胱氨酸病时肾脏的病理学表现。

The pathology of the kidney in cystinosis.

作者信息

Spear G S

出版信息

Birth Defects Orig Artic Ser. 1974;10(4):87-8.

PMID:4620308

Abstract

The etiology and pathogenesis of the variable glomerular, tubular and interstitial functional and anatomic abnormalities in the kidney in cystinosis are not known. The doctrine that the abnormalities are consequent to accumulation of crystals of cystine must be questioned. Not previously appreciated, the podocyte is notably affected in cystinosis.

摘要

胱氨酸病患者肾脏中肾小球、肾小管及间质出现的功能性和解剖学异常变化多样，其病因和发病机制尚不清楚。认为这些异常是胱氨酸晶体蓄积所致的学说值得质疑。此前未被充分认识到的是，胱氨酸病中足细胞受到显著影响。