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肺泡蛋白沉积症:新生儿慢性呼吸窘迫的罕见病因。

Pulmonary alveolar proteinosis: an uncommon cause of chronic neonatal respiratory distress.

作者信息

Coleman M, Dehner L P, Sibley R K, Burke B A, L'Heureux P R, Thompson T R

出版信息

Am Rev Respir Dis. 1980 Mar;121(3):583-6. doi: 10.1164/arrd.1980.121.3.583.

DOI:10.1164/arrd.1980.121.3.583
PMID:7416585
Abstract

A full-term newborn infant had the onset of respiratory distress immediately after birth. She required supplemental oxygen from birth, and had pulmonary alveolar proteinosis (PAP) documented by open lung biopsies at 6 and at 12 weeks of age. Light and electron microscopy showed dense, PAS-positive granular, homogeneous material within the alveolar spaces, diagnostic of PAP. No Pneumocystic carinii organisms were demonstrated on silver staining, and bacterial and viral cultures were negative. The infant died of progressive respiratory insufficiency at 1 yr or age. Pulmonary alveolar proteinosis should considered a rare cause of chronic respiratory distress in the newborn infant.

摘要

一名足月儿出生后立即出现呼吸窘迫。自出生起她就需要吸氧,并在6周和12周龄时通过开胸肺活检确诊为肺泡蛋白沉积症(PAP)。光镜和电镜检查显示肺泡腔内有致密的、PAS阳性的颗粒状均匀物质,可诊断为PAP。银染色未发现卡氏肺孢子虫,细菌和病毒培养均为阴性。该婴儿1岁时死于进行性呼吸功能不全。肺泡蛋白沉积症应被视为新生儿慢性呼吸窘迫的罕见原因。

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