anita Dhanrajani, Mercedes Chan, Stephanie Pau, Janet Ellsworth, Jaime Guzman, Florence A. Aeschlimann, Marinka Twilt, Simon W. Eng, Shehla Sheikh, Ronald M. Laxer, Diane Hebert, Damien Noone, Christian Pagnoux, Susanne M. Benseler, Rae S. Yeung, Christoph Kessel, Katrin Lippitz, Toni Weinhage, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Niklas Grün, Dirk Föll, Pieter Van Dijkhuizen, Federica Del Chierico, Clara Malattia, Alessandra Russo, Denise Pires Marafon, Nienke M. ter Haar, Silvia Magni-Manzoni, Sebastiaan J. Vastert, Bruno Dallapiccola, Berent Prakken, Fabrizio De Benedetti, Lorenza Putignani, Berna Eren Fidanci, Kenan Barut, Serap Arıcı, Dogan Simsek, Mustafa Cakan, Ezgi D. Batu, Sezgin Şahin, Ayşenur Kısaarslan, Ebru Yilmaz, Özge Basaran, Ferhat Demir, Kubra Ozturk, Zübeyde Gunduz, Betül Sozeri, Balahan Makay, Nuray Ayaz, Onder Yavascan, Ozlem Aydog, Yelda Bilginer, Zelal Ekinci, Dilek Yıldız, Faysal Gök, Muferret Erguven, Erbil Unsal, Ozgur Kasapcopur, For the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR), Hafize E. Sönmez, Betül Sözeri, Yonatan Butbul, Seza Özen, Claudia Bracaglia, Giusi Prencipe, Manuela Pardeo, Geneviève Lapeyre, Emiliano Marasco, Walter Ferlin, Robert Nelson, Cristina de Min, N. Ruperto, H. I. Brunner, P. Quartier, T. Constantin, E. Alexeeva, K. Marzan, N. Wulffraat, R. Schneider, S. Padeh, V. Chasnyk, C. Wouters, J. B. Kuemmerle-Deschner, T. Kallinich, B. Lauwerys, E. Haddad, E. Nasonov, M. Trachana, O. Vougiouka, K. Leon, E. Vritzali, A. Martini, D. Lovell, PRINTO/PRCSG, Stefano Volpi, Claudia Pastorino, Francesca Kalli, Alessia Omenetti, Sabrina Chiesa, Arinna Bertoni, Paolo Picco, Gilberto Filaci, Elisabetta Traggiai, Marie-Louise Fremond, Naoki Kitabayashi, Olivero Sacco, Isabelle Meyts, Marie-Anne Morren, Carine Wouters, Eric Legius, Isabelle Callebaut, Christine Bodemer, Frederic Rieux-Laucat, Mathieu Rodero, Nadia Jeremiah, Alexandre Belot, Eric Jeziorski, Didier Bessis, Guilhem Cros, Gillian I. Rice, Bruno Charbit, Anne Hulin, Nihel Khoudour, Consuelo Modesto Caballero, Monique Fabre, Laureline Berteloot, Muriel Le Bourgeois, Philippe Reix, Thierry Walzer, Despina Moshous, Stéphane Blanche, Alain Fischer, Brigitte Bader-Meunier, Frédéric Rieux-Laucat, K. Annink, N. ter Haar, S. Al-Mayouf, G. Amaryan, K. Barron, S. Benseler, P. Brogan, L. Cantarini, M. Cattalini, A. Cochino, F. Dedeoglu, A. De Jesus, O. Dellacasa, E. Demirkaya, P. Dolezalova, K. Durrant, G. Fabio, R. Gallizzi, R. Goldbach-Mansky, E. Hachulla, V. Hentgen, T. Herlin, M. Hofer, H. Hoffman, A. Insalaco, A. Jansson, I. Koné-Paut, A. Kozlova, J. Kuemmerle-Deschner, R. Laxer, S. Nielsen, I. Nikishina, A. Ombrello, E. Papadopoulou-Alataki, A. Ravelli, D. Rigante, R. Russo, Y. Uziel, Nienke ter Haar, Jerold Jeyaratnam, Anna Simon, Matteo Doglio, Jordi Anton, Consuelo Modesto, Pierre Quartier, Esther Hoppenreijs, Luca Cantarini, Loredana Lepore, Inmaculada Calvo Penades, Christina Boros, Rita Consolini, Donato Rigante, Ricardo Russo, Jana Pachlopnik Schmid, Thirusha Lane, Nicolino Ruperto, Joost Frenkel, Chiara Passarelli, Elisa Pisaneschi, Virginia Messia, Antonio Novelli, Fabrizio Debenedetti, P. A. Brogan, X. Wei, Martina Finetti, Francesca Orlando, Elisabetta Cortis, Angela Miniaci, Nicola Ruperto, Charlotte Eijkelboom, Pavla Dolezalova, Isabelle Koné-Paut, Marija Jelusic-Drazic, Liliana Bezrodnik, Mari Carmen Pinedo, Valda Stanevicha, Marielle van Gijn, Silvia Federici, Hermann Girschick, Gerd Ganser, Susan Nielsen, Troels Herlin, Sulaiman Mohammed Al-Mayouf, Michael Hofer, Jasmin Kuemmerle-Deschner, Susanne Schalm, Annette Jansson, on behalf of PRINTO and Eurofever registry, Marta Marchi, Chiara Marini, Angelo Ravelli, Alberto Garaventa, Sonia Carta, Enrica Balza, Patrizia Castellani, Caterina Pellecchia, Silvia Borghini, Maria Libera Trotta, Anna Rubartelli, Andrew Henrey, Thomas Loughin, Roberta Berard, Natalie Shiff, Roman Jurencak, Susanne Benseler, Lori Tucker, on behalf of ReACCh-Out Investigators, Charalampia Papadopoulou, Ying Hong, Petra Krol, Yiannis Ioannou, Clarissa Pilkington, Hema Chaplin, Stephania Simou, Marietta Charakida, Lucy Wedderburn, Lynn R. Spiegel, Sara Ahola Kohut, Jennifer Stinson, Paula Forgeron, Miriam Kaufman, Nadia Luca, Khush Amaria, Mary Bell, J Swart, F. Boris, E. Castagnola, A. Groll, G. Giancane, G. Horneff, H. I. Huppertz, T. Wolfs, E. Alekseeva, V. Panaviene, F. Uettwiller, V. Stanevicha, L. M. Ailioaie, E. Tsitami, S. Kamphuis, G. Susic, F. Sztajnbok, B. Flato, A. Pistorio, Stephanie J. W. Shoop, Suzanne M. M. Verstappen, Janet E. McDonagh, Wendy Thomson, Kimme L. Hyrich, CAPS, Maarit Tarkiainen, Pirjo Tynjala, Pekka Lahdenne, Janne Martikainen, Acute-JIA Study Group, Meredyth Wilkinson, Christopher Piper, Georg Otto, Claire T. Deakin, Stefanie Dowle, Stefania Simou, Daniel Kelberman, Claudia Mauri, Elizabeth Jury, David Isenberg, Lucy R. Wedderburn, Kiran Nistala, I. Foeldvari, D. J. Lovell, G. Simonini, M. Bereswill, J. Kalabic, Kiem Oen, Brian M. Feldman, Brenden Dufault, Jennifer Lee, Karen Watanabe Duffy, Ciaran Duffy, ReACCh-Out Investigators, N. Tzaribachev, G. Vega-Cornejo, I. Louw, A. Berman, I. Calvo, R. Cuttica, F. Avila-Zapata, R. Cimaz, E. Solau-Gervais, R. Joos, G. Espada, X. Li, M. Nys, R. Wong, S. Banerjee, For Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology Collaborative Study Group (PRCSG), Rebecca Nicolai, Margherita Verardo, Adele D’Amico, Luisa Bracci-Laudiero, Gian Marco Moneta, Gillian Rice, Anne-Laure Mathieu, Sulliman O. Omarjee, Tracy A. Briggs, James O’Sullivan, Simon Williams, Rolando Cimaz, Eve Smith, Michael W. Beresford, Yanick J. Crow, GENIAL Investigators, UK JSLE Study Group, Madeleine Rooney, Nick Bishop, joyce davidson, Clarissa pilkington, Michael Beresford, Jacqui Clinch, Rangaraj Satyapal, Helen Foster, Janet Gardner Medwin, Janet McDonagh, Sue Wyatt, On Behalf of the British Society for Paediatric and Adolescent Rheumatology, Valentina Litta Modignani, Francesco Baldo, Stefano Lanni, Alessandro Consolaro, Giovanni Filocamo, Helen J. Lachmann, on behalf of Eurofever Registry, Gianmarco Moneta, Camilla Celani, Bilade Cherqaoui, Linda Rossi-Semerano, Perrine Dusser, Véronique Hentgen, Claire Grimwood, Linda Rossi, Isabelle Kone Paut, Veronique Hentgen, Denise Lasigliè, Denise Ferrera, Giulia Amico, Marco Di Duca, Laura Obici, Roberto Ravazzolo, Ryuta Nishikomori, Juan Arostegui, Andrea Petretto, Chiara Lavarello, Elvira Inglese, Federica Vanoni, Michaël Hofer, on behalf of EUROFEVER PROJECT, P. N. Hawkins, T. van der Poll, U. A. Walker, H. H. Tilson, Pascal N. Tyrrell, Raphaela Goldbach-Mansky, Norbert Blank, Hal M. Hoffman, Elisabeth Weissbarth-Riedel, Boris Huegle, Tilmann Kallinich, Ahmet Gul, Marlen Oswald, Fatma Dedeoglu, Aki Hanaya, Takako Miyamae, Manabu Kawamoto, Yumi Tani, Takuma Hara, Yasushi Kawaguchi, Satoru Nagata, Hisashi Yamanaka, Almira Ćosićkić, Fahrija Skokić, Belkisa Čolić, Sanimir Suljendić, Anna Kozlova, Irina Mersiyanova, Mariya Panina, Lily Hachtryan, Vasiliy Burlakov, Elena Raikina, Alexey Maschan, Anna Shcherbina, Banu Acar, Meryem Albayrak, Betul Sozeri, Sezgin Sahin, Amra Adrovic, Nese Inan, Serhan Sevgi, Caroline M. Andreasen, Anne Grethe Jurik, Mia B. Glerup, Christian Høst, Birgitte T. Mahler, Ellen-Margrethe Hauge, Cecilia Lazea, Laura Damian, Calin Lazar, Rodica Manasia, Chloe M. Stephenson, Vimal Prajapati, Paivi M. Miettunen, Dilek Yılmaz, Yavuz Tokgöz, Yasin Bulut, Harun Çakmak, Ferah Sönmez, Elif Comak, Gülşah Kaya Aksoy, Mustafa Koyun, Sema Akman, Yunus Arıkan, Ender Terzioğlu, Osman Nidai Özdeş, İbrahim Keser, Hüseyin Koçak, Ayşen Bingöl, Aygen Yılmaz, Reha Artan, X. Xu, Fatemeh F. Mehregan, Vahid Ziaee, Mohammad H. Moradinejad, Francesco La Torre, Clotilde Alizzi, Pio D’Adamo, G. Junge, J. Gregson, Hasmik Sargsyan, Hulya Zengin, Berna E. Fidanci, Cagla Kaymakamgil, Dilek Konukbay, Dilek Yildiz, Faysal Gok, Iris Stoler, Judith Freytag, Banu Orak, Christine Seib, Lars Esmann, Eva Seipelt, Faekah Gohar, Dirk Foell, Ismail Dursun, Sebahat Tulpar, Sibel Yel, Demet Kartal, Murat Borlu, Funda Bastug, Hakan Poyrazoglu, Zubeyde Gunduz, Kader Kose, Mehmet E. Yuksel, Abdullah Calıskan, Ahmet B. Cekgeloglu, Ruhan Dusunsel, Katerina Bouchalova, Jana Franova, Marcel Schuller, Marie Macku, Katerina Theodoropoulou, Raffaella Carlomagno, Annette von Scheven-Gête, Claudia Poloni, Laura O. Damian, Dan Cosma, Amanda Radulescu, Dan Vasilescu, Liliana Rogojan, Simona Rednic, Mihaela Lupse, Lien De Somer, Pierre Moens, Rocio Galindo Zavala, Laura Martín Pedraz, Esmeralda Núñez Cuadros, Gisela Díaz-Cordovés Rego, Antonio L. Urda Cardona, Ilaria Dal Forno, Sara Pieropan, Ombretta Viapiana, Davide Gatti, Gloria Dallagiacoma, Paola Caramaschi, Domenico Biasi, Daniel Windschall, Ralf Trauzeddel, Hartwig Lehmann, Rainer Berendes, Maria Haller, Manuela Krumrey-Langkammerer, Antje Nimtz-Talaska, Philipp Schoof, Ralf Felix Trauzeddel, Christine Nirschl, Estefania Quesada-Masachs, Carla Aguilar Blancafort, Sara Marsal Barril, Francisca Aguiar, Rita Fonseca, Duarte Alves, Ana Vieira, Alberto Vieira, Jorge A. Dias, Iva Brito, Gordana Susic, Vera Milic, Goran Radunovic, Ivan Boricic, Pauline Marteau, Catherine Adamsbaum, Michel De Bandt, Irène Lemelle, Chantal Deslandre, Tu Anh Tran, Anne Lohse, Elisabeth Solau-Gervais, Pascal Pillet, Julien Wipff, Cécile Gaujoux-Viala, Sylvain Breton, Valérie Devauchelle-Pensec, Sandra Gran, Olesja Fehler, Stefanie Zenker, Michael Schäfers, Thomas Vogl, Severine Guillaume Czitrom, EH Pieter Van Dijkhuizen, Silvia Magni Manzoni, Francesca Magnaguagno, Laura Tanturri de Horatio, Nienke M. Ter Haar, Annemieke S. Littooij, Vitor A. Teixeira, Raquel Campanilho-Marques, Ana F. Mourão, Filipa O. Ramos, Manuela Costa, Wafa A. Madan, Orla G. Killeen, Adriana Rodriguez Vidal, Diana Sueiro Delgado, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Aleksey Kozhevnikov, Nina Pozdeeva, Mikhail Konev, Evgeniy Melchenko, Vladimir Kenis, Gennadiy Novik, Aysenur Pac Kısaarslan, Butsabong Lerkvaleekul, Suphaneewan Jaovisidha, Witaya Sungkarat, Niyata Chitrapazt, Praman Fuangfa, Thumanoon Ruangchaijatuporn, Soamarat Vilaiyuk, Dan Ø. Pradsgaard, Arne Hørlyck, Anne H. Spannow, Carsten W. Heuck, Talia Diaz, Fernando Garcia, Lorenia De La Cruz, Nadina Rubio, Joanna Świdrowska-Jaros, Elzbieta Smolewska, Mirta Lamot, Lovro Lamot, Mandica Vidovic, Edi Paleka Bosak, Ivana Rados, Miroslav Harjacek, Nikolay Tzaribachev, Polymnia Louka, Romiesa Hagoug, Chiara Trentin, Olga Kubassova, Mark Hinton, Mikael Boesen, Olena A. Oshlianska, Illya A. Chaikovsky, G. Mjasnikov, A. Kazmirchyk, Umberto Garagiola, Irene Borzani, Paolo Cressoni, Fabrizia Corona, Eszter Dzsida, Giampietro Farronato, Antonella Petaccia, Alenka Gagro, Agneza Marija Pasini, Goran Roic, Ozren Vrdoljak, Lucija Lujic, Matija Zutelija-Fattorini, Monika M. Esser, Deepthi R. Abraham, Craig Kinnear, Glenda Durrheim, Mike Urban, Eileen Hoal, Victoria B. Nikolayenko, Kubilay Şahin, Yasar Karaaslan, Adele Civino, Giovanni Alighieri, Sergio Davì, Roberto Rondelli, Andrea Magnolato, Francesca Ricci, Alma Olivieri, Valeria Gerloni, Bianca Lattanzi, Francesca Soscia, Alessandro De Fanti, Stefania Citiso, Lorenzo Quartulli, Maria Cristina Maggio, Manuela Marsili, Maria Antonietta Pelagatti, Valentino Conter, Franca Fagioli, Andrea Pession, Marco Garrone, Mariangela Rinaldi, Jaime De Inocencio, Stella Garay, Daniel J. Lovell, Berit Flato, EPOCA Study Group, Angela Aquilani, Simona Cascioli, Ivan Caiello, Denise Pires-Marafón, Rita Carsetti, Emily Robinson, Salvatore Albani, Wilco de Jager, Sytze de Roock, Trang Duong, Justine Ellis, Kimme Hyrich, Laetitia Jervis, Daniel Lovell, Lucy Marshall, Elizabeth D. Mellins, Kirsten Minden, Jane Munro, Peter A. Nigrovic, Jason Palman, Sunil Sampath, Laura E. Schanberg, Susan D. Thompson, Richard Vesely, Chris Wallace, Chris Williams, Qiong Wu, Nico Wulffraat, Rae S. M. Yeung, M. B. Seyger, D. Arikan, J. K. Anderson, A. Lazar, D. A. Williams, C. Wang, R. Tarzynski-Potempa, J. S. Hymans, Gabriele Simonini, Erika Scoccimarro, Irene Pontikaki, Teresa Giani, Alessandro Ventura, Pier Luigi Meroni, Gaetana Minnone, Marzia Soligo, Luigi Manni, Luisa Bracci Laudiero, Noortje Groot, I. Grein, N. M. Wulffraat, R. Schepp, G. Berbers, C. C. Barbosa Sandoval de Souza, V. Paes Leme Ferriani, G. Pileggi, S. de Roock, Ingrid H. R. Grein, Silvia Scala, Elisa Patrone, Casper Schoemaker, on behalf of Dutch JIA patient organization, Wendy Costello, on behalf of ENCA, Suzanne Parsons, Jean-David Cohen, Damien Bentayou, Marc-Antoine Bernard Brunel, Sonia Trope, Jens Klotsche, Miriam Listing, Martina Niewerth, Gerd Horneff, Angelika Thon, Hans-Iko Huppertz, Kirsten Mönkemöller, Ivan Foeldvari, ICON study group, Achille Marino, Stefano Stagi, Niccolò Carli, Federico Bertini, Adriana S. Díaz-Maldonado, Sally Pino, Pilar Guarnizo, Alfonso Ragnar Torres-Jimenez, Berenice Sanchez-Jara, Eunice Solis-Vallejo, Adriana Ivonne Cespedes-Cruz, Maritza Zeferino-Cruz, Julia Veronica Ramirez-Miramontes, Ankur Kumar, Anju Gupta, Deepti Suri, Amit Rawat, Nandita Kakkar, Surjit Singh, Özge A. Gücenmez, Erbil Ünsal, Bo Magnusson, Karina Mördrup, Anna Vermé, Christina Peterson, Board of the Swedish Pediatric Rheumatology Registry, Caroline Freychet, Jean Louis Stephan, Cathryn E. Harkness, Leanne Foster, Emma Henry, Pauline Taggart, Coskun F. Ozkececi, Esra Kurt, Gokalp Basbozkurt, Daiva Gorczyca, Jacek Postępski, Aleksandra Czajkowska, Bogumiła Szponar, Mariola Paściak, Anna Gruenpeter, Iwona Lachór-Motyka, Daria Augustyniak, Edyta Olesińska, Emediong S. Asuka, Tatyana Golovko, Samuel U. Aliejim, Emilio Inarejos Clemente, Estibaliz Iglesias Jimenez, Joan Calzada Hernandez, Sergi Borlan Fernandez, Clara Gimenez Roca, David Moreno Romo, Natalia Rodriguez Nieva, Juan Manuel Mosquera Angarita, Jordi Anton Lopez, Esmeralda Nuñez-Cuadros, Gisela Diaz-Cordovés, Rocío Galindo-Zavala, Antonio Urda-Cardona, Antonio Fernández-Nebro, Daniel Álvarez de la Sierra, Marina Garcia Prat, Mónica Martínez Gallo, Ricardo Pujol Borrell, Ana M. Marín Sánchez, Etienne Merlin, Sylvie Fraitag, Jean-Louis Stephan, Federico Annoni, Giancarla Di Landro, Sofia Torreggiani, Marta Torcoletti, Georgina Tiller, Jo Buckle, Angela Cox, Peter Gowdie, Roger C. Allen, Jonathan D. Akikusa, Hayde G. Hernández-Huirache, Edel R. Rodea-Montero, William Fahy, Christelle Sordet, Karin B. Berggren, Johanna T. Kembe, Joyce Bos, Wineke Armbrust, Marco van Brussel, Jeanette Cappon, Pieter Dijkstra, Jan Geertzen, Elizabeth Legger, Marion van Rossum, Pieter Sauer, Otto Lelieveld, Levent Buluc, Gur Akansel, Bahar Muezzinoglu, Ljubov Rychkova, Tatyana Knyazeva, Anna Pogodina, Tatyana Belova, Tamara Mandzyak, Ekaterina Kulesh, Alessandro Cafarotti, Cosimo Giannini, Roberta Salvatore, Giuseppe Lapergola, Caterina Di Battista, Maria Loredana Marcovecchio, Raffaella Basilico, Piernicola Pelliccia, Francesco Chiarelli, Luciana Breda, Beverley Almeida, Sarah Tansley, Harsha Gunawardena, Neil McHugh, Juvenile Dermatomyositis Research Group (JDRG), Jessie Aouizerate, Marie De Antonio, Christine Barnerias, Guillaume Bassez, Isabelle Desguerre, Romain Gherardi, Jean-Luc Charuel, François-Jérôme Authier, Cyril Gitiaux, C. H. Spencer, Rabheh Abdul Aziz, Chack-Yung Yu, Brent Adler, Sharon Bout-Tabaku, Katherine Lintner, Melissa Moore-Clingenpeel, Liza McCann, Nicola Ambrose, Mario Cortina-Borja, Juvenile Dermatomyositis Cohort and Biomarker Study (JCDBS), Prasad T. Oommen, Fabian Speth, Johannes-Peter Haas, Working Group “Juvenile Dermatomyositis” of the German Society for Paediatric and Adolescent Rheumatology (GKJR), Claudio Lavarello, Gabriella Giancane, Angela Pistorio, Lisa Rider, Rohit Aggarwal, Sheila K. Oliveira, Ruben Cuttica, Michel Fischbach, Gary Sterba, Karine Brochard, Frank Dressler, Patrizia Barone, Ruben Burgos-Vargas, Elizabeth Candell Chalom, Marine Desjonqueres, Graciela Espada, Anders Fasth, Stella Maris Garay, Rose-Marie Herbigneaux, Claire Hoyoux, Chantal Job Deslandre, Frederick W. Miller, Jiri Vencovsky, Erdal Sag, Gulsev Kale, Haluk Topaloglu, Beril Talim, Francesco Zulian, Tadej Avcin, Roberto Marini, Anne Pagnier, Michel Rodiere, Christine Soler, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Ana V. Villarreal, Nydia Acevedo, Yuridiana Ramirez, Enrique Faugier, Rocio Maldonado, Bita Arabshahi, John H. Lee, Ian Leibowitz, Lawrence O. Okong’o, Jo Wilmshurst, Monika Esser, Christiaan Scott, Ezgi Deniz Batu, Nagehan Emiroglu, Hafize Emine Sonmez, Gokcen Dilsa Tugcu, Zehra Serap Arici, Ebru Yalcin, Deniz Dogru, Ugur Ozcelik, Mithat Haliloglu, Nural Kiper, Masato Yashiro, Mutsuko Yamada, Toshihiko Yabuuchi, Tomonobu Kikkawa, Nobuyuki Nosaka, Yosuke Fujii, Yukie Saito, Hirokazu Tsukahara, Sulaiman M. Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-shukair, Zeyad El-Habahbeh, Abdullah Alsonbul, Aleksandra Szabat, Monika Chęć, Violetta Opoka-Winiarska, Biman Saikia, Ranjana W. Minz, Christine Arango, Clara Malagon, Maria D. P. Gomez, Angela C. Mosquera, Ricardo Yepez, Tatiana Gonzalez, Camilo Vargas, GRIP study group, Marta Balzarin, Biagio Castaldi, Elena Reffo, Francesca Sperotto, Giorgia Martini, Alessandra Meneghel, Ornella Milanesi, Ozgur Kasapçopur, Maria Teresa Terreri, Ekaterina Alexeeva, Maria Katsicas, Mikhail Kostik, Thomas Lehman, W.-Alberto Sifuentes-Giraldo, Vanessa Smith, Flavio Sztajnbok, Tadey Avcin, Maria Jose Santos, Dana Nemcova, Cristina Battagliotti, Liora Harel, Mahesh Janarthanan, Kathryn Torok, Nicola Helmus, Eileen Baildem, Michael Blakley, Kim Fligelstone, Antonia Kienast, Clare Pain, Amanda Saracino, Gabriele Simoni, Lisa Weibel, Maria K. Osminina, Nathalia A. Geppe, Olga V. Niconorova, Olesya V. Karashtina, Oksana V. Abbyasova, Olga V. Shpitonkova, Sinem Durmus, Hafize Uzun, Angela Mauro, Eleonora Fanti, Fabio Voller, Franca Rusconi, Fernando Garcia-Rodriguez, Ana V. Villarreal-Treviño, Angel J. Flores-Pineda, Paola B. Lara-Herrea, Diego R. Salinas-Encinas, Talia Diaz-Prieto, Maria R. Maldonado-Velazquez, Sarbelio Moreno-Espinosa, Enrique Faugier-Fuentes, Mirella Crapanzano, Ilaria Parissenti, Man S. Parihar, Pandiarajan Vignesh, ManojKumar Rohit, Kavitha Gopalan, Savita V. Attri, Alan Salama, David Jayne, Mark Little, Yulia Kostina, Galina Lyskina, Olga Shpitonkova, Alena Torbyak, Olga Shirinsky, Maria Francesca Gicchino, Maria Cristina Smaldone, Mario Diplomatico, Alma Nunzia Olivieri, C H. Spencer, Richard McClead, Hiren Patel, Chung-Yung Yu, Dita Cebecauerová, Tomáš Dallos, Edita Kabíčková, Martin Kynčl, Daniela Chroustová, Jozef Hoza, Dana Němcová, Vladimír Tesař, Pavla Doležalová, Tuncay Hazirolan, Fatih Ozaltin, Fabiola Almeida, Isabela H. Faria de Paula, Maíra M. Sampaio, Fernando N. Arita, Andressa G. Alves, Maria Carolina Santos, Eunice M. Okuda, Silvana B. Sacchetti, Fernanda Falcini, Marini Francesca, Gemma Lepri, Marco Matucci-Cerinic, Maria Luisa Brandi, Hakan Kisaoglu, Sema Misir, Selim Demir, Yuksel Aliyazicioglu, Mukaddes Kalyoncu, Carlos Eduardo Ramalho, Fabiola D. Almeida, Joan Calzada-Hernández, Rosa Bou, Estíbaliz Iglesias, Judith Sánchez-Manubens, Fredy Hermógenes Prada Martínez, Clara Giménez Roca, Sergi Borlan Fernández, Marek Bohm, Kamran Mahmood, Valentina Leone, Mark Wood, Ken-Ichi Yamaguchi, Satoshi Fujikawa, Working Group of Behçet’s Disease, Pediatric Rheumatology Association of Japan (PRAJ), Kyu Yeun Kim, Do Young Kim, Dong Soo Kim, Maka Ioseliani, Ivane Chkhaidze, Maia Lekishvili, Nana Tskhakaia, Shorena Tvalabeishvili, Aleksandre Kajrishvili, Maiko Takakura, Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Akihiro Yachie, Giovanni Corsello, Maryam Piram, Carla Maldini, Sandra Biscardi, Nathalie Desuremain, Catherine Orzechowski, Emilie Georget, Delphine Regnard, Isabelle Kone-Paut, Alfred Mahr, Mihaela Sparchez, Zeno Sparchez, Nydia Acevedo Silva, Ana V. Villarreal Treviño, Yuridiana Ramirez Loyola, Talia Diaz Prieto, Enrique Faugier Fuentes, Maria D. R. Maldonado Velazquez, Pilar Perez, Sagar Bhattad, Ranjana Minz, Jitendra Shandilya, Pediatric Allergy and Immunology Unit, PGIMER, Chandigarh, Ana Villarreal, Yuridiana Ramírez, Zeynep Birsin Özçakar, Suat Fitoz, Fatos Yalcinkaya, Annacarin Horne, Francesca Minoia, Francesca Bovis, Sergio Davi, Priyankar Pal, Kimo Stein, Sandra Enciso, Michael Jeng, Despoina Maritsi, Randy C. Cron, Anne Thorwarth, Sae Lim von Stuckrad, Angela Rösen-Wolff, Hella Luksch, Patrick Hundsdoerfer, Peter Krawitz, Nuray Aktay Ayaz, Doğan Simsek, Şebnem Sara Kılıc, Emine Sonmez, Aysenur Pac Kisaarslan, Ozge Altug Gucenmez, Z. Serap Arıcı, Fatih Kelesoglu, Zelal Ekinci Ekinci, Maria Miranda-Garcia, Carolin Pretzer, Michael Frosch, F. Gohar, Angela McArdle, Niamh Callan, Belinda Hernandez, Miha Lavric, Oliver FitzGerald, Stephen R. Pennington, Joachim Peitz, Joern Kekow, Ariane Klein, Anna C. Schulz, Frank Weller-Heinemann, Anton Hospach, J-Peter Haas, BIKER collaborative group, Karen Put, Jessica Vandenhaute, Anneleen Avau, Annemarie van Nieuwenhuijze, Ellen Brisse, Tim Dierckx, Omer Rutgeerts, Josselyn E. Garcia-Perez, Jaan Toelen, Mark Waer, Georges Leclercq, An Goris, Johan Van Weyenbergh, Adrian Liston, Patrick Matthys, Carine H. Wouters, Yasuo Nakagishi, Michael J. Ombrello, Victoria Arthur, Anne Hinks, Patricia Woo, International Childhood Arthritis Genetics (INCHARGE) Consortium, Barbara Stanimirovic, Biljana Djurdjevic-Banjac, Olivera Ljuboja, Boris Hugle, MArgarita Onoufriou, Olga Vougiouka, Kenza Bouayed, Sanae El Hani, Imane Hafid, Nabiha Mikou, Nunu Shelia, Mari Laan, Jaanika Ilisson, and Chris Pruunsild

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
Full Text
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13. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part two

Author

Olga Lomakina, Ekaterina Alekseeva, Sania Valieva, Tatiana Bzarova, Irina Nikishina, Elena Zholobova, Svetlana Rodionovskaya, Maria Kaleda, Yasuo Nakagishi, Masaki Shimizu, Mao Mizuta, Akihiro Yachie, Yuko Sugita, Nami Okamoto, Kousuke Shabana, Takuji Murata, Hiroshi Tamai, Eve M. Smith, Peng Yin, Andrea L. Jorgensen, Michael W. Beresford, on behalf of On behalf of the UK JSLE Cohort Study, Antonio Eleuteri, Beatrice Goilav, Laura Lewandowski, Angel Phuti, Dawn Wahezi, Tamar Rubinstein, Caroline Jones, Paul Newland, Stephen Marks, Rachel Corkhill, Diana Ekdawy, Clarissa Pilkington, Kjell Tullus, Chaim Putterman, Chris Scott, Antony C. Fisher, Andrea Jorgensen, Ezgi Deniz Batu, Can Kosukcu, Ekim Taskiran, Sema Akman, Kubra Ozturk, Betul Sozeri, Erbil Unsal, Zelal Ekinci, Yelda Bilginer, Mehmet Alikasifoglu, Seza Ozen, Hanna Lythgoe, Hermine I. Brunner, Gaurav Gulati, Jordan T. Jones, Mekibib Altaye, Jamie Eaton, Mark Difrancesco, Joo Guan Yeo, Jingyao Leong, Loshinidevi D/O Thana Bathi, Thaschawee Arkachaisri, Salvatore Albani, Nagla Abdelrahman, Michael W Beresford, Valentina Leone, UK JSLE study group supported by the National Institute of Health Research Clinical Research Network, Noortje Groot, D. Shaikhani, I. E. M. Bultink, M. Bijl, R. J. E. M. Dolhain, Y. K. O. Teng, E. Zirkzee, K. de Leeuw, R. Fritsch-Stork, S. S. M. Kamphuis, Rachael D. Wright, Reem Abdawani, Laila Al Shaqshi, Ibrahim Al Zakwani, Natali W. Gormezano, David Kern, Oriany L. Pereira, Gladys C. C. Esteves, Adriana M. Sallum, Nadia E. Aikawa, Rosa M. Pereira, Clovis A. Silva, Eloisa Bonfa, Jessica Beckmann, Nora Bartholomä, Nils Venhoff, Philipp Henneke, Ulrich Salzer, Ales Janda, Alina Lucica Boteanu, Sandra Garrote Corral, Alberto Sifuentes Giraldo, Mariluz Gámir Gámir, Antonio Zea Mendoza, Amra Adrovic, Reyhan Dedeoglu, Sezgin Sahin, Kenan Barut, Aida Koka, Funda Oztunc, Ozgur Kasapcopur, Ana Luisa Rodriguez-Lozano, Francisco Rivas-Larrauri, Silvestre García de la Puente, Andressa G. F. Alves, Maria F. D. A. Giacomin, Juliana Farhat, Alfésio L. F. Braga, Adriana M. E. Sallum, Lúcia M. D. A. Campos, Luiz A. A. Pereira, Ana J. D. F. C. Lichtenfels, Clóvis A. Silva, Sylvia C. L. Farhat, Banu Acar, Z. Birsin Ozcakar, Nilgün Çakar, Nermin Uncu, Gökçe Gür, Semanur Özdel, Fatoş Yalçınkaya, Christiaan Scott, Nicky Brice, Peter Nourse, Christine Arango, Angela C. Mosquera, Clara Malagon, Ana P. Sakamoto, Marco F. C. D. Silva, Ananadreia S. Lopes, Gleice C. S. Russo, Adriana E. M. Sallum, Katia Kozu, Eloisa Bonfá, Claudia Saad-Magalhães, Rosa M. R. Pereira, Claudio A. Len, Maria T. Terreri, Deepti Suri, Siyaram Didel, Amit Rawat, Surjit Singh, Despoina Maritsi, MArgarita Onoufriou, Olga Vougiouka, Maria Tsolia, Edi Paleka Bosak, Mandica Vidović, Mirta Lamot, Lovro Lamot, Miroslav Harjaček, Erika Van Nieuwenhove, Adrian Liston, Carine Wouters, Fatemeh Tahghighi, Vahid Ziaee, Seid-Reza Raeeskarami, Francisca Aguiar, Sandra Pereira, Mariana Rodrigues, Cláudia Moura, Gustavo Rocha, Hercília Guimarães, Iva Brito, Rita Fonseca, Gerd Horneff, Ariane Klein, Kirsten Minden, Hans-Iko Huppertz, Frank Weller-Heinemann, Jasmin Kuemmerle-Deschner, J-Peter Haas, Anton Hospach, BIKER collaborative group, Ricardo Menendez-Castro, Boris Huegle, Johannes-Peter Haas, Joost Swart, Gabriella Giancane, Francesca Bovis, Elio Castagnola, Andreas Groll, Daniel J. Lovell, Tom Wolfs, Michael Hofer, Violeta Panaviene, Susan Nielsen, Jordi Anton, Florence Uettwiller, Valda Stanevicha, Maria Trachana, Denise Pires Marafon, Constantin Ailioaie, Elena Tsitsami, Sylvia Kamphuis, Troels Herlin, Pavla Doležalová, Gordana Susic, Berit Flatø, Flavio Sztajnbok, Angela Pistorio, Alberto Martini, Nico Wulffraat, Nicolino Ruperto, Marco Gattorno, Antonio Brucato, Martina Finetti, George Lazaros, Silvia Maestroni, Mara Carraro, Davide Cumetti, Alessandra Carobbio, Monia Lorini, Alessandro Rimini, Renzo Marcolongo, Anna Valenti, Gian Luca Erre, Riccardo Belli, Fiorenzo Gaita, Maria Pia Sormani, Massimo Imazio, Mario Abinun, Nicola Smith, Tim Rapley, Flora McErlane, Lianne Kearsley-Fleet, Kimme L. Hyrich, Helen Foster, Nikolay Tzaribachev, Andrew Zeft, Rolando Cimaz, John Bohnsack, Thomas Griffin, Ruy Carrasco, Jason Dare, Ivan Foeldvari, Richard Vehe, Teresa Simon, Hermine Brunner, S. Verazza, S. Davì, A. Consolaro, A. Insalaco, V. Gerloni, R. Cimaz, F. Zulian, S. Pastore, F. Corona, G. Conti, P. Barone, M. Cattalini, E. Cortis, L. Breda, A. N. Olivieri, A. Civino, R. Podda, D. Rigante, F. La Torre, G. D’Angelo, M. Jorini, R. Gallizzi, M. C. Maggio, R. Consolini, A. De Fanti, M. G. Alpigiani, A. Martini, A. Ravelli, on behalf of Italian Pediatric Rheumatology Study Group, Aysenur Pac Kısaarslan, Zubeyde Gunduz, Ruhan Dusunsel, Ismail Dursun, Hakan Poyrazoglu, Ekaterina Kuchinskaya, Farida Abduragimova, Mikhail Kostik, Erik Sundberg, Soley Omarsdottir, Lena Klevenvall, Helena Erlandsson-Harris, Gokalp Basbozkurt, Ozge Erdemli, Dogan Simsek, Fatih Yazici, Yildirim Karsioglu, Aysen Tezcaner, Dilek Keskin, Huseyin Ozkan, Cengizhan Acikel, Erkan Demirkaya, Ilonka Orbán, Krisztina Sevcic, Valentin Brodszky, Emese Kiss, Ismaiel A. Tekko, Madeleine Rooney, James McElnay, Cliff Taggart, Helen McCarthy, Ryan F. Donnelly, Drug Delivery Group, Mary Slatter, Zohreh Nademi, Mark Friswell, Sharmila Jandial, Terence Flood, Sophie Hambleton, Andrew Gennery, Andrew Cant, Phoi-Ngoc Duong, Isabelle Koné-Paut, Giovanni Filocamo, María Luz Gamir, Helga Sanner, Laura Carenini, Mesut Topdemir, Yildirim Karslioglu, Faysal Gok, Nadezhda Tsurikova, Elena Ligostaeva, Navdha R. Ramchurn, O. Kostareva, I. Nikishina, S. Arsenyeva, S. Rodionovskaya, M. Kaleda, D. Alexeev, Ismail Dursun Dursun, Sara Murias, Estefania Barral, Rosa Alcobendas, Eugenia Enriquez, Agustin Remesal, Jaime de Inocencio, Tania M. Castro, Simone A. Lotufo, Tatjana Freye, Raffaella Carlomagno, Thomas Zumbrunn, Jan Bonhoeffer, Elvira Cannizzaro Schneider, Daniela Kaiser, Michaël Hofer, Véronique Hentgen, Andreas Woerner, Juvenile Inflammatory Rheumatism (JIR) Cohort, Tobias Schwarz, Jens Klotsche, Martina Niewerth, Gerd Ganser, ICON study group, Jerold Jeyaratnam, Nienke ter Haar, Donato Rigante, Fatma Dedeoglu, Ezgi Baris, Sebastiaan Vastert, Joost Frenkel, Jonathan S. Hausmann, Kathleen G. Lomax, Ari Shapiro, Karen L. Durrant, P. A. Brogan, M. Hofer, J. B. Kuemmerle-Deschner, B. Lauwerys, A. Speziale, K. Leon, X. Wei, R. M. Laxer, Sara Signa, Marta Rusmini, Elena Campione, Sabrina Chiesa, Alice Grossi, Alessia Omenetti, Roberta Caorsi, Gianmaria Viglizzo, Isabella Ceccherini, Silvia Federici, Helen Lachmann, Nicola Ruperto, on behalf of PRINTO and Eurofever Registry, Federica Vanoni, on behalf of PRINTO and Eurofever Project, Sonia Melo Gomes, Ebun Omoyinmi, Juan I. Arostegui, Eva Gonzalez-Roca, Despina Eleftheriou, Nigel Klein, Paul Brogan, Stefano Volpi, Elettra Santori, Paolo Picco, Claudia Pastorino, Gillian Rice, Alessandra Tesser, Yanick Crow, Fabio Candotti, Ada B. Sinoplu, Gozde Yucel, Gizem Pamuk, Laura O. Damian, Cecilia Lazea, Mihaela Sparchez, Paulina Vele, Laura Muntean, Adriana Albu, Simona Rednic, Calin Lazar, Leonardo O. Mendonça, Alessandra Pontillo, Jorge Kalil, Fabio M. Castro, Myrthes T. Barros, Manuela Pardeo, Virginia Messia, Fabrizio De Benedetti, Antonella Insalaco, Giorgia Malighetti, Chiara Gorio, Francesca Ricci, Ilaria Parissenti, Paola Montesano, Barbara Bonafini, Veronica Medeghini, Marco Cattalini, Lucio Giordano, Giulia Zani, Rosalba Ferraro, Donatella Vairo, Silvia Giliani, Maria Cristina Maggio, Girolamo Luppino, Giovanni Corsello, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Adriana Rodriguez Vidal, Juan I. Arostegui Gorospe, Inmaculada Calvo Penades, Nadia K. Rafiq, Karen Wynne, Khalid Hussain, Paul A. Brogan, Elizabeth Ang, Nicholas Ng, Ayla Kacar, Ozge Altug Gucenmez, Balahan Makay, Sevket Erbil Unsal, Yasin Sahin, Tufan Kutlu, Fugen Cullu-Cokugras, Hasret Ayyildiz-Civan, Tulay Erkan, Sana Al Zuhbi, Eiman Abdalla, Ricardo A. Russo, María M. Katsicas, Francesca Minoia, Angelo Ravelli, Sagar Bhattad, Anju Gupta, Vignesh Pandiarajan, Ritambhra Nada, Kaara Tiewsoh, Philip Hawkins, Dorota Rowczenio, Sarka Fingerhutova, Jana Franova, Leona Prochazkova, Eva Hlavackova, Pavla Dolezalova, Havva Evrengül, Selçuk Yüksel, Mustafa Doğan, Dolunay Gürses, Harun Evrengül, Silvia De Pauli, Serena Pastore, Anna Monica Bianco, Giovanni Maria Severini, Andrea Taddio, Alberto Tommasini, Svetlana O. Salugina, Evgeny Fedorov, Elena Kamenets, Ekaterina Zaharova, Tatiana Sleptsova, Ekaterina Alexeeva, Kirill Savostyanov, Alexander Pushkov, Tatyana Bzarova, Saniya Valieva, Rina Denisova, Kseniya Isayeva, Evgeniya Chistyakova, Margarita Soloshenko, Elena Kaschenko, Utako Kaneko, Chihaya Imai, Akihiko Saitoh, Vitor A. Teixeira, Filipa O. Ramos, Manuela Costa, Yonatan Butbul Aviel, Shafe Fahoum, Riva Brik, Zeynep Birsin Özçakar, Banu Acar Celikel, Fatos Yalcinkaya, Benedetta Schiappapietra, Sergio Davi’, Federica Mongini, Luisa Giannone, Cecilia Bava, Maria Giannina Alpigiani, Alessandro Consolaro, Dragana S. Lazarevic, Jelena Vojinovic, Jelena Basic, Valentina Muratore, Valentina Marzetti, Neus Quilis, Belen Serrano Benavente, Alessandra Alongi, Adele Civino, Lorenzo Quartulli, Giedre Januskeviciute, Pieter van Dijkhuizen, N. Groot, W. van Dijk, A. Kardolus, Raul Gutiérrez Suárez, Ellen B. Nordal, Veronika G. Rypdal, Lillemor Berntson, Maria Ekelund, Kristiina Aalto, Suvi Peltoniemi, Marek Zak, Mia Glerup, Ellen D. Arnstad, Anders Fasth, Marite Rygg, the Nordic Study Group of Pediatric Rheumatology (NoSPeR), Ana Catarina Duarte, Sandra Sousa, Lídia Teixeira, Ana Cordeiro, Mª José Santos, Ana Filipa Mourão, Maria José Santos, Mónica Eusébio, Ana Lopes, Filipa Oliveira-Ramos, Manuel Salgado, Paula Estanqueiro, José Melo-Gomes, Fernando Martins, José Costa, Carolina Furtado, Ricardo Figueira, Jaime C. Branco, João E. Fonseca, Helena Canhão, Ana F. Mourão, Maria Jose Santos, Andrea Coda, Samuel Cassidy, Kerry West, Gordon Hendry, Debra Grech, Julie Jones, Fiona Hawke, Davinder Singh Grewal, Charlene Foley, Orla Killeen, Emma MacDermott, Douglas Veale, Ursula Fearon, Dilek Konukbay, Ela Tarakci, Nilay Arman, Sezgin Şahin, Jane Munro, Esi Morgan, Meredith Riebschleger, Jennifer Horonjeff, Vibeke Strand, Clifton Bingham, Ma. Theresa M. Collante, Margarita Ganeva, Stefan Stefanov, Albena Telcharova, Dimitrina Mihaylova, Radoslava Saraeva, Reni Tzveova, Radka Kaneva, Adelina Tsakova, Katya Temelkova, GRANT Medical University, Sofia 68/, Maria Mercedes C. Picarelli, Luiz C. Danzmann, Florencia Barbé-Tuana, Lucas K. Grun, Marcus H. Jones, Marijan Frković, Karla Ištuk, Ika Birkić, Saša Sršen, Marija Jelušić, Alan Easton, Rachael Quarmby, Raju Khubchandani, Mercedes Chan, Radoslav Srp, Katerina Kobrova, Dana Nemcova, Jozef Hoza, Michal Uher, Melania Saifridova, Lenka Linkova, Sirirat Charuvanij, Isree Leelayuwattanakul, Thita Pacharapakornpong, Sakda A.-O. Vallipakorn, Butsabong Lerkvaleekul, Soamarat Vilaiyuk, Stefano Lanni, Sergio Davì, Randy Q. Cron, Chiara Passarelli, Elisa Pisaneschi, Antonio Novelli, Claudia Bracaglia, Ivan Caiello, Kathy de Graaf, Florence Guilhot, Walter Ferlin, Grant Schulert, Alexi A. Grom, Robert Nelson, Cristina de Min, Dirk Holzinger, Christoph Kessel, Ndate Fall, Alexei Grom, Wilco de Jager, Raffaele Strippoli, Anna Horne, Stephan Ehl, Sandra Ammann, Kai Lehmberg, Karin Beutel, Dirk Foell, AnnaCarin Horne, Laura Pagani, Graciela Espada, Yi-jin Gao, Susan Shenoi, Sheila Weitzman, Giusi Prencipe, Antonia Pascarella, Walter G. Ferlin, Laurence Chatel, Philippe Jacqmin, Kathy De Graaf, Maria Ballabio, Zoë Johnson, Geneviève Lapeyre, Fabrizio de Benedetti, de Min Cristina, Hiroyuki Wakiguchi, Shunji Hasegawa, Reiji Hirano, Fumiko Okazaki, Tamaki Nakamura, Hidenobu Kaneyasu, Shouichi Ohga, Kazuko Yamazaki, Tomo Nozawa, Taichi Kanetaka, Shuichi Ito, Shumpei Yokota, Kirsty McLellan, Ishbel MacGregor, Neil Martin, Joyce Davidson, Sandra Hansmann, Andreas Eikelberg, Iris Haug, Sabrina Schuller, Susanne M. Benseler, Single Hub and Access point for paediatric Rheumatology in Europe (SHARE), Liliia S. Nazarova, Kseniia V. Danilko, Viktor A. Malievsky, Tatiana V. Viktorova, Angela Mauro, Angela Barnicoat, Jane Hurst, Nathalie Canham, Sandrine Lacassagne, Anastasia Wiener, Boris Hügle, Bernd Denecke, Ivan Costa-Filho, Johannes Peter Haas, Klaus Tenbrock, David Popp, Arjan Boltjes, Frank Rühle, Stefanie Herresthal, Femke van Wijk, Joachim Schultze, Monika Stoll, Luisa Klotz, Thomas Vogl, Johannes Roth, Estefania Quesada-Masachs, Daniel Álvarez de la Sierra, Marina Garcia Prat, Ana M. Marín Sánchez, Ricardo Pujol Borrell, Sara Marsal Barril, Mónica Martínez Gallo, Consuelo Modesto Caballero, Iryna Chyzheuskaya, Lyudmyla M. Byelyaeva, Rostislav M. Filonovich, Helena K. Khrustaleva, Larisa I. Zajtseva, Tamara M. Yuraga, Thomas Giner, Lukas Hackl, Julia Albrecht, Reinhard Würzner, Juergen Brunner, Marta Minute, Fulvio Parentin, Agostino Nocerino, Mette Nørgaard, Mikel Alberdi-Saugstrup, Marek S. Zak, Susan M. Nielsen, Ellen Nordal, Klaus G. Müller, Nordic Study Group of Pediatric Rheumatology (NoSPeR), Mojca Zajc Avramovič, Vita Dolžan, Nataša Toplak, Tadej Avčin, N. Ruperto, D. J. Lovell, C. Wallace, M. Toth, I. Foeldvari, J. Bohnsack, D. Milojevic, C. Rabinovich, D. Kingsbury, K. Marzan, P. Quartier, K. Minden, E. Chalom, G. Horneff, R. M. Kuester, J. Dare, M. Heinrich, H. Kupper, J. Kalabic, H. I. Brunner, on behalf of PRINTO and PRCSG, Ruben Burgos-Vargas, Tamas Constantin, Joke Dehoorne, Valda Stanevica, Katarzyna Kobusinska, Zbigniew Zuber, Richard Mouy, Ingrida Rumba-Rozenfelde, Chantal Job-Deslandre, Ronald Pederson, Jack Bukowski, Tina Hinnershitz, Bonnie Vlahos, Paula Keskitalo, Salla Kangas, Paula Vähäsalo, Raul A. Chavez Valencia, David Martino, Anne-Louise Ponsonby, Rachel Chiaroni-Clarke, Braydon Meyer, Roger C. Allen, Jonathan D. Akikusa, Jeffrey M. Craig, Richard Saffrey, Justine A. Ellis, Carol Wallace, Yosef Uziel, Gary Sterba, Rayfel Schneider, Ricardo Russo, Athimalaipet V. Ramanan, Jana Pachlopnik Schmid, Kim E Nichols, Paivi Miettunen, Toshiyuki Kitoh, Norman T. Ilowite, Jan-Inge Henter, Alexei A Grom, Edward M. Behrens, Tadej Avcin, Maurizio Aricò, Sriharsha Grevich, Peggy Lee, Sarah Ringold, Brian Leroux, Hannah Leahey, Megan Yuasa, Jessica Foster, Jeremy Sokolove, Lauren Lahey, William Robinson, Joshua Newson, Anne Stevens, Stephanie J. W. Shoop, Suzanne M. M. Verstappen, Wendy Thomson, Janet E. McDonagh, CAPS, Timothy Beukelman, Yuki Kimura, Marc Natter, Norm Ilowite, Kelly Mieszkalski, Grendel Burrell, Brian Best, Helen Bristow, Shannon Carr, Anne Dennos, Rachel Kaufmann, Laura Schanberg, for the CARRA Registry Investigators, Gabriele Simonini, Francesca Lancini, Margaux Gerbaux, Phu-Quoc Lê, Laurence Goffin, Valérie Badot, Céline La, Laure Caspers, François Willermain, Alina Ferster, Maria Ceci, Francesco Licciardi, Marco Turco, Francesca Santarelli, Davide Montin, Claudia Toppino, Clotilde Alizzi, Bruno Papia, Beatrice Vergara, Umberto Corpora, Luca Messina, Maria Tsinti, Vasiliko Dermentzoglou, Panagiotis Tziavas, Marija Perica, Lana Tambić Bukovac, Mustafa Çakan, Nuray Aktay Ayaz, Gonca Keskindemirci, Michael Lang, Catherine Laing, Susanne Benseler, Tommy Gerschman, Nadia Luca, Heinrike Schmeling, Anastasia Dropol, Jaymi Taiani, Nicole Johnson, Brian Rusted, Panagiota Nalbanti, Polyxeni Pratsidou, Grigoris Pardalos, Vasiliki Tzimouli, Anna Taparkou, Maria Stavrakidou, Fotios Papachristou, Florence Kanakoudi-Tsakalidou, Peter Bale, Emily Robinson, Jason Palman, Elizabeth Ralph, Kimberly Gilmour, Clare Heard, Lucy R. Wedderburn, Yara Barrense-Dias, Antonarakis Gregory, Dhouib Amira, Scolozzi Paolo, Hanquinet Sylviane, Hofer Michaël, Nataliya Panko, Salah Shokry, Liudmila Rakovska, Sally Pino, Adriana Diaz-Maldonado, Pilar Guarnizo, Sofia Torreggiani, Paolo Cressoni, Umberto Garagiola, Giancarla Di Landro, Giampietro Farronato, Fabrizia Corona, Samantha Bell, Parveen Bhatti, Lee Nelson, Beth A. Mueller, T. A. Simon, A. Baheti, N. Ray, Z. Guo, Anasuya Hazra, Thomas Stock, Ronnie Wang, Charles Mebus, Christine Alvey, Manisha Lamba, Sriram Krishnaswami, Umberto Conte, Min Wang, Daniel Kingsbury, Elena Koskova, Elzbieta Smolewska, Richard K. Vehe, Daniel Lovell, Tomohiro Kubota, Junko Yasumura, Toshitaka Kizawa, Masato Yashiro, Tsuyoshi Yamatou, Yuichi Yamasaki, Syuji Takei, Yoshifumi Kawano, Ulrika Järpemo Nykvist, Bo Magnusson, Rikard Wicksell, Karin Palmblad, Gunnar L. Olsson, Mohammadreza Modaressi, Mohammad-Hassan Moradinejad, Valentina Seraya, Alisa Vitebskaya, Veronica Moshe, Gil Amarilyo, Liora Harel, Phillip J Hashkes, Amir Mendelson, Noa Rabinowicz, Yonit Reis, Zane Dāvidsone, Arina Lazareva, Ruta Šantere, Dace Bērziņa, Valda Staņēviča, Giulia Camilla Varnier, Susan Maillard, Cristina Ferrari, Silvia Zaffarano, Juvenile Dermatomyositis Research Group and European Federation of Immunological Societies, Judith Wienke, Felicitas Bellutti Enders, Lucas L. van den Hoogen, Jorre S. Mertens, Timothy R. Radstake, Henny G. Hotten, Ruth Fritsch, Lucy Wedderburn, Kiran Nistala, Berent Prakken, Annet van Royen-Kerkhof, Mohammad Alhemairi, Mohammed Muzaffer, Pieter Van Dijkhuizen, Claire T. Deakin, Stefania Simou, Maria De Iorio, Qiong Wu, Tania Amin, Lee Dossetter, Juvenile Dermatomyositis Research Group (JDRG), Raquel Campanilho-Marques, Claire Deakin, Clarissa A. Pilkington, on behalf of Juvenile Dermatomyositis Research Group (JDRG), Silvia Rosina, Sirisucha Soponkanaporn, on behalf of the UK Juvenile Dermatomyositis Research Group (JDRG), Zehra S. Arıcı, Gökçen D. Tuğcu, Ezgi D. Batu, Hafize E. Sönmez, Deniz Doğru-Ersöz, Beril Talim, Nural Kiper, Seza Özen, Alexander Solyom, Ezgi Batu, John Mitchell, Ariana Kariminejad, Fatemeh Hadipour, Zahra Hadipour, Marta Torcoletti, Carlo Agostoni, Maja Di Rocco, Pranoot Tanpaiboon, Andrea Superti-Furga, Luisa Bonafé, Nur Arslan, Norberto Guelbert, Karoline Ehlert, Giedre Grigelioniene, Ratna Puri, Edward Schuchman, Pilar Gomez, Tatiana Gonzalez, Ricardo Yepez, Camilo Vargas, GRIP study group, Falcini Fernanda, Gemma Lepri, Alessandra Ferrari, Marco Matucci-Cerinic, Antonella Meini, Gian Marco Moneta, Emiliano Marasco, Rebecca Nicolai, Luisa Bracci-Laudiero, Olga Kopchak, Alexander Mushkin, Alexey Maletin, Catalina Mosquera, Rita A. Amorim, Juliana Molina, Gustavo Moreira, Flávia H. Santos, Melissa Fraga, Livia Keppeke, Vanessa M. Silva, Camila Hirotsu, Sergio Tufik, Maria Teresa Terreri, Vinícius L. Braga, Maria Beatriz Fonseca, Vania Schinzel, Maria Teresa R. Terreri, Liliana Jorge, Liana Guerra, Edson Amaro Junior, Maria Cristina Castiglione, Alessandra Tricarico, Emily Boulter, Andre Schultz, Kevin Murray, Fernanda Falcini, Stefano Stagi, Eleonora Bellucci, Ingrid H. R. Grein, Gecilmara Pileggi, Natália B. F. Pinto, Aline L. de Oliveira, Lyudmila Belyaeva, Rostislav Filonovich, Helena Khrustaleva, Larisa Zajtseva, Jaanika Ilisson, Chris Pruunsild, Olivier Gilliaux, Francis Corazza, Christophe Lelubre, on behalf of PANLAR Pediatric Rheumatology Study Group, Zoilo Morel, Claudia Saad-Magalhães C, Luis Lira, Mabel Ladino, Ruth Eraso, Ivonne Arroyo, Clovis Silva, Carlos Rose, and PANLAR Pediatric Rheumatology Study Group

Subjects
Pediatrics, RJ1-570, Diseases of the musculoskeletal system, RC925-935
Published
2017
Full Text
View/download PDF

14. Congenital Lung Malformations: Shifting from Open to Thoracoscopic Surgery

Author

Girolamo Mattioli, Luca Pio, Nicola Massimo Disma, Michele Torre, Oliviero Sacco, Angela Pistorio, Clelia Zanaboni, Giovanni Montobbio, Fabio Barra, and Luca Antonio Ramenghi

Subjects
congenital lung malformations, CPAM, thoracoscopy, Pediatrics, RJ1-570
Abstract

Over the years the need for surgical treatment, timing of intervention, and the type of surgical approach have been discussed, but the treatment of congenital lung malformations remains controversial. The aim of this study was to compare the thoracotomy approach with the thoracoscopic technique by evaluating different surgical outcomes (duration of surgery, postoperative hospital stay, and complications). Methods: All patients operated from January 2011 to March 2015 for suspected congenital cystic lung were included in the study. Patients treated for congenital lobar emphysema and tracheobronchial neoplasms were excluded from the study. Results: In the analyzed period, 31 asymptomatic patients were treated: 18 lung resections were performed with thoracotomy (Group A) and 13 with the thoracoscopic approach (Group B). No significant differences were observed between the age and weight at surgery, length of the procedures, complications, and the need for postoperative intensive care between the two groups. The postoperative hospital stay in Group A was twice that for Group 2 (p = 0.0009). Conclusion: Comparing thoracoscopic surgery with the traditional open approach, we confirmed the superiority of minimally invasive treatment in terms of postoperative hospital stay. Common technical recommendations can help pediatric centers to develop the thoracoscopic approach for the treatment of congenital pulmonary malformations.

Published
2016
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15. Endothelial and smooth muscle cells from abdominal aortic aneurysm have increased oxidative stress and telomere attrition.

Author

Giuseppe Cafueri, Federica Parodi, Angela Pistorio, Maria Bertolotto, Francesco Ventura, Claudio Gambini, Paolo Bianco, Franco Dallegri, Vito Pistoia, Annalisa Pezzolo, and Domenico Palombo

Subjects
Medicine, Science
Abstract

BACKGROUND: Abdominal aortic aneurysm (AAA) is a complex multi-factorial disease with life-threatening complications. AAA is typically asymptomatic and its rupture is associated with high mortality rate. Both environmental and genetic risk factors are involved in AAA pathogenesis. Aim of this study was to investigate telomere length (TL) and oxidative DNA damage in paired blood lymphocytes, aortic endothelial cells (EC), vascular smooth muscle cells (VSMC), and epidermal cells from patients with AAA in comparison with matched controls. METHODS: TL was assessed using a modification of quantitative (Q)-FISH in combination with immunofluorescence for CD31 or α-smooth muscle actin to detect EC and VSMC, respectively. Oxidative DNA damage was investigated by immunofluorescence staining for 7, 8-dihydro-8-oxo-2'-deoxyguanosine (8-oxo-dG). RESULTS AND CONCLUSIONS: Telomeres were found to be significantly shortened in EC, VSMC, keratinocytes and blood lymphocytes from AAA patients compared to matched controls. 8-oxo-dG immunoreactivity, indicative of oxidative DNA damage, was detected at higher levels in all of the above cell types from AAA patients compared to matched controls. Increased DNA double strand breaks were detected in AAA patients vs controls by nuclear staining for γ-H2AX histone. There was statistically significant inverse correlation between TL and accumulation of oxidative DNA damage in blood lymphocytes from AAA patients. This study shows for the first time that EC and VSMC from AAA have shortened telomeres and oxidative DNA damage. Similar findings were obtained with circulating lymphocytes and keratinocytes, indicating the systemic nature of the disease. Potential translational implications of these findings are discussed.

Published
2012
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16. Dexamethasone Prophylaxis in Pediatric Open Heart Surgery Is Associated with Increased Blood Long Pentraxin PTX3: Potential Clinical Implications

Author

Franco Lerzo, Giuseppe Peri, Andrea Doni, Paola Bocca, Fabio Morandi, Angela Pistorio, Anna Maria Carleo, Alberto Mantovani, Vito Pistoia, and Ignazia Prigione

Subjects
Immunologic diseases. Allergy, RC581-607
Abstract

Glucocorticoid administration before cardiopulmonary bypass (CPB) can reduce the systemic inflammatory response and improve clinical outcome. Long pentraxin PTX3 is a novel inflammatory parameter that could play a protective cardiovascular role by regulating inflammation. Twenty-nine children undergoing open heart surgery were enrolled in the study. Fourteen received dexamethasone (1st dose 1.5 mg/Kg i.v. or i.m. the evening before surgery; 2nd dose 1.5 mg/kg i.v. before starting bypass) and fifteen children served as control. Blood PTX3, short pentraxin C-reactive protein (CRP), interleukin-1 receptor II (IL-1RII), fibrinogen and partial thromboplastin time (PTT) were assayed at different times. PTX3 levels significantly increased during CPB in dexamethasone-treated (+D) and dexamethasone-untreated (−D) subjects, but were significantly higher in +D than −D patients. CRP levels significantly increased both in +D and −D patients in the postoperative days, with values significantly higher in −D than +D patients. Fibrinogen and PTT values were significantly higher in −D than +D patients in the 1st postoperative day. IL-1RII plasma levels increased in the postoperative period in both groups. Dexamethasone prophylaxis in pediatric patients undergoing CPB for cardiac surgery is associated with a significant increase of blood PTX3 that could contribute to decreasing inflammatory parameters and improving patient clinical outcome.

Published
2011
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17. Morbidity and mortality after anesthesia in early life in Italy. A subgroup analysis of the NECTARINE Trial

Author

Bonfiglio, Rachele, Francavilla, Andrea, Gregori, Dario, Lenares, Elena, Kuppers, Beate, Mondarini, Maria C, Camporesi, Anna, Sbaraglia, Fabio, Disma, Nicola, Cristina AGAPITI, Marinella ASTUTO, Elena ATTANASI, Stefania BIANZINA, Federico BILOTTA, Giulia BOTTAZZI, Edoardo CALDERINI, Fabio CARAMELLI, Gilda CINNELLA, Laura COLANTONIO, Andrea CORTEGIANI, Antonella COTOIA, Brita DE LORENZO, Moreno FAVARATO, Simona Anna FINAMORE, Giulia FRASACCO, Dario GALANTE, Rossella GARRA, Chiara GRASSO, Maurizia GRAZZINI, Anna GUDDO, Francesca IZZO, Svetlana KOTZEVA, Roberto LEONE, Daniela LERRO, Bruno G. LOCATELLI, Micol MAFFIOLETTI, Leila MAMELI, Girolamo MATTIOLI, Alessandra MOSCARELLI, Camilla MICALIZZI, Carmelo MINARDI, Alessia MONTAGUTI, Gerald R. NEBA, Francesca PINZONI, Giuliana A. PORRO, Sergio PICARDO, Angela PISTORIO, Emanuele ROSSETTI, Maria SAMMARTINO, Rita SCALISI, Valter SONZOGNI, Simonetta TESORO, Cesare VEZZOLI, Alessandro VITTORI, Andrea WOLFLER, Nicola ZADRA, Clelia ZANABONI, Bonfiglio, Rachele, Francavilla, Andrea, Gregori, Dario, Lenares, Elena, Kuppers, Beate, Mondarini, Maria C, Camporesi, Anna, Sbaraglia, Fabio, Disma, Nicola, and Cristina AGAPITI, Marinella ASTUTO, Elena ATTANASI, Stefania BIANZINA, Federico BILOTTA, Giulia BOTTAZZI, Edoardo CALDERINI, Fabio CARAMELLI, Gilda CINNELLA, Laura COLANTONIO, Andrea CORTEGIANI, Antonella COTOIA, Brita DE LORENZO, Moreno FAVARATO, Simona Anna FINAMORE, Giulia FRASACCO, Dario GALANTE, Rossella GARRA, Chiara GRASSO, Maurizia GRAZZINI, Anna GUDDO, Francesca IZZO, Svetlana KOTZEVA, Roberto LEONE, Daniela LERRO, Bruno G. LOCATELLI, Micol MAFFIOLETTI, Leila MAMELI, Girolamo MATTIOLI, Alessandra MOSCARELLI, Camilla MICALIZZI, Carmelo MINARDI, Alessia MONTAGUTI, Gerald R. NEBA, Francesca PINZONI, Giuliana A. PORRO, Sergio PICARDO, Angela PISTORIO, Emanuele ROSSETTI, Maria SAMMARTINO, Rita SCALISI, Valter SONZOGNI, Simonetta TESORO, Cesare VEZZOLI, Alessandro VITTORI, Andrea WOLFLER, Nicola ZADRA, Clelia ZANABONI

Subjects
Neonatal anesthesia
Abstract

Background: Recent literature on neonatal anesthesia focuses on the importance of keeping physiology within the ranges of normality to improve the long-term neurological outcome. The Neonate and Children audit of Anesthesia pRactice IN Europe (NECTARINE) showed a derangement of one or more than one physiological parameters during anesthesia needing a medical intervention in 35.2% of 6592 anesthesia procedure performed in infants up to 60 weeks postmenstrual age. Methods: Subanalysis of the Italian NECTARINE cohort providing a snapshot of anesthesia management, incidence of clinical events requiring intervention during anesthesia, and morbidity and mortality at 30 and 90 days. Secondary aim was to compare outcomes between Italy and Europe. Results: Twenty-three Italian centers recruited 501 patients (63% male, 37% female) undergoing 611 procedures (441 surgical and 170 non-surgical) with a mean gestational age at birth of 38 weeks. Events requiring a medical intervention during anesthesia occurred in 177 cases (28.9%), lower than those reported in Europe (35.3%). The majority of events concerned episodes of cardiovascular instability, most commonly due to hypotension. The incidence of mortality at 30 days was 2.7%, consistent with the European incidence. Conclusions: Anesthetizing neonates is challenging. It is crucial that neonatal anesthesia practice is performed in specialized centers to maximize the potential positive outcome. We recommend a certification of quality for Institutions providing care for very young patients.

Published
2023

18. Neonates undergoing pyloric stenosis repair are at increased risk of difficult airway management

Author

Nicola Disma, Thomas Engelhardt, Tom G. Hansen, Jurgen C. de Graaff, Katalin Virag, Walid Habre, Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, Francis Veyckemans, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Julie Lauweryns, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, René Urbanec, Petra Cajková, Vladimír Mixa, Yvona Sedláčková, Lenka Knoppová, Alena Zlámalová (neé Květoňová), Martin Vavřina, Jiří Žurek, Tom Hansen, Arash Afshari, Anders Bastholm Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Väli, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vähätalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Amory Catherine, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Hervé Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, François Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau Cécile Magne, Léa Petry, Lilica Ros, Aurélien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurélien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, François de la Brière, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Jost Kaufmann, Michael Laschat, Frank Wappler, Karin Becke, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Löffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schäfer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Schöler, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yücetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Söhle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Székely, Nandor Kosik, Veronika Maráczi, Janos Schnur, Judit Csillag, János Gál, Gergely Göbl, Balázs Hauser, András Petróczy, Gyula Tövisházi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O’Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria Cristina Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Francesca Izzo, Edoardo Calderini, Laura Brigitta Colantonio, Simona Anna Finamore, Giuliana Anna Porro, Rachele Bonfiglio, Svetlana Kotzeva, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Anna Guddo, Gerald Rogan Neba, Moreno Favarato, Bruno Guido Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Bilotta Federico, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Jurgen de Graaff, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Eva Schafrat, Jan Schreiber, Petronella Mari Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R. Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M. Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B. Boerke, Nil Ekiz, Kristoffer Stensrud, Inger Marie Drage, Erik Ramon Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Marzena Zielinska, Teresa Leal, Maria Soares, Pedro Pina, Sílvia Pinho, Maria Domingas Patuleia, Catarina Cruz Esteves, Helena Salgado, Maria João Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyíri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Dusica Simic, Irena Simic, Marija Stevic, Irena Vulicevic, Banská Bystrica, Barbora Cabanová, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela Pirtovšek Štupnik, Dolores Méndez, Gema Pino, Paloma Rubio, Alberto Izquierdo, Silvia López, Cristina González Serrano, Jesús Cebrián, Ana Peleteiro, Pilar Del Rey de Diego, Ernesto Martínez García, Carolina Tormo de las Heras, Pablo Troncoso Montero, Celia Arbona, David Artés, Alicia Chamizo, Silvia Serrano, Montserrat Suarez Comas, Francisco Escribá, Cristina Auli, Osvaldo Pérez Pardo, Natalia Sierra Biddle, Ceferina Suárez Castaño, María Isabel Villalobos Rico, Susana Manrique Muñoz, Irene García Martínez, Nuria Montferrer Estruch, Elena Vilardell Ortíz, Rodrigo Poves-Álvarez, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandström, Sporre Bengt, Rainer Dörenberg, Peter Frykholm, Maria Garcia, Ann Kvarnström, Emma Pontén, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Thomas Riva, Stefan Seiler, Yann Bögli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Laszlo Vutskits, Mattias Casutt, Martin Hölzle, Thomas Hurni, Martin Jöhr, Anna-Ursina Malär, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse Çiğdem Tutuncu, Zehra Hatipoğlu, Dilek Özcengiz, Hale Aksu Erdost, Elvan Öçmen, Çimen Olguner, Hilmi Ayanoglu, Pelin Corman Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbiçer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L. Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet Wolfe Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano Scalia Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, Dowell Julia, Feijten Prisca, Harlet Pierre, Herbineaux Sarah, Leva Brigitte, Plichon Benoît, Virág Katalin, and Anesthesiology

Subjects
Europe, Anesthesiology and Pain Medicine, Infant, Newborn, Intubation, Intratracheal, Humans, Infant, airway management, complications, difficult airway, neonatal anaesthesia, pyloric stenosis, tracheal intubation, Anesthesia, Pyloric Stenosis, Hypertrophic, Airway Management, Child, Hypoxia
Abstract

Background: Hypertrophic pyloric stenosis in otherwise healthy neonates frequently requires urgent surgical procedure but anaesthesia care may result in respiratory complications, such as hypoxaemia, pulmonary aspiration of gastric contents, and postoperative apnoea. The primary aim was to study whether or not the incidence of difficult airway management and of hypoxaemia in neonates undergoing pyloric stenosis repair was higher than that in neonates undergoing other surgeries. Methods: Data on neonates and infants undergoing anaesthesia and surgery for pyloric stenosis were extracted from the NEonate and Children audiT of Anesthesia pRactice In Europe (NECTARINE) database, for secondary analysis. Results: We identified 310 infants who had anaesthesia for surgery for pyloric stenosis. Difficult airway management (more than two attempts at laryngoscopy) was higher in children with pyloric stenosis when compared with the entire NECTARINE cohort (7.9% [95% confidence interval {CI}, 5.22–11.53] vs 4.4% [95% CI, 1.99–6.58]; relative risk [RR]=1.81 [95% CI, 1.21–2.69]; P=0.004), whereas transient hypoxaemia with oxygen saturation

Published
2022

19. Predictive Value of Magnetic Resonance Imaging in Patients With Juvenile Idiopathic Arthritis in Clinical Remission

Author

Francesca Magnaguagno, Gian Michele Magnano, Angelo Ravelli, Marta Mazzoni, Stefania Viola, Alessia Urru, Clara Malattia, and Angela Pistorio

Subjects
medicine.medical_specialty, Synovitis, Tenosynovitis, Adolescent, business.industry, Radiography, Arthritis, Disease, Symptom Flare Up, medicine.disease, Magnetic Resonance Imaging, Predictive value, Arthritis, Juvenile, Rheumatology, Internal medicine, Humans, Edema, Medicine, In patient, business, Bone Marrow Diseases, Retrospective Studies, Subclinical infection
Abstract

To define the prevalence of subclinical synovitis on magnetic resonance imaging (MRI) in a large cohort of patients with juvenile idiopathic arthritis (JIA) in clinical remission and to evaluate its predictive value in terms of disease flare and joint deterioration.Ninety patients with clinically inactive JIA who underwent a contrast-enhanced (CE)-MRI of a previously affected joint were retrospectively included. Each joint was evaluated for synovitis, tenosynovitis, and bone marrow edema. Baseline and follow-up radiographs were assessed to evaluate structural damage progression.CE-MRI was acquired in 45 wrists, 30 hips, 13 ankles, and 2 knees. Subclinical synovitis was detected in 59 (65.5%) of 90 patients and bone marrow edema in 42 (46.7%) of 90 patients. Fifty-seven of 90 (63.3%) patients experienced a disease flare during follow-up. Forty-four of 59 (74.6%) patients with subclinical synovitis experienced a disease flare versus 13 (41.9%) of 31 patients with no residual synovitis on MRI (P = 0.002). The presence of subclinical synovitis was the best predictor of disease flare on multivariable regression analysis (hazard ratio [HR] 2.45, P = 0.003). Baseline and follow-up radiographs were available for 54 patients, and 17 (31.5%) of 54 patients experienced radiographic damage progression. The presence of bone marrow edema (HR 4.40, P = 0.045) and being17 years old (HR 3.51, P = 0.04) were strong predictors of joint damage progression in the multivariable analysis.MRI-detected subclinical inflammation was present in a large proportion of patients with JIA despite clinical remission. Subclinical synovitis and bone marrow edema have been shown to play a role in predicting the risk of disease relapse and joint deterioration, with potential implications for patients' management of the disease.

Published
2022

20. Genotype–phenotype correlation in contactin-associated protein-like 2 (CNTNAP-2) developmental disorder

Author

Gianluca D’Onofrio, Andrea Accogli, Mariasavina Severino, Haluk Caliskan, Tomislav Kokotović, Antonela Blazekovic, Kristina Gotovac Jercic, Silvana Markovic, Tamara Zigman, Krnjak Goran, Nina Barišić, Vlasta Duranovic, Ana Ban, Fran Borovecki, Danijela Petković Ramadža, Ivo Barić, Walid Fazeli, Peter Herkenrath, Carla Marini, Roberta Vittorini, Vykuntaraju Gowda, Arjan Bouman, Clarissa Rocca, Issam Azmi Alkhawaja, Bibi Nazia Murtaza, Malik Mujaddad Ur Rehman, Chadi Al Alam, Gisele Nader, Maria Margherita Mancardi, Thea Giacomini, Siddharth Srivastava, Javeria Raza Alvi, Hoda Tomoum, Sara Matricardi, Michele Iacomino, Antonella Riva, Marcello Scala, Francesca Madia, Angela Pistorio, Vincenzo Salpietro, Carlo Minetti, Jean-Baptiste Rivière, Myriam Srour, Stephanie Efthymiou, Reza Maroofian, Henry Houlden, Sonja Catherine Vernes, Federico Zara, Pasquale Striano, Vanja Nagy, Clinical Genetics, University of St Andrews. School of Biology, University of St Andrews. Institute of Behavioural and Neural Sciences, and University of St Andrews. St Andrews Bioinformatics Unit

Subjects
MCC, NDAS, Genetics, QH426 Genetics, QH426, Genetics (clinical)
Abstract

Contactin-associated protein-like 2 (CNTNAP2) gene encodes for CASPR2, a presynaptic type 1 transmembrane protein, involved in cell–cell adhesion and synaptic interactions. Biallelic CNTNAP2 loss has been associated with “Pitt-Hopkins-like syndrome-1” (MIM#610042), while the pathogenic role of heterozygous variants remains controversial. We report 22 novel patients harboring mono- (n = 2) and bi-allelic (n = 20) CNTNAP2 variants and carried out a literature review to characterize the genotype–phenotype correlation. Patients (M:F 14:8) were aged between 3 and 19 years and affected by global developmental delay (GDD) (n = 21), moderate to profound intellectual disability (n = 17) and epilepsy (n = 21). Seizures mainly started in the first two years of life (median 22.5 months). Antiseizure medications were successful in controlling the seizures in about two-thirds of the patients. Autism spectrum disorder (ASD) and/or other neuropsychiatric comorbidities were present in nine patients (40.9%). Nonspecific midline brain anomalies were noted in most patients while focal signal abnormalities in the temporal lobes were noted in three subjects. Genotype–phenotype correlation was performed by also including 50 previously published patients (15 mono- and 35 bi-allelic variants). Overall, GDD (p p p = 0.012), ASD (p = 0.009), language impairment (p = 0.020) and severe cognitive impairment (p = 0.031) were significantly associated with the presence of biallelic versus monoallelic variants. We have defined the main features associated with biallelic CNTNAP2 variants, as severe cognitive impairment, epilepsy and behavioral abnormalities. We propose CASPR2-deficiency neurodevelopmental disorder as an exclusively recessive disease while the contribution of heterozygous variants is less likely to follow an autosomal dominant inheritance pattern.

Published
2023

21. Progetto di validazione di uno strumento per la valutazione della complessità assistenziale del paziente pediatrico: il Gaslini Pediatric Nursing Score

Author

Roberta Da Rin Della Mora, Simona Calza, Milva Subbrero, Roberta Tirone, Stefano Parodi, Angela Pistorio, Maria Grazia Calevo, Riccardo Haupt, and Silvia Scelsi

Abstract

Un servizio sanitario di qualità, oltre a possedere requisiti di alto livello e operare in sicurezza, deve organizzare le risorse in modo efficace prevedendo l'assegnazione dei pazienti al setting di cura appropriato e la riorganizzazione delle risorse sulla base delle loro necessità. Classificare i pazienti secondo i loro bisogni può fornire informazioni utili per l'organizzazione e l'erogazione dell'assistenza infermieristica. In ambito pediatrico diversi strumenti per la classificazione dei pazienti non soddisfano tutti i criteri metodologico-scientifici per essere ritenuti affidabili e validi, o sono difficilmente applicabili nella pratica. E' stato quindi costruito uno strumento (Gaslini Pediatric Nursing Score) per la valutazione della complessità dell'assistenza infermieristica pediatrica necessaria per soddisfare bisogni, identificati come core, di bambini o adolescenti ricoverati e delle loro famiglie.

Published
2022

22. Anakinra in Patients With Systemic Juvenile Idiopathic Arthritis

Author

Gabriella Giancane, Riccardo Papa, Sebastiaan Vastert, Francesca Bagnasco, Joost F. Swart, Pierre Quartier, Jordi Antón, Sylvia Kamphuis, Helga Sanner, Mia Glerup, Fabrizio De Benedetti, Elena Tsitsami, Agustin Remesal, Estefania Moreno, Jaime De Inocencio, Charlotte Myrup, Chiara Pallotti, Isabelle Koné-Paut, Karin Franck-Larsson, Håkan Malmström, Susanna Cederholm, Angela Pistorio, Nico Wulffraat, Nicolino Ruperto, and Pediatrics

Subjects
musculoskeletal diseases, Interleukin 1 Receptor Antagonist Protein, Treatment Outcome, Rheumatology, systemic juvenile idiopathic arthritis, Antirheumatic Agents, Immunology, Humans, Immunology and Allergy, long-term adverse effects, Registries, Arthritis, Juvenile, anakinra
Abstract

ObjectiveTo evaluate the long-term safety profile of anakinra in patients with systemic juvenile idiopathic arthritis (sJIA).MethodsData from patients with sJIA enrolled in the Pharmachild registry (ClinicalTrials.gov: NCT03932344) prior to September 30, 2018, and treated with anakinra were analyzed. The study endpoints were the occurrence of non-serious adverse events (SAEs) of at least moderate severity and SAEs, including macrophage activation syndrome (MAS), and the duration of anakinra treatment with reasons for discontinuation. All endpoints were analyzed overall by 6-month time windows, and in different treatment sets represented by those patients treated continuously with anakinra for at least 12, 18, and 24 months (set-12, -18, and -24, respectively).ResultsThree hundred six patients were enrolled. Of these patients, 46%, 34%, and 28% had been treated for at least 12, 18, and 24 months, respectively. Two hundred and one AEs, mostly represented by infections, were reported for 509.3 patient-years (PY) with an overall incidence rate (IR) of 39.5 per 100 PY. Among 56 SAEs (IR 11.0/100 PY), 23.2% were infections and 19.6% MAS episodes. The IR of AEs was higher during the first 6 months of anakinra treatment, followed by decreasing IRs in the long-term treatment sets. Treatment discontinuation occurred in 76% of patients, most frequently in the first 6 months, because of inefficacy (43%), remission (31%), or AEs/intolerance (15%). No deaths or malignancies occurred during anakinra treatment.ConclusionThe results of the present study confirm the long-term safety profile of anakinra in patients with sJIA and demonstrate an overall decreasing incidence of AEs over time. [ClinicalTrials.gov: NCT01399281 and NCT03932344]

Published
2022

23. Supplementary text from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

Supplementary legends

Published
2023

24. Supplementary table 4 from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

Microarray data analysis

Published
2023

25. Supplementary Figure 2 from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

PML and HIF1a expression

Published
2023

26. Supplementary table 2 from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

New neuroblastoma patient cohort

Published
2023

27. Data from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

Purpose: Neuroblastoma is a childhood malignancy originating from the sympathetic nervous system with a complex biology, prone to metastasize and relapse. High-risk, metastatic cases are explained in part by amplification or mutation of oncogenes, such as MYCN and ALK, and loss of tumor suppressor genes in chromosome band 1p. However, it is fundamental to identify other pathways responsible for the large portion of neuroblastomas with no obvious molecular alterations.Experimental Design: Neuroblastoma cell lines were used for the assessment of tumor growth in vivo and in vitro. Protein expression in tissues and cells was assessed using immunofluorescence and IHC. The association of promyelocytic leukemia (PML) expression with neuroblastoma outcome and relapse was calculated using log-rank and Mann–Whitney tests, respectively. Gene expression was assessed using chip microarrays.Results: PML is detected in the developing and adult sympathetic nervous system, whereas it is not expressed or is low in metastatic neuroblastoma tumors. Reduced PML expression in patients with low-risk cancers, that is, localized and negative for the MYCN proto-oncogene, is strongly associated with tumor recurrence. PML-I, but not PML-IV, isoform suppresses angiogenesis via upregulation of thrombospondin-2 (TSP2), a key inhibitor of angiogenesis. Finally, PML-I and TSP2 expression inversely correlates with tumor angiogenesis and recurrence in localized neuroblastomas.Conclusions: Our work reveals a novel PML-I–TSP2 axis for the regulation of angiogenesis and cancer relapse, which could be used to identify patients with low-risk, localized tumors that might benefit from chemotherapy. Clin Cancer Res; 22(13); 3398–409. ©2016 AACR.

Published
2023

28. Supplementary Table 3 from A Promyelocytic Leukemia Protein–Thrombospondin-2 Axis and the Risk of Relapse in Neuroblastoma

Author

Paolo Salomoni, Arturo Sala, Vito Pistoia, Neil Sebire, John Anderson, Hugues De The, Luigi Bagella, Kairbaan Hodivala-Dilke, Nigel J. Saunders, Alan J. Burns, Morgane Lebras, Daniel Morgenstern, Nicola Brindle, Tanguy Lechertier, Ximena Montano, Jack Barton, Alex Virasami, Angela Rita Sementa, Angela Pistorio, Tarik Regad, Olesya Chayka, Ana Paula Leite, Sandra Cantilena, Annalisa Pezzolo, Shalini Chakelam, Valentina Nieddu, and Maria Dvorkina

Abstract

Pathway analysis

Published
2023

30. 'To Randomize, or Not to Randomize, That is the Question'

Author

Nicolino Ruperto, Alberto Martini, and Angela Pistorio

Subjects
Biological Products, medicine.medical_specialty, Evidence-Based Medicine, business.industry, Immunology, MEDLINE, Arthritis, medicine.disease, Arthritis, Juvenile, Rheumatology, Antirheumatic Agents, Family medicine, Humans, Immunology and Allergy, Medicine, business, Randomized Controlled Trials as Topic
Abstract

Until the end of the last century there were no drugs approved for the treatment of juvenile idiopathic arthritis (JIA). This situation was revolutionized in the past 20 years thanks to 3 factors: the introduction in the USA and in Europe of paediatric legislations(1, 2), the availability of biologic disease-modifying anti-rheumatic drugs (b-DMARDs), and the existence of not-for-profit organizations with the mission to foster, facilitate and co-ordinate the development, conduct, analysis, and reporting of multi-centres studies in children with paediatric rheumatic diseases.

Published
2021

31. The pandemic seen through the eyes of the youngest people: evaluating psychological impact of the early COVID-19 related confinement on children and adolescents through the analysis of drawings and of an e-survey on their parents

Author

Sonia, DI Profio, Sara, Uccella, Paola, Cimellaro, Alessandra, Biolcati Rinaldi, Maria, D'Apruzzo, Serena, Rebora, Ludovica, Primavera, Alice, Zanetti, Sara, DE Giuseppe, Sabrina, Robotti, Camilla, Sacanna, Chiara, Perico, Deborah, Preiti, Lino, Nobili, Angela, Pistorio, and Cristina, Venturino

Subjects
Pediatrics, Perinatology and Child Health
Abstract

COVID-19 is having a significant impact on long term children' and adolescents' psychological health. We aimed to evaluate the direct early psychological and behavioural signs related to the COVID-19 pandemic outbreak and related confinement on children and adolescents.Children and adolescents' drawings were collected for a limited time window (16th March-10th April 2020) and analyzed. Their parents were asked in the following month to answer a qualitative e-survey on somatic complaints and behavioral changes of the participating children/adolescents.Ninety-eight drawings by children/adolescents (mean age 7.01±2.83 years) were analysed. Analyses of the 98 drawings reported signs of trauma in all (of them, 60.2% with moderate-to high levels). Children aged 3-5 years were more impacted, followed by preadolescents/adolescents aged 11-17 years. Parents reported somatic complaints in the 71.1% of their children/adolescents: the most frequent were increased appetite (35.6%), abdominal pain (20.0%) and headache (20.0%). Behavioral changes were observed in 77.8% of subjects: increased appetite (35.6%), abdominal pain (20.0%) and headache (20.0%) were more represented.Early psychological distress related to COVID-19 pandemic was observed both in children and in adolescents by the analysis of drawings and confirmed by their parents. Implementation of mental health-care services for preventing future psychopathological problems is mandatory.

Published
2022

32. The comparison between children and adolescents with asthma provided by the real-world 'ControL’Asma' study

Author

Michela Silvestri, Maria Angela Tosca, Gian Luigi Marseglia, Angela Pistorio, and Giorgio Ciprandi

Subjects
Male, Pulmonary and Respiratory Medicine, Allergy, Pediatrics, medicine.medical_specialty, Visual Analog Scale, Visual analogue scale, Physical examination, Disease, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Surveys and Questionnaires, Asthma control, medicine, Humans, Immunology and Allergy, 030212 general & internal medicine, Lung function, Rhinitis, Asthma, medicine.diagnostic_test, business.industry, medicine.disease, Comorbidity, respiratory tract diseases, Cross-Sectional Studies, 030228 respiratory system, Pediatrics, Perinatology and Child Health, Female, business
Abstract

Objective: Because asthma is a disease that changes over time, the Italian Society of Pediatric Allergy and Immunology launched a nationwide study on asthma control (the ControL'Asma study). The intent was to test the hypothesis that children with asthma could present a different pattern compared to adolescents. In the study, we compared children with adolescents in a real-world setting by analyzing the asthma control grade and other asthma-related parameters. Methods: This cross-sectional real-world study included 471 asthmatic children (< age 12) and adolescents (≥ age 12), mostly male (n = 326; 69.2%), who were consecutively enrolled at 10 tertiary pediatric allergy clinics. Asthma control level was evaluated according to the Global Initiative for Asthma (GINA) guidelines, the Childhood Asthma Control Test (cACT) questionnaire for children, and the Asthma Control Test (ACT) questionnaire for adolescents, history, rhinitis comorbidity, allergy, clinical examination, lung function, and perception of asthma symptoms assessed by visual analogue scale (VAS). Results: There was no difference between the age groups in asthma control as determined by both GINA criteria and the cACT/ACT questionnaires. However, adolescents with asthma had significantly more frequent rhinitis comorbidity (p = 0.02; OR =2.07) and allergy (p = 0.012; OR =3.72) than children. Asthma severity, lung function, and symptom perception were not different between age groups. Conclusions: The current study showed that asthma control is not associated with age in young people. Adolescents with asthma did experience rhinitis and allergy more frequently than children. These findings reflect the progressive nature of the allergic phenotype in young patients.

Published
2021

33. Difficult tracheal intubation in neonates and infants. NEonate and Children audiT of Anaesthesia pRactice IN Europe (NECTARINE)

Author

Nicola Disma, Katalin Virag, Thomas Riva, Jost Kaufmann, Thomas Engelhardt, Walid Habre, Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, Francis Veyckemans, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Julie Lauweryns, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, René Urbanec, Petra Cajková, Vladimír Mixa, Yvona Sedláčková, Lenka Knoppová, Alena Zlámalová (neé Květoňová), Martin Vavřina, Jiří Žurek, Tom Hansen, Arash Afshari, Anders Bastholm Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Väli, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vähätalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Catherine Amory, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Hervé Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, François Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau, Cécile Magne, Léa Petry, Lilica Ros, Aurélien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurélien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, François de la Brière, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Michael Laschat, Frank Wappler, Karin Becke, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Löffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schäfer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Schöler, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yücetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Söhle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Székely, Nandor Kosik, Veronika Maráczi, Janos Schnur, Judit Csillag, János Gál, Gergely Göbl, Balázs Hauser, András Petróczy, Gyula Tövisházi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O’Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria Cristina Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Andrea Wolfler, Edoardo Calderini, Laura Brigitta Colantonio, Simona Anna Finamore, Giuliana Anna Porro, Rachele Bonfiglio, Svetlana Kotzeva, Leila Mameli, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Clelia Zanaboni, Anna Guddo, Gerald Rogan Neba, Moreno Favarato, Bruno Guido Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Federico Bilotta, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Jurgen de Graaff, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Wolfgang Buhre, Eva Schafrat, Jan Schreiber, Petronella Mari Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R. Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M. Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B. Boerke, Nil Ekiz, Kristoffer Stensrud, Inger Marie Drage, Erik Ramon Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Marzena Zielinska, Teresa Leal, Maria Soares, Pedro Pina, Sílvia Pinho, Maria Domingas Patuleia, Catarina Cruz Esteves, Helena Salgado, Maria João Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyíri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Dusica Simic, Irena Simic, Marija Stevic, Irena Vulicevic, Barbora Cabanová, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela Pirtovšek Štupnik, Dolores Méndez, Gema Pino, Paloma Rubio, Alberto Izquierdo, Silvia López, Cristina González Serrano, Jesús Cebrián, Ana Peleteiro, Pilar Del Rey de Diego, Ernesto Martínez García, Carolina Tormo de las Heras, Pablo Troncoso Montero, Celia Arbona, David Artés, Alicia Chamizo, Silvia Serrano, Montserrat Suarez Comas, Francisco Escribá, Cristina Auli, Osvaldo Pérez Pardo, Natalia Sierra Biddle, Ceferina Suárez Castaño, María Isabel Villalobos Rico, Susana Manrique Muñoz, Irene García Martínez, Nuria Montferrer Estruch, Elena Vilardell Ortíz, Rodrigo Poves-Álvarez, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandström, Sporre Bengt, Rainer Dörenberg, Peter Frykholm, Maria Garcia, Ann Kvarnström, Emma Pontén, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Stefan Seiler, Yann Bögli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Laszlo Vutskits, Mattias Casutt, Martin Hölzle, Thomas Hurni, Martin Jöhr, Anna-Ursina Malär, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse Çiğdem Tutuncu, Zehra Hatipoğlu, Dilek Özcengiz, Hale Aksu Erdost, Elvan Öçmen, Çimen Olguner, Hilmi Ayanoglu, Pelin Corman Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbiçer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L. Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet Wolfe Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano Scalia Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, Dowell Julia, Feijten Prisca, Harlet Pierre, Herbineaux Sarah, Leva Brigitte, Plichon Benoît, Virág Katalin, Critical care, Anesthesiology, Peri-operative and Emergency medicine (CAPE), Anesthesiology, HUS Children and Adolescents, Children's Hospital, Clinicum, Anestesiologian yksikkö, Disma, Nicola, Virag, Katalin, Riva, Thoma, Kaufmann, Jost, Engelhardt, Thoma, Habre, Walid, Breschan, Christian, Likar, Rudolf, Platzer, Manuela, Edelman, Isole, Eger, Johane, Heschl, Stefan, Messerer, Brigitte, Vittinghof, Maria, Kroess, Ruth, Stichlberger, Martina, Kahn, David, Pirotte, Thierry, Pregardien, Caroline, Veyckemans, Franci, Stevens, France, Berghmans, Johan, Bauters, Annemie, De Baerdemaeker, Luc, De Hert, Stefan, Lapage, Koen, Parashchanka, Aliaksandra, Van Limmen, Jurgen, Wyffels, Piet, Lauweryns, Julie, Najafi, Nadia, Vundelinckx, Jori, Butković, Diana, Kerovec Sorić, Ivana, Kralik, Sandra, Markić, Ana, Azman, Josip, Markic, Josko, Pupacic, Daniela, Frelich, Michal, Reimer, Petr, Urbanec, René, Cajková, Petra, Mixa, Vladimír, Sedláčková, Yvona, Knoppová, Lenka, Zlámalová (neé Květoňová), Alena, Vavřina, Martin, Žurek, Jiří, Hansen, Tom, Afshari, Arash, Bille, Anders Bastholm, Ellekvist, Marguerite, Ilmoja, Mari-Lii, Moor, Reet, Kikas, Reet, Väli, Merle, Kallio, Kariantti, Reponen, Elisa, Suominen, Pertti, Suvanto, Sami, Vähätalo, Raisa, Kokki, Hannu, Kokki, Merja, Harju, Jarkko, Kokkonen, Miia, Vieri, Jenni, Manner, Tuula, Amory, Catherine, Ludot, Hugue, Bert, Dina, Godart, Juliette, Laffargue, Anne, Dupont, Hervé, Urbina, Benjamin, Baujard, Catherine, Roulleau, Philippe, Staiti, Giuseppe, Bordes, Maryline, Nouette Gaulain, Karine, Hamonic, Yann, Semjen, Françoi, Jacqmarcq, Olivier, Lejus-Bourdeau, Caroline, Magne, Cécile, Petry, Léa, Ros, Lilica, Zang, Aurélien, Bennis, Mehdi, Coustets, Bernard, Fesseau, Rose, Constant, Isabelle, Khalil, Eliane, Sabourdin, Nada, Audren, Noemie, Descarpentries, Thoma, Fabre, Fanny, Legrand, Aurélien, Druot, Emilie, Orliaguet, Gille, Sabau, Lucie, Uhrig, Lynn, de la Brière, Françoi, Jonckheer, Karin, Mission, Jean-Paul, Scordo, Lucia, Couchepin, Caroline, Dadure, Christophe, De la Arena, Pablo, Hertz, Laurent, Pirat, Philippe, Sola, Chrystelle, Bellon, Myriam, Dahmani, Souhayl, Julien-Marsollier, Florence, Michelet, Daphne, Depret-Donatien, Veronique, Lesage, Anne, Laschat, Michael, Wappler, Frank, Becke, Karin, Brunner, Lena, Oppenrieder, Karin, Badelt, Gregor, Hochmuth, Karin, Koller, Bernhard, Reil, Anita, Richter, Sebastian, Fischer, Thoma, Diers, Anja, Schorer, Clemen, Weyland, Andrea, Cohausz, Ruth, Kretz, Franz-Josef, Löffler, Michaela, Wilbs, Marku, Hoehne, Claudia, Ulrici, Johanna, Goeters, Christiane, Flinspach, Armin, Klages, Matthia, Lindau, Simone, Messroghli, Leila, Zacharowski, Kai, Eisner, Christoph, Mueller, Thoma, Richter, Daniel, Schäfer, Melanie, Weigand, Marku, Weiterer, Sebastian, Ochsenreiter, Miriam, Schöler, Michael, Terboven, Tom, Eggemann, Isabel, Haussmann, Sascha, Leister, Nicola, Menzel, Christoph, Trieschmann, Uwe, Yücetepe, Sirin, Keilig, Susanna, Kranke, Peter, Jelting, Yvonne, Baehner, Torsten, Ellerkmann, Richard, Ghamari, Shahab, Neumann, Claudia, Söhle, Martin, Chloropoulou, Pelagia, Ntritsou, Vagia, Papagiannopoulou, Pinelopi, Garini, Eleana, Karafotia, Afroditi, Mammi, Panagoula, Bali, Evangelia, Iordanidou, Despoina, Malisiova, Anna, Polyzoi, Artemi, Tsiotou, Adelai, Sapi, Erzsebet, Székely, Edgar, Kosik, Nandor, Maráczi, Veronika, Schnur, Jano, Csillag, Judit, Gál, Jáno, Göbl, Gergely, Hauser, Baláz, Petróczy, Andrá, Tövisházi, Gyula, Blain, Stuart, Gallagher, Sarah, Harte, Sinead, Jackson, Mandy, Meehan, Emma, Nawoor, Zeenat, O’Hare, Brendan, Ross, Mark, Lerro, Daniela, Astuto, Marinella, Grasso, Chiara, Scalisi, Rita, Frasacco, Giulia, Lenares, Elena, Leone, Roberto, Grazzini, Maurizia, Minardi, Carmelo, Zadra, Nicola, Cinnella, Gilda, Cotoia, Antonella, Galante, Dario, De Lorenzo, Brita, Kuppers, Beate, Bottazzi, Giulia, Caramelli, Fabio, Mondardini, Maria Cristina, Rossetti, Emanuele, Picardo, Sergio, Vittori, Alessandro, Camporesi, Anna, Wolfler, Andrea, Calderini, Edoardo, Colantonio, Laura Brigitta, Finamore, Simona Anna, Porro, Giuliana Anna, Bonfiglio, Rachele, Kotzeva, Svetlana, Mameli, Leila, Mattioli, Girolamo, Micalizzi, Camilla, Montaguti, Alessia, Pistorio, Angela, Zanaboni, Clelia, Guddo, Anna, Neba, Gerald Rogan, Favarato, Moreno, Locatelli, Bruno Guido, Maffioletti, Micol, Sonzogni, Valter, Garra, Rossella, Sammartino, Maria, Sbaraglia, Fabio, Cortegiani, Andrea, Moscarelli, Alessandra, Attanasi, Elena, Tesoro, Simonetta, Agapiti, Cristina, Pinzoni, Francesca, Vezzoli, Cesare, Bilotta, Federico, Barzdina, Arta, Straume, Zane, Zundane, Anda, Lukosiene, Laura, Maraulaite, Irena, Razlevice, Ilona, Schmitz, Bernd, Mifsud, Stephanie, Aehling, Carolin, Allison, Celia, De Boer, Rient, Emal, Dina, Stevens, Marku, Buitenhuis, Marielle, de Graaff, Jurgen, De Liefde, Inge, Machotta, Andrea, Scoones, Gail, Staals, Lonneke, Tomas, Jeremy, Van der Knijff-van Dortmont, Anouk, Veldhuizen, Marianne, Alders, David, Buhre, Wolfgang, Schafrat, Eva, Schreiber, Jan, Vermeulen, Petronella Mari, Hendriks, Mark, Lako, Sandra, Voet-Lindner, Marieke, Pieters, Barbe, Scheffer, Gert-Jan, Tielens, Luc, Absalom, Anthony R., Bergsma, Margot, De Ruiter, Joke, Meier, Sascha, Volkers, Martin, Zweers, Tjerk, Beukers, Anne M., Boer, Christa, Dertinger, Jurgen, Numan, Sandra, Van Zaane, Ba, Boerke, Wenche B., Ekiz, Nil, Stensrud, Kristoffer, Drage, Inger Marie, Isern, Erik Ramon, Bartkowska-Sniatkowska, Alicja, Grzeskowiak, Malgorzata, Juzwa-Sobieraj, Magdalena, Rosada-Kurasińska, Jowita, Baranowski, Artur, Jakubowska, Karina, Lewandowska, Dorota, Mierzewska-Schmidt, Magdalena, Sawicki, Piotr, Urban-Lechowicz, Magdalena, Przemyslaw, Pomianek, Zielinska, Marzena, Leal, Teresa, Soares, Maria, Pina, Pedro, Pinho, Sílvia, Patuleia, Maria Dominga, Esteves, Catarina Cruz, Salgado, Helena, Santos, Maria João, Badeti, Rodica, Cindea, Iulia, Oana, Loredana, Gurita, Adriana, Ilie, Luminita, Mocioiu, Gabriel, Tabacaru, Radu, Trante, Irina, Munteanu, Valentin, Morariu, Mihai, Nyíri, Emese, Budic, Ivana, Marjanovic, Vesna, Drašković, Biljana, Pandurov, Marina, Ilic, Jordanka, Mandras, Ana, Rados, Zdenka, Stankovic, Nikola, Suica, Maja, Vasiljevic, Sladjana, Knezevic, Mirjana, Milojevic, Irina, Petrov, Ivana, Puric Racic, Selena, Simic, Dusica, Simic, Irena, Stevic, Marija, Vulicevic, Irena, Cabanová, Barbora, Hanula, Miloslav, Berger, Jelena, Janjatovic, Darja, Pirtovšek Štupnik, Špela, Méndez, Dolore, Pino, Gema, Rubio, Paloma, Izquierdo, Alberto, López, Silvia, González Serrano, Cristina, Cebrián, Jesú, Peleteiro, Ana, Del Rey de Diego, Pilar, Martínez García, Ernesto, Tormo de las Heras, Carolina, Troncoso Montero, Pablo, Arbona, Celia, Artés, David, Chamizo, Alicia, Serrano, Silvia, Suarez Comas, Montserrat, Escribá, Francisco, Auli, Cristina, Pérez Pardo, Osvaldo, Sierra Biddle, Natalia, Suárez Castaño, Ceferina, Villalobos Rico, María Isabel, Manrique Muñoz, Susana, García Martínez, Irene, Montferrer Estruch, Nuria, Vilardell Ortíz, Elena, Poves-Álvarez, Rodrigo, Kohn, Ivan, Lindestam, Ulf, Reinhard, Jarl, Castellheim, Albert, Sandström, Kerstin, Bengt, Sporre, Dörenberg, Rainer, Frykholm, Peter, Garcia, Maria, Kvarnström, Ann, Pontén, Emma, Bruelisauer, Thoma, Erdoes, Gabor, Kaiser, Heiko, Marchon, Mathia, Seiler, Stefan, Bögli, Yann, Dolci, Mirko, Marcucci, Carine, Pichon, Isabelle, Vutskits, Laszlo, Casutt, Mattia, Hölzle, Martin, Hurni, Thoma, Jöhr, Martin, Malär, Anna-Ursina, Mauch, Jacqueline, Erb, Thoma, Oeinck, Karin, Akin, Mine, Keskin, Gulsen, Senayli, Yesim, Kaya, Guner, Kendigelen, Pinar, Tutuncu, Ayse Çiğdem, Hatipoğlu, Zehra, Özcengiz, Dilek, Erdost, Hale Aksu, Öçmen, Elvan, Olguner, Çimen, Ayanoglu, Hilmi, Dincer, Pelin Corman, Umuroglu, Tumay, Azizoglu, Mustafa, Birbiçer, Handan, Doruk, Nurcan, Sagun, Aslı, Baris, Sibel, Dmytriiev, Dmytro, Kuchi, Sridevi, Masip, Nuria, Brooks, Peter, Hare, Alison, Ahmad, Nargi, Casey, Michelle, De Silva, Sam, Dobby, Nadine, Krishnan, Prakash, Sogbodjor, L. Amaki, Walker, Ellie, Walker, Suellen, King, Stephanie, Nicholson, Katy, Quinney, Michelle, Stevens, Paul, Blevin, Andrew, Giombini, Mariangela, Goonasekera, Chulananda, Adil, Sadia, Bew, Stephanie, Bodlani, Carol, Gilpin, Dan, Jinks, Stephanie, Malarkkan, Nalini, Miskovic, Alice, Pad, Rebecca, Wolfe Barry, Juliet, Abbott, Joy, Armstrong, Jame, Cooper, Natalie, Crate, Lindsay, Emery, John, James, Kathryn, King, Hannah, Martin, Paul, Scalia Catenacci, Stefano, Bomont, Rob, Smith, Paul, Mele, Sara, Verzelloni, Alessandra, Dix, Philippa, Bell, Graham, Gordeva, Elena, McKee, Lesley, Ngan, Esther, Scheffczik, Jutta, Tan, Li-En, Worrall, Mark, Cassar, Carmel, Goddard, Kevin, Barlow, Victoria, Oshan, Vimmi, Shah, Khairi, Bell, Sarah, Daniels, Lisa, Gandhi, Monica, Pachter, David, Perry, Chri, Robertson, Andrew, Scott, Carmen, Waring, Lynne, Barnes, David, Childs, Sophie, Norman, Joanne, Sunderland, Robin, Julia, Dowell, Prisca, Feijten, Pierre, Harlet, Sarah, Herbineaux, Brigitte, Leva, Benoît, Plichon, Katalin, Virág, IOO, ACS - Microcirculation, APH - Quality of Care, and ANS - Neuroinfection & -inflammation

Subjects
Male, Time Factors, paediatric, medicine.medical_treatment, morbidity, Severity of Illness Index, 0302 clinical medicine, 030202 anesthesiology, Risk Factors, Clinical endpoint, Anesthesia, Prospective Studies, 610 Medicine & health, Hypoxia, Medical Audit, medicine.diagnostic_test, ddc:617, infants, Incidence (epidemiology), Incidence, Age Factors, anaesthesia, respiratory system, Europe, Treatment Outcome, Cohort, Female, medicine.symptom, Bradycardia, Laryngoscopy, Anesthesia/adverse effects/mortality, Risk Assessment, Europe/epidemiology, 03 medical and health sciences, Laryngoscopy/adverse effects/mortality, Hypoxia/diagnosis/epidemiology/mortality, medicine, Intubation, Intratracheal, Humans, difficult intubation, business.industry, Tracheal intubation, Infant, Newborn, Infant, Perioperative, Newborn, 3126 Surgery, anesthesiology, intensive care, radiology, mortality, neonates, Clinical trial, Intratracheal, Anesthesiology and Pain Medicine, airway, Intubation, Intratracheal/adverse effects/mortality, neonate, Intubation, business, airways
Abstract

Background: Neonates and infants are susceptible to hypoxaemia in the perioperative period. The aim of this study was to analyse interventions related to anaesthesia tracheal intubations in this European cohort and identify their clinical consequences. Methods: We performed a secondary analysis of tracheal intubations of the European multicentre observational trial (NEonate and Children audiT of Anaesthesia pRactice IN Europe [NECTARINE]) in neonates and small infants with difficult tracheal intubation. The primary endpoint was the incidence of difficult intubation and the related complications. The secondary endpoints were the risk factors for severe hypoxaemia attributed to difficult airway management, and 30 and 90 day outcomes. Results: Tracheal intubation was planned in 4683 procedures. Difficult tracheal intubation, defined as two failed attempts of direct laryngoscopy, occurred in 266 children (271 procedures) with an incidence (95% confidence interval [CI]) of 5.8% (95% CI, 5.1e6.5). Bradycardia occurred in 8% of the cases with difficult intubation, whereas a significant decrease in oxygen saturation (SpO2

Published
2021

34. Peri-operative red blood cell transfusion in neonates and infants: NEonate and Children audiT of Anaesthesia pRactice IN Europe: A prospective European multicentre observational study

Author

Fuchs A., Disma N., Virag K., Ulmer F., Habre W., de Graaff J. C., Riva T., NECTARINE Group of the European Society of Anaesthesiology and Intensive Care Clinical Trial Network: Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, Francis Veyckemans, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, René Urbanec, Petra Cajková, Vladimír Mixa, Yvona Sedláčková, Lenka Knoppová, Alena Zlámalová, Martin Vavřina, Jiří Žurek, Tom Hansen, Arash Afshari, Anders Bastholm Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Väli, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vähätalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Catherine Amory, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Hervé Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, François Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau, Cécile Magne, Léa Petry, Lilica Ros, Aurélien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurélien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, François de la Brière, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Jost Kaufmann, Michael Laschat, Frank Wappler, Karin Becke, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Löffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schäfer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Schöler, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yücetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Söhle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Székely, Nandor Kosik, Veronika Maráczi, Janos Schnur, Judit Csillag, János Gál, Gergely Göbl, Balázs Hauser, András Petróczy, Gyula Tövisházi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O'Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria Cristina Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Andrea Wolfler, Edoardo Calderini, Laura Brigitta Colantonio, Simona Anna Finamore, Giuliana Anna Porro, Rachele Bonfiglio, Svetlana Kotzeva, Leila Mameli, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Clelia Zanaboni, Anna Guddo, Gerald Rogan Neba, Moreno Favarato, Bruno Guido Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Federico Bilotta, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Wolfgang Buhre, Eva Schafrat, Jan Schreiber, Petronella Mari Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B Boerk, Nil Ekiz, Kristoffer Stensrud, Inger Marie Drage, Erik Ramon Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Marzena Zielinska, Teresa Leal, Maria Soares, Pedro Pina, Sílvia Pinho, Maria Domingas Patuleia, Catarina Cruz Esteves, Helena Salgado, Maria João Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyíri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Dusica Simic, Irena Simic, Marija Stevic, Irena Vulicevic, Barbora Cabanová, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela Pirtovšek Štupnik, Dolores Méndez, Gema Pino, Paloma Rubio, Alberto Izquierdo, Silvia López, Cristina González Serrano, Jesús Cebrián, Ana Peleteiro, Pilar Del Rey de Diego, Ernesto Martínez García, Carolina Tormo de Las Heras, Pablo Troncoso Montero, Celia Arbona, David Artés, Alicia Chamizo, Silvia Serrano, Montserrat Suarez Comas, Francisco Escribá, Cristina Auli, Osvaldo Pérez Pardo, Natalia Sierra Biddle, Ceferina Suárez Castaño, María Isabel Villalobos Rico, Susana Manrique Muñoz, Irene García Martínez, Nuria Montferrer Estruch, Elena Vilardell Ortíz, Rodrigo Poves-Álvarez, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandström, Sporre Bengt, Rainer Dörenberg, Peter Frykholm, Maria Garcia, Ann Kvarnström, Emma Pontén, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Stefan Seiler, Yann Bögli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Laszlo Vutskits, Mattias Casutt, Martin Hölzle, Thomas Hurni, Martin Jöhr, Anna-Ursina Malär, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse Çiğdem Tutuncu, Zehra Hatipoğlu, Dilek Özcengiz, Hale Aksu Erdost, Elvan Öçmen, Çimen Olguner, Hilmi Ayanoglu, Pelin Corman Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbiçer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet Wolfe Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano Scalia Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, Fuchs A., Disma N., Virag K., Ulmer F., Habre W., de Graaff J.C., Riva T., NECTARINE Group of the European Society of Anaesthesiology and Intensive Care Clinical Trial Network: Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, Francis Veyckemans, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, René Urbanec, Petra Cajková, Vladimír Mixa, Yvona Sedláčková, Lenka Knoppová, Alena Zlámalová, Martin Vavřina, Jiří Žurek, Tom Hansen, Arash Afshari, Anders Bastholm Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Väli, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vähätalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Catherine Amory, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Hervé Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, François Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau, Cécile Magne, Léa Petry, Lilica Ros, Aurélien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurélien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, François de la Brière, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Jost Kaufmann, Michael Laschat, Frank Wappler, Karin Becke, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Löffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schäfer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Schöler, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yücetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Söhle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Székely, Nandor Kosik, Veronika Maráczi, Janos Schnur, Judit Csillag, János Gál, Gergely Göbl, Balázs Hauser, András Petróczy, Gyula Tövisházi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O'Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria Cristina Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Andrea Wolfler, Edoardo Calderini, Laura Brigitta Colantonio, Simona Anna Finamore, Giuliana Anna Porro, Rachele Bonfiglio, Svetlana Kotzeva, Leila Mameli, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Clelia Zanaboni, Anna Guddo, Gerald Rogan Neba, Moreno Favarato, Bruno Guido Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Federico Bilotta, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Wolfgang Buhre, Eva Schafrat, Jan Schreiber, Petronella Mari Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B Boerk, Nil Ekiz, Kristoffer Stensrud, Inger Marie Drage, Erik Ramon Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Marzena Zielinska, Teresa Leal, Maria Soares, Pedro Pina, Sílvia Pinho, Maria Domingas Patuleia, Catarina Cruz Esteves, Helena Salgado, Maria João Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyíri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Dusica Simic, Irena Simic, Marija Stevic, Irena Vulicevic, Barbora Cabanová, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela Pirtovšek Štupnik, Dolores Méndez, Gema Pino, Paloma Rubio, Alberto Izquierdo, Silvia López, Cristina González Serrano, Jesús Cebrián, Ana Peleteiro, Pilar Del Rey de Diego, Ernesto Martínez García, Carolina Tormo de Las Heras, Pablo Troncoso Montero, Celia Arbona, David Artés, Alicia Chamizo, Silvia Serrano, Montserrat Suarez Comas, Francisco Escribá, Cristina Auli, Osvaldo Pérez Pardo, Natalia Sierra Biddle, Ceferina Suárez Castaño, María Isabel Villalobos Rico, Susana Manrique Muñoz, Irene García Martínez, Nuria Montferrer Estruch, Elena Vilardell Ortíz, Rodrigo Poves-Álvarez, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandström, Sporre Bengt, Rainer Dörenberg, Peter Frykholm, Maria Garcia, Ann Kvarnström, Emma Pontén, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Stefan Seiler, Yann Bögli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Laszlo Vutskits, Mattias Casutt, Martin Hölzle, Thomas Hurni, Martin Jöhr, Anna-Ursina Malär, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse Çiğdem Tutuncu, Zehra Hatipoğlu, Dilek Özcengiz, Hale Aksu Erdost, Elvan Öçmen, Çimen Olguner, Hilmi Ayanoglu, Pelin Corman Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbiçer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet Wolfe Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano Scalia Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, and Anesthesiology

Subjects
Red Blood Cell Transfusion, NEONATE, Haemoglobin levels, 610 Medicine & health, Peri-operative, red blood cell transfusion, neonates, infants, High morbidity, Hemoglobins, TRANSFUSION, medicine, Clinical endpoint, Humans, Anesthesia, Prospective Studies, business.industry, Postmenstrual Age, Infant, Newborn, Perioperative, Europe, Red blood cell, Anesthesiology and Pain Medicine, medicine.anatomical_structure, Observational study, business, 610 Medizin und Gesundheit, Erythrocyte Transfusion
Abstract

BACKGROUND Little is known about current clinical practice concerning peri-operative red blood cell transfusion in neonates and small infants. Guidelines suggest transfusions based on haemoglobin thresholds ranging from 8.5 to 12���g���dl-1, distinguishing between children from birth to day 7 (week 1), from day 8 to day 14 (week 2) or from day 15 (���week 3) onwards. OBJECTIVE To observe peri-operative red blood cell transfusion practice according to guidelines in relation to patient outcome. DESIGN A multicentre observational study. SETTING The NEonate-Children sTudy of Anaesthesia pRactice IN Europe (NECTARINE) trial recruited patients up to 60 weeks' postmenstrual age undergoing anaesthesia for surgical or diagnostic procedures from 165 centres in 31 European countries between March 2016 and January 2017. PATIENTS The data included 5609 patients undergoing 6542 procedures. Inclusion criteria was a peri-operative red blood cell transfusion. MAIN OUTCOME MEASURES The primary endpoint was the haemoglobin level triggering a transfusion for neonates in week 1, week 2 and week 3. Secondary endpoints were transfusion volumes, 'delta haemoglobin' (preprocedure - transfusion-triggering) and 30-day and 90-day morbidity and mortality. RESULTS Peri-operative red blood cell transfusions were recorded during 447 procedures (6.9%). The median haemoglobin levels triggering a transfusion were 9.6 [IQR 8.7 to 10.9] g���dl-1 for neonates in week 1, 9.6 [7.7 to 10.4] g���dl-1 in week 2 and 8.0 [7.3 to 9.0] g���dl-1 in week 3. The median transfusion volume was 17.1 [11.1 to 26.4] ml���kg-1 with a median delta haemoglobin of 1.8 [0.0 to 3.6] g���dl-1. Thirty-day morbidity was 47.8% with an overall mortality of 11.3%. CONCLUSIONS Results indicate lower transfusion-triggering haemoglobin thresholds in clinical practice than suggested by current guidelines. The high morbidity and mortality of this NECTARINE sub-cohort calls for investigative action and evidence-based guidelines addressing peri-operative red blood cell transfusions strategies. TRIAL REGISTRATION ClinicalTrials.gov, identifier: NCT02350348.

Published
2021

35. Sleep and Sleep Complaints in Juvenile Fibromyalgia Syndrome

Author

Clara Malattia, Lorenzo Chiarella, Miriam Sansone, Angela Pistorio, Claudio Lavarello, Manuela Carpaneto, Raffaele Ferri, Angelo Ravelli, and Lino Nobili

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

ObjectiveTo investigate sleep quality in juvenile fibromyalgia syndrome (JFS) and its effect on the global burden of the disease.MethodsConsecutive patients with JFS who performed full-night polysomnography (PSG) were included in this cross-sectional study. JFS-related symptoms, neuropsychiatric features, and sleep quality were assessed using self-report measures. PSG sleep parameters, including N3 distribution index, were obtained from patients and age-matched healthy controls.ResultsWe included 25 patients (20 females, median age 15.7 yrs). Nonrestorative sleep was reported by 22 of 25 (88%) patients. Patients with JFS showed significantly longer sleep period time (P= 0.004) and increased wake after sleep onset (P= 0.03) compared to healthy peers. The N3 distribution index was significantly lower in patients than in the control group (P= 0.02). Subjective poor sleep quality was related to Widespread Pain Index (WPI;rs–0.65), Symptom Severity Scale (rs–0.64), depressive symptoms (rs–0.58), fatigue (rs–0.44), and symptom severity upon awakening (rs–0.65). The N3 distribution index was correlated to depressive symptoms (rs0.41) and irritability (rs0.40). On multiple regression analysis, WPI was predicted by subjective sleep quality (β –0.32,P= 0.04), whereas depressive symptoms were predicted by subjective sleep measures (β –0.32,P= 0.04) and PSG parameters (N3 min: β –0.07,P= 0.03).ConclusionSleep complaints are a key hallmark of JFS and have significant effect on relevant clinical domains of the disease, such as pain and depression.

Published
2023

36. Clinico-radiological features, molecular spectrum, and identification of prognostic factors in developmental and epileptic encephalopathy due to inosine triphosphate pyrophosphatase (ITPase) deficiency

Author

Marcello Scala, Saskia B. Wortmann, Namik Kaya, Menno D. Stellingwerff, Angela Pistorio, Emma Glamuzina, Clara D. Karnebeek, Cristina Skrypnyk, Katarzyna Iwanicka‐Pronicka, Dorota Piekutowska‐Abramczuk, Elżbieta Ciara, Frederic Tort, Beth Sheidley, Annapurna Poduri, Parul Jayakar, Anuj Jayakar, Jariya Upadia, Nicolette Walano, Tobias B. Haack, Holger Prokisch, Hesham Aldhalaan, Ehsan G. Karimiani, Yilmaz Yildiz, Ahmet C. Ceylan, Teresa Santiago‐Sim, Amy Dameron, Hui Yang, Mehran B. Toosi, Farah Ashrafzadeh, Javad Akhondian, Shima Imannezhad, Hanieh S. Mirzadeh, Shazia Maqbool, Aisha Farid, Mohamed A. Al‐Muhaizea, Meznah O. Alshwameen, Lama Aldowsari, Maysoon Alsagob, Ashwaq Alyousef, Rawan AlMass, Aljouhra AlHargan, Ali H. Alwadei, Maha M. AlRasheed, Dilek Colak, Hanan Alqudairy, Sameena Khan, Matthew A. Lines, M. Ángeles García Cazorla, Antonia Ribes, Eva Morava, Farah Bibi, Shahzad Haider, Matteo P. Ferla, Jenny C. Taylor, Hessa S. Alsaif, Abdulwahab Firdous, Mais Hashem, Chingiz Shashkin, Kairgali Koneev, Rauan Kaiyrzhanov, Stephanie Efthymiou, Queen Square Genomics, Thomas Schmitt‐Mechelke, Andreas Ziegler, Mahmoud Y. Issa, Hasnaa M. Elbendary, Pasquale Striano, Fowzan S. Alkuraya, Maha S. Zaki, Joseph G. Gleeson, Tahsin Stefan Barakat, Jorgen Bierau, Marjo S. Knaap, Reza Maroofian, Henry Houlden, Pediatric surgery, Amsterdam Neuroscience - Cellular & Molecular Mechanisms, Integrative Neurophysiology, Clinical Genetics, ANS - Cellular & Molecular Mechanisms, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, MUMC+: DA KG Lab Centraal Lab (9), and RS: FHML non-thematic output

Subjects
MUTATIONS, Metabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6], ASSOCIATION, heart disease, white matter abnormalities, Prognosis, Inosine, Itpa, Itpase, Congenital Microcephaly, Developmental And Epileptic Encephalopathy 35, Heart Disease, White Matter Abnormalities, ITPA, SDG 3 - Good Health and Well-being, Mutation, Genetics, Microcephaly, GROWTH, Humans, Inosine Triphosphate, Epilepsy, Generalized, ITPase, NUCLEOTIDE POOLS, Pyrophosphatases, congenital microcephaly, RETARDATION, developmental and epileptic encephalopathy 35, Genetics (clinical)
Abstract

Contains fulltext : 283128.pdf (Publisher’s version ) (Open Access) Developmental and epileptic encephalopathy 35 (DEE 35) is a severe neurological condition caused by biallelic variants in ITPA, encoding inosine triphosphate pyrophosphatase, an essential enzyme in purine metabolism. We delineate the genotypic and phenotypic spectrum of DEE 35, analyzing possible predictors for adverse clinical outcomes. We investigated a cohort of 28 new patients and reviewed previously described cases, providing a comprehensive characterization of 40 subjects. Exome sequencing was performed to identify underlying ITPA pathogenic variants. Brain MRI (magnetic resonance imaging) scans were systematically analyzed to delineate the neuroradiological spectrum. Survival curves according to the Kaplan-Meier method and log-rank test were used to investigate outcome predictors in different subgroups of patients. We identified 18 distinct ITPA pathogenic variants, including 14 novel variants, and two deletions. All subjects showed profound developmental delay, microcephaly, and refractory epilepsy followed by neurodevelopmental regression. Brain MRI revision revealed a recurrent pattern of delayed myelination and restricted diffusion of early myelinating structures. Congenital microcephaly and cardiac involvement were statistically significant novel clinical predictors of adverse outcomes. We refined the molecular, clinical, and neuroradiological characterization of ITPase deficiency, and identified new clinical predictors which may have a potentially important impact on diagnosis, counseling, and follow-up of affected individuals.

Published
2022

37. A prospective observational cohort study on the incidence of postoperative sore throat in the pediatric population

Author

Angela Pistorio, Alice Miskovic, Louise Frost, Mae Johnson, Elena Fernandez, and Nicola Disma

Subjects
Male, Adolescent, Vomiting, Stridor, Anesthesia, General, Laryngeal Masks, Cohort Studies, Postoperative Complications, Laryngeal mask airway, Risk Factors, Intubation, Intratracheal, otorhinolaryngologic diseases, Sore throat, Humans, Medicine, Postoperative Period, Prospective Studies, Laryngospasm, Child, business.industry, Incidence, Pharyngitis, Perioperative, United Kingdom, stomatognathic diseases, Anesthesiology and Pain Medicine, Child, Preschool, Anesthesia, Pediatrics, Perinatology and Child Health, Female, medicine.symptom, business
Abstract

BACKGROUND Postoperative sore throat is common after general anesthesia. The incidence in pediatric anesthesia is variable, and the etiology unclear. Establishing risk factors would enable prevention and could improve quality of care. AIMS We performed a prospective single-center cohort study aiming to establish the incidence of postoperative sore throat in children undergoing GA with an endotracheal tube or laryngeal mask airway. Secondary aims were to identify independent risk factors for sore throat and the incidence of other postoperative complications including stridor, laryngospasm, nausea and vomiting, and delayed oral intake. METHODS Between November 2017 and April 2018, perioperative data were collected from children aged 5-16 years undergoing general, plastic, urology, renal, and orthopedic surgery. Patients completed a postoperative questionnaire within 24 hours of surgery. RESULTS We screened 334 children for inclusion at a tertiary pediatric hospital in the United Kingdom. One hundred and ninety-seven patients were included in the final analysis. The frequency of postoperative sore throat was 36.5%. Stridor occurred in 1.5%, laryngospasm 1.0%, postoperative nausea 59.4%, vomiting in recovery 4.6%, and delayed oral intake due to postoperative sore throat 30%. Nausea, vomiting, thirst, and pain were associated with a sore throat. Univariate analysis showed anesthesia longer than 2 hours, and use of an endotracheal tube was statistically associated with higher risk of sore throat. Over 50% of children with an endotracheal tube cuff pressure

Published
2019

38. The practical clinical relevance of rhinitis classification in children with asthma

Author

Ilaria Brambilla, Maria Angela Tosca, Valentina De Vittori, Angela Pistorio, M. Leone, Luciana Indinnimeo, Michela Silvestri, Ahmad Kantar, Loredana Chini, Viviana Moschese, Elisa Anastasio, Gian Luigi Marseglia, Marzia Duse, Diego Peroni, Maria Di Cicco, Carlo Caffarelli, Paolo Del Barba, Giorgio Ciprandi, Riccardo Ciprandi, Roberta Olcese, Anna Maria Zicari, and Amelia Licari

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, business.industry, Immunology, MEDLINE, medicine.disease, Comorbidity, Text mining, Severity of illness, medicine, Immunology and Allergy, Clinical significance, Intensive care medicine, business, Asthma
Published
2019

39. Reply

Author

Nicolino, Ruperto, Alberto, Martini, and Angela, Pistorio

Published
2021

40. MEDB-35. Relationship between genetic profile, histology, clinical features and long-term outcome in young children medulloblastoma (YCMB) treated with upfront high dose chemotherapy (HDCT) in Italy

Author

Maria Luisa Garrè, Maura Massimino, Francesca Romana Buttarelli, Lorenza Gandola, Salvina Barra, Felice Giangaspero, Tobias Goschzik, Veronica Biassoni, Lorenza Pastorino, Angela Pistorio, and Torsten Pietsch

Subjects
Cancer Research, Oncology, Neurology (clinical)
Abstract

AIMS: We report a cohort of YCMB cases homogeneously treated with HDCT in two Italian institutions, and the prognostic impact of histology and genetics retrospectively evaluated. METHODS: All YCMB (aged≤3 years) treated with upfront HDCT in the period 1998-2019 were included, reclassified according to the WHO2021 classification of CNS tumours. Mutational status ofPTCH1, SUFU, and TP53 was analysed in selected cases. Histology and genetics were correlated with survival, secondary tumours(STs), and cancer predisposition syndromes(CPSs). RESULTS: Fifty-three patients were enrolled (62.3% male), median age 2.2 years. 21 had classic(CMB), 15 desmoplastic/nodular(DMB), 11 MBEN and 6 large-cell/anaplastic(AMB/LCMB) medulloblastoma. Metastases were present in 18. Genomic pattern showed SHH-TP53wt in 29 cases, non-WNT/non-SHH in 22; 2 were SHH-TP53mut. Induction chemotherapy (VCR/HDMTX, HDVP16, VCR/HDCTX and HDCARBO) was followed by 2-3 HDCT courses; irradiation reserved to cases with metastatic disease and/or residual tumours. 22 patients never received irradiation. SHH-TP53wt cases had significantly less metastasis (p=0.002), while non-WNT/non-SHH received more often irradiation (p

Published
2022

41. Corrigendum to ‘Difficult tracheal intubation in neonates and infants. NEonate and Children audiT of Anaesthesia pRactice IN Europe (NECTARINE):a prospective European multicentre observational study’ (Br J Anaesth 2021; 126: 1173–81) (British Journal of Anaesthesia (2021) 126(6) (1173–1181), (S0007091221001161), (10.1016/j.bja.2021.02.021))

Author

Nicola Disma, Katalin Virag, Thomas Riva, Jost Kaufmann, Thomas Engelhardt, Walid Habre, Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, Francis Veyckemans, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Julie Lauweryns, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, René Urbanec, Petra Cajková, Vladimír Mixa, Yvona Sedláčková, Lenka Knoppová, Alena Zlámalová (neé Květoňová), Martin Vavřina, Jiří Žurek, Tom Hansen, Arash Afshari, Anders Bastholm Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Väli, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vähätalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Catherine Amory, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Hervé Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, François Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau, Cécile Magne, Léa Petry, Lilica Ros, Aurélien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurélien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, François de la Brière, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Michael Laschat, Frank Wappler, Karin Becke, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Löffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schäfer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Schöler, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yücetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Söhle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Székely, Nandor Kosik, Veronika Maráczi, Janos Schnur, Judit Csillag, János Gál, Gergely Göbl, Balázs Hauser, András Petróczy, Gyula Tövisházi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O’Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria Cristina Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Andrea Wolfler, Edoardo Calderini, Laura Brigitta Colantonio, Simona Anna Finamore, Giuliana Anna Porro, Rachele Bonfiglio, Svetlana Kotzeva, Leila Mameli, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Clelia Zanaboni, Anna Guddo, Gerald Rogan Neba, Moreno Favarato, Bruno Guido Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Federico Bilotta, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Jurgen de Graaff, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Wolfgang Buhre, Eva Schafrat, Jan Schreiber, Petronella Mari Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R. Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M. Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B. Boerke, Nil Ekiz, Kristoffer Stensrud, Inger Marie Drage, Erik Ramon Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Marzena Zielinska, Teresa Leal, Maria Soares, Pedro Pina, Sílvia Pinho, Maria Domingas Patuleia, Catarina Cruz Esteves, Helena Salgado, Maria João Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyíri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Dusica Simic, Irena Simic, Marija Stevic, Irena Vulicevic, Barbora Cabanová, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela Pirtovšek Štupnik, Dolores Méndez, Gema Pino, Paloma Rubio, Alberto Izquierdo, Silvia López, Cristina González Serrano, Jesús Cebrián, Ana Peleteiro, Pilar Del Rey de Diego, Ernesto Martínez García, Carolina Tormo de las Heras, Pablo Troncoso Montero, Celia Arbona, David Artés, Alicia Chamizo, Silvia Serrano, Montserrat Suarez Comas, Francisco Escribá, Cristina Auli, Osvaldo Pérez Pardo, Natalia Sierra Biddle, Ceferina Suárez Castaño, María Isabel Villalobos Rico, Susana Manrique Muñoz, Irene García Martínez, Nuria Montferrer Estruch, Elena Vilardell Ortíz, Rodrigo Poves-Álvarez, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandström, Sporre Bengt, Rainer Dörenberg, Peter Frykholm, Maria Garcia, Ann Kvarnström, Emma Pontén, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Stefan Seiler, Yann Bögli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Laszlo Vutskits, Mattias Casutt, Martin Hölzle, Thomas Hurni, Martin Jöhr, Anna-Ursina Malär, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse Çiğdem Tutuncu, Zehra Hatipoğlu, Dilek Özcengiz, Hale Aksu Erdost, Elvan Öçmen, Çimen Olguner, Hilmi Ayanoglu, Pelin Corman Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbiçer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L. Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet Wolfe Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano Scalia Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, Dowell Julia, Feijten Prisca, Harlet Pierre, Herbineaux Sarah, Leva Brigitte, Plichon Benoît, Virág Katalin, Disma N., Virag K., Riva T., Kaufmann J., Engelhardt T., Habre W., Breschan C., Likar R., Platzer M., Edelman I., Eger J., Heschl S., Messerer B., Vittinghof M., Kroess R., Stichlberger M., Kahn D., Pirotte T., Pregardien C., Veyckemans F., Stevens F., Berghmans J., Bauters A., De Baerdemaeker L., De Hert S., Lapage K., Parashchanka A., Van Limmen J., Wyffels P., Lauweryns J., Najafi N., Vundelinckx J., Butkovic D., Kerovec Soric I., Kralik S., Markic A., Azman J., Markic J., Pupacic D., Frelich M., Reimer P., Urbanec R., Cajkova P., Mixa V., Sedlackova Y., Knoppova L., Zlamalova (nee Kvetonova) A., Vavrina M., Zurek J., Hansen T., Afshari A., Bille A.B., Ellekvist M., Ilmoja M.-L., Moor R., Kikas R., Vali M., Kallio K., Reponen E., Suominen P., Suvanto S., Vahatalo R., Kokki H., Kokki M., Harju J., Kokkonen M., Vieri J., Manner T., Amory C., Ludot H., Bert D., Godart J., Laffargue A., Dupont H., Urbina B., Baujard C., Roulleau P., Staiti G., Bordes M., Nouette Gaulain K., Hamonic Y., Semjen F., Jacqmarcq O., Lejus-Bourdeau C., Magne C., Petry L., Ros L., Zang A., Bennis M., Coustets B., Fesseau R., Constant I., Khalil E., Sabourdin N., Audren N., Descarpentries T., Fabre F., Legrand A., Druot E., Orliaguet G., Sabau L., Uhrig L., de la Briere F., Jonckheer K., Mission J.-P., Scordo L., Couchepin C., Dadure C., De la Arena P., Hertz L., Pirat P., Sola C., Bellon M., Dahmani S., Julien-Marsollier F., Michelet D., Depret-Donatien V., Lesage A., Laschat M., Wappler F., Becke K., Brunner L., Oppenrieder K., Badelt G., Hochmuth K., Koller B., Reil A., Richter S., Fischer T., Diers A., Schorer C., Weyland A., Cohausz R., Kretz F.-J., Loffler M., Wilbs M., Hoehne C., Ulrici J., Goeters C., Flinspach A., Klages M., Lindau S., Messroghli L., Zacharowski K., Eisner C., Mueller T., Richter D., Schafer M., Weigand M., Weiterer S., Ochsenreiter M., Scholer M., Terboven T., Eggemann I., Haussmann S., Leister N., Menzel C., Trieschmann U., Yucetepe S., Keilig S., Kranke P., Jelting Y., Baehner T., Ellerkmann R., Ghamari S., Neumann C., Sohle M., Chloropoulou P., Ntritsou V., Papagiannopoulou P., Garini E., Karafotia A., Mammi P., Bali E., Iordanidou D., Malisiova A., Polyzoi A., Tsiotou A., Sapi E., Szekely E., Kosik N., Maraczi V., Schnur J., Csillag J., Gal J., Gobl G., Hauser B., Petroczy A., Tovishazi G., Blain S., Gallagher S., Harte S., Jackson M., Meehan E., Nawoor Z., O'Hare B., Ross M., Lerro D., Astuto M., Grasso C., Scalisi R., Frasacco G., Lenares E., Leone R., Grazzini M., Minardi C., Zadra N., Cinnella G., Cotoia A., Galante D., De Lorenzo B., Kuppers B., Bottazzi G., Caramelli F., Mondardini M.C., Rossetti E., Picardo S., Vittori A., Camporesi A., Wolfler A., Calderini E., Colantonio L.B., Finamore S.A., Porro G.A., Bonfiglio R., Kotzeva S., Mameli L., Mattioli G., Micalizzi C., Montaguti A., Pistorio A., Zanaboni C., Guddo A., Neba G.R., Favarato M., Locatelli B.G., Maffioletti M., Sonzogni V., Garra R., Sammartino M., Sbaraglia F., Cortegiani A., Moscarelli A., Attanasi E., Tesoro S., Agapiti C., Pinzoni F., Vezzoli C., Bilotta F., Barzdina A., Straume Z., Zundane A., Lukosiene L., Maraulaite I., Razlevice I., Schmitz B., Mifsud S., Aehling C., Allison C., De Boer R., Emal D., Stevens M., Buitenhuis M., de Graaff J., De Liefde I., Machotta A., Scoones G., Staals L., Tomas J., Van der Knijff-van Dortmont A., Veldhuizen M., Alders D., Buhre W., Schafrat E., Schreiber J., Vermeulen P.M., Hendriks M., Lako S., Voet-Lindner M., Pieters B., Scheffer G.-J., Tielens L., Absalom A.R., Bergsma M., De Ruiter J., Meier S., Volkers M., Zweers T., Beukers A.M., Boer C., Dertinger J., Numan S., Van Zaane B., Boerke W.B., Ekiz N., Stensrud K., Drage I.M., Isern E.R., Bartkowska-Sniatkowska A., Grzeskowiak M., Juzwa-Sobieraj M., Rosada-Kurasinska J., Baranowski A., Jakubowska K., Lewandowska D., Mierzewska-Schmidt M., Sawicki P., Urban-Lechowicz M., Przemyslaw P., Zielinska M., Leal T., Soares M., Pina P., Pinho S., Patuleia M.D., Esteves C.C., Salgado H., Santos M.J., Badeti R., Cindea I., Oana L., Gurita A., Ilie L., Mocioiu G., Tabacaru R., Trante I., Munteanu V., Morariu M., Nyiri E., Budic I., Marjanovic V., Draskovic B., Pandurov M., Ilic J., Mandras A., Rados Z., Stankovic N., Suica M., Vasiljevic S., Knezevic M., Milojevic I., Petrov I., Puric Racic S., Simic D., Simic I., Stevic M., Vulicevic I., Cabanova B., Hanula M., Berger J., Janjatovic D., Pirtovsek Stupnik S., Mendez D., Pino G., Rubio P., Izquierdo A., Lopez S., Gonzalez Serrano C., Cebrian J., Peleteiro A., Del Rey de Diego P., Martinez Garcia E., Tormo de las Heras C., Troncoso Montero P., Arbona C., Artes D., Chamizo A., Serrano S., Suarez Comas M., Escriba F., Auli C., Perez Pardo O., Sierra Biddle N., Suarez Castano C., Villalobos Rico M.I., Manrique Munoz S., Garcia Martinez I., Montferrer Estruch N., Vilardell Ortiz E., Poves-Alvarez R., Kohn I., Lindestam U., Reinhard J., Castellheim A., Sandstrom K., Bengt S., Dorenberg R., Frykholm P., Garcia M., Kvarnstrom A., Ponten E., Bruelisauer T., Erdoes G., Kaiser H., Marchon M., Seiler S., Bogli Y., Dolci M., Marcucci C., Pichon I., Vutskits L., Casutt M., Holzle M., Hurni T., Johr M., Malar A.-U., Mauch J., Erb T., Oeinck K., Akin M., Keskin G., Senayli Y., Kaya G., Kendigelen P., Tutuncu A.C., Hatipoglu Z., Ozcengiz D., Erdost H.A., Ocmen E., Olguner C., Ayanoglu H., Dincer P.C., Umuroglu T., Azizoglu M., Birbicer H., Doruk N., Sagun A., Baris S., Dmytriiev D., Kuchi S., Masip N., Brooks P., Hare A., Ahmad N., Casey M., De Silva S., Dobby N., Krishnan P., Sogbodjor L.A., Walker E., Walker S., King S., Nicholson K., Quinney M., Stevens P., Blevin A., Giombini M., Goonasekera C., Adil S., Bew S., Bodlani C., Gilpin D., Jinks S., Malarkkan N., Miskovic A., Pad R., Wolfe Barry J., Abbott J., Armstrong J., Cooper N., Crate L., Emery J., James K., King H., Martin P., Scalia Catenacci S., Bomont R., Smith P., Mele S., Verzelloni A., Dix P., Bell G., Gordeva E., McKee L., Ngan E., Scheffczik J., Tan L.-E., Worrall M., Cassar C., Goddard K., Barlow V., Oshan V., Shah K., Bell S., Daniels L., Gandhi M., Pachter D., Perry C., Robertson A., Scott C., Waring L., Barnes D., Childs S., Norman J., Sunderland R., Julia D., Prisca F., Pierre H., Sarah H., Brigitte L., Benoit P., Katalin V., Anesthesiology, APH - Quality of Care, and Amsterdam Neuroscience - Neuroinfection & -inflammation

Subjects
Anesthesiology and Pain Medicine, business.industry, medicine.medical_treatment, Anesthesia, Tracheal intubation, Neonates, anaesthesia, medicine, MEDLINE, Observational study, Audit, business
Abstract

The authors regret that errors were present in the above article. On page 1174, in the second paragraph of the Statistical methods section, the second sentence should read as follows: The incidence of difficult intubation was determined including those whose tracheas were already intubated and is reported as a percentage with a 95% exact binomial CI. On page 1175, in the third paragraph of the Statistical methods section ‘mean standardised difference (MSD)’ should read ‘standardised mean difference (SMD)’ The authors would like to apologise for any inconvenience caused.

Published
2021

42. Morbidity and mortality after anaesthesia in early life:results of the European prospective multicentre observational study, neonate and children audit of anaesthesia practice in Europe (NECTARINE)

Author

Nicola Disma, Francis Veyckemans, Katalin Virag, Tom G. Hansen, Karin Becke, Pierre Harlet, Laszlo Vutskits, Suellen M. Walker, Jurgen C. de Graaff, Marzena Zielinska, Dusica Simic, Thomas Engelhardt, Walid Habre, Christian Breschan, Rudolf Likar, Manuela Platzer, Isole Edelman, Johanes Eger, Stefan Heschl, Brigitte Messerer, Maria Vittinghof, Ruth Kroess, Martina Stichlberger, David Kahn, Thierry Pirotte, Caroline Pregardien, France Stevens, Johan Berghmans, Annemie Bauters, Luc De Baerdemaeker, Stefan De Hert, Koen Lapage, Aliaksandra Parashchanka, Jurgen Van Limmen, Piet Wyffels, Julie Lauweryns, Nadia Najafi, Joris Vundelinckx, Diana Butković, Ivana Kerovec Sorić, Sandra Kralik, Ana Markić, Josip Azman, Josko Markic, Daniela Pupacic, Michal Frelich, Petr Reimer, Rene Urbanec, Petra Cajkova, Vladimir Mixa, Yvona Sedlačkova, Lenka Knoppova, Alena Zlamalova (nee Květoňova), Martin Vavřina, Jiři Žurek, Tom Hansen, Arash Afshari, Anders B. Bille, Marguerite Ellekvist, Mari-Liis Ilmoja, Reet Moor, Reet Kikas, Merle Vali, Kariantti Kallio, Elisa Reponen, Pertti Suominen, Sami Suvanto, Raisa Vahatalo, Hannu Kokki, Merja Kokki, Jarkko Harju, Miia Kokkonen, Jenni Vieri, Tuula Manner, Catherine Amory, Hugues Ludot, Dina Bert, Juliette Godart, Anne Laffargue, Herve Dupont, Benjamin Urbina, Catherine Baujard, Philippe Roulleau, Giuseppe Staiti, Maryline Bordes, Karine Nouette Gaulain, Yann Hamonic, Francois Semjen, Olivier Jacqmarcq, Caroline Lejus-Bourdeau, Cecile Magne, Lea Petry, Lilica Ros, Aurelien Zang, Mehdi Bennis, Bernard Coustets, Rose Fesseau, Isabelle Constant, Eliane Khalil, Nada Sabourdin, Noemie Audren, Thomas Descarpentries, Fanny Fabre, Aurelien Legrand, Emilie Druot, Gilles Orliaguet, Lucie Sabau, Lynn Uhrig, Francois de la Briere, Karin Jonckheer, Jean-Paul Mission, Lucia Scordo, Caroline Couchepin, Christophe Dadure, Pablo De la Arena, Laurent Hertz, Philippe Pirat, Chrystelle Sola, Myriam Bellon, Souhayl Dahmani, Florence Julien-Marsollier, Daphne Michelet, Veronique Depret-Donatien, Anne Lesage, Jost Kaufmann, Michael Laschat, Frank Wappler, Lena Brunner, Karin Oppenrieder, Gregor Badelt, Karin Hochmuth, Bernhard Koller, Anita Reil, Sebastian Richter, Thomas Fischer, Anja Diers, Clemens Schorer, Andreas Weyland, Ruth Cohausz, Franz-Josef Kretz, Michaela Loffler, Markus Wilbs, Claudia Hoehne, Johanna Ulrici, Christiane Goeters, Armin Flinspach, Matthias Klages, Simone Lindau, Leila Messroghli, Kai Zacharowski, Christoph Eisner, Thomas Mueller, Daniel Richter, Melanie Schafer, Markus Weigand, Sebastian Weiterer, Miriam Ochsenreiter, Michael Scholer, Tom Terboven, Isabel Eggemann, Sascha Haussmann, Nicolas Leister, Christoph Menzel, Uwe Trieschmann, Sirin Yucetepe, Susanna Keilig, Peter Kranke, Yvonne Jelting, Torsten Baehner, Richard Ellerkmann, Shahab Ghamari, Claudia Neumann, Martin Sohle, Pelagia Chloropoulou, Vagia Ntritsou, Pinelopi Papagiannopoulou, Eleana Garini, Afroditi Karafotia, Panagoula Mammi, Evangelia Bali, Despoina Iordanidou, Anna Malisiova, Artemis Polyzoi, Adelais Tsiotou, Erzsebet Sapi, Edgar Szekely, Nandor Kosik, Veronika Maraczi, Janos Schnur, Judit Csillag, Janos Gal, Gergely Gobl, Balazs Hauser, Andras Petroczy, Gyula Tovishazi, Stuart Blain, Sarah Gallagher, Sinead Harte, Mandy Jackson, Emma Meehan, Zeenat Nawoor, Brendan O’Hare, Mark Ross, Daniela Lerro, Marinella Astuto, Chiara Grasso, Rita Scalisi, Giulia Frasacco, Elena Lenares, Roberto Leone, Maurizia Grazzini, Carmelo Minardi, Nicola Zadra, Gilda Cinnella, Antonella Cotoia, Dario Galante, Brita De Lorenzo, Beate Kuppers, Giulia Bottazzi, Fabio Caramelli, Maria C. Mondardini, Emanuele Rossetti, Sergio Picardo, Alessandro Vittori, Anna Camporesi, Andrea Wolfler, Edoardo Calderini, Laura B. Colantonio, Simona A. Finamore, Giuliana A. Porro, Rachele Bonfiglio, Svetlana Kotzeva, Leila Mameli, Girolamo Mattioli, Camilla Micalizzi, Alessia Montaguti, Angela Pistorio, Clelia Zanaboni, Anna Guddo, Gerald R. Neba, Moreno Favarato, Bruno G. Locatelli, Micol Maffioletti, Valter Sonzogni, Rossella Garra, Maria Sammartino, Fabio Sbaraglia, Andrea Cortegiani, Alessandra Moscarelli, Elena Attanasi, Simonetta Tesoro, Cristina Agapiti, Francesca Pinzoni, Cesare Vezzoli, Federico Bilotta, Arta Barzdina, Zane Straume, Anda Zundane, Laura Lukosiene, Irena Maraulaite, Ilona Razlevice, Bernd Schmitz, Stephanie Mifsud, Carolin Aehling, Celia Allison, Rients De Boer, Dina Emal, Markus Stevens, Marielle Buitenhuis, Jurgen de Graaff, Inge De Liefde, Andreas Machotta, Gail Scoones, Lonneke Staals, Jeremy Tomas, Anouk Van der, Knijff-van Dortmont, Marianne Veldhuizen, David Alders, Wolfgang Buhre, Eva Schafrat, Jan Schreiber, Petronella M. Vermeulen, Mark Hendriks, Sandra Lako, Marieke Voet-Lindner, Barbe Pieters, Gert-Jan Scheffer, Luc Tielens, Anthony R. Absalom, Margot Bergsma, Joke De Ruiter, Sascha Meier, Martin Volkers, Tjerk Zweers, Anne M. Beukers, Christa Boer, Jurgen Dertinger, Sandra Numan, Bas Van Zaane, Wenche B. Boerke, Nil Ekiz, Kristoffer Stensrud, Inger M. Drage, Erik R. Isern, Alicja Bartkowska-Sniatkowska, Malgorzata Grzeskowiak, Magdalena Juzwa-Sobieraj, Jowita Rosada-Kurasińska, Artur Baranowski, Karina Jakubowska, Dorota Lewandowska, Magdalena Mierzewska-Schmidt, Piotr Sawicki, Magdalena Urban-Lechowicz, Pomianek Przemyslaw, Teresa Leal, Maria Soares, Pedro Pina, Silvia Pinho, Maria Domingas Patuleia, Catarina C. Esteves, Helena Salgado, Maria J. Santos, Rodica Badeti, Iulia Cindea, Loredana Oana, Adriana Gurita, Luminita Ilie, Gabriel Mocioiu, Radu Tabacaru, Irina Trante, Valentin Munteanu, Mihai Morariu, Emese Nyiri, Ivana Budic, Vesna Marjanovic, Biljana Drašković, Marina Pandurov, Jordanka Ilic, Ana Mandras, Zdenka Rados, Nikola Stankovic, Maja Suica, Sladjana Vasiljevic, Mirjana Knezevic, Irina Milojevic, Ivana Petrov, Selena Puric Racic, Irena Simic, Marija Stevic, Irena Vulicevic, Barbora Cabanova, Miloslav Hanula, Jelena Berger, Darja Janjatovic, Špela P. Štupnik, Ivan Kohn, Ulf Lindestam, Jarl Reinhard, Albert Castellheim, Kerstin Sandstrom, Sporre Bengt, Rainer Dorenberg, Peter Frykholm, Maria Garcia, Ann Kvarnstrom, Emma Ponten, Thomas Bruelisauer, Gabor Erdoes, Heiko Kaiser, Mathias Marchon, Thomas Riva, Stefan Seiler, Yann Bogli, Mirko Dolci, Carine Marcucci, Isabelle Pichon, Mattias Casutt, Martin Holzle, Thomas Hurni, Martin Johr, null Anna-Ursina, null Malar, Jacqueline Mauch, Thomas Erb, Karin Oeinck, Mine Akin, Gulsen Keskin, Yesim Senayli, Guner Kaya, Pinar Kendigelen, Ayse C. Tutuncu, Zehra Hatipoğlu, Dilek Ozcengiz, Hale Aksu Erdost, Elvan Ocmen, Cimen Olguner, Hilmi Ayanoglu, Pelin C. Dincer, Tumay Umuroglu, Mustafa Azizoglu, Handan Birbicer, Nurcan Doruk, Aslı Sagun, Sibel Baris, Dmytro Dmytriiev, Sridevi Kuchi, Nuria Masip, Peter Brooks, Alison Hare, Nargis Ahmad, Michelle Casey, Sam De Silva, Nadine Dobby, Prakash Krishnan, L. Amaki Sogbodjor, Ellie Walker, Suellen Walker, Stephanie King, Katy Nicholson, Michelle Quinney, Paul Stevens, Andrew Blevin, Mariangela Giombini, Chulananda Goonasekera, Sadia Adil, Stephanie Bew, Carol Bodlani, Dan Gilpin, Stephanie Jinks, Nalini Malarkkan, Alice Miskovic, Rebecca Pad, Juliet W. Barry, Joy Abbott, James Armstrong, Natalie Cooper, Lindsay Crate, John Emery, Kathryn James, Hannah King, Paul Martin, Stefano S. Catenacci, Rob Bomont, Paul Smith, Sara Mele, Alessandra Verzelloni, Philippa Dix, Graham Bell, Elena Gordeva, Lesley McKee, Esther Ngan, Jutta Scheffczik, Li-En Tan, Mark Worrall, Carmel Cassar, Kevin Goddard, Victoria Barlow, Vimmi Oshan, Khairi Shah, Sarah Bell, Lisa Daniels, Monica Gandhi, David Pachter, Chris Perry, Andrew Robertson, Carmen Scott, Lynne Waring, David Barnes, Sophie Childs, Joanne Norman, Robin Sunderland, Critical care, Anesthesiology, Peri-operative and Emergency medicine (CAPE), UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Service d'anesthésiologie, Tampere University, Department of Prehospital Emergency Care, Pain Management and Anaesthesiology, Anesthesiology, APH - Quality of Care, ANS - Neuroinfection & -inflammation, Disma, Nicola, Veyckemans, Franci, Virag, Katalin, Hansen, Tom G., Becke, Karin, Harlet, Pierre, Vutskits, Laszlo, Walker, Suellen M., de Graaff, Jurgen C., Zielinska, Marzena, Simic, Dusica, Engelhardt, Thoma, Habre, Walid, Breschan, Christian, Likar, Rudolf, Platzer, Manuela, Edelman, Isole, Eger, Johane, Heschl, Stefan, Messerer, Brigitte, Vittinghof, Maria, Kroess, Ruth, Stichlberger, Martina, Kahn, David, Pirotte, Thierry, Pregardien, Caroline, Stevens, France, Berghmans, Johan, Bauters, Annemie, De Baerdemaeker, Luc, De Hert, Stefan, Lapage, Koen, Parashchanka, Aliaksandra, Van Limmen, Jurgen, Wyffels, Piet, Lauweryns, Julie, Najafi, Nadia, Vundelinckx, Jori, Butković, Diana, Sorić, Ivana Kerovec, Kralik, Sandra, Markić, Ana, Azman, Josip, Markic, Josko, Pupacic, Daniela, Frelich, Michal, Reimer, Petr, Urbanec, Rene, Cajkova, Petra, Mixa, Vladimir, Sedlačkova, Yvona, Knoppova, Lenka, Zlamalova (nee Květoňova), Alena, Vavřina, Martin, Žurek, Jiři, Hansen, Tom, Afshari, Arash, Bille, Anders B., Ellekvist, Marguerite, Ilmoja, Mari-Lii, Moor, Reet, Kikas, Reet, Vali, Merle, Kallio, Kariantti, Reponen, Elisa, Suominen, Pertti, Suvanto, Sami, Vahatalo, Raisa, Kokki, Hannu, Kokki, Merja, Harju, Jarkko, Kokkonen, Miia, Vieri, Jenni, Manner, Tuula, Amory, Catherine, Ludot, Hugue, Bert, Dina, Godart, Juliette, Laffargue, Anne, Dupont, Herve, Urbina, Benjamin, Baujard, Catherine, Roulleau, Philippe, Staiti, Giuseppe, Bordes, Maryline, Gaulain, Karine Nouette, Hamonic, Yann, Semjen, Francoi, Jacqmarcq, Olivier, Lejus-Bourdeau, Caroline, Magne, Cecile, Petry, Lea, Ros, Lilica, Zang, Aurelien, Bennis, Mehdi, Coustets, Bernard, Fesseau, Rose, Constant, Isabelle, Khalil, Eliane, Sabourdin, Nada, Audren, Noemie, Descarpentries, Thoma, Fabre, Fanny, Legrand, Aurelien, Druot, Emilie, Orliaguet, Gille, Sabau, Lucie, Uhrig, Lynn, de la Briere, Francoi, Jonckheer, Karin, Mission, Jean-Paul, Scordo, Lucia, Couchepin, Caroline, Dadure, Christophe, De la Arena, Pablo, Hertz, Laurent, Pirat, Philippe, Sola, Chrystelle, Bellon, Myriam, Dahmani, Souhayl, Julien-Marsollier, Florence, Michelet, Daphne, Depret-Donatien, Veronique, Lesage, Anne, Kaufmann, Jost, Laschat, Michael, Wappler, Frank, Brunner, Lena, Oppenrieder, Karin, Badelt, Gregor, Hochmuth, Karin, Koller, Bernhard, Reil, Anita, Richter, Sebastian, Fischer, Thoma, Diers, Anja, Schorer, Clemen, Weyland, Andrea, Cohausz, Ruth, Kretz, Franz-Josef, Loffler, Michaela, Wilbs, Marku, Hoehne, Claudia, Ulrici, Johanna, Goeters, Christiane, Flinspach, Armin, Klages, Matthia, Lindau, Simone, Messroghli, Leila, Zacharowski, Kai, Eisner, Christoph, Mueller, Thoma, Richter, Daniel, Schafer, Melanie, Weigand, Marku, Weiterer, Sebastian, Ochsenreiter, Miriam, Scholer, Michael, Terboven, Tom, Eggemann, Isabel, Haussmann, Sascha, Leister, Nicola, Menzel, Christoph, Trieschmann, Uwe, Yucetepe, Sirin, Keilig, Susanna, Kranke, Peter, Jelting, Yvonne, Baehner, Torsten, Ellerkmann, Richard, Ghamari, Shahab, Neumann, Claudia, Sohle, Martin, Chloropoulou, Pelagia, Ntritsou, Vagia, Papagiannopoulou, Pinelopi, Garini, Eleana, Karafotia, Afroditi, Mammi, Panagoula, Bali, Evangelia, Iordanidou, Despoina, Malisiova, Anna, Polyzoi, Artemi, Tsiotou, Adelai, Sapi, Erzsebet, Szekely, Edgar, Kosik, Nandor, Maraczi, Veronika, Schnur, Jano, Csillag, Judit, Gal, Jano, Gobl, Gergely, Hauser, Balaz, Petroczy, Andra, Tovishazi, Gyula, Blain, Stuart, Gallagher, Sarah, Harte, Sinead, Jackson, Mandy, Meehan, Emma, Nawoor, Zeenat, O’Hare, Brendan, Ross, Mark, Lerro, Daniela, Astuto, Marinella, Grasso, Chiara, Scalisi, Rita, Frasacco, Giulia, Lenares, Elena, Leone, Roberto, Grazzini, Maurizia, Minardi, Carmelo, Zadra, Nicola, Cinnella, Gilda, Cotoia, Antonella, Galante, Dario, De Lorenzo, Brita, Kuppers, Beate, Bottazzi, Giulia, Caramelli, Fabio, Mondardini, Maria C., Rossetti, Emanuele, Picardo, Sergio, Vittori, Alessandro, Camporesi, Anna, Wolfler, Andrea, Calderini, Edoardo, Colantonio, Laura B., Finamore, Simona A., Porro, Giuliana A., Bonfiglio, Rachele, Kotzeva, Svetlana, Mameli, Leila, Mattioli, Girolamo, Micalizzi, Camilla, Montaguti, Alessia, Pistorio, Angela, Zanaboni, Clelia, Guddo, Anna, Neba, Gerald R., Favarato, Moreno, Locatelli, Bruno G., Maffioletti, Micol, Sonzogni, Valter, Garra, Rossella, Sammartino, Maria, Sbaraglia, Fabio, Cortegiani, Andrea, Moscarelli, Alessandra, Attanasi, Elena, Tesoro, Simonetta, Agapiti, Cristina, Pinzoni, Francesca, Vezzoli, Cesare, Bilotta, Federico, Barzdina, Arta, Straume, Zane, Zundane, Anda, Lukosiene, Laura, Maraulaite, Irena, Razlevice, Ilona, Schmitz, Bernd, Mifsud, Stephanie, Aehling, Carolin, Allison, Celia, De Boer, Rient, Emal, Dina, Stevens, Marku, Buitenhuis, Marielle, de Graaff, Jurgen, De Liefde, Inge, Machotta, Andrea, Scoones, Gail, Staals, Lonneke, Tomas, Jeremy, Van der, Anouk, Dortmont, Knijff-van, Veldhuizen, Marianne, Alders, David, Buhre, Wolfgang, Schafrat, Eva, Schreiber, Jan, Vermeulen, Petronella M., Hendriks, Mark, Lako, Sandra, Voet-Lindner, Marieke, Pieters, Barbe, Scheffer, Gert-Jan, Tielens, Luc, Absalom, Anthony R., Bergsma, Margot, De Ruiter, Joke, Meier, Sascha, Volkers, Martin, Zweers, Tjerk, Beukers, Anne M., Boer, Christa, Dertinger, Jurgen, Numan, Sandra, Van Zaane, Ba, Boerke, Wenche B., Ekiz, Nil, Stensrud, Kristoffer, Drage, Inger M., Isern, Erik R., Bartkowska-Sniatkowska, Alicja, Grzeskowiak, Malgorzata, Juzwa-Sobieraj, Magdalena, Rosada-Kurasińska, Jowita, Baranowski, Artur, Jakubowska, Karina, Lewandowska, Dorota, Mierzewska-Schmidt, Magdalena, Sawicki, Piotr, Urban-Lechowicz, Magdalena, Przemyslaw, Pomianek, Leal, Teresa, Soares, Maria, Pina, Pedro, Pinho, Silvia, Patuleia, Maria Dominga, Esteves, Catarina C., Salgado, Helena, Santos, Maria J., Badeti, Rodica, Cindea, Iulia, Oana, Loredana, Gurita, Adriana, Ilie, Luminita, Mocioiu, Gabriel, Tabacaru, Radu, Trante, Irina, Munteanu, Valentin, Morariu, Mihai, Nyiri, Emese, Budic, Ivana, Marjanovic, Vesna, Drašković, Biljana, Pandurov, Marina, Ilic, Jordanka, Mandras, Ana, Rados, Zdenka, Stankovic, Nikola, Suica, Maja, Vasiljevic, Sladjana, Knezevic, Mirjana, Milojevic, Irina, Petrov, Ivana, Racic, Selena Puric, Simic, Irena, Stevic, Marija, Vulicevic, Irena, Cabanova, Barbora, Hanula, Miloslav, Berger, Jelena, Janjatovic, Darja, Štupnik, Špela P., Kohn, Ivan, Lindestam, Ulf, Reinhard, Jarl, Castellheim, Albert, Sandstrom, Kerstin, Bengt, Sporre, Dorenberg, Rainer, Frykholm, Peter, Garcia, Maria, Kvarnstrom, Ann, Ponten, Emma, Bruelisauer, Thoma, Erdoes, Gabor, Kaiser, Heiko, Marchon, Mathia, Riva, Thoma, Seiler, Stefan, Bogli, Yann, Dolci, Mirko, Marcucci, Carine, Pichon, Isabelle, Casutt, Mattia, Holzle, Martin, Hurni, Thoma, Johr, Martin, Anna-Ursina, null, Malar, null, Mauch, Jacqueline, Erb, Thoma, Oeinck, Karin, Akin, Mine, Keskin, Gulsen, Senayli, Yesim, Kaya, Guner, Kendigelen, Pinar, Tutuncu, Ayse C., Hatipoğlu, Zehra, Ozcengiz, Dilek, Erdost, Hale Aksu, Ocmen, Elvan, Olguner, Cimen, Ayanoglu, Hilmi, Dincer, Pelin C., Umuroglu, Tumay, Azizoglu, Mustafa, Birbicer, Handan, Doruk, Nurcan, Sagun, Aslı, Baris, Sibel, Dmytriiev, Dmytro, Kuchi, Sridevi, Masip, Nuria, Brooks, Peter, Hare, Alison, Ahmad, Nargi, Casey, Michelle, De Silva, Sam, Dobby, Nadine, Krishnan, Prakash, Sogbodjor, L. Amaki, Walker, Ellie, Walker, Suellen, King, Stephanie, Nicholson, Katy, Quinney, Michelle, Stevens, Paul, Blevin, Andrew, Giombini, Mariangela, Goonasekera, Chulananda, Adil, Sadia, Bew, Stephanie, Bodlani, Carol, Gilpin, Dan, Jinks, Stephanie, Malarkkan, Nalini, Miskovic, Alice, Pad, Rebecca, Barry, Juliet W., Abbott, Joy, Armstrong, Jame, Cooper, Natalie, Crate, Lindsay, Emery, John, James, Kathryn, King, Hannah, Martin, Paul, Catenacci, Stefano S., Bomont, Rob, Smith, Paul, Mele, Sara, Verzelloni, Alessandra, Dix, Philippa, Bell, Graham, Gordeva, Elena, McKee, Lesley, Ngan, Esther, Scheffczik, Jutta, Tan, Li-En, Worrall, Mark, Cassar, Carmel, Goddard, Kevin, Barlow, Victoria, Oshan, Vimmi, Shah, Khairi, Bell, Sarah, Daniels, Lisa, Gandhi, Monica, Pachter, David, Perry, Chri, Robertson, Andrew, Scott, Carmen, Waring, Lynne, Barnes, David, Childs, Sophie, Norman, Joanne, Sunderland, Robin, HUS Children and Adolescents, Children's Hospital, and Clinicum

Subjects
Male, Time Factors, Health Status, Comorbidity, Intraoperative Complications/diagnosis/epidemiology/mortality/therapy, 0302 clinical medicine, Postoperative Complications, 030202 anesthesiology, Risk Factors, patient safety, Medicine, Anesthesia, Prospective Studies, Prospective cohort study, Intraoperative Complications, Medical Audit, ddc:617, Incidence (epidemiology), Incidence, Age Factors, Gestational age, critical events, Europe, Anesthetics/adverse effects, quality, Surgical Procedures, Operative, outcome, Female, Infant, Premature, Gestational Age, Anesthesia/adverse effects/mortality, Risk Assessment, Europe/epidemiology, 03 medical and health sciences, Humans, Anesthetics, business.industry, Postoperative Complications/diagnosis/epidemiology/mortality/therapy, Postmenstrual Age, Infant, Newborn, Infant, medicine.disease, 3126 Surgery, anesthesiology, intensive care, radiology, neonates, Clinical trial, Surgical Procedures, Operative/adverse effects/mortality, Anesthesiology and Pain Medicine, Blood pressure, Observational study, critical event, neonate, business
Abstract

Background: Neonates and infants requiring anaesthesia are at risk of physiological instability and complications, but triggers for peri-anaesthetic interventions and associations with subsequent outcome are unknown. Methods: This prospective, observational study recruited patients up to 60 weeks' postmenstrual age undergoing anaesthesia for surgical or diagnostic procedures from 165 centres in 31 European countries between March 2016 and January 2017. The primary aim was to identify thresholds of pre-determined physiological variables that triggered a medical intervention. The secondary aims were to evaluate morbidities, mortality at 30 and 90 days, or both, and associations with critical events. Results: Infants (n=5609) born at mean (standard deviation [SD]) 36.2 (4.4) weeks postmenstrual age (35.7% preterm) underwent 6542 procedures within 63 (48) days of birth. Critical event(s) requiring intervention occurred in 35.2% of cases, mainly hypotension (>30% decrease in blood pressure) or reduced oxygenation (SpO2

Published
2021

43. Clustering analysis in outpatients with allergic rhinitis in clinical practice

Author

Giorgio Ciprandi, Angela Pistorio, Maria Angela Tosca, Michela Silvestri, and Ignazio Cirillo

Subjects
medicine.medical_specialty, Cross-sectional study, Immunology, MEDLINE, 01 natural sciences, 03 medical and health sciences, 0302 clinical medicine, Text mining, Public health surveillance, Outpatients, medicine, Cluster Analysis, Humans, Immunology and Allergy, Public Health Surveillance, Practice Patterns, Physicians', 0101 mathematics, Cluster analysis, business.industry, Practice patterns, 010102 general mathematics, Rhinitis, Allergic, Clinical Practice, Cross-Sectional Studies, 030228 respiratory system, Family medicine, business
Published
2018

44. Management and outcome of benign acute childhood myositis in pediatric emergency department

Author

Claudio Bruno, Marta Romanengo, Chiara Panicucci, Emanuela Piccotti, Alberto Gaiero, Giacomo Brisca, Marcello Mariani, Angela Pistorio, and Daniela Pirlo

Subjects
Male, Pediatrics, medicine.medical_specialty, Adolescent, Rest, Physical examination, Neurological examination, Rhabdomyolysis, 03 medical and health sciences, 0302 clinical medicine, Clinical pathway, Recurrence, medicine, Humans, Creatine kinase, Child, Children, Myositis, Retrospective Studies, Neurologic Examination, Analgesics, medicine.diagnostic_test, business.industry, Research, Medical record, Myoglobinuria, lcsh:RJ1-570, lcsh:Pediatrics, 030208 emergency & critical care medicine, Retrospective cohort study, Emergency department, medicine.disease, Hospitalization, Virus Diseases, Gait abnormalities, Child, Preschool, Acute Disease, Fluid Therapy, Female, Emergency Service, Hospital, business, 030217 neurology & neurosurgery
Abstract

Background Benign acute childhood myositis (BACM) is a self-limited syndrome associated with viral infections characterized by symmetric lower extremity pain typically affecting school-aged children. Evolution in rhabdomyolysis and kidney damage is rarely reported. Despite this, the acute presentation commonly concerns both parents and health care providers, often leading to unnecessary workup. The aim of the study was to determine the features and outcome of a large series of children with BACM identifying a management pathway for pediatricians in Emergency Department (ED). Methods We conducted a retrospective study of patients with BACM managed in 2 Italian pediatric ED during a period of 8 and a half years. Demographic data, clinical, and laboratory results were extracted from electronic medical records. Recurrence, complications, treatments, and outcomes were also recorded. Descriptive statistics were produced for first-episode patients and for those with recurrence of myositis. A comparison between groups was performed. Results One hundred and thirteen patients with BACM were identified. Ninety-two children (65 males) had a single episode, while ten (nine males) had recurrence. The mean age at presentation was 6.0 years (range 2–13,2). All patients had normal neurological examination and no one developed myoglobinuria, or renal failure. At first evaluation median CK level was 1413 UI/l (normal values Median CK of “recurrent” patients was higher than “non-recurrent” (2330 vs 1150 U/L, p = 0.009). Viral studies were positive in 51/74 cases, with high prevalence of Influenza viruses. Ninety-six patients (85%) were hospitalized with a median of 4 days. No patients had any residual muscular impairment. Conclusions BACM has an excellent prognosis. Severe pathological conditions can be excluded with a complete history and clinical examination and simple blood and urine tests, avoiding unnecessary diagnostic investigations. Most patients may be discharged home from ED recommending hydration, rest, analgesics and careful follow-up.

Published
2021

45. The treatment of allergic rhinitis in asthmatic children and adolescents: practical outcomes 2 from the real-world 'ControL’Asma' study

Author

Maria Angela Tosca, Gian Luigi Marseglia, Giorgio Ciprandi Elisa Anastasio, Ilaria, Brambilla, Carlo 13 Caffarelli, Loredana, Chini, Riccardo, Ciprandi, Valentina De Vittori, Marzia, Duse, Maria Elisa 14 Di Cicco, Luciana, Indinnimeo, Ahmad, Kantar, Maddalena, Leone, Amelia, Licari, Guido 15 Marinelli, Viviana, Moschese, Roberta, Olcese, Diego, G Peroni, Angela, Pistorio, Claudia 16 Salmaso, Michela, Silvestri, and Zicari, Anna Maria

Published
2021

46. CASK related disorder: Epilepsy and developmental outcome

Author

Romina Romaniello, Enrico Bertini, Lino Nobili, Renato Borgatti, Chiara Pepi, Claudio Zucca, Angela Pistorio, Sara Nuovo, Elisa De Grandis, Maria Margherita Mancardi, Sara Uccella, Mariasavina Severino, Pasquale Striano, Enza Maria Valente, Roberta Battini, Ginevra Zanni, Thea Giacomini, Giulia Prato, Raffaella Cusmai, Annarita Ferrari, and Livia Pisciotta

Subjects
Male, Pediatrics, medicine.medical_specialty, Microcephaly, Developmental delay, Developmental Disabilities, Electroencephalography, Cohort Studies, 03 medical and health sciences, Epilepsy, 0302 clinical medicine, Atrophy, CASK pathogenic Variants, 030225 pediatrics, Late-onset spasms, Intellectual disability, Spindles abnormalities, medicine, Humans, Child, Preschool, Cerebellar hypoplasia, Retrospective Studies, Brain Diseases, medicine.diagnostic_test, business.industry, Infant, Child, Preschool, Female, Guanylate Kinases, Mutation, General Medicine, medicine.disease, Comorbidity, 3. Good health, Pediatrics, Perinatology and Child Health, Cohort, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

Objective CASK pathogenic variants are associated with variable features, as intellectual disability, optic atrophy, brainstem/cerebellar hypoplasia, and epileptic encephalopathy. Few studies describe the electroclinical features of epilepsy in patients with CASK pathogenic variants and their relationship with developmental delay. Methods this national multicentre cohort included genetically confirmed patients with different CASK pathogenic variants. Our findings were compared with cohorts reported in the literature. Results we collected 34 patients (29 females) showing from moderate (4 patients) to severe (22) and profound (8) developmental delay; all showed pontine and cerebellar hypoplasia, all except three with microcephaly. Seventeen out of 34 patients (50%) suffered from epileptic seizures, including spasms (11 patients, 32.3%), generalized (5) or focal seizures (1). In 8/17 individuals (47.1%), epilepsy started at or beyond the age of 24 months. Seven (3 males) out of the 11 children with spasms showed EEG features and a course supporting the diagnosis of a developmental and epileptic encephalopathy (DEE). Drug resistance was frequent in our cohort (52.9% of patients with epilepsy). EEG abnormalities included poorly organized background activity with diffuse or multifocal epileptiform abnormalities and sleep-activation, with possible appearance over the follow-up period. Developmental delay degree was not statistically different among patients with or without seizures but feeding difficulties were more frequent in patients with epilepsy. Conclusions epilepsy is a frequent comorbidity with a high incidence of spasms and drug resistance. Overall developmental disability does not seem to be more severe in the group of patients with epilepsy nor to be linked to specific epilepsy/EEG characteristics. A childhood onset of epilepsy is frequent, with possible worsening over time, so that serial and systematic monitoring is mandatory.

Published
2021

47. Italian cohort of Lafora disease: Clinical features, disease evolution, and genotype-phenotype correlations

Author

Antonella Riva, Pasquale Striano, Paolo Tinuper, Roberto Michelucci, Marcello Scala, Silvana Franceschetti, Giuseppe d'Orsi, Berge A. Minassian, Angela Pistorio, Maria Teresa Di Claudio, Alfredo D'Aniello, Pierangelo Veggiotti, Carlo Avolio, Carla Marini, Maurizio Elia, Vito Sofia, Elena Freri, Maria Tappatà, Adriana Magaudda, Vittoria Taramasso, Antonino Romeo, Francesca Bisulli, Federico Zara, Giancarlo Di Gennaro, Amedeo Bianchi, Cinzia Costa, Carlo Minetti, Alessandro Orsini, Edoardo Ferlazzo, Salvatore Striano, Laura Canafoglia, Elena Gennaro, Riva A., Orsini A., Scala M., Taramasso V., Canafoglia L., d'Orsi G., Di Claudio M.T., Avolio C., D'Aniello A., Elia M., Franceschetti S., Di Gennaro G., Bisulli F., Tinuper P., Tappata M., Romeo A., Freri E., Marini C., Costa C., Sofia V., Ferlazzo E., Magaudda A., Veggiotti P., Gennaro E., Pistorio A., Minetti C., Bianchi A., Striano S., Michelucci R., Zara F., Minassian B.A., and Striano P.

Subjects
Adult, Ubiquitin-Protein Ligase, medicine.medical_specialty, Adolescent, Ubiquitin-Protein Ligases, EPM2A, EPM2B, Genetic Association Studie, medicine.disease_cause, Lafora disease, Article, 03 medical and health sciences, Epilepsy, Young Adult, 0302 clinical medicine, Progressive myoclonu, Internal medicine, medicine, Humans, Non-Receptor, 030212 general & internal medicine, Young adult, Neurodegeneration, Child, Genetic Association Studies, Mutation, business.industry, Middle Aged, medicine.disease, Protein Tyrosine Phosphatases, Non-Receptor, Progressive myoclonus, Italy, Lafora Disease, Neurology, Cohort, Neurology (clinical), medicine.symptom, Protein Tyrosine Phosphatases, business, Laforin, Myoclonus, 030217 neurology & neurosurgery, Human
Abstract

Background Lafora disease (LD) is characterized by progressive myoclonus, refractory epilepsy, and cognitive deterioration. This complex neurodegenerative condition is caused by pathogenic variants in EPM2A/EPM2B genes, encoding two essential glycogen metabolism enzymes known as laforin and malin. Long-term follow-up data are lacking. We describe the clinical features and genetic findings of a cohort of 26 Italian patients with a long clinical follow-up. Methods Patients with EPM2A/EPM2B pathogenic variants were identified by direct gene sequencing or gene panels with targeted re-sequencing. Disease progression, motor functions, and mental performance were assessed by a simplified disability scale. Spontaneous/action myoclonus severity was scored by the Magaudda Scale. Results Age range was 12.2–46.2 years (mean:25.53 ± 9.14). Age at disease onset ranged from 10 to 22 years (mean:14.04 ± 2.62). The mean follow-up period was 11.48 ± 7.8 years. Twelve out of the 26 (46%) patients preserved walking ability and 13 (50%) maintained speech. A slower disease progression with preserved ambulation and speech after ≥4 years of follow-up was observed in 1 (11%) out of the 9 (35%) EPM2A patients and in 6 (35%) out of the 17 (65%) EPM2B patients. Follow-up was >10 years in 7 (41.2%) EPM2B individuals, including two harbouring the homozygous p.(D146N) pathogenic variant. Conclusions This study supports an overall worse disease outcome with severe deterioration of ambulation and speech in patients carrying EPM2A mutations. However, the delayed onset of disabling symptoms observed in the EPM2B subjects harbouring the p.(D146N) pathogenic variant suggests that the underlying causative variant may still influence LD severity.

Published
2021

48. Development and Testing of Reduced Versions of the Manual Muscle Test-8 in Juvenile Dermatomyositis

Author

Angelo Ravelli, Graciela Espada, Claudia Bracaglia, Rolando Cimaz, Angela Pistorio, Giulia Camilla Varnier, Silvia Rosina, Marija Jelušić, Nicolino Ruperto, Adele Civino, Clara Malattia, Anand Prahalad Rao, Elena Tsitsami, Gerard Couillault, S Maillard, Pierre Quartier, Clarissa Pilkington, Rik Joos, Alessandro Consolaro, and Adriana Cespedes-Cruz

Subjects
medicine.medical_specialty, Immunology, Elbow, Dermatomyositis, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal consistency, mental disorders, Outcome Assessment, Health Care, medicine, Retrospective analysis, Immunology and Allergy, Humans, 030212 general & internal medicine, Muscle Strength, Muscle, Skeletal, Juvenile dermatomyositis, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Manual Muscle Testing-8 (MMT-8), juvenile dermatomyositis (JDM), Muscles, Outcome measures, Construct validity, medicine.disease, Clinical Practice, Clinical trial, medicine.anatomical_structure, Physical therapy, business
Abstract

Objective.To develop and test shortened versions of the Manual Muscle Test-8 (MMT-8) in juvenile dermatomyositis (JDM).Methods.Construction of reduced tools was based on a retrospective analysis of individual scores of MMT-8 muscle groups in 3 multinational datasets. The 4 and 6 most frequently impaired muscle groups were included in MMT-4 and MMT-6, respectively. Metrologic properties of reduced tools were assessed by evaluating construct validity, internal consistency, discriminant ability, and responsiveness to change.Results.Neck flexors, hip extensors, hip abductors, and shoulder abductors were included in MMT-4, whereas MMT-6 also included elbow flexors and hip flexors. Both shortened tools revealed strong correlations with MMT-8 and other muscle strength measures. Correlations with other JDM outcome measures were in line with predictions. Internal consistency was good (0.88–0.96) for both MMT-4 and MMT-6. Both reduced tools showed strong ability to discriminate between disease activity states, assessed by the caring physician or a parent (P < 0.001), and between patients whose parents were satisfied or not satisfied with illness course (P < 0.001). Responsiveness to change (assessed by both standardized response mean and relative efficiency) of MMT-4 and, to a lesser degree, MMT-6, was slightly superior to that of MMT-8.Conclusion.Overall, the metrologic performance of MMT-4 and MMT-6 was comparable to that of the other established muscle strength tools, which indicates that they may be suitable for use in clinical practice and research, including clinical trials. The measurement properties of these tools should be further tested in other patient populations and evaluated prospectively.

Published
2020

49. Endocrine Outcomes In Central Diabetes Insipidus: the Predictive Value of Neuroimaging 'Mismatch Pattern'

Author

Anna Elsa Maria Allegri, Anastasia Ibba, Giuseppa Patti, Marco Crocco, Mohamad Maghnie, Flavia Napoli, Daniela Fava, Marilea Lezzi, Deborah Bianco, Natascia Di Iorgi, Andrea Rossi, Maurizio Schiavone, Giovanni Morana, Andrea Calandrino, Angela Pistorio, Hanan F. Thiabat, Mariasavina Severino, Tiziana Camia, and Domenico Tortora

Subjects
Male, medicine.medical_specialty, Pituitary gland, Pathology, Adolescent, Endocrinology, Diabetes and Metabolism, Clinical Biochemistry, Neurogenic, Context (language use), Biochemistry, Growth hormone deficiency, Pituitary stalk, Endocrinology, Anterior pituitary, Langerhans cell histiocytosis, Internal medicine, medicine, Humans, Central diabetes insipidus, Posterior pituitary hyperintensity, Child, Child, Preschool, Diabetes Insipidus, Neurogenic, Female, Histiocytosis, Langerhans-Cell, Pituitary Gland, Retrospective Studies, Magnetic Resonance Imaging, Langerhans-Cell, Preschool, business.industry, Biochemistry (medical), medicine.disease, medicine.anatomical_structure, Diabetes insipidus, business, Histiocytosis, Neurogenic diabetes insipidus, Diabetes Insipidus
Abstract

Context The etiology of central diabetes insipidus (CDI) in children is often unknown. Clinical and radiological features at disease onset do not allow discrimination between idiopathic forms and other conditions or to predict anterior pituitary dysfunction. Objective To evaluate the evolution of pituitary stalk (PS) thickening and the pattern of contrast-enhancement in relation with etiological diagnosis and pituitary function. Methods We enrolled 39 children with CDI, 29 idiopathic and 10 with Langerhans cell histiocytosis (LCH). Brain magnetic resonance images taken at admission and during follow-up (332 studies) were examined, focusing on PS thickness, contrast-enhancement pattern, and pituitary gland size; T2-DRIVE and postcontrast T1-weighted images were analyzed. Results Seventeen of 29 patients (58.6%) with idiopathic CDI displayed “mismatch pattern,” consisting in a discrepancy between PS thickness in T2-DRIVE and postcontrast T1-weighted images; neuroimaging findings became stable after its appearance, while “mismatch” appeared in LCH patients after chemotherapy. Patients with larger PS displayed mismatch more frequently (P = 0.003); in these patients, reduction of proximal and middle PS size was documented over time (P = 0.045 and P = 0.006). The pituitary gland was smaller in patients with mismatch (P < 0.0001). Patients with mismatch presented more frequently with at least one pituitary hormone defect, more often growth hormone deficiency (P = 0.033). Conclusions The PS mismatch pattern characterizes patients with CDI, reduced pituitary gland size, and anterior pituitary dysfunction. The association of mismatch pattern with specific underlying conditions needs further investigation. As patients with mismatch show stabilization of PS size, we assume a prognostic role of this peculiar pattern, which could be used to lead follow-up.

Published
2020

50. Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT)

Author

Sabine Irtan, Luca Pio, Vincent Couloigner, Gianni Bisogno, Calogero Virgone, Gabriela Guillén Burrieza, Gastón Bellia Munzón, Naziha Khen Dunlop, Rebecca Maunsell, M. Larroquet, Jaime Penchyna Grub, Michael J. Rutter, Giovanni Cecchetto, Martin J Eliott, Patricio Varela, Michele Torre, Victor Rachkov, Madhavan Ramaswamy, Sabine Sarnacki, Irene Paraboschi, Angela Pistorio, Mariano Boglione, Claudia Schweiger, and Andrea Ferrari

Subjects
Male, medicine.medical_specialty, Adolescent, Malignancy, 03 medical and health sciences, 0302 clinical medicine, pediatric tracheobronchial tumors, Mucoepidermoid carcinoma, 030225 pediatrics, Internal medicine, medicine, Humans, Child, airway team, Survival rate, Retrospective Studies, Pediatric airway tumors, Pediatric airway tumors, airway team, pediatric tracheobronchial tumors, business.industry, Medical record, Bronchial Neoplasms, Infant, Retrospective cohort study, Evidence-based medicine, medicine.disease, Survival Rate, Otorhinolaryngology, Child, Preschool, 030220 oncology & carcinogenesis, Female, Tracheal Neoplasms, Pediatric airway, Presentation (obstetrics), business
Abstract

OBJECTIVE Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS Patients

Published
2020

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