婴儿肺泡蛋白沉积症

Pulmonary alveolar proteinosis in infants.

作者信息

Sakai Y, Abo W, Yoshimura H, Sano H, Kuroki Y, Satoh M, Kaimori M

机构信息

Department of Paediatrics, Aomori Central Hospital, Higashi-Tsukurimichi, Japan.

出版信息

Eur J Pediatr. 1999 May;158(5):424-6. doi: 10.1007/s004310051107.

DOI:10.1007/s004310051107

PMID:10333130

Abstract

UNLABELLED

We investigated the histological and molecular characteristics of pulmonary alveolar proteinosis (PAP) in two siblings (a brother and sister) who did not exhibit respiratory distress at birth but who each developed symptoms during infancy. Histological analysis of lung specimens showed positive staining for surfactant proteins in both patients. The polymerase chain reaction revealed expression of messenger RNA for surfactant protein B (SP-B) in the lung specimens. No defect in SP-B which is characteristic of the congenital form of PAP was observed. The concentration of surfactant protein A (SP-A) in bronchial alveolar lavage (BAL) fluid was elevated in patient 1 suggesting the BAL concentration of SP-A may be a clue to the diagnosis of this form of PAP.

CONCLUSION

The accumulation of surfactant protein A in two siblings with an infantile form of pulmonary alveolar proteinosis could be a clue to the diagnosis.

摘要

未标注

我们研究了两名兄弟姐妹（一兄一妹）肺泡蛋白沉积症（PAP）的组织学和分子特征，他们出生时未出现呼吸窘迫，但在婴儿期均出现了症状。肺标本的组织学分析显示，两名患者的表面活性物质蛋白染色均为阳性。聚合酶链反应显示肺标本中有表面活性物质蛋白B（SP-B）信使核糖核酸的表达。未观察到先天性PAP特征性的SP-B缺陷。患者1支气管肺泡灌洗（BAL）液中表面活性物质蛋白A（SP-A）的浓度升高，提示SP-A的BAL浓度可能是这种形式PAP诊断的线索。