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Aims: Cardiac surgery has improved life expectancy of patients with congenital heart diseases (CHDs). Exercise capacity is an important determinant of survival in patients with CHDs. There is a lack of studies focusing on the role of resting respiratory performance in reducing exercise tolerance in these patients., Objectives: To determine the prevalence and severity of respiratory functional impairment in different types of corrected/palliated CHDs, and its impact on an exercise test., Materials and Methods: Retrospective single-center study involving 168 corrected/palliated patients with CHD and 52 controls. Patients CHD were divided into subgroups according to the presence of native pulmonary blood flow or total cavopulmonary connection (TCPC). All subjects performed complete pulmonary function tests and gas diffusion; patients with CHD also performed cardiopulmonary exercise test (CPX)., Results: Mean values of lung volumes were within the normal range in all CHD groups. Comparing to controls, patients with the reduced pulmonary flow and with TCPC had the highest reduction in lung volumes. CPX was reduced in all groups, most severely in TCPC, and it was correlated to decreased dynamic volumes in all CHD groups except in TCPC. Younger age at intervention and number of surgical operations negatively affected lung volumes., Conclusions: Respiratory function is within the normal range in our patients with different CHDs at rest but altered in all CHDs during exercise when cardiorespiratory balance is likely to be inadequate. Comparing the different groups, patients with reduced pulmonary flow and TCPC are the most impaired., (© 2020 Wiley Periodicals, Inc.)

Kawasaki disease (KD)is an idiopathic acute febrile illness that primarily occurs in children <5 years of age and can lead to artery lesions if not promptly treated. Recent studies have shown possible correlations between KD onset and currents and air pollutants.The present study describes results on the correlation between environmental conditions, frequency, and variability ofKD onset in children under five years of age in Emilia-Romagna, a region of Northern Italy, over the period from 2000 to 2017. Since there are substantial climatic differences between the western-central (Emilia) and the eastern area (Romagna) of the region, the data for these areas are analyzed separately. The environmental factors considered are observed local surface daily temperature, daily precipitation, upper air wind regimes, and local air pollution. The results indicate that in Emilia-Romagna, KD onset occurs mainly during late autumn and early spring, which is in agreement with the literature. The frequency of KD onset in Emilia is significantly higher in months characterized by a high frequency of southerly flow, which is associated with milder than average night-time temperature, and in years with a prevailing south-westerly mean flow. These results are consistent with other studies, suggesting that certain wind conditions are more favorable for disease onset, which are possibly associated with one or more airborne agents., Competing Interests: The authors declare no conflict of interest.

Introduction: Nursing home residents represent a particularly vulnerable population experiencing high risk of unplanned hospital admissions, but few interventions have proved effective in reducing this risk. The aim of this research will be to verify the effects of a hospital-based multidisciplinary mobile unit (MMU) team intervention delivering urgent care to nursing home residents directly at their bedside., Methods and Analysis: Four nursing homes based in the Parma province, in Northern Italy, will be involved in this prospective, pragmatic, multicentre, 18-month quasiexperimental study (sequential design with two cohorts). The residents of two nursing homes will receive the MMU team care intervention. In case of urgent care needs, the nursing home physician will contact the hospital physician responsible for the MMU team by phone. The case will be triaged as (a) manageable by phone advice, (b) requiring urgent assessment by the MMU team or (c) requiring immediate emergency department (ED) referral. MMU team is composed of one senior physician and one emergency-medicine resident chosen within the staff of Internal Medicine and Critical Subacute Care Unit of Parma University-Hospital, usually with different specialty background, and equipped with portable ultrasound, set of drugs and devices useful in urgency. The MMU visits patients in nursing homes, with the mission to stabilise clinical conditions and avoid hospital admission. Residents of the other two nursing homes will receive usual care, that is, ED referral in every case of urgency. Study endpoints include unplanned hospital admissions (primary), crude all-cause mortality, hospital mortality, length of stay and healthcare-related costs (secondary)., Ethics and Dissemination: The study protocol was approved by the Ethics Committee of Area Vasta Emilia Nord (Emilia-Romagna region). Informed consent will be collected from patients or legal representatives. The results will be actively disseminated through peer-reviewed journals and conference presentations, in compliance with the Italian law., Trial Registration Number: ClinicalTrials.gov Registry (NCT04085679); Pre-results., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To describe clinical presentation, electrocardiographic, and echocardiographic characteristics of carditis at the time of diagnosis of acute rheumatic fever (ARF) over a 13-year period., Study Design: A single-center retrospective chart analysis was conducted involving all consecutive patients diagnosed with ARF between 2003 and 2015. Patient age, sex, clinical characteristics, recent medical history for group A streptococcal pharyngotonsillitis and antibiotic treatment, and laboratory, echocardiographic, and electrocardiographic findings were recorded., Results: Of 98 patients (62 boys, mean age 8.81 ± 3.04 years), 59 (60.2%) reported a positive history of pharyngotonsillitis; 48 (49%) had received antibiotic (mean duration of treatment of 5.9 ± 3.1 days), and, among these, 28 (58.3%) had carditis. Carditis was the second most frequent finding, subclinical in 27% of patients. Mitral regurgitation was present in 49 of 56 patients (87.5%) and aortic regurgitation in 36/56 (64.3%) no stenosis was documented., Conclusions: ARF is still present in high-income countries and can develop despite primary prophylaxis, especially when given for a short course. Our findings highlight the need for 10 days of antistreptococcal treatment to prevent ARF. Echocardiography is important because 27% of cases with carditis were subclinical., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: In older patients with acute illness, a condition of "hospital-dependence" may arise: patients get adapted to the hospital care and, once discharged, may experience health status decline, requiring repeated readmissions despite appropriate treatments., Aims: The objective of this case-series study was to describe the characteristics of 118 patients (72 F) aged ≥75 (mean 83.7 ± 4.9) who were urgently admitted to our institution at least 4 times in 2015., Methods: For each patient and admission, data on multimorbidity (Cumulative Illness Rating Scale Comorbidity Score and Severity Index), frailty (Rockwood Clinical Frailty Scale), functional dependence, functional status, polypharmacy, length of stay and interval between admissions were extrapolated from clinical records. Mortality during the years 2015 and 2016 was assessed on the institutional database., Results: At the first admission, patients had a high burden of polypharmacy (median number of drugs 8.5, IQR 6-11) and multimorbidity (Comorbidity Score 15.8 ± 4.1, Severity Index 2.9 ± 1.1). However, most (55.5%) were fit or pre-frail according to Clinical Frailty Scale (score 1-4). At multivariate models, Severity Index was significantly correlated with the length of stay (β ± SE 2.23 ± 0.89, p = .01) and readmission interval (β ± SE -22.49 ± 9.27, p = .02). Significantly increasing trends of multimorbidity and disability occurred across admissions. By the end of 2016, 66% of patients had died. Frailty (RR 2.005, 95%CI 1.054-3.814, p = .007) and cancer were the only predictors of mortality., Conclusions: Hospital-dependent patients had severe multimorbidity, but exhibited an unexpectedly low prevalence of frailty/disability at baseline, though increasing across admissions. Trends of frailty and multimorbidity are paramount for profiling the hospital-dependence risk., (Copyright © 2019 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

Competing Interests: Competing interests: None declared.

Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor.Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment. What is Known: • There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions. • Data are scarce on their effectiveness in non-Asian children. What is New: • We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement. • Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities.

Background: Proper integration of multiple imaging modalities in the routine follow-up of patients with repaired tetralogy of Fallout (TOF) is poorly supported by data. We report our single center comparative study between cardiac magnetic resonance (CMR) and echocardiography to assess equipoise in the clinical utility of these two imaging methods in an unselected consecutive cohort of TOF patients referred to our outpatient clinic., Material and Methods: In this cross-sectional study, repaired TOF patients who underwent CMR and echocardiography within a 4-week period between 2010 and 2011 at our Center were included. Linear regression was used to analyze degree of inter modality correlation. A prediction model tested the association between functional data/probrain natriuretic peptide (Pro-BNP) with CMR., Results: Fifty patients were included in the study (mean age 31 ± 18 years). The best predictors of right ventricle (RV) ejection fraction at CMR were tricuspid anular plane systolic excursion (tricuspid valve anular plane systolic excursion, R 0.37, P < 0.0001) and RV peak S-wave velocity (R 0.40, P < 0.001). Pro-BNP levels did present weak correlation with New York Heart Association functional class (R 0.31, P < 0.002) and QRS duration (R 0.32, P < 0.002) and a moderate correlation with right atrium area at CMR (R 0.46, P < 0.0001)., Conclusion: We found limited correlation between the two imaging modalities in the evaluation of RV after intracardiac repair of TOF. Pro-BNP level presents moderate correlation with right atrium area measured with echocardiography. Serial CMR evaluations are needed in this patient population, but they may be interchanged by routine echocardiography in particular in patients with normal or stable echocardiographic parameters.

Background: Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease., Objective: To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions., Methods: Retrospective study of KD patients diagnosed between 2000 and 2015 in 13 pediatric units in Italy. Patients were divided into 2 groups according to the presence or absence of abdominal manifestations at onset. We compared their demographic and clinical data, IVIG-responsiveness, coronary ectasia/aneurysms, laboratory findings from the acute and subacute phases., Results: 302 patients (181 boys) were enrolled: 106 patients with, and 196 patients without presenting abdominal features. Seasonality was different between the groups (p = 0.034). Patients with abdominal manifestations were younger (p = 0.006) and more frequently underwent delayed treatment (p = 0.014). In the acute phase, patients with abdominal presentation had higher platelet counts (PLT) (p = 0.042) and lower albuminemia (p = 0.009), while, in the subacute phase, they had higher white blood cell counts (WBC) and PLT (p = 0.002 and p < 0.005, respectively) and lower red blood cell counts (RBC) and hemoglobin (Hb) (p = 0.031 and p 0.009). Moreover, the above mentioned group was more likely to be IVIG-resistant (p < 0.005) and have coronary aneurysms (p = 0.007). In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, IVIG- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms (respectively p<0.005, <0.005, = 0.005 and 0.009)., Conclusions: This is the first multicenter report demonstrating that presenting gastrointestinal features in KD identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population., Clinical Trial Registration: 8/20014/O/OssN., Competing Interests: The authors have declared that no competing interests exist.

Background: Cardiotoxic effects of anthracycline therapy are a major cause of morbidity for childhood cancer survivors. The aim of this retrospective evaluation is to assess the efficacy of Tissue Doppler Imaging in the early detection of myocardial alterations in these patients., Methods: A population of 50 childhood cancer survivors (32 males and 18 females) who have been treated with anthracyclines was evaluated by standard and TDI echocardiographic examination of the basal and median region of the interventricular septum (IVSb, IVSm), of the left ventricular posterior wall (LVPWb, LVPWm), and of the mitral annulus; the results were compared with those obtained from a population of 50 healthy age-matched and sex-matched controls by using the Student test. The clinical and echocardiographic data of the two groups were compared also with the independent samples t-test. All data were expressed as mean ± standard deviation. A two-tailed P -value < 0.05 was considered statistically significant. Statistical analysis was performed using STATA 7.0., Results: The case-control analysis showed statistically significant differences ( p  < 0,05) between the patients and the controls values. The systolic performance of the patients was normal (LVEF ( p  = 0,0029) and LVFS ( p  = 0,0002)). Statistically significant differences between patients and controls were found for diastolic function measurements obtained with PW Doppler such as IVRT ( p  = 0,0000), DT ( p  = 0,0041), E ( p  = 0,0000), A ( p  = 0,0458), even if E/A ratio was not altered. TDI analysis also show significant differences between patients and controls in both LVPW and IVS (basal and middle segments); E/E' ratio and E'/A' ratio did not vary significantly. Linear Regression and multivariate analysis showed that Hematopoietic Stem Cell Transplantation had the highest impact on our measurements., Conclusions: The results showed a myocardial diastolic impairment with preserved ejection fraction. Since the median follow-up time of our cohort was 2 years, further evaluation is needed to better define the diastolic alterations. TDI analysis showed high sensitivity for the detection of mild myocardial dysfunction; the implementation of this novel method as standard practice in the follow-up of selected childhood cancer survivors might help to achieve a better management of long-term complications of cardiotoxic chemotherapy., Competing Interests: Competing interestsThe authors declare that they have no competing interests., (© The Author(s). 2018.)

The article explains the main technical and methodological references of the reorganization of the main surgical activities (for production volumes and case mixes) of the Parma University Hospital. In defining the organizational design we considered the compliance with national laws and regional guidelines, the vision of the Public Enterprise Strategic Management, the attempt to bring the continuity of care through hospital paths, the valorization of the high technical specialty of a hub university center, the recognition of the ability and vocations of Professionals and the search for efficiency of a complex public production system. The project has also responded to the need to pursue greater production efficiency by the entire local health system of Parma, optimizing, at the same time, training courses for Medical Students and Health Professions of the University of Parma transversely with respect to surgical clinical pathways and clinical research.

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Argentinian Spanish language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 373 JIA patients (23.1% systemic, 30.8% oligoarticular, 28.1% RF negative polyarthritis, 18% other categories) and 100 healthy children were enrolled in five centres. The JAMAR components discriminated well healthy subjects from JIA patients. Notably, there was no significant difference between healthy subjects and their affected peers in the school-related item. All JAMAR components revealed good psychometric performances. In conclusion, the Argentinian Spanish version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

The article addresses the increasingly important issue of organizational ethics in healthcare. Assuming that the governance of organizational change consists of a seriousness of continuous choices and of objectives to be determined and achieved, a possible definition of this new discipline is then deepened, and its fields of application are identified. In the discussion therefore emerge the close link between ethical choice in organization and legality, passing through transparency and prevention of corruptive phenomena. After a focus on the strategic role played by the Medical Doctor of the healthcare management team, in conclusion, the need arises to support a strengthened supervisory and evaluation system and a culture of the ethical organizational choice of health managers, a rooting of the sense of identity and belonging to the healthcare organization and its mission by Professionals. Also governing this last aspect is the new challenge for the management of public healthcare enterprises.

Background: The upside-down position is a little known modified Valsalva manoeuvre (VM). The aim of this study was to investigate the safety and the efficacy of the upside-down position for the treatment of paroxysmal SVT in children., Methods: Twenty-four paediatric patients followed for SVT were enrolled. The patients were assigned (1:1) to a standard VM or to an upside-down position at the first episode of SVT at home. If no cardioversion occurred, a second attempt was undertaken with the other VM. At the patient's first relapse, the intervention protocol was applied in the opposite order at home., Results: The upside-down position compared to standard VM reached 67% vs 33% rate of cardioversion at a first attempt, followed by 50% vs 0% rate of cardioversion in patients who had failed the first attempt. After having reversed the order of intervention in case of SVT recurrence, we recorded 67% vs 25% and 71% vs 42% success rates in favour of the upside-down position. There were no adverse events., Conclusion: The upside-down position was safe and tended to be more effective than standard VM for out of hospital SVT treatment. Doctors and parents should be more aware of this effective but overlooked manoeuvre., (Copyright © 2017 Elsevier B.V. All rights reserved.)

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A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierre's syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes-Stovin syndrome was diagnosed. Hughes-Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case. The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.

Background: Manual measurement of 4-meter gait speed by a stopwatch is the gold standard test for functional assessment in older adults. However, the accuracy of this technique may be biased by several factors, including intra- and inter-operator variability. Instrumental techniques of measurement using accelerometers may have a higher accuracy. Studies addressing the concordance between these two techniques are missing. The aim of the present community-based observational study was to compare manual and instrumental measurements of 4-meter gait speed in older individuals and to assess their relationship with other indicators of physical performance., Methods: One-hundred seventy-two (69 men, 103 women) non-disabled community-dwellers aged ≥65 years were enrolled. They underwent a comprehensive geriatric assessment including physical function by Short Physical Performance Battery (SPPB), hand grip strength, and 6-minute walking test (6MWT). Timed usual walking speed on a 4-meter course was assessed by using both a stopwatch (4-meter manual measurement, 4-MM) and a tri-axial accelerometer (4-meter automatic measurement, 4-MA). Correlations between these performance measures were evaluated separately in men and women by partial correlation coefficients., Results: In both genders, 4-MA was associated with 4-MM (men r = 0.62, p<0.001; women r = 0.73, p<0.001), handgrip strength (men r = 0.40, p = 0.005; women r = 0.29, p = 0.001) and 6MWT (men r = 0.50, p = 0.0004; women r = 0.22, p = 0.048). 4-MM was associated with handgrip strength and 6MWT in both men and women. Considering gait speed <0.6 m/s as diagnostic of dismobility syndrome, the two methods of assessment disagreed, with a different categorization of subjects, in 19% of men and 23% of women. The use of accelerometer resulted in 29 (13 M, 16 F) additional diagnoses of dismobility, compared with the 4-MM., Conclusions: In an older population, the concordance of gait speeds manually or instrumentally assessed is not optimal. The results suggest that manual measures might lead to misclassification of a substantial number of subjects. However, longitudinal studies using standardized and validated procedures aimed at the comparison of different techniques are needed before recommending the use of accelerometers in comprehensive geriatric assessment.

Background: Kawasaki disease (KD) is a systemic vasculitis with an acute and self-limited course. The incidence of KD differs widely among ethnic groups and is higher in the Asian population. In Italy, no recent data are available. Our purpose is to define the epidemiology of Kawasaki disease in the years 2008-2013 in children aged < 14 years in the Italian regions of Tuscany and Emilia Romagna through administrative data., Methods: We studied the epidemiology of KD in the years 2008-2013 in children 0-14 years old resident in Tuscany and in Emilia Romagna regions using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates., Results: The distribution of the KD patients across ages was similar for the two regions with a peak in the second year of life. When considering data of the two regions together, the rate of incidence was 17.6 for 100,000 children under 5 years. For both Regions the incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring and winter., Conclusion: This is the first Italian study performed through the use of administrative data. Figures are in line but slightly higher than those published in other European countries.

Univentricular heart is a rare congenital heart malformation. Nowadays prognosis is considered to be strictly linked to surgical intervention, and survival into late adulthood is unusual. In some patients native haemodynamic circulation balances pulmonary and systemic blood flow, allowing long-term survival without the need for surgery. We report the case of a 74-year-old man with a univentricular heart in natural history, and we discuss the factors that might contribute to his extraordinary long-term survival.

Pulmonary embolism is an uncommon but potentially fatal disease in children, especially with congenital heart disease where pulmonary perfusion depends on caval flow. Anticoagulation is the mainstay of therapy for children with pulmonary embolism. However, thrombolytic therapy can also be considered. The outcome of pediatric pulmonary embolism is uncertain and needs further investigation. We suggest increased awareness and a high index of suspicion in the presence of suggestive clinical signs in high-risk patients in order to initiate prompt diagnostic imaging and treatment.

Background: The Comunity Health Centre (CHC) primary care model is a team-based health care delivery model intended to provide comprehensive and continuous medical care to patients within a defined community. The CHC, Case della Salute in Italian, model was introduced in the Emilia-Romagna Region in 2010., Methods: We present updated data on the implementation on the CHC Case della Salute primary care model in the Emilia-Romagna Region., Results: There are 67 operating CHCs in Emilia-Romagna (update March 2015); 26 small (39%), 24 medium (36%) and 17 large (25%). Since 2011 the number of operating CHCs has increased by 60%, reaching 55% of the target planned CHCs (n. = 122). There is, on average, one running CHC per 66.524 inhabitants. 16% of total general practitioners (GPs) and 8.4% of total family paediatricians working in Emilia-Romagna have their practice in CHCs. CHCs offer primary and specialist integrated care, prevention services, health education and social care., Discussion: Although preliminary results suggest CHCs have fostered primary care's quality and efficiency, more research is needed to assess their impact on improving clinical, social and economic outcomes.

Objectives: There is consolidated evidence that the burden of inappropriate laboratory test requests is very high, up to 70%. We describe here the function of a computerized alert system linked to the order entry, designed to limit the number of potentially inappropriate laboratory test requests., Design and Methods: A computerized alert system based on re-testing intervals and entailing the generation of pop-up alerts when preset criteria of appropriateness for 15 laboratory tests were violated was implemented in two clinical wards of the University Hospital of Parma. The effectiveness of the system for limiting potentially inappropriate tests was monitored for 6months., Results: Overall, 765/3539 (22%) test requests violated the preset criteria of appropriateness and generated the appearance of electronic alert. After alert appearance, 591 requests were annulled (17% of total tests requested and 77% of tests alerted, respectively). The total number of test requests violating the preset criteria of inappropriateness constantly decreased over time (26% in the first three months of implementation versus 17% in the following period; p<0.001). The total financial saving of test withdrawn was 3387 Euros (12.8% of the total test cost) throughout the study period., Conclusions: The results of this study suggest that a computerized alert system may be effective to limit the inappropriateness of laboratory test requests, generating significant economic saving and educating physicians to a more efficient use of laboratory resources., (Copyright © 2015 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Coarctation of the aorta (CofA) has been associated with an increased risk of intracranial aneurysm (IA). This magnetic resonance angiography (MRA) study investigates the prevalence of IAs in 80 children treated in early life for CofA. MRA was performed at mean age of 15.7 ± 7.1 years, and surgical or endovascular treatment for CofA occurred at a mean age of 2.6 ± 4.4 years. No IA was found. In contrast with earlier findings in adult patients with late treatment for CofA, this first systematic study of very early treated patients for CofA failed to confirm the association between CofA and IAs. Our results call the abnormal developmental relation between CofA and IAs into question and suggest that modifiable risk factors like hypertension may be responsible for IA development in patients with CofA with adult diagnosis and treatment. In conclusion, our data suggest that early treatment of CofA can reduce the formation of IAs in children so as to make MRA screening less valuable in this young population., (Copyright © 2015 Elsevier Inc. All rights reserved.)

This study investigates the organizational culture and associated characteristics of the newly established primary care units (PCUs)-collaborative teams of general practitioners (GPs) who provide patients with integrated health care services-in the Emilia-Romagna Region (RER), Italy. A survey instrument covering 6 cultural dimensions was administered to all 301 GPs in 21 PCUs in the Local Health Authority (LHA) of Parma, RER; the response rate was 79.1%. Management style, organizational trust, and collegiality proved to be more important aspects of PCU organizational culture than information sharing, quality, and cohesiveness. Cultural dimension scores were positively associated with certain characteristics of the PCUs including larger PCU size and greater proportion of older GPs. The presence of female GPs in the PCUs had a negative impact on collegiality, organizational trust, and quality. Feedback collected through this assessment will be useful to the RER and LHAs for evaluating and guiding improvements in the PCUs., (© 2013 by the American College of Medical Quality.)

Background: Cardiovascular disease (CVD) is a common cause of morbidity and mortality in children with chronic kidney disease (CKD). Left ventricular hypertrophy (LVH) and diastolic dysfunction (LVDD) are early markers. The aims of this study were to evaluate (1) LVH and LVDD, using both conventional echocardiographic evaluation and Tissue Doppler Imaging (TDI), and (2) the correlation between cardiac disease and possible risk factors, in children with CKD., Methods: The study cohort comprised 34 paediatric patients with CKD and 34 healthy children (mean ± standard deviation: age 9 ± 4.6 and 8.2 ± 4.3 years, respectively). Thirteen (38 %) patients were in CKD stage 2, 15 (44 %) in stage 3 and six (18 %) in stage 4-5. LVH was defined as a left ventricular mass index (LVMI) of >95th percentile (38 g/h(2.7))., Results: Left ventricular hypertrophy was present in 13 patients (38 %). Diastolic function evaluated with TDI (E'/A' = early/late diastolic myocardial velocity) worsened with the reduction of glomerular filtration rate (p = 0.020). There was a positive correlation between LVMI and body mass index-standard deviation score (p = 0.020) and a negative correlation between E'/A' and serum phosphorus and calcium levels and their respective product (p = 0.004, p = 0.017, p < 0.001). The relaxation index E' was reduced in 68 % of patients., Conclusion: Based on our results, TDI is a simple procedure and would appear to be a more accurate diagnostic tool than conventional echocardiography in the early diagnosis of LVDD.

Background: Surfactant treatment reduces respiratory morbidity and mortality in preterm infants. Data on its haemodynamic consequences are conflicting. The aim was to characterise the haemodynamic effects of surfactant treatment on cardiac function in preterm newborns with respiratory distress syndrome (RDS)., Methods: Preterm infants (gestational age <34 weeks, birth weight <2000 g) with RDS, who received surfactant within 72 hours of life, were recruited.Echocardiography was performed before surfactant, and 2 and 24 hours after. Left and right ventricular peak systolic, early diastolic and late diastolic myocardial velocities were measured using Tissue Doppler Imaging (TDI), while characteristics of the ductus arteriosus, pulmonary artery pressure, right ventricular (RVO) and left ventricular output were measured by standard echocardiography. Tricuspidal Annular Plane Systolic Excursion (TAPSE) was measured on the free wall of the tricuspid annulus., Results: Fourteen patients were studied. Surfactant was associated with a decrease in pulmonary pressure and an increase in RVO. The improvement of right ventricular function was also confirmed by a significant increase in right peak systolic velocity and in TAPSE. Left ventricular velocities did not change significantly after surfactant., Conclusions: Surfactant administration in preterm infants with RDS did not impair myocardial contractility and was followed by increased RVO, in agreement with other parameters of right ventricular function. TDI and TAPSE appeared to be reliable and feasible in this population. The addition of TDI and TAPSE to standard neonatal echocardiography may provide additional information about cardiac function.

Nutritional rickets is still occasionally found in high-income countries, especially in populations at risk, and induced hypocalcaemia is a rare but possible cause of dilated cardiomyopathy. Although rare, physicians need to consider nutritional rickets in the differential diagnosis of hypocalcaemia cardiac failure, especially in high-risk populations such as immigrants. Despite being a reversible condition, the prognosis depends on the severity and time of diagnosis. We report two cases of exclusively breastfed infants with congestive cardiac failure due to hypokinetic dilated cardiomyopathy who had completely different outcomes. This report supports the need for prevention of this deficiency and underlies the role of vitamin D supplementation.

MEF2B encodes a transcriptional activator and is mutated in ∼11% of diffuse large B cell lymphomas (DLBCLs) and ∼12% of follicular lymphomas (FLs). Here we found that MEF2B directly activated the transcription of the proto-oncogene BCL6 in normal germinal-center (GC) B cells and was required for DLBCL proliferation. Mutation of MEF2B resulted in enhanced transcriptional activity of MEF2B either through disruption of its interaction with the corepressor CABIN1 or by rendering it insensitive to inhibitory signaling events mediated by phosphorylation and sumoylation. Consequently, the transcriptional activity of Bcl-6 was deregulated in DLBCLs with MEF2B mutations. Thus, somatic mutations of MEF2B may contribute to lymphomagenesis by deregulating BCL6 expression, and MEF2B may represent an alternative target for blocking Bcl-6 activity in DLBCLs.

Takotsubo cardiomyopathy, or broken heart syndrome, is characterized by transient left ventricular dysfunction associated to chest pain, elevation of cardiac enzymes, and electrocardiographic changes, mimicking an acute coronary syndrome, especially in older women after a physical or emotional stress. It is extremely infrequent in children as well as after infective stress. We described a celiac 4-year-old girl, following a gluten-free diet, who developed features of cardiac failure few days after episodes of acute diarrhea with fever. The patient was treated with oral anticongestive therapy and intravenous immunoglobulins, and she had a dramatic and rapid improvement; echocardiographic features normalized in 48 hours., (© 2012 Wiley Periodicals, Inc.)

Sinus of Valsalva aneurysm can be congenital or acquired. The major complication is rupture: this can represent an evolution or an abrupt event. In case of rupture or of large aneurysm, a surgical approach is justified. We report a case of ruptured sinus of Valsalva aneurysm in a seventeen years old girl, who had underwent surgical correction of Fallot tetralogy. As soon as the rupture of sinus of Valsalva aneurysm is suspected, echocardiographic examination is the easiest and most straightforward tool to make a correct diagnosis.

Catheter-related central thrombosis is a rare complication of long-term central line. We describe the case of an asymptomatic boy who was diagnosed a calcified thrombus in right atrium eight years after the removal of a long-term central venous device. Although the most appropriate therapeutic approach for managing floating right heart thrombi remains to be determined, surgical removal is an effective and safe procedure for calcified long-standing thrombus and it is to be preferred in elective conditions especially in young asymptomatic patients without hemodynamic involvement, that are at low risk of surgery-related morbidity and mortality.

Background: Percutaneous aortic valvuloplasty is an effective means of treatment for congenital aortic valve stenosis. The aim of this study was to evaluate the immediate results of valvuloplasty, to analyze the medium to long-term outcome and to assess its efficacy in preventing or postponing a new percutaneous valvuloplasty or aortic valve surgery., Methods: We retrospectively analyzed the reports of 37 patients aged > 1 month (mean age 6.3 years) who underwent aortic valvuloplasty for severe aortic stenosis. Associated congenital cardiac defects were present in 16% of the patients. The average time of follow-up was 5.07 years. Particular attention was focused on occurrence and progression of aortic regurgitation., Results: Hemodynamic gradient after aortic valvuloplasty decreased from 58.5 to 22.5 mmHg, with an average decrease of 61.5%. On echography, the maximum gradient decreased from 93.0 to 40.5 mmHg, with an average decrease of 56.5%; mean gradient decreased from 52.0 to 20.5 mmHg with an average decrease of 60.6%. At last follow-up the average maximum and mean gradient on echo were 50.0 and 27.0 mmHg. A reintervention was needed in 21.6% of cases: a second valvuloplasty in 8.1% and aortic surgery in 13.5%. The mortality rate was 2.7%. Survival after 14 years was 97.2%; freedom from aortic valve surgery was 85.5%, from a second valvuloplasty was 89.5%, and from any type of procedure was 76.1%., Conclusions: Percutaneous aortic valvuloplasty is a safe and effective treatment for congenital aortic stenosis in patients aged > 1 month. Aortic regurgitation is the main concern in the follow-up. Nonetheless, 14 years after valvuloplasty, over 75% of patients are free from any type of aortic valve reintervention.

Infantile subglottic hemangioma is a pediatric tumor of endothelial cells characterized by an initial phase of rapid proliferation (around 6 months), followed by slow involution, often leading to complete regression following the first year of life. It is most frequently found in females and it usually it occurs also in the skin. From its position it can cause a progressive airway obstruction, so early diagnosis and treatment are very important. Many treatments have been described in the literature, including systemic steroids, intralesional steroid injection, carbon dioxide laser therapy, submucous resection, interferon alfa-2 and also tracheostomy as last approach. This case report discusses a 6-month old infant, that arrived to our attention for an acute two-way stridor. Laringoscopy under general anesthesia showed a subocclusive subglottic haemangioma that closed 70% of the laryngeal airway. In agreement with our ENT specialist it was decided to begin systemic steroid therapy, first by i.v. ingection during intensive therapy with rinotracheal intubation and mechanic ventilation; after the canula removal and the hemangioma reduction, the patient took oral steroids with a gradual reduction of the dose. This case evidences the importance of laryngoscopy in the diagnosis of subglottic haemangioma; it also proves the importance of multi-disciplinary collaboration with ENT specialist and dermatologist for the diagnosis and treatment of this kind of patient. It also shows that systemic steroids are an effective alternative in the management of obstructive pediatric subglottic hemangiomas.

Derivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians. We report here a case of cardiovascular and neurological depression induced by oxymetalzoline in a toddler.

Background: Acute large-bowel obstruction represents a surgical challenge whose management is controversial, usually requiring emergent surgery in adverse circumstances with increased morbidity and mortality compared with elective situations., Methods: We report our experience with self-expanding metal stents in the initial management of acute neoplastic colorectal obstruction. From December 2000 to February 2003, insertion of self-expanding metal stents under endoscopic and fluoroscopic guidance was attempted in 22 patients, in 9 as primary palliative measure (group A) whereas in 13 as a bridge to surgery (group B)., Results: Stenting was technically successful in 20 patients. There were 2 perforations, and one patient died as a consequence. Nine of the 11 patients in the group B underwent elective colonic resection while in two patients with advanced malignancy the stent was considered as a definitive palliative treatment. In all 10 patients with ultimate prosthesis (8 group A, 2 group B) two dislocations and one recurrent obstruction were noted in the mean follow-up of 12 months., Conclusion: Self expanding metal stents represent a good option in the treatment of large bowel obstruction, providing time for a complete preoperative evaluation and mechanical bowel preparation as well as for restoring patient's general conditions thus avoiding emergency surgery with considerably lower morbidity and mortality rates. In patients with advanced cancer, they provide an alternative to surgery with satisfactory results.

In this random study, the efficacy of either colloidal bismuth subcitrate (CBS) or metronidazole in combination with an H2-antagonist in the treatment of various gastric pathologies was evaluated, along with the trends in antibody levels. Among the 40 Helicobacter pylori-positive patients with various gastroduodenal pathologies who underwent chemotherapy, 27 were treated with CBS and 13 with metronidazole. H. pylori was eradicated in 48.14% of the patients treated with CBS and 53.8% of those treated with metronidazole. After therapy, no statistically significant or slight decrease in the serum levels of antibodies was found.

External pancreatic fistula is a common complication after pancreatic resection. We report a case successfully treated by endoscopic papillotomy and a naso-pancreatic tube.