@article{WiegeringSchickBeeretal.2011, author = {Wiegering, Verena and Schick, Judith and Beer, Meinrad and Gattenl{\"o}hner, Stefan and Girschick, Hermann and Liese, Johannes and Schlegel, Paul and Eyrich, Matthias}, title = {Varicella-zoster virus infections in immunocompromised patients - a single centre 6-years analysis}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-68723}, year = {2011}, abstract = {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.}, subject = {Varizellen-Virus}, language = {en} } @article{SchoenbergerLudwigWildneretal.2013, author = {Sch{\"o}nberger, Katharina and Ludwig, Maria-Sabine and Wildner, Manfred and Weissbrich, Benedikt}, title = {Epidemiology of Subacute Sclerosing Panencephalitis (SSPE) in Germany from 2003 to 2009: A Risk Estimation}, series = {PLoS ONE}, volume = {8}, journal = {PLoS ONE}, number = {7}, doi = {10.1371/journal.pone.0068909}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-130089}, pages = {e68909}, year = {2013}, abstract = {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 W{\"u}rzburg. 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.}, language = {en} } @article{SchmidLoisMetzetal.2022, author = {Schmid, Andrea and Lois, Anna-Maria and Metz, Corona and Grunz, Jan-Peter and Veldhoen, Simon}, title = {Not all that looks fractured is broken - multipartite humeral epicondyles in children}, series = {European Radiology}, volume = {32}, journal = {European Radiology}, number = {8}, doi = {10.1007/s00330-022-08670-1}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-324987}, pages = {5045-5052}, year = {2022}, abstract = {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.}, language = {en} } @article{KuehnischHerbstAl‐Wakeel‐Marquardetal.2019, author = {K{\"u}hnisch, Jirko and Herbst, Christopher and Al-Wakeel-Marquard, Nadya and Dartsch, Josephine and Holtgrewe, Manuel and Baban, Anwar and Mearini, Giulia and Hardt, Juliane and Kolokotronis, Konstantinos and Gerull, Brenda and Carrier, Lucie and Beule, Dieter and Schubert, Stephan and Messroghli, Daniel and Degener, Franziska and Berger, Felix and Klaassen, Sabine}, title = {Targeted panel sequencing in pediatric primary cardiomyopathy supports a critical role of TNNI3}, series = {Clinical Genetics}, volume = {96}, journal = {Clinical Genetics}, number = {6}, doi = {10.1111/cge.13645}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-213958}, pages = {549 -- 559}, year = {2019}, abstract = {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.}, language = {en} } @article{FuhrmannTeschRomanosetal.2020, author = {Fuhrmann, Saskia and Tesch, Falko and Romanos, Marcel and Abraham, Susanne and Schmitt, Jochen}, title = {ADHD in school-age children is related to infant exposure to systemic H1-antihistamines}, series = {Allergy}, volume = {75}, journal = {Allergy}, number = {11}, doi = {10.1111/all.14411}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-215982}, pages = {2956 -- 2957}, year = {2020}, language = {en} } @article{EyrichRachorSchreiberetal.2013, author = {Eyrich, Matthias and Rachor, Johannes and Schreiber, Susanne C. and W{\"o}lfl, Matthias and Schlegel, Paul G.}, title = {Dendritic cell vaccination in pediatric gliomas: lessons learnt and future perspectives}, series = {Frontiers in Pediatrics}, journal = {Frontiers in Pediatrics}, doi = {10.3389/fped.2013.00012}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-96937}, year = {2013}, abstract = {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.}, language = {en} }