Klinische Padiatrie最新文献_第8页

Evaluation of Long-Term Late Side Effects in High-Risk Neuroblastoma Patients Undergoing Autologous Bone Marrow Transplantation. [接受自体骨髓移植的高危神经母细胞瘤患者的长期后遗症评估]。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-06 DOI: 10.1055/a-2815-2484

Rıza Yıldırım, Gulcihan Ozek, Eda Ataseven, Serap Aksoylar

{"title":"Evaluation of Long-Term Late Side Effects in High-Risk Neuroblastoma Patients Undergoing Autologous Bone Marrow Transplantation.","authors":"Rıza Yıldırım, Gulcihan Ozek, Eda Ataseven, Serap Aksoylar","doi":"10.1055/a-2815-2484","DOIUrl":"10.1055/a-2815-2484","url":null,"abstract":"Survival in high-risk neuroblastoma has improved with multimodal treatment approaches and numerous studies have focused on treatment-related side effects. This study examines the prevalence, severity and risks of late effects following high-dose chemotherapy and autologous stem cell transplantation in high risk neuroblastoma patients.This study included high-risk neuroblastoma patients who underwent autologous stem cell transplantation and survived 5 years without relapse in a single center.Of the 41 patients who received high-dose chemotherapy and autologous stem cell transplantation, 20 patients survived without relapse for at least 5 years and were included in the analysis. Amid 20 patients, 12 patients (60%) were men. The median age of the patients was 13.5 years at the last follow up. After six cycles of induction chemotherapy, 11 patients (55%) received busulfan-melphalan as the consolidation regimen, while 9 patients (45%) received carboplatin-etoposide-melphalan. The median follow-up after transplantation was 9 years. At least one complication occurred in 19 out of 20 patients (95%). Severe late complications were observed in seven patients. Two patients developed treatment-related secondary neoplasms. In contrast to the literature, the most common adverse event was focal nodular hyperplasia (40%) in the liver. The second most common adverse event was ovarian failure (37.5%) and the third most common was hearing loss (35%). Endocrine pathologies were more common in patients receiving busulfan-melphalan as the consolidation regimen.This study highlights the future risks of side effects in high-risk neuroblastoma survivors and emphasizes the need for a long-term follow-up.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146220059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome without Camptodactyly: A Pediatric Case and Review of the Literature. 无喜树趾畸形的喜树趾-关节病-髋内翻-心包炎综合征：1例儿科病例及文献回顾。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-04 DOI: 10.1055/a-2814-3127

Firat Gundogmus, Halil Kazanasmaz, Ender Can Eroglu, Mukaddes Kalyoncu

{"title":"Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome without Camptodactyly: A Pediatric Case and Review of the Literature.","authors":"Firat Gundogmus, Halil Kazanasmaz, Ender Can Eroglu, Mukaddes Kalyoncu","doi":"10.1055/a-2814-3127","DOIUrl":"https://doi.org/10.1055/a-2814-3127","url":null,"abstract":"Background: Camptodactyly-arthropathy-coxa vara-pericarditis syndrome is a rare autosomal recessive disorder with camptodactyly, non-inflammatory arthropathy, coxa vara, and pericarditis. Symptoms usually begin in early childhood with swelling of interphalangeal joints, wrists, and knees. Pericarditis is rare. It is caused by proteoglycan 4 gene mutations and often misdiagnosed as juvenile idiopathic arthritis due to overlapping features.Case presentation: A 4-year-old boy presented with 6 months of knee and wrist swelling and nocturnal pain. His parents were first-degree cousins, and a maternal uncle had rheumatoid arthritis. He had prior hip surgeries at 2.5 years. Examination showed swelling in knees, ankles, and wrists, and 30° wrist extension limitation. No systemic symptoms were noted. The erythrocyte sedimentation rate, C-reactive protein level, and antinuclear antibody test results were normal. Magnetic resonance imaging showed effusion and synovial thickening. Initially diagnosed with polyarticular juvenile idiopathic arthritis, he received methotrexate and etanercept. Due to progressive gait issues and radiographic coxa vara, diagnosis was revised. Genetic testing confirmed homozygous proteoglycan 4 (c.915delC) mutation.Conclusions: This case underscores an atypical camptodactyly-arthropathy-coxa vara-pericarditis presentation without camptodactyly. Persistent non-inflammatory arthropathy despite immunosuppression should raise suspicion for camptodactyly-arthropathy-coxa vara-pericarditis.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147355501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Outpatient parenteral antibiotic therapy in pediatric oncology patients with fever in neutropenia: literature review and scoping review]. 【小儿肿瘤发热中性粒细胞减少患者的门诊肠外抗生素治疗：文献回顾和范围回顾】。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-03 DOI: 10.1055/a-2775-4289

S Barbulescu, A Wagner, D Schoendorf, R Toenne, D Schneider, T Simon, M Remke, D Reinhard, A Simon

{"title":"[Outpatient parenteral antibiotic therapy in pediatric oncology patients with fever in neutropenia: literature review and scoping review].","authors":"S Barbulescu, A Wagner, D Schoendorf, R Toenne, D Schneider, T Simon, M Remke, D Reinhard, A Simon","doi":"10.1055/a-2775-4289","DOIUrl":"https://doi.org/10.1055/a-2775-4289","url":null,"abstract":"Fever in neutropenia (FN) is a common complication in pediatric oncology patients receiving chemotherapy. These patients are usually treated as inpatients. Alternatively, under certain conditions, pediatric outpatient parenteral antibiotic therapy (pAPAT) may be an option.Literature search from PubMed and the Cochrane Library. Review of the included studies in a Summary of Findings table. Interpretation of the results by pediatric oncologists and infectious disease specialists.Twelve original studies involving 653 pAPAT courses were evaluated. pAPAT was used in stable patients in good general condition, with reliable adherence and accessibility (as outpatients), without a focus of infection and with negative blood cultures during the course of treatment. pAPAT was successfully completed in 75-95% of cases and was not inferior to inpatient treatment in comparative studies. There were no infection-associated deaths in the pAPAT group. The vast majority of patients and their families rated the pAPAT experience positively.The available studies are very heterogeneous in terms of definitions, risk stratification, selection of antibiotics, and the structural, organizational, and personnel processes involved in pAPAT. Taking these limitations into account, pAPAT after at least 24 hours of inpatient monitoring appears to be an effective and safe alternative to inpatient treatment for pediatric oncology patients with fever in neutropenia who display a low risk of severe complications.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Congenital Disorders of Glycosylation Type I: A Report of Two Cases. 先天性糖基化I型疾病附2例报告。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-02 DOI: 10.1055/a-2799-8968

Semra Atasoy Yilmaz, Burcu Güven, Ilker Eyüboğlu, Beril Dilber, Aslı Durmus, Basire Nur Yıldız

引用次数: 0

[Alveolar capillary dysplasia with FOXF1 mutation as differential diagnosis in refractory persistent pulmonary hypertension (PPHN)]. [肺泡毛细血管发育不良伴FOXF1突变作为难治性持续性肺动脉高压（PPHN）的鉴别诊断]。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-11-24 DOI: 10.1055/a-2710-7277

Hannah Weller, Anne Hickmann, Julia Heiter, Dennis Klinkenberg, Jochen Herrmann, Dominique Singer, Mario Lange

引用次数: 0

Severe Asthma in Children and Adolescents: an AWMF Guideline by the German Society for Pediatric Pulmonology. 儿童和青少年严重哮喘：德国儿科肺病学会的AWMF指南。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-12-23 DOI: 10.1055/a-2752-7457

Eckard Hamelmann, Christina Schorlemer, Nico Derichs, Ernst Eber, Michael Gerstlauer, Andreas Jung, Michael Kabesch, Matthias Volkmar Kopp, Susanne Lau, Christiane Lex, Alex Möller, Bianca Schaub, Nicolaus Schwerk, Thomas Spindler, Christian Taube, Christian Vogelberg, Angela Zacharasiewicz, Stefan Zielen, Antje Schuster, Monika Gappa

{"title":"Severe Asthma in Children and Adolescents: an AWMF Guideline by the German Society for Pediatric Pulmonology.","authors":"Eckard Hamelmann, Christina Schorlemer, Nico Derichs, Ernst Eber, Michael Gerstlauer, Andreas Jung, Michael Kabesch, Matthias Volkmar Kopp, Susanne Lau, Christiane Lex, Alex Möller, Bianca Schaub, Nicolaus Schwerk, Thomas Spindler, Christian Taube, Christian Vogelberg, Angela Zacharasiewicz, Stefan Zielen, Antje Schuster, Monika Gappa","doi":"10.1055/a-2752-7457","DOIUrl":"10.1055/a-2752-7457","url":null,"abstract":"Asthma is the most common chronic respiratory disease in children and adolescents. While most patients achieve good control with guideline-based treatment, a significant proportion experience persistent symptoms, frequent exacerbations, and impaired quality of life.This guideline aims to define severe and difficult-to-treat asthma in children and adolescents, support diagnostic precision, and provide practical, evidence-based recommendations for assessment and management, including biological therapies.The S1 guideline was developed under the coordination of the German Society for Pediatric Pulmonology following AWMF procedures. A structured consensus process involving experts from pediatric pulmonology, allergology, and general pediatrics was conducted. Existing national and international guidelines and new evidence were systematically reviewed and adapted.Key elements include a stepwise diagnostic algorithm to distinguish difficult-to-treat from truly severe asthma, guidance on assessing adherence, comorbidities, and inflammation biomarkers, and recommendations for targeted biological treatment. This guideline addresses monitoring tools, transition to adult care, and the role of rehabilitation.Children and adolescents with severe asthma require early referral to specialized centers and a structured, interdisciplinary approach. Personalized treatment strategies-including biologics-should be guided by phenotyping and biomarkers. Registry data are essential to improve care quality and generate real-world evidence.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"65-73"},"PeriodicalIF":0.9,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145819900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

ALL Figured Out? Primary Leukemic Pulmonary Infiltration in an Adolescent with Acute Lymphoblastic Leukemia: A Case Report and a Brief Review of the Literature. 都明白了吗？青少年急性淋巴细胞白血病的原发性白血病肺浸润：1例报告及文献回顾。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-12-09 DOI: 10.1055/a-2738-7397

Rachel Bortnick, Herwig Brandtner, Corinne Vasilico, Pavel Basek, Simon Grewendorf

引用次数: 0

[Pediatric pneumological aspects in the care of children with Down Syndrome]. 【唐氏综合症患儿护理中的儿科肺炎方面】。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-12-11 DOI: 10.1055/a-2748-4649

Hans Fuchs, Lennart Gunst, Anke Wendt, Sebastian Becker, Ruth Margarethe Grychtol, Dejan Vlajnic, Dorit Aschmann-Muehlhans, Christiane Wuerfel, Mathis Steindor, Svea Muehlberg, Florian Stehling

{"title":"[Pediatric pneumological aspects in the care of children with Down Syndrome].","authors":"Hans Fuchs, Lennart Gunst, Anke Wendt, Sebastian Becker, Ruth Margarethe Grychtol, Dejan Vlajnic, Dorit Aschmann-Muehlhans, Christiane Wuerfel, Mathis Steindor, Svea Muehlberg, Florian Stehling","doi":"10.1055/a-2748-4649","DOIUrl":"10.1055/a-2748-4649","url":null,"abstract":"Pulmonary problems are common in children with Downsyndrome/trisomy 21, alongside other health issues, but are often given too little attention. The aim of this review is to summarize these aspects for pediatric pulmonologists. Narrow nasal passages, a small pharynx and larynx, in combination with relative macroglossia, other airway malformations, and generalized muscular hypotonia, lead to glossoptosis, which in very young infants frequently causes obstructive sleep apnea syndrome. If left untreated, this is associated with impaired cognitive development. The children also suffer from chronic rhinitis. Together with recurrent silent aspirations resulting from the typical dysphagia of children with Trisomie 21 and immune dysregulation, lower respiratory tract infections are common and often severe. Viral infections caused by RSV, influenza, and SARS-CoV-2 more frequently lead to hospitalizations and have a much higher mortality rate than in healthy children. Children with Down syndrome are also more likely to develop chronic wheezing. The development of pulmonary hypertension may rarely occur even without an associated heart defect. This article summarizes the diagnostic and therapeutic tasks related to pulmonary problems in children with Down syndrome for the pediatric pulmonologist.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"59-64"},"PeriodicalIF":0.9,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145743245","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Near-Fatal Asthma Due to Severe Airway Mucus Plugging in a 12-Year-Old Boy. 一名12岁男孩严重气道粘液堵塞导致的近乎致命的哮喘。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2026-02-10 DOI: 10.1055/a-2787-2111

Wanda Naumann, Dominik Leitz, Mirjam Völler, Vladimir Skrypnikov, Martin Ruß, Björn Weiß, Viktoria Martiny, Stefanie Hort, Anke Wendt, Susanne Lau, Alexander Gratopp, Marcus Alexander Mall

引用次数: 0

Rare Pediatric Pulmonary Diseases: Insights from a Survey of Pediatric Pulmonologists in German-Speaking Countries. 罕见的儿科肺部疾病：来自德语国家儿科肺病学家调查的见解。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-10-31 DOI: 10.1055/a-2713-3667

Julia Carlens, Srdjan Micic, Nicolaus Schwerk, Matthias Griese, Alexander Moeller, Elias Seidl

{"title":"Rare Pediatric Pulmonary Diseases: Insights from a Survey of Pediatric Pulmonologists in German-Speaking Countries.","authors":"Julia Carlens, Srdjan Micic, Nicolaus Schwerk, Matthias Griese, Alexander Moeller, Elias Seidl","doi":"10.1055/a-2713-3667","DOIUrl":"10.1055/a-2713-3667","url":null,"abstract":"Rare pediatric pulmonary diseases, such as childhood interstitial lung disease and congenital thoracic malformations, pose diagnostic and therapeutic challenges due to their low prevalence and clinical heterogeneity. In contrast to cystic fibrosis and primary ciliary dyskinesia, which are supported by dedicated care networks, many other rare pediatric pulmonary diseases lack structured management pathways. This study aimed to assess pediatric pulmonologists' clinical exposure, confidence, and educational needs related to rare pediatric pulmonary diseases. A web-based survey was distributed to all 914 members of the German Society for Pediatric Pulmonology. The questionnaire evaluated clinical experience, diagnostic confidence, and preferences for educational contents and formats. Responses were analyzed using descriptive statistics and group comparisons. Among 209 respondents (22.9% response rate), clinical exposure was low (median: 3 patients/y; interquartile range: 1-5), with 22.5% treating none. Only 36.7% of respondents felt confident in suspecting a rare pediatric pulmonary disease, 22.0% in diagnosis, and 13.3% in treatment. Educational interest was high (68.8%), particularly in clinical presentation (70.7%), treatment (69.8%), and imaging (59.0%). Clinicians with<10 years of experience reported greater interest in clinical presentation than those with≥10 years (84.5% vs. 57.3%, p=0.0002). Workshops, webinars, and online discussions were the most preferred learning formats. Despite limited exposure and low reported confidence, pediatric pulmonologists express strong interest in further education on rare pediatric pulmonary diseases. Tailored, accessible educational strategies are essential to improve awareness, diagnosis, and care for children with rare pulmonary conditions.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"86-92"},"PeriodicalIF":0.9,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145421979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0