Diffuse alveolar hemorrhage in children with interstitial lung disease: Determine etiologies!
Diffuse alveolar hemorrhage in children with interstitial lung disease: Determine etiologies!
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DOI：
10.1002/ppul.26301
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发表时间：
2023-04
影响因子：
3.1
通讯作者：
Griese, Matthias
中科院分区：
文献类型：
--
作者：
Knoflach, Katrin;Rapp, Christina Katharina;Schwerk, Nicolaus;Carlens, Julia;Wetzke, Martin;Emiralioglu, Nagehan;Kiper, Nural;Ring, Astrid Madsen;Buchvald, Frederik;Manali, Effrosyni;Papiris, Spyros;Reu-Hofer, Simone;Kappler, Matthias;Schieber, Alexandra;Seidl, Elias;Gothe, Florian;Robinson, Peter N.;Griese, Matthias
关键词：
儿童弥漫性肺泡出血是一种由多种潜在疾病引发的罕见病症。本研究旨在描述患有间质性肺病（儿童间质性肺病，chILD）和弥漫性肺泡出血（DAH）的儿童的特征及诊断方法，通过提高临床医生对诊断缺陷的认识来改进诊断方式。
我们对1997年7月1日至2020年12月31日期间在我院或合作中心接受治疗的ILD和DAH患者进行了回顾性数据分析。以病例简述的形式系统检索并研究了临床病程和诊断方法的数据。为评估诊断软件算法的适用性，对人类表型本体（HPO）进行了修订和扩充，以优化其相关工具“Phenomizer”的使用条件。
在131例患者中，97例（74%）明确了肺出血的病因。34例患者（26%）未发现潜在病因（称为特发性肺出血，IPH）。根据实验室检查结果或临床表型/合并症，其中20例患者被归入描述性类别：与自身免疫特征相关的IPH（9例）、嗜酸性粒细胞增多（5例）、肾脏疾病（3例）或多器官受累（3例）。对于14例患者，无法进一步分类。
完整且有时需重复进行的诊断对于确诊DAH儿童至关重要。我们建议将IPH患者归入描述性类别，这也可为进一步研究提供指导。像Phenomizer/HPO这样的数字化工具很有前景，但需要扩展完善以提高诊断准确性。
Diffuse alveolar hemorrhage in children is a rare condition resulting from different underlying diseases. This study aimed at describing characteristics and diagnostic measures in children with ILD (children’s interstitial lung disease, chILD) and diffuse alveolar hemorrhage (DAH) to improve the diagnostic approach by increasing clinician’s awareness of diagnostic shortcomings. A retrospective data analysis of patients with ILD and DAH treated in our own or collaborating centers between 01/07/1997 and 31/12/2020 was performed. Data on clinical courses and diagnostic measures were systematically retrieved as case-vignettes and investigated. To assess suitability of diagnostic software-algorithms, the Human Phenotype Ontology (HPO) was revised and expanded to optimize conditions of its associated tool the “Phenomizer”. For 97 (74%) of 131 patients, etiology of pulmonary hemorrhage was clarified. For 34 patients (26%), no underlying condition was found (termed as idiopathic pulmonary hemorrhage, IPH). Based on laboratory findings or clinical phenotype/ comorbidities, 20 of these patients were assigned to descriptive clusters: IPH associated with autoimmune features (9), eosinophilia (5), renal disease (3) or multi-organ involvement (3). For 14 patients, no further differentiation was possible. Complete and sometimes repeated diagnostics are essential for establishing the correct diagnosis in children with DAH. We suggest assignment of patients with IPH to descriptive clusters, which may also guide further research. Digital tools such as the Phenomizer/ HPO are promising, but need to be extended to increase diagnostic accuracy.
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基准对床的评论：肺肾脏综合征 - 强化主义者的更新。
影响因子：
15.1
作者：
Papiris, Spyros A.;Manali, Effrosyni D.;Kalomenidis, Ioannis;Kapotsis, Giorgios E.;Karakatsani, Anna;Roussos, Charis
通讯作者：
Roussos, Charis
影响因子：
13.3
作者：
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通讯作者：
Goldbach-Mansky, Raphaela
影响因子：
1.4
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通讯作者：
Kasapcopur, Ozgur
影响因子：
3.7
作者：
Griese, Matthias;Irnstetter, Armin;Kroener, Carolin
通讯作者：
Kroener, Carolin
影响因子：
2.7
作者：
Aksu, G.;Gulez, N.;Kutukculer, N.
通讯作者：
Kutukculer, N.