Peters M G, Hoffnagle J H, McGarvey C, Fox I, Gregg R E, Jones E A
Liver Diseases Section, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Maryland.
J Clin Gastroenterol. 1989 Dec;11(6):694-7.
We report a patient with advanced primary biliary cirrhosis associated with Sjögren's syndrome, xanthelasma, and extensive, painful xanthomata involving cutaneous lipid deposits on her face, abdomen, hands, and buttocks and extensor surfaces over many joints. Despite conventional dietary and drug therapy, these lesions progressed rapidly over 3 years. There was symptomatic improvement of the xanthomata, but no objective amelioration of the xanthomatosis with the use of plasmapheresis over an 18-month period. Liver transplantation was undertaken for decompensated chronic liver disease and poor quality of life due to complications of xanthomatosis. Twelve months after transplantation, all xanthomata and xanthelasma and symptoms attributable to xanthomata had disappeared. Liver transplantation is a drastic but successful remedy for complications of abnormal lipid metabolism associated with primary biliary cirrhosis.
我们报告了一名患有晚期原发性胆汁性肝硬化的患者，该患者伴有干燥综合征、睑黄瘤，以及广泛且疼痛的黄色瘤，这些黄色瘤累及她面部、腹部、手部、臀部的皮肤脂质沉积处以及多个关节的伸侧表面。尽管采用了传统的饮食和药物治疗，这些病变在3年中仍迅速进展。黄色瘤有症状改善，但在18个月期间使用血浆置换术对黄瘤病并无客观改善。因失代偿性慢性肝病以及黄瘤病并发症导致生活质量差，遂进行了肝移植。移植后12个月，所有黄色瘤、睑黄瘤以及黄色瘤所致的症状均消失。肝移植是治疗与原发性胆汁性肝硬化相关的异常脂质代谢并发症的一种激进但成功的疗法。
