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Background: Rituximab (RTX) resistance or early B-cells repopulation were observed in children but only few publications reported the use of Obinutuzumab and no recommendations were made concerning the dosage for children., Methods: This study was a single-center retrospective cohort study of all the children followed-up in the Pediatric Neurology Department of Necker-Enfants malades Hospital in Paris, France, and treated with obinutuzumab, between November 1, 2019, and November 1, 2021., Results: A total of eight children (three females, median age 4.5 years) were treated. Seven patients presented with autoimmune encephalitis and one with myeloradiculitis. The median delay of B-cell repopulation after a course of RTX was 87 days (range 41 to 160). A switch to obinutuzumab (anti-CD20) was performed for eight children. The median duration between the first RTX infusion and obinutuzumab administration was 6.6 months. The dosage regimen for obinutuzumab was one infusion of 1000 mg/1.73 m 2 , that is to say 580 mg/m 2 (maximum 1000 mg/infusion), by extrapolation from the adult dosage. The median delay of B-cell repopulation after one course of obinutuzumab was 230 days (range 66 to 303 days) vs 87 days after one course of RTX (P < 0.01). None of the patients presented side effects with obinutuzumab treatment. All patients had a favorable evolution at the last-follow up. Median follow-up was 1.6 years., Conclusions: This study reports the use of obinutuzumab in neurological inflammatory diseases in a pediatric population. Obinutuzumab seems to have a better biological efficacy than RTX with a longer time of B-cell repopulation., Competing Interests: Declaration of competing interest The authors declare that no conflicts of interest that could potentially influence or bias the submitted work exists., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study was to assess the clinical impact of indeterminate pulmonary nodules (no more than four pulmonary nodules of less than 5 mm or one nodule measuring between 5 and less than 10 mm by computed tomography [CT]) in children and adolescents with adult-type non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) at diagnosis., Methods: Patients with NRSTS treated in 11 centers as part of the European paediatric Soft Tissue Sarcoma Study Group (EpSSG) were retrospectively assessed. Local radiologists, blinded to clinical information except for patients' age and tumor histotype, reviewed the chest CT at diagnosis and filled out a case report form. Because patients with or without indeterminate nodules in the EpSSG NRSTS 2005 study received the same type of treatment, event-free survival (EFS) and overall survival (OS) between groups by log-rank test were compared., Results: Overall, 206 patients were examined: 109 (52.9%) were without any nodules, 78 (38%) had at least one indeterminate nodule, and 19 (9.2%) had nodules meeting the definition of metastases, which were then considered to be misclassified and were excluded from further analyses. Five-year EFS was 78.5% (95% CI, 69.4%-85.1%) for patients without nodules and 69.6% (95% CI, 57.9%-78.7%) for patients with indeterminate nodules (p = .135); 5-year OS was 87.4% (95% CI, 79.3%-92.5%) and 79.0% (95% CI, 67.5%-86.8%), respectively (p = .086)., Conclusions: This study suggests that survival does not differ in otherwise nonmetastatic patients with indeterminate pulmonary nodules compared to nonmetastatic patients without pulmonary nodules., Plain Language Summary: Radiologists should be aware of the classification of indeterminate pulmonary nodules in non-rhabdomyosarcoma soft tissue sarcomas and use it in their reports. More than a third of patients with non-rhabdomyosarcoma soft tissue sarcoma can be affected by indeterminate pulmonary nodules. Indeterminate pulmonary nodules do not significantly affect the overall survival of pediatric patients with non-rhabdomyosarcoma soft tissue sarcoma., (© 2023 American Cancer Society.)

Background: RTX is used off-label in several neurological inflammatory diseases in adults children patients. We conducted a study to assess indications and safety of rituximab (RTX) for children and to identify risk factors for early B-cell repopulation., Methods: A single-center retrospective study of children treated with RTX for a neurological disease between May 31, 2010, and May 31, 2020, was performed., Results: A total of 77 children (median age, 8.9 years) were included. RTX was mostly used as second-line therapy in all groups of diseases (68%). Median dose was 1500 mg/m 2 for each patient. There were 13 clinical relapses (17%), 5 when B-cell depletion was complete. Adverse events were present in 6% of the cases. The factors influencing early B-cell repopulation were the recent infusion of intravenous Ig (P < 0.01) and the administration of less than 1500 mg/m 2 during the first RTX treatment (P = 0.04). The median time to B-cell repopulation seemed to be shorter (160 vs 186 days) when patients had plasmapheresis even when a 48-hour delay was observed with RTX infusions., Conclusions: This study confirms the good tolerance of RTX in the treatment of specific neurological disorders in a pediatric population. It also highlights risk factors for early B-cell repopulation and underlines the importance of B-cell monitoring., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Magnetic resonance imaging (MRI) for radiotherapy is often based on 3D acquisitions, but suffers from low signal-to-noise ratio due to immobilization device and flexible coil use. The aim of this study was to investigate if Compressed Sensing (CS) improves image quality for 3D Turbo Spin Echo acquisitions compared with Controlled Aliasing k-space-based parallel imaging in equivalent acquisition time for intracranial T1, T2-Fluid-Attenuated Inversion Recovery (FLAIR) and pelvic T2 imaging. Qualitative ratings suffered from large inter-rater variability. CS-T1 brain MRI was superior numerically and qualitatively. CS-T2-FLAIR brain MRI was numerically superior, but rater equivalent. CS-T2 pelvic MRI was equivalent without gain., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Authors. Published by Elsevier B.V. on behalf of European Society of Radiotherapy & Oncology.)

Introduction: Complete surgical resection constitutes the mainstay of treatment for locally aggressive, rarely metastazing tumor and low-grade soft tissue sarcomas (LAS). Local relapse is the most common tumor event, especially in the presence of positive margins (R1 margins). The aims of this study are to assess the impact of the national network on patient care and to evaluate the role of immediate re-excision in children, adolescents and young adults with incompletely resected LAS., Methods: National retrospective multicenter study of all young patients (≤25 years) included in the Sarcoma "ConticaBase" treated for LAS between 2005 and 2017 for whom pathology/biology review was available via the national NETSARC + network., Results: A total of 96 patients were identified (median age: 16 years). Tumors were localized in 99% of cases (1 N+ tumor). With a median follow-up of 4.7 years (range: 0.1-11.9), eight local relapses and two distant metastases were observed. No patient died. Overall 5-year event-free survival (EFS) was 90.4% [95%CI, 84.3-97]. Five year EFS for R1 patients (n = 51) with (n = 24) and without (n = 27) immediate re-excision was 90.5% [95%CI, 78.8-100.0] and 80.3% [95%CI, 64.7-99.9], respectively (p = 0.34). The 37 patients directly treated in a reference center more commonly had a diagnostic biopsy (78% vs. 21%; p < 0.001), more complete surgery (R0: 65% vs. 14%; p < 0.001) and less commonly underwent re-excision (16% vs. 54%; p < 0.001)., Conclusions: This large series indicates that LAS are rare in young patients and have a favorable prognosis. Immediate management in reference centers is associated with better standard of care. The main tumor events are local relapses., Competing Interests: Declaration of competing interest None., (Copyright © 2021 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Objectives: The objective is to check the maintaining performance of filter barrier in sterilizations containers with the leakage rupture the junction tank/lid of the container. In order to control the good sealing performance, we use the water leak test as described in the normative document AFNOR FD S98-053. Does the leakage rupture cause the contamination of containers during the storage or transport between two sterilization units?, Method: We use 5 sets of 3 different containers (the first without barrier system (positive control), the others with barrier system (the second with positive water test and the third with negative water test)). The containers are sterilized after having positioned triptyc soy agar (TSA) plates at the bottom. In a closed chamber test, the containers are exposed with an aerosol of Micrococcus luteus under stress of an overpressure (25, 50 or 75 mbar) simulating atmospheric pressure variations. Each triptyc soy agar (TSA) plates are incubated during 5 days at 37°C for bacterial identification., Results: At 25 mbar, 74% of containers having a leakage with a water test let the microorganisms ingress despite the filter barrier. The results confirm that the overpressure increases the level of bacteria in containers. Furthermore, the containers with positive water test are more contaminated than containers with perfect sealing. The effectiveness of the sterile barrier system is evaluated by the logarithmic reduction value (LRV)., Conclusions: The water test could be a good indication to evaluate the effectiveness of the sterile barrier system of rigid sterilization containers., (Copyright © 2018 Académie Nationale de Pharmacie. Published by Elsevier Masson SAS. All rights reserved.)

Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma that typically affects pediatric and young adult patients with a median age in the general and in the pediatric population of 24.6 years (range 4-58 years) and 15.0 years (range 0-21 years) respectively, with a strong male predominance. This tumor is characterized by a specific t(11;22)(p13;q12) that results in fusion of EWS and WT1 genes which can be demonstrated by RT-PCR or by FISH. DSRCT most frequently presents as an intra-abdominal primary mass associated with peritoneal seeding and a highly aggressive pattern of spread. Generally, all tumors showed the typical histologic findings of variably sized clusters of poly-phenotypic small, round, or spindled cells lying in a desmoplastic stroma. Treatment of this malignancy remains a challenge. The combination of polychemotherapy regimens and aggressive surgery followed by whole abdomen radiation therapy represents nowadays a classical protocol for DSRCT. The survival rate of DSRCT patients is still disappointing around 20 %. However, the survival of patients who had complete resection of the tumor appears better. Hopes are turning to targeted therapeutics against this simple genomic sarcoma. Authors summarize medical knowledge of this rare tumor., (Copyright © 2018 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Buruli ulcer (BU), the third most frequent mycobacteriosis worldwide, is a neglected tropical disease caused by Mycobacterium ulcerans. We report the clinical description and extensive genetic analysis of a consanguineous family from Benin comprising two cases of unusually severe non-ulcerative BU. The index case was the most severe of over 2,000 BU cases treated at the Centre de Dépistage et de Traitement de la Lèpre et de l'Ulcère de Buruli, Pobe, Benin, since its opening in 2003. The infection spread to all limbs with PCR-confirmed skin, bone and joint infections. Genome-wide linkage analysis of seven family members was performed and whole-exome sequencing of both patients was obtained. A 37 kilobases homozygous deletion confirmed by targeted resequencing and located within a linkage region on chromosome 8 was identified in both patients but was absent from unaffected siblings. We further assessed the presence of this deletion on genotyping data from 803 independent local individuals (402 BU cases and 401 BU-free controls). Two BU cases were predicted to be homozygous carriers while none was identified in the control group. The deleted region is located close to a cluster of beta-defensin coding genes and contains a long non-coding (linc) RNA gene previously shown to display highest expression values in the skin. This first report of a microdeletion co-segregating with severe BU in a large family supports the view of a key role of human genetics in the natural history of the disease.

Background: Steatosis assessed by histology is commonly considered to be a significant risk factor for liver surgery. MRI is considered as the new gold standard for noninvasive liver fat quantification. The purpose was to assess whether liver steatosis determined by preoperative MRI is an independent risk factor of complications after major liver resection., Methods: All patients who underwent liver MRI before major liver resection in our institution between January 2001 and December 2011 were included in this retrospective study. The liver fat fraction (LFF) was assessed on in- and opposed-phase T1-weighted dual echo gradient echo MRI and steatosis was defined as a MRI LFF of ≥ 5%. The association between steatosis and postoperative complications (Clavien-Dindo classification, ascites > 500 mL at day 5, 50-50 criteria, fistula/collection, blood liver test alterations, pulmonary complications, nonpulmonary complications, >1 complication, duration of stay in the intensive care unit, duration of hospital stay, and death) was assessed by multivariate analysis using the appropriate model., Results: A MRI LFF of ≥ 5% was associated with severe postoperative complications (Clavien-Dindo score ≥ IIIa; P = .04), more pulmonary complications (P = .02), and longer duration of hospital stay (P = .02) on the multivariate model adjusted for confounding factors. The postoperative aminotransferase levels were higher in patients with a MRI LFF of ≥ 5%, than in other patients (P = .0008)., Conclusion: Liver steatosis assessed by routine preoperative MRI is shown to be an independent risk factor of severe postoperative complications after major liver resection., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Good sterilization practices include discarding items containing residual moisture after steam sterilization. In this small laboratory study, however, the presence of residual water did not appear to compromise the sterility of surgical instruments in 2 commonly used types of packaging during routine storage after steam sterilization.

Objective: Conventional scales may help with the identification of depression but are generally too lengthy for clinical practice and perform poorly against anxiety and distress. We therefore examined the value of a single item NCCN Distress Thermometer and an enhanced visual-analogue method (Emotion Thermometers, ET) that incorporates four emotion thermometers., Methods: We examined 228 patients with mixed cardiovascular conditions of whom 200 completed questionnaires. 64.5% suffered from cardiomyopathy/congestive heart failure, 9.5% had coronary artery disease, 4.5% had multiple cardiac diagnoses, 3% suffered from hypertension, 2% had rhythm problem, 2% had valve problems and 1.5% were diagnosed with atrial fibrillation. We used DSM-IV criteria to define current depression, the GAD7 to define current anxiety and the HADS-T to define distress. 13% had DSM-IV MDD and 19.1% had major or minor depression using DSM-IV (any depression). There were also 59 people (29.6%) with clinically significant distress and 46 with clinically significant anxiety (23.1%)., Results: The optimal accuracy for major depression was either the Depression thermometer (DepT) or the Help thermometer (HelpT), as both performed well. They had a sensitivity and specificity of 73.1%, 89.7% and 84.6%, 85.6%, respectively. The DepT was also best for detecting any DSM-IV depression (sensitivity 68.4% and specificity 93.2%) and HAD-T based distress (sensitivity 79.7% and specificity 82.9%). The Anxiety thermometer (AnxT) performed best against the GAD7 (sensitivity 84.8% and specificity 83.7%)., Conclusion: Innovative visual-analogue screening tools for mood appear to perform well in cardiovascular settings., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Levodopa induces long-term motor complications in Parkinson's disease (PD). Therapeutic strategies that prevent motor complications are needed. Our aim was to evaluate the impact of recommendations of a French consensus conference published in 2000 on initial PD therapy. We identified 308 PD patients as part of a population-based study performed within the Mutualité Sociale Agricole in five French districts (2007). Neurologists confirmed PD diagnosis. We compared initial therapy in 102 patients treated before 12/31/2000 to that of 206 patients treated afterwards. Initial treatment was in agreement with the recommendations if dopamine agonists were used in patients <60 years (n = 49) and levodopa in patients ≥70 years (n = 133). Agreement with the recommendations increased after 2000 (66.0%) compared to before (46.3%, p = 0.025). For patients <60 years, agreement increased (64.0% vs 20.2%, p = 0.017) while it remained stable (66.4% vs 70.6%, p = 0.73) in patients ≥70 years. The publication of recommendations has influenced initial treatment choices for PD in France., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Pelvic abscesses are not always amenable to percutaneous drainage. Transrectal drainage of pouch of Douglas abscesses, performed blindly by surgeons for many years, is not familiar to radiologists. The purpose of this article is to describe our technique for the drainage of pouch of Douglas abscesses under suprapubic US guidance and report our results in a series of 7 pediatric patients with deep pelvic abscess not amenable to percutaneous drainage after failure of antibiotics. The procedure was performed under general anesthesia. All procedures were successful and without complication. The drainage catheter was well tolerated in all cases with short post-drainage hospital stay. After failure of medical management, this technique can be used irrespective of patient age or sex using US transducers and drainage catheters routiney available in any radiology department.

Retrospective study of 14 cases of Staphylococcus aureus meningitis and of data from the literature shows that this as yet infrequent disease which carries a high mortality rate (40%) is always related to neurosurgery, primary or iatrogenic septicemia, or local infection, usually in the lumbar area (spinal, abscess, spondylitis). The major clinical problem is the search for the meningeal lead point of Staphylococcus aureus penetration, whose eradication is more essential than in usual bacterial meningitis. Prognosis is mainly dependent upon the eventual existence of associated severe septicemia. Antibiotics active on Peni-M resistant Staphylococcus aureus and meningeal infection are reviewed.

Several applications, both on the ground and in space, are envisaged for large, light parabolic reflectors which are compact when folded. In this paper the aim is to calculate by a differential method the deformed meridian line and the creased zone of an elastic inflatable revolution membrane. Conversely, one can calculate the original meridian line which, under a given pressure, transforms into a selected meridian line, in this case parabolic. The theory is confirmed by the deformation measurements of a cap in 36-microm thick polyester film cut out of a sphere 4 m in diameter.