Popović-Rolović M, Kostić M, Sindjić M, Jovanović O, Peco-Antić A, Kruscić D
University Children's Hospital, Belgrade, Yugoslavia, Serbia.
Pediatr Nephrol. 1993 Aug;7(4):396-400. doi: 10.1007/BF00857550.
We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenal involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.
我们报告了4例患有一种独特肝肾疾病的患者的临床和形态学特征，并回顾了文献中另外5例类似患者的情况。主要临床特征为胆汁淤积性肝病早期发病，以及进行性肾小管间质性肾炎，导致幼儿期肾衰竭死亡。肝脏组织学显示结构紊乱，有结节和腺泡形成、门脉纤维化和胆管增生。肾脏的组织学异常为严重的间质纤维化、肾小管萎缩和扩张，而缺乏肾单位肾痨的典型特征。这些临床和形态学特征使我们的患者有别于大多数已描述的患有肾单位肾痨和先天性肝纤维化或任何其他伴有肝肾受累的已知综合征的患者。我们认为胆汁淤积性肝病与进行性肾小管间质性肾炎的关联代表一种新的综合征。
