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表面活性物质蛋白B缺乏症:影像学表现。

Surfactant protein B deficiency: radiographic manifestations.

作者信息

Herman T E, Nogee L M, McAlister W H, Dehner L P

机构信息

Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis Children's Hospital Department of Radiology, Missouri 63110.

出版信息

Pediatr Radiol. 1993;23(5):373-5. doi: 10.1007/BF02011963.

DOI:10.1007/BF02011963
PMID:8233692
Abstract

Surfactant is a complex structure primarily composed of phospholipids, but containing essential proteins as well. Congenital deficiency of Surfactant Protein-B (SPB) has recently been documented for the first time in two siblings. The pathologic findings in these infants was that of congenital pulmonary alveolar proteinosis and the radiographic manifestations were strikingly similar to hyaline membrane disease.

摘要

表面活性剂是一种主要由磷脂组成的复杂结构,但也含有必需蛋白质。最近首次在两名兄弟姐妹中发现了先天性表面活性蛋白B(SPB)缺乏症。这些婴儿的病理表现为先天性肺泡蛋白沉积症,其影像学表现与透明膜病极为相似。

相似文献

1
Surfactant protein B deficiency: radiographic manifestations.表面活性物质蛋白B缺乏症:影像学表现。
Pediatr Radiol. 1993;23(5):373-5. doi: 10.1007/BF02011963.
2
Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis.简短报告:先天性肺泡蛋白沉积症中肺表面活性物质蛋白B缺乏
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4
Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency.与遗传性表面活性物质蛋白B缺乏相关的先天性肺泡蛋白沉积症综合征的分子和表型变异性。
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Ultrastructure of lung in surfactant protein B deficiency.表面活性蛋白B缺乏时肺的超微结构
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[Neonatal pulmonary alveolar proteinosis and SP-B deficiency: pulmonary surfactant at the time of molecular pathology].
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Surfactant protein-A concentration in bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis.肺泡蛋白沉积症患者支气管肺泡灌洗液中表面活性蛋白A的浓度
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Surfactant Protein B Deficiency Caused by Homozygous C248X Mutation-A Case Report and Review of the Literature.纯合子C248X突变导致的表面活性蛋白B缺乏症——病例报告及文献复习
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Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis.简短报告:先天性肺泡蛋白沉积症中肺表面活性物质蛋白B缺乏
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