TY - JOUR A1 - Wiegering, Verena A1 - Schick, Judith A1 - Beer, Meinrad A1 - Gattenlöhner, Stefan A1 - Girschick, Hermann A1 - Liese, Johannes A1 - Schlegel, Paul A1 - Eyrich, Matthias T1 - Varicella-zoster virus infections in immunocompromised patients - a single centre 6-years analysis N2 - Background: Infection with varicella-zoster virus (VZV) contemporaneously with malignant disease or immunosuppression represents a particular challenge and requires individualized decisions and treatment. Although the increasing use of varicella-vaccines in the general population and rapid initiation of VZVimmunoglobulins and acyclovir in case of exposure has been beneficial for some patients, immunocompromised individuals are still at risk for unfavourable courses. Methods: In this single center, 6-year analysis we review incidence, hospitalization and complication rates of VZVinfections in our center and compare them to published data. Furthermore, we report three instructive cases. Results: Hospitalization rate of referred children with VZV-infections was 45%, among these 17% with malignancies and 9% under immunosuppressive therapy. Rate of complications was not elevated in these two high-risk cohorts, but one ALL-patient died due to VZV-related complications. We report one 4-year old boy with initial diagnosis of acute lymphoblastic leukemia who showed a rapidly fatal outcome of his simultaneous varicella-infection, one 1.8-year old boy with an identical situation but a mild course of his disease, and an 8.5-year old boy with a steroiddependent nephrotic syndrome. This boy developed severe hepatic involvement during his varicella-infection but responded to immediate withdrawl of steroids and administration of acyclovir plus single-dose cidofovir after nonresponse to acyclovir after 48 h. Conclusion: Our data show that patients with malignant diseases or immunosuppressive therapy should be hospitalized and treated immediately with antiviral agents. Despite these measures the course of VZV-infections can be highly variable in these patients. We discuss aids to individual decision-making for these difficult situations. KW - Varizellen-Virus KW - varicella-zoster virus immunosuppression KW - pediatrics KW - cidofovir Y1 - 2011 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-68723 ER - TY - JOUR A1 - Schönberger, Katharina A1 - Ludwig, Maria-Sabine A1 - Wildner, Manfred A1 - Weissbrich, Benedikt T1 - Epidemiology of Subacute Sclerosing Panencephalitis (SSPE) in Germany from 2003 to 2009: A Risk Estimation JF - PLoS ONE N2 - Subacute sclerosing panencephalitis (SSPE) is a fatal long-term complication of measles infection. We performed an estimation of the total number of SSPE cases in Germany for the period 2003 to 2009 and calculated the risk of SSPE after an acute measles infection. SSPE cases were collected from the Surveillance Unit for Rare Paediatric Diseases in Germany and the Institute of Virology and Immunobiology at the University of Würzburg. The total number of SSPE cases was estimated by capture-recapture analysis. For the period 2003 to 2009, 31 children with SSPE who were treated at German hospitals were identified. The capture-recapture estimate was 39 cases (95% confidence interval: 29.2–48.0). The risk of developing SSPE for children contracting measles infection below 5 years of age was calculated as 1∶1700 to 1∶3300. This risk is in the same order of magnitude as the risk of a fatal acute measles infection. KW - Germany KW - pediatric infections KW - age groups KW - measels virus KW - German people KW - measles KW - pediatrics KW - vaccination and immunization Y1 - 2013 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-130089 VL - 8 IS - 7 ER - TY - JOUR A1 - Schmid, Andrea A1 - Lois, Anna-Maria A1 - Metz, Corona A1 - Grunz, Jan-Peter A1 - Veldhoen, Simon T1 - Not all that looks fractured is broken - multipartite humeral epicondyles in children JF - European Radiology N2 - Objective Multipartite epicondyles may mimic fractures in the setting of pediatric elbow trauma. This study examines the prevalence of multipartite epicondyles during skeletal development and their association with pediatric elbow fractures. Materials and methods In this retrospective analysis, 4282 elbow radiographs of 1265 elbows of 1210 patients aged 0–17 years were reviewed. The radiographs were analyzed by two radiologists in consensus reading, and the number of visible portions of the medial and lateral epicondyles was noted. For elbows in which epicondylar ossification was not yet visible, the epicondyles were already fused with the humerus or could not be sufficiently evaluated due to projection issues or because osteosynthesis material was excluded. In total, 187 elbows were included for the lateral and 715 for the medial epicondyle analyses. Results No multipartite medial epicondyles were found in patients without history of elbow fracture, whereas 9% of these patients had multipartite lateral epicondyles (p < 0.01). Current or previous elbow fractures increased the prevalence of multipartite epicondyles, with significant lateral predominance (medial epicondyle + 9% vs. lateral + 24%, p < 0.0001). Including all patients regardless of a history of elbow fracture, multipartite medial epicondyles were observed in 3% and multipartite lateral epicondyles in 18% (p < 0.0001). There was no gender difference in the prevalence of multipartition of either epicondyle, regardless of a trauma history. Conclusion Multipartite medial epicondyles occur in patients with current or previous elbow fractures only, whereas multipartite lateral epicondyles may be constitutional. Elbow fractures increase the prevalence of multipartite epicondyles on both sides, with significant lateral predominance. Key Points • Multipartite medial epicondyles should be considered of traumatic origin. • Multipartite lateral epicondyles may be constitutional. • Elbow fractures increase the prevalence of multipartite epicondyles on both sides with lateral predominance. KW - elbow joint KW - epicondyles KW - bone fractures KW - pediatrics KW - radiography Y1 - 2022 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-324987 VL - 32 IS - 8 ER - TY - JOUR A1 - Kühnisch, Jirko A1 - Herbst, Christopher A1 - Al‐Wakeel‐Marquard, Nadya A1 - Dartsch, Josephine A1 - Holtgrewe, Manuel A1 - Baban, Anwar A1 - Mearini, Giulia A1 - Hardt, Juliane A1 - Kolokotronis, Konstantinos A1 - Gerull, Brenda A1 - Carrier, Lucie A1 - Beule, Dieter A1 - Schubert, Stephan A1 - Messroghli, Daniel A1 - Degener, Franziska A1 - Berger, Felix A1 - Klaassen, Sabine T1 - Targeted panel sequencing in pediatric primary cardiomyopathy supports a critical role of TNNI3 JF - Clinical Genetics N2 - The underlying genetic mechanisms and early pathological events of children with primary cardiomyopathy (CMP) are insufficiently characterized. In this study, we aimed to characterize the mutational spectrum of primary CMP in a large cohort of patients ≤18 years referred to a tertiary center. Eighty unrelated index patients with pediatric primary CMP underwent genetic testing with a panel‐based next‐generation sequencing approach of 89 genes. At least one pathogenic or probably pathogenic variant was identified in 30/80 (38%) index patients. In all CMP subgroups, patients carried most frequently variants of interest in sarcomere genes suggesting them as a major contributor in pediatric primary CMP. In MYH7, MYBPC3, and TNNI3, we identified 18 pathogenic/probably pathogenic variants (MYH7 n = 7, MYBPC3 n = 6, TNNI3 n = 5, including one homozygous (TNNI3 c.24+2T>A) truncating variant. Protein and transcript level analysis on heart biopsies from individuals with homozygous mutation of TNNI3 revealed that the TNNI3 protein is absent and associated with upregulation of the fetal isoform TNNI1. The present study further supports the clinical importance of sarcomeric mutation—not only in adult—but also in pediatric primary CMP. TNNI3 is the third most important disease gene in this cohort and complete loss of TNNI3 leads to severe pediatric CMP. KW - cardiomyopathy KW - genetics KW - pediatrics KW - sarcomere KW - TNNI3 Y1 - 2019 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-213958 VL - 96 IS - 6 SP - 549 EP - 559 ER - TY - JOUR A1 - Fuhrmann, Saskia A1 - Tesch, Falko A1 - Romanos, Marcel A1 - Abraham, Susanne A1 - Schmitt, Jochen T1 - ADHD in school‐age children is related to infant exposure to systemic H1‐antihistamines JF - Allergy KW - atopic dermatitis KW - histamine KW - pediatrics Y1 - 2020 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-215982 VL - 75 IS - 11 SP - 2956 EP - 2957 ER - TY - JOUR A1 - Eyrich, Matthias A1 - Rachor, Johannes A1 - Schreiber, Susanne C. A1 - Wölfl, Matthias A1 - Schlegel, Paul G. T1 - Dendritic cell vaccination in pediatric gliomas: lessons learnt and future perspectives JF - Frontiers in Pediatrics N2 - Immunotherapy of malignant gliomas with autologous dendritic cells (DCs) in addition to surgery and radiochemotherapy has been a focus of intense research during the past decade. Since both children and adults are affected by this highly aggressive brain tumor, 10–15% of the several hundred vaccinated patients represent children, making pediatric glioma patients the largest uniform pediatric vaccination cohort so far. In general, DC vaccination in malignant gliomas has been shown to be safe and several studies with a non-vaccinated control group could clearly demonstrate a survival benefit for the vaccinated patients. Interestingly, children and adolescents below 21 years of age seem to benefit even more than adult patients. This review summarizes the findings of the 25 clinical trials published so far and gives a perspective how DC vaccination could be implemented as part of multimodal therapeutic strategies in the near future. KW - pediatrics KW - malignant glioma KW - DC vaccination KW - clinical trial KW - immunotherapy Y1 - 2013 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-96937 ER -