Henrot B, Vermylen C, Caus D, Cosyns J P, Gosseye S, Verellen G, Cornu G
Department of Paediatrics, St. Luc University Hospital, Brussels, Belgium.
Eur J Pediatr. 1990 Feb;149(5):365-7. doi: 10.1007/BF02171570.
We describe an infant with renal and liver disease who died at the age of 10 months from chronic renal failure. Hepatosplenomegaly was present along with biochemical findings of cholestasis and cytolysis. The diagnosis of tubulointerstitial nephropathy with cortical microcysts associated with hepatic fibrosis was based upon laboratory, radiological, and histological data. This rarely described disorder is characterized by an early clinical onset and unfavourable progression to end-stage renal failure before the age of 2.
我们描述了一名患有肾脏和肝脏疾病的婴儿，该婴儿在10个月大时死于慢性肾衰竭。存在肝脾肿大以及胆汁淤积和细胞溶解的生化检查结果。基于实验室、放射学和组织学数据，诊断为伴有肝纤维化的肾小管间质性肾病合并皮质微囊肿。这种很少被描述的疾病的特点是临床发病早，且在2岁前进展为终末期肾衰竭的预后不佳。
