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[特发性肺含铁血黄素沉着症:2例病例的解剖临床研究]

[Idiopathic pulmonary hemosiderosis: anatomoclinical study of 2 cases].

作者信息

Fidalgo I, Simón R, Rivera J M, Vázquez C, Benzanilla J L

出版信息

An Esp Pediatr. 1982 Aug;17(2):130-6.

PMID:7149480
Abstract

Two children with idiopathic pulmonary hemosiderosis are described. Both of them presented initially a clinical picture of recurring iron deficiency anemia, before pulmonary symptoms and signs made it possible to establish correct diagnosis. In one case diagnosis was considered only when an overwhelming pulmonary hemorrhage occurred, leading to the patient's death. At necropsy, areas of fresh alveolar hemorrhage and hemosiderin loaded macrophages were found, as well as moderate to severe interstitial fibrosis and hyperplastic alveolar cells. There was not disruption of the basal alveolocapillary membrane.

摘要

本文描述了两名患有特发性肺含铁血黄素沉着症的儿童。他们最初均表现为反复缺铁性贫血的临床症状,之后出现肺部症状和体征才得以确诊。其中一例仅在发生严重肺出血导致患者死亡后才得以诊断。尸检时发现有新鲜肺泡出血区域、含铁血黄素沉积的巨噬细胞,以及中度至重度的间质纤维化和肺泡细胞增生。肺泡毛细血管基底膜未被破坏。

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