結節性硬化症合併腎臟血管肌脂肪瘤與腎衰竭－病例報告

Tuberous Sclerosis with Renal Angiomyolipoma and Renal Failure-A Case Report

杜興孟(Hsing-Mong Tu) ； 曾廣基(Kwong-Kee Tsang) ； 范成得(Cherng-Der Fan) ； 陳學文(Shwe-Winn Chen) ； 曾建平(Chirn-Ping Tsang)

《Journal of Medical Sciences》 14卷1期 (1993/08) Pp. 51-58

摘要

一名五十三歲婦女因發燒噁心嘔吐及腹部疼痛而住院，經檢查病人有明顯的結節性硬化症的皮膚病變，另外在頭骨及指骨上出現結節性硬化症的典型病變，頭部電腦斷層攝影亦出現皮質結節及多處鈣化現象，但是病人過去並無癲癇病史，亦無智力障礙；另外在病人腹部發現有巨大的腫塊，經診斷為腎臟血管肌脂肪瘤，同時亦出現慢性腎衰竭及嚴重貧血，但是病人並無血尿或大便潛血反應，故病人的貧血可因腎衰竭所引起，結節性硬化症患者一般均以腦神經病變而就醫，在過去的病例報告上以腎衰竭為表徵的病例較少。

關鍵字

結節性硬化症 ； 血管肌脂肪瘤

並列摘要

A 53-year-old female was admitted on account of fever and abdominal pain. Prominent skin lesion of tuberous sclerosis was noted, but neither seizure attack nor mental retardation occured before. Huge abdominal mass was found, and renal angiomyolipoma was diagnosed. Chronic renal failure with severe anemia was found too. Bony lesion over her skull and phalangeal bones, and the finding of brain CT also showed typical abnormality of tuberous sclerosis. Although tuberous sclerosis with renal angiomyolipoma was reported frequently but associated renal failure was rarely reported. Nevertheless, the incidence of complicating renal failure could be increased in the near future, because longer survival might be achieved by the advanced medical care.

並列關鍵字

Tuberous sclerosis ； angiomyolipoma