Grüne S, Müllhaupt B, Moradpour D, Münch R, Siegenthaler-Zuber G, Siegenthaler W
Departement für Innere Medizin, Universitätsspital Zürich.
Dtsch Med Wochenschr. 1991 Jul 12;116(28-29):1095-100. doi: 10.1055/s-2008-1063722.
In a 40-year-old patient unexplained recurrent attacks of epigastric colic with transient cholestatic icterus occurred over a 9-year period. When the patient was again hospitalised because of progressive pain-free icterus associated with mild pruritus (alkaline phosphatase 900 U/l, direct bilirubin 305 mumol/l, GOT 187 U/l, GPT 103 U/l) sonography revealed liver enlargement to 17 cm, extended intrahepatic bile ducts and an echodense area of about 1 cm size in the region of the bifurcation of the common hepatic duct. Fine-needle puncture did not yield clear cytological findings. Endoscopic retrograde cholangiopancreatography pointed to sclerosing cholangitis. This diagnosis was confirmed by liver punch biopsy. Since the patient did not agree to a liver transplantation, he was treated with 450 mg ursodeoxycholic acid twice daily, resulting in marked reduction of the liver parameters until severe cholangiosepsis and acute renal failure occurred about 4 months later. The septic condition and its complications could not be managed despite thorough intensive-care measures so that a liver transplant had to be performed after all. Histology of the explantate revealed a cholangiocarcinoma in the region of the bifurcation of the common hepatic duct. At first the patient's condition improved markedly but one and half months later the transplant was rejected and the patient died.
一名40岁患者在9年期间反复出现原因不明的上腹部绞痛并伴有短暂性胆汁淤积性黄疸。当患者因进行性无痛性黄疸伴轻度瘙痒再次住院时（碱性磷酸酶900 U/l，直接胆红素305 μmol/l，谷草转氨酶187 U/l，谷丙转氨酶103 U/l），超声检查显示肝脏增大至17 cm，肝内胆管扩张，在肝总管分叉处有一个大小约1 cm的回声密集区。细针穿刺未获得明确的细胞学结果。内镜逆行胰胆管造影提示硬化性胆管炎。肝穿刺活检证实了这一诊断。由于患者不同意进行肝移植，遂给予熊去氧胆酸450 mg，每日2次治疗，肝脏参数显著降低，直至约4个月后发生严重胆管败血症和急性肾衰竭。尽管采取了全面的重症监护措施，败血症及其并发症仍无法控制，最终不得不进行肝移植。切除肝脏的组织学检查显示肝总管分叉处有胆管癌。起初患者病情明显改善，但1个半月后移植肝脏被排斥，患者死亡。
