Herman T E, Nogee L M, McAlister W H, Dehner L P
Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis Children's Hospital Department of Radiology, Missouri 63110.
Pediatr Radiol. 1993;23(5):373-5. doi: 10.1007/BF02011963.
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.
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。