Schmeisser W, Eggstein M, Maulbetsch R, Dölle W
Schweiz Med Wochenschr. 1977 Nov 12;107(45):1613-20.
The case of a 23-year-old male patient with benign intrahepatic recurrent cholestasis of Summerskill-Tygstrup and Walshe type is presented. The patient had suffered 9 attacks up to 1974. The clinical and histological data of this patient were compared with those in the 65 cases described previously. The most characteristic signs were that the disease began before puberty and the attacks of jaundice with direct hyper bilirubmemia were induced by infections. Histology obtained in acute phase of the disease revealed intrahepatic cholestasis, and there were signs of a non-specific mesenchymal reaction of the liver tissue while the patients were asymptomatic. The serum activity of alkaline phosphatase was increased, gamma-glutamyltranspeptidase and transaminases being normal or close to normal. The pattern of the biochemical findings was characteristic and the patient was recognized among 19,035 other patients hospitalized from 1972-1975 by means of a special computer program. This program may be helpful in detecting patients with this disease from computerized data of hospitalized patients and avoiding unnecessary surgical intervention.
本文报告了一例23岁男性患者，患有Summerskill - Tygstrup和Walshe型良性肝内复发性胆汁淤积症。截至1974年，该患者已发作9次。将该患者的临床和组织学数据与之前描述的65例病例进行了比较。最具特征性的体征是疾病在青春期前开始，感染可诱发黄疸发作并伴有直接胆红素血症。疾病急性期的组织学检查显示肝内胆汁淤积，在患者无症状时肝组织有非特异性间叶反应的迹象。碱性磷酸酶的血清活性升高，γ-谷氨酰转肽酶和转氨酶正常或接近正常。生化检查结果的模式具有特征性，通过一个特殊的计算机程序，该患者在1972年至1975年住院的19035名其他患者中被识别出来。该程序可能有助于从住院患者的计算机数据中检测出患有这种疾病的患者，并避免不必要的手术干预。
