Congenital Nephrotic Syndrome: Report of an Infant with Diffuse Mesangial Sclerosis

先天性腎病症候群：瀰漫性腎間質硬化症之一案例報告

張瑞文(Jei-Wen Chang) ； 楊令瑀(Ling-Yu Yang)

《Acta Paediatrica Taiwanica》 44卷5期 (2003/10) Pp. 300-302

https://doi.org/10.7097/APT.200310.0300

摘要

本文報告因瀰漫性腎間質硬化症引起先天性腎病症候群之一案例。女嬰出生體重為2600克，35週出生，出生後出現早發性全身水腫、嚴重蛋白尿、寡尿、低白蛋白及快速進展至腎衰竭。即使已用強效性抗生素病童仍因敗血性休克，於七天大時死亡，死後腎臟病理切片檢查，顯示為瀰漫性腎間質硬化症。此案例顯示早產、低出生體重、早發性腎病症候群不僅見於芬蘭型腎病症候群，亦可發生於瀰漫性腎間質硬化症。除此之外，應早期使用足以含概院內菌株之廣效消炎藥以防嚴重感染。

關鍵字

先天性腎病症候群 ； 芬蘭型腎病症候群 ； 瀰漫性腎間質硬化症

並列摘要

A case of congenital nephrotic syndrome (CNS) caused by diffuse mesangial sclerosis (DMS) is presented. A female baby weighting 2,680 gm was delivered at 35 weeks’ gestation. She had early onset of general ized edema, heavy proteinuria, oligouria, uncorrectable hypoalbuminemia, and rapid progression to renal failure. Even after being treated with strong antibiotics (teicoplanin and ceftazidine), the infant died of septic shock with Enterobacter cloacea, only sensitive to imipenem, at the age of 7 days. The necropsy showed diffuse mesangial sclerosis. This case demonstrates that prematurity, low birth body weight and early onset of symptoms are not pathognomonic of the congenital nephrotic syndrome of the Finnish type (CNF). It can also occur in DMS. Besides, empirical antibiotics should be started promptly and should cover the major hospital strains of bacteria if the patient is not well.

並列關鍵字

congenital nephrotic syndrome ； congenital nephrotic syndrome of the Finnish type ； diffuse mesangial sclerosis