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[芬兰型先天性肾病综合征。2例病例评论]

[The Finnish-type congenital nephrotic syndrome. Comments on 2 cases].

作者信息

Sabău I, Potencz E

出版信息

Rev Pediatr Obstet Ginecol Pediatr. 1989 Jan-Mar;38(1):79-86.

PMID:2505364
Abstract

The authors described the clinical, biologic and histologic particularities of two cases of congenital nephrotic syndrome, Finnish type. Clinically, the hydropic syndrome was represented by anasarca: biologically by marked proteinuria, hypoproteinemia with dysproteinemia and hyperlipemia with dyslipemia. Histologically both cases presented microcystic dilatation of the proximal tubuli contorti. The evolution was unfavorable and ended in death.

摘要

作者描述了两例芬兰型先天性肾病综合征的临床、生物学和组织学特征。临床上,水肿综合征表现为全身水肿;生物学上表现为显著蛋白尿、伴有蛋白异常血症的低蛋白血症以及伴有血脂异常的高脂血症。组织学上,两例均呈现近端曲管的微囊性扩张。病情进展不利,最终导致死亡。

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