Sisto A, Van Doesburg N, Deal C, Brochu P, Leung T K, Roy C C
Department of Pediatrics and Pathology, Hôpital Ste.-Justine, Montreal, Canada.
J Pediatr Gastroenterol Nutr. 1989 Jul;9(1):126-30.
The name of a 13-year-old girl diagnosed as having idiopathic dilated cardiomyopathy was removed from the cardiac transplant list because a hepatitis-like picture developed that coincided with evidence of decompensation of her cardiac function. On admission, there was only modest evidence of cytolysis (ALT level, 115 U/L) and of cholestasis (bilirubin level, 3.0 mg/dl), but there was severe prolongation of her prothrombin time (28 s). This was followed by elevation of both her transaminases and bilirubin levels. A liver biopsy sample showed extensive necrosis involving both the central and midlobular zones, while periportal areas revealed dilated sinusoids and steatotic multinucleated hepatocytes. A brief improvement of both her liver and her heart was followed by rapid deterioration of the functions of both. Attention is drawn to the relationship between reduced cardiac output and hepatic dysfunction secondary to massive cytolysis.
一名被诊断患有特发性扩张型心肌病的13岁女孩从心脏移植名单中被移除,因为出现了类似肝炎的症状,同时伴有心功能失代偿的证据。入院时,仅有轻微的细胞溶解证据(谷丙转氨酶水平,115 U/L)和胆汁淤积证据(胆红素水平,3.0 mg/dl),但她的凝血酶原时间严重延长(28秒)。随后,她的转氨酶和胆红素水平均升高。肝活检样本显示广泛坏死,累及中央区和小叶中区,而汇管区可见扩张的血窦和脂肪变性的多核肝细胞。她的肝脏和心脏功能短暂改善后,两者功能迅速恶化。需注意心输出量减少与大量细胞溶解继发的肝功能障碍之间的关系。