Canny G J, Cutz E, MacLusky I B, Levison H
Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Thorax. 1991 Nov;46(11):851-3. doi: 10.1136/thx.46.11.851.
A six year old girl with diffuse pulmonary angiomatosis presented with haemoptysis and diffuse interstitial changes with bilateral pleural effusions on the chest radiograph. The lung lesion as seen on biopsy specimens (and confirmed at necropsy) consisted of bloodless, thin walled, endothelium lined channels, affecting the interstitial septae, pleura, bronchi, and adventitia of large vessels. There was no response to oral corticosteroids or a trial of cyclosphamide. This lesion may be an example of an angiogenic disease.
一名患有弥漫性肺血管瘤病的6岁女孩出现咯血，胸部X线片显示弥漫性间质改变伴双侧胸腔积液。活检标本所见（尸检证实）的肺部病变由无血、薄壁、内衬内皮的通道组成，累及间质间隔、胸膜、支气管和大血管外膜。口服皮质类固醇或环磷酰胺试验均无反应。这种病变可能是一种血管生成性疾病的实例。
