Nevens F, Staessen D, Sciot R, Van Damme B, Desmet V, Fevery J, De Groote J, Van Steenbergen W
Department of Internal Medicine, University Hospital Gasthuisberg, Catholic University of Leuven, Belgium.
Gastroenterology. 1994 Feb;106(2):459-63. doi: 10.1016/0016-5085(94)90605-x.
BACKGROUND/AIMS: Incomplete septal cirrhosis (ISC) is a form of macronodular cirrhosis characterized by slender, incomplete septa that demarcate inconspicuous nodules. Its clinical features have not been investigated in a large series. The aims of this study were to review the clinical symptoms and evolution of ISC in 42 patients.
Forty-two patients with at least one liver biopsy strongly suggestive of ISC were selected for the study covering a period between 1968 and 1987. Data for these patients were compared with the evolution of 49 patients with classical macronodular cirrhosis after chronic active hepatitis type B or C.
Possible etiological factors for ISC were alcohol abuse, arsenic treatment, and hepatitis B infection. In three cases, a genetic factor could not be excluded. Patients with ISC had significantly lower serum concentrations of transaminases and bilirubin at diagnosis. Compared with macronodular cirrhosis, bleeding varices were more frequent (57% vs. 22%) in ISC. Ten-year survivals in the ISC and the macronodular cirrhosis groups were 54% and 57%, respectively.
ISC represents a relatively stable burnt-out form of macronodular cirrhosis with an unusually high incidence of variceal bleeding. This could be explained by a superimposed insufficiency of the portal vascular supply.
背景/目的:不完全间隔性肝硬化(ISC)是大结节性肝硬化的一种形式,其特征为纤细、不完全的间隔,分隔不明显的结节。尚未对其临床特征进行大样本研究。本研究的目的是回顾42例ISC患者的临床症状及病情演变。
选取42例经至少一次肝活检强烈提示为ISC的患者进行研究,研究时间跨度为1968年至1987年。将这些患者的数据与49例慢性乙型或丙型活动性肝炎后经典大结节性肝硬化患者的病情演变进行比较。
ISC可能的病因包括酒精滥用、砷剂治疗和乙型肝炎感染。3例患者不能排除遗传因素。ISC患者诊断时血清转氨酶和胆红素浓度显著较低。与大结节性肝硬化相比,ISC患者静脉曲张出血更为常见(57%对22%)。ISC组和大结节性肝硬化组的10年生存率分别为54%和57%。
ISC是大结节性肝硬化一种相对稳定的终末期形式,静脉曲张出血发生率异常高。这可能是由于门静脉血管供应叠加不足所致。