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儿童弥漫性肺疾病的诊断病理学

Diagnostic Pathology of Diffuse Lung Disease in Children.

作者信息

Dishop Megan K

机构信息

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

出版信息

Pediatr Allergy Immunol Pulmonol. 2010 Mar;23(1):69-85. doi: 10.1089/ped.2010.0007.

DOI:10.1089/ped.2010.0007
PMID:22332032
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3269262/
Abstract

The pathologic classification of diffuse lung disease in children and adolescents has undergone revision in recent years in response to rapid developments and new discoveries in the field. A number of important advancements have been made in the last 10 years including the description of new genetic mutations causing severe lung disease in infants and children, as well as the description of new pathologic entities in infants. These recently described entities, including ABCA3 surfactant disorders, pulmonary interstitial glycogenosis, and neuroendocrine cell hyperplasia of infancy, are being recognized with increasing frequency. This review will include brief discussion of the etiology and pathogenesis of the major groups of diffuse lung disease in children. Histopathologic features are discussed for each of the major categories of diffuse lung disease in children, beginning with the genetic, developmental, and alveolar growth disorders common in infancy, followed by brief discussion of airway diseases, immunologic diseases, and pulmonary vascular diseases seen more commonly in older children. A protocol for handling pediatric wedge lung biopsies is also discussed, which optimizes the diagnostic yield of lung biopsies in this population.

摘要

近年来,随着儿童和青少年弥漫性肺疾病领域的快速发展和新发现,其病理分类已经历修订。在过去10年里取得了一些重要进展,包括描述了导致婴幼儿严重肺部疾病的新基因突变,以及描述了婴儿期新的病理实体。这些最近描述的实体,包括ABCA3表面活性剂疾病、肺间质糖原沉积症和婴儿期神经内分泌细胞增生,正越来越频繁地被认识到。本综述将简要讨论儿童弥漫性肺疾病主要类型的病因和发病机制。讨论了儿童弥漫性肺疾病各主要类别的组织病理学特征,首先是婴儿期常见的遗传、发育和肺泡生长障碍,随后简要讨论了年龄较大儿童中更常见的气道疾病、免疫性疾病和肺血管疾病。还讨论了处理儿童楔形肺活检的方案,该方案可优化该人群肺活检的诊断率。

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本文引用的文献

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Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT.婴儿期神经内分泌细胞增生:高分辨率 CT 诊断。
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Genomic and genic deletions of the FOX gene cluster on 16q24.1 and inactivating mutations of FOXF1 cause alveolar capillary dysplasia and other malformations.16号染色体长臂24.1区域FOX基因簇的基因组和基因缺失以及FOXF1的失活突变会导致肺泡毛细血管发育异常和其他畸形。
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Diffuse lung disease in infancy: a proposed classification applied to 259 diagnostic biopsies.婴儿期弥漫性肺疾病:应用于259例诊断性活检的一种拟议分类法
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Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA.由CSF2RA基因突变引起的家族性肺泡蛋白沉积症。
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