兄弟患原发性胆汁性肝硬化
Primary biliary cirrhosis in brothers.
作者信息
Bown R, Clark M L, Doniach D
出版信息
Postgrad Med J. 1975 Feb;51(592):110-5. doi: 10.1136/pgmj.51.592.110.
Abstract
This is the first report of two brothers who demonstrated the classical clinical course, histology, biochemistry and auto-antibodies of primary biliary cirrhosis. Both also exhibited an associated keratoconjunctivitis sicca and, in one, renal tubular acidosis resulted in severe systemic acidosis after lactulose therapy and with a subsequent intraperitoneal variceal rupture. Screening of the relatives recalled a high incidence of ‘auto-immune’ disease and auto-antibodies.
摘要
本文首次报道了两兄弟患有原发性胆汁性肝硬化，他们表现出了该病典型的临床病程、组织学、生物化学特征及自身抗体。两人均伴有干燥性角结膜炎，其中一人在接受乳果糖治疗后出现肾小管酸中毒，进而导致严重的全身酸中毒，随后发生腹腔静脉曲张破裂。对其亲属进行筛查后发现，“自身免疫性”疾病和自身抗体的发病率很高。
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Primary biliary cirrhosis and chronic active hepatitis in two sisters.两姐妹患原发性胆汁性肝硬化和慢性活动性肝炎。
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