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一女性患儿,3岁8个月。平素健康,门诊检查发现肝脾显著肿大,无黄疸及搔痒史,谷丙及谷草转氨酶、硷性磷酸酶明显增高,血清胆红素、总蛋白和凝血象正常,HBsAg和抗核抗体阴性。肝穿刺发现为原因不明之肝硬化静止期。门诊曾用肾上腺皮质激素治疗。1个月后住院检查发现,巩膜不黄,肝于肋下8cm,质硬光滑,脾肋下7cm,腹胀,可见腹壁静脉曲胀,但无腹水。面部有蜘蛛痣。谷丙及谷草转氨酶,硷性磷酸酶分别为344、570及416IU/L,空腹血胆酸盐浓度为375μg％(正常<60),餐后增至
A female child, 3 years and 8 months. Usually healthy, outpatient examination found that liver and spleen were significantly enlarged, no history of jaundice and itching, valleys and aspartate aminotransferase, alkaline phosphatase was significantly elevated serum bilirubin, total protein and coagulation as normal, HBsAg and anti-nuclear antibody negative. Liver puncture found for unknown causes of cirrhosis quiescence. Outpatient adrenal cortical hormone treatment. 1 month after the hospital examination found that the sclera is not yellow, liver in the ribs 8cm, hard and smooth, spleen ribs 7cm, abdominal distension, abdominal paralysis visible veins, but no ascites. Facial spider mole. Alanine and aspartate aminotransferase, alkaline phosphatase were 344,570 and 416IU / L, fasting blood bile acid salt concentration 375μg% (normal <60), postprandial increase