Diffuse lung disease in young children - Application of a novel classification scheme
Diffuse lung disease in young children - Application of a novel classification scheme
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DOI：
10.1164/rccm.200703-393oc
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发表时间：
2007-12-01
影响因子：
24.7
通讯作者：
White, Frances V.
中科院分区：
文献类型：
--
作者：
Deutsch, Gail H.;Young, Lisa R.;White, Frances V.
基本原理：由于成人和幼儿之间疾病谱的相对罕见和差异，关于小儿弥漫性肺病的命名、分类和管理存在相当大的混乱：目的：形成一个多学科工作组：（1）应用共识术语和诊断标准，用于婴儿期弥漫性肺病的疾病;和（2）描述目前在北美进行肺活检的幼儿的疾病实体、临床特征和结果的分布。11个中心提供了病理材料、临床数据，和影像学检查的所有儿童小于2岁谁接受了肺活检弥漫性肺疾病从1999年至2004年。测量和主要结果：多学科审查对187例病例中的88%进行了分类。婴儿期更常见的疾病，包括原发性发育和肺生长异常、婴儿期神经内分泌细胞增生和表面活性物质功能障碍，构成了大多数病例（60%）。肺生长障碍通常在临床上未被怀疑，在组织学上被低估。已知表面活性物质突变的病例具有特征性病理特征。活检时的年龄和临床表现因类别而异。肺动脉高压，存在原发性发育异常，或ABCA 3突变与高死亡率，而没有死亡发生在肺间质糖原，或神经内分泌细胞增生的婴儿期。结论：这项回顾性队列研究确定了一个不同的频谱的肺部疾病，主要是独特的幼儿。应用分类方案将具有不同活检年龄和死亡率的临床不同患者分组。标准化的术语和分类将提高这些疾病的准确描述和诊断。
Rationale: Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and differences in the spectrum of disease between adults and young children.Objectives: A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical features, and outcome in young children who currently undergo lung biopsy in North America.Methods: Eleven centers provided pathologic material, clinical data, and imaging from all children less than 2 years of age who underwent lung biopsy for diffuse lung disease from 1999 to 2004.Measurements and Main Results: Multidisciplinary review categorized 88% of 187 cases. Disorders more prevalent in infancy, including primary developmental and lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and surfactant-dysfunction disorders, constituted the majority of cases (60%). Lung growth disorders were often unsuspected clinically and under-recognized histologically. Cases with known surfactant mutations had characteristic pathologic features. Age at biopsy and clinical presentation varied among categories. Pulmonary hypertension, presence of a primary developmental abnormality, or ABCA3 mutation was associated with high mortality, while no deaths occurred in cases of pulmonary interstitial glycogenosis, or neuroendocrine cell hyperplasia of infancy.Conclusions: This retrospective cohort study identifies a diverse spectrum of lung disorders, largely unique to young children. Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality. Standardized terminology and classification will enhance accurate description and diagnosis of these disorders.