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儿科间质性肺疾病:分类与定义。

Paediatric interstitial lung disease: classification and definitions.

机构信息

Department of Pathology, The Children's Hospital, University of Colorado-Denver, USA.

出版信息

Paediatr Respir Rev. 2011 Dec;12(4):230-7. doi: 10.1016/j.prrv.2011.01.002. Epub 2011 Mar 5.

DOI:10.1016/j.prrv.2011.01.002
PMID:22018036
Abstract

Classifications of interstitial (diffuse) lung disease in adults and children have undergone significant revision in recent years, with advances in our understanding of new entities and the biology and prognostic significance of certain histologic patterns. The contributions of the European Respiratory Society Task Force on Interstitial Lung Disease in Children and the North American Children's Interstitial Lung Disease Group are reviewed, and a clinicopathologic classification of paediatric diffuse lung disease is summarized. Clinical characteristics and histologic definitions are also presented for selected entities within this classification, specifically, acinar dysgenesis, congenital alveolar dysplasia, alveolar capillary dysplasia with misalignment of pulmonary veins, abnormalities of alveolar growth, pulmonary interstitial glycogenosis, neuroendocrine cell hyperplasia of infancy, surfactant dysfunction disorders, obliterative bronchiolitis, hypersensitivity pneumonitis, and immunologic disorders. More uniform application of this diagnostic terminology in the future will allow more meaningful comparisons of different patient populations, radiologic-pathologic correlation, and development of disease-specific therapeutic strategies.

摘要

近年来,成人和儿童间质性(弥漫性)肺疾病的分类发生了重大修订,这得益于我们对新实体以及某些组织学模式的生物学和预后意义的理解的进步。回顾了欧洲呼吸学会儿童间质性肺疾病工作组和北美儿童间质性肺疾病小组的贡献,并总结了儿科弥漫性肺疾病的临床病理分类。本文还介绍了该分类中某些特定实体的临床特征和组织学定义,具体包括腺泡发育不良、先天性肺泡发育不良、肺静脉排列异常的肺泡毛细血管发育不良、肺泡生长异常、肺间质糖原沉积症、婴儿神经内分泌细胞增生、表面活性剂功能障碍、闭塞性细支气管炎、过敏性肺炎和免疫性疾病。未来更统一地应用这一诊断术语将能够对不同患者群体、影像学-病理学相关性进行更有意义的比较,并制定针对特定疾病的治疗策略。

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