Klinische Padiatrie最新文献_第9页

Successful methionine treatment for diffuse lung disease in a child with pathogenic MARS-1 gene variants. 蛋氨酸治疗致病性MARS-1基因变异患儿弥漫性肺病的成功

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-12-11 DOI: 10.1055/a-2744-0192

Aylin Pörtecene, Julia Carlens, Anibh Martin Das, Maurice Petrasch, Nicolaus Schwerk, Pia Maier

引用次数: 0

Tuberculosis in a Large Day Care Center: A Case Report and Review of the Literature. 大型日托中心肺结核病例报告及文献回顾。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2026-01-16 DOI: 10.1055/a-2763-5966

Franziska Zeyer, Hanna Renk, Elke Maritz, Matthias Kumpf, Markus Kratz

引用次数: 0

Correction: Severe Asthma in Children and Adolescents: an AWMF Guideline by the German Society for Pediatric Pulmonology. 更正：儿童和青少年的严重哮喘：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: 2026-04-01 DOI: 10.1055/a-2838-1477

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

引用次数: 0

Chiari Malformation Type I with Compression of the Cervical Myelon as a Cause of Laryngomalacia. I型Chiari畸形伴颈髓受压，是喉软化症的病因。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2025-12-23 DOI: 10.1055/a-2744-7399

Anna Pauly, Mathis Steindor, Adela Della Marina, Heike Kölbel, Philipp Dammann, Marcel Opitz, Florian Stehling

引用次数: 0

[Infant with cyanosis and hypoxemia]. [婴儿发绀和低氧血症]。

IF 0.8 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2026-01-19 DOI: 10.1055/a-2763-4491

Simone Nicolaus, Ute Friederike Jarmola, Sebastian Becker

{"title":"[Infant with cyanosis and hypoxemia].","authors":"Simone Nicolaus, Ute Friederike Jarmola, Sebastian Becker","doi":"10.1055/a-2763-4491","DOIUrl":"10.1055/a-2763-4491","url":null,"abstract":"We report on an 8-week-old infant who presented as an outpatient due to abnormal skin color a few hours after surgical achillotomy under local anesthesia. The infant showed a dirty brownish, cyanotic skin color with reduced general condition and hypoxemia at SaO2 84% without improvement by oxygen supplementation. The blood gas analysis showed lactic acidosis and a significantly elevated methemoglobin level of 38% (reference value≤1.5%). Due to the temporal correlation, this is most likely due to the infiltration anesthesia with mepivacaine, which was performed for the achillotomy. Treatment with methylene blue was administered, resulting in a restitutio ad integrum.Methemoglobin is formed by the oxidation of iron contained in hemoglobin, which prevents oxygen from binding. Significant cyanosis can occur when the methemoglobin level exceeds 10%. The reduction of methemoglobin to oxyhemoglobin by the enzyme NADH cytochrome b5 reductase is physiologically not fully developed in young infants. Furthermore, the hemoglobin of young infants is more easily oxidized, which makes these children susceptible to methemoglobinaemia. As the methemoglobin content increases, the blood turns brown with increasing levels of methemoglobin, which is visible clinically and in blood samples.Local anesthetics applied cutaneously, subcutaneously or mucous membranes can cause clinically relevant methemoglobinemia, which can result in potentially severe hypoxemia. The risk is increased in young infants. It is important to recognize methemoglobinemia as the cause of cyanosis in time, as it is easily treatable.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"93-96"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146003234","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

[Procedural options in the prescription of outpatient intensive care and potential assessment in children and adolescents.] [儿童和青少年门诊重症监护处方的程序选择及潜力评估]

IF 0.8 4区医学

Klinische Padiatrie Pub Date : 2026-03-01 Epub Date: 2026-02-04 DOI: 10.1055/a-2787-2051

Sebastian Becker, Florian Stehling, Hans Fuchs, Lennart Gunst

引用次数: 0

Lamp Oil Poisoning: Pulmonary Hypertension Following Oil Aspiration Successfully Treated with Inhaled Nitric Oxide. 灯油中毒：吸入一氧化氮成功治疗吸油后肺动脉高压。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-02-27 DOI: 10.1055/a-2814-3185

Demet Tosun, İlyas Bingöl, Nihal Akçay

引用次数: 0

Long-Term Outcomes of Successful Treatment with Ruxolitinib in a Pediatric Patient with Autoimmune Lymphoproliferative Syndrome and a Signal Transducer and Activator of Transcription 3 Gain-of-Function Mutation. Ruxolitinib治疗自身免疫性淋巴细胞增生综合征和转录3功能获得突变的信号传感器和激活因子的成功治疗的长期结果

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-02-27 DOI: 10.1055/a-2788-7908

Sultan Aydin, Ayse Oz, Tarkan Kalkan, Güney Kücük, Fatih Celmeli

{"title":"Long-Term Outcomes of Successful Treatment with Ruxolitinib in a Pediatric Patient with Autoimmune Lymphoproliferative Syndrome and a Signal Transducer and Activator of Transcription 3 Gain-of-Function Mutation.","authors":"Sultan Aydin, Ayse Oz, Tarkan Kalkan, Güney Kücük, Fatih Celmeli","doi":"10.1055/a-2788-7908","DOIUrl":"https://doi.org/10.1055/a-2788-7908","url":null,"abstract":"Loss or gain-of-function mutations in signal transducer and transcription activator genes, classified as combined immunodeficiency, can present with highly heterogeneous and life-threatening clinical presentations. We report an adolescent girl who has combined immunodeficiency associated with signal transducer and activator of transcription 3 gain-of-function, c.1032G > C, p.Met344Ile located in exon 10, identified by a next-generation sequencing panel performed due to short stature, hepatosplenomegaly, and decreased levels of both immunoglobulin G and immunoglobulin M. At 12 years of age, the patient was referred to the pediatric hematology outpatient clinic owing to hepatosplenomegaly and pancytopenia after initiating growth hormone treatment due to short stature. On physical examination, bilateral multiple posterior and anterior cervical micro-lymphadenopathies were detected, her liver was palpated 6 cm below the costal margin, and her spleen was palpated 10 cm below the costalmargin. The whole blood count revealed a leukocyte count of 2,300/mm3, neutrophils of 1,330/mm3, lymphocytes of 730/mm3, hemoglobin of 8 g/dL, platelets of 96,000/mm3, and reticulocytes of 2%. The bone marrow biopsy revealed a cellularity of 30-35%. After steroid-dependent treatment resulted in Cushing syndrome and treatment with sirolimus was ineffective, treatment with ruxolitinib 20 mg twice daily significantly resolved cytopenias and hepatosplenomegaly due to autoimmune lymphoproliferative syndrome.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147317423","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

[Pneumocystis Pneumonia Following Chemotherapy-Induced Immunosuppression - A Rare Differential Diagnosis in Children and Adolescence]. 化疗诱导免疫抑制后肺囊虫性肺炎——儿童和青少年罕见的鉴别诊断。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-02-17 DOI: 10.1055/a-2798-4613

Carolin Czekalla, Helena Posch, Mirjam Stahl

引用次数: 0

Malaria in Children is Associated with a High Rate of Neurological Sequelae and Need for Rehabilitative Therapies in Germany. 在德国，儿童疟疾与神经系统后遗症的高发率和对康复治疗的需求有关。

IF 0.9 4区医学

Klinische Padiatrie Pub Date : 2026-02-06 DOI: 10.1055/a-2784-6563

Jonas Erasmus Adolph, Christina Pentek, Lydia Rink, Clara Johanna Held, Adela Della Marina, Andrea Gangfuß, Heike Kölbel, Jan Dziobaka, Peter-Michael Rath, Hedda Luise Verhasselt, Ursula Felderhoff-Müser, Christian Dohna-Schwake, Sarah Christina Goretzki

{"title":"Malaria in Children is Associated with a High Rate of Neurological Sequelae and Need for Rehabilitative Therapies in Germany.","authors":"Jonas Erasmus Adolph, Christina Pentek, Lydia Rink, Clara Johanna Held, Adela Della Marina, Andrea Gangfuß, Heike Kölbel, Jan Dziobaka, Peter-Michael Rath, Hedda Luise Verhasselt, Ursula Felderhoff-Müser, Christian Dohna-Schwake, Sarah Christina Goretzki","doi":"10.1055/a-2784-6563","DOIUrl":"https://doi.org/10.1055/a-2784-6563","url":null,"abstract":"Malaria can cause severe complications including cerebral involvement and long-term neurocognitive impairment, especially in young children. Our study presents data on pediatric malaria cases with a focus on long-term neurological and neurocognitive outcomes following standardized treatment.This retrospective, single-centre study analyzed all pediatric malaria cases treated at our tertiary care hospital in 2023. Follow-ups included neurological examinations, standardized intelligence testing, electroencephalography, and cranial magnetic resonance imaging, with additional assessments provided as needed.Eleven patients (median age: 9.5 y) were included, with Plasmodium falciparum identified in 91% of cases. Eight (72.7%) patients were diagnosed with severe malaria. Artesunate was used as first-line therapy in 64% of patients. Residual neurological symptoms were observed in 82% of patients. Neurocognitive testing revealed deficits in 44% of the tested patients. Electroencephalographic abnormalities were noted in four patients; three patients developed epilepsies. Cranial magnetic resonance imaging findings included cytotoxic lesions of the corpus callosum and trigonal lesions in three patients. At 12 months, 77.8% of patients showed clinical improvement.Despite prompt, guideline-appropriate treatment, pediatric malaria patients in our cohort exhibited high rates of neurological sequelae requiring rehabilitative and pharmacological treatments. These findings highlight the need for a coordinated follow-up even in non-endemic countries.","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146132313","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