Pereira E, Kannebley J, Carvalho M, Mendes R, Marques M, Franco M
Departmento de Patologia, Faculdade de Medicina, Universidade Estadual Paulista-UNESP, Botucatu, SP.
Arq Gastroenterol. 1994 Apr-Jun;31(2):63-8.
Primary intrahepatic lithiasis is an entity defined by intrahepatic stones exclusively located in the IH ducts above the emergence of the common bile duct. The disease is classified in two types: Eastern type (stones formed primarily in intrahepatic ducts; frequent in Japan) and Western type (stones formed in the extrahepatic bile system, usually in gallbladder, which migrate up to the intra-hepatic ducts). The mechanisms of lithogenesis in the entity are as yet not fully understood; multiple factors seem to operate synergistically: anatomical changes of the intrahepatic ducts, metabolic disorders, infections, idiopathic alteration. All these factors may facilitate biliary stasis leading ultimately to stone formation. We report on a case of and review the literature on primary intrahepatic lithiasis, which is a rare occurrence in the West and a disease of difficult surgical approach and high mortality.
原发性肝内胆管结石是一种仅位于肝内胆管（在胆总管开口以上）的结石所定义的疾病。该疾病分为两种类型：东方型（结石主要在肝内胆管形成；在日本常见）和西方型（结石在肝外胆管系统形成，通常在胆囊，然后向上迁移至肝内胆管）。该疾病的结石形成机制尚未完全明确；多种因素似乎协同作用：肝内胆管的解剖结构改变、代谢紊乱、感染、特发性改变。所有这些因素都可能促使胆汁淤积，最终导致结石形成。我们报告一例原发性肝内胆管结石病例并复习相关文献，该病在西方较为罕见，手术难度大且死亡率高。
