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- W2437007167 abstract "进行性家族性肝内胆汁淤积症3型(progressive familial intrahepatic cholestasis type 3,PFIC3)是一种常染色体隐性遗传性疾病,由编码多药耐药蛋白3(multidrug resistance protein 3,MDR3)的AB CB4(ATP-bindingcassette,sub-family B,member 4)基因突变引起~([1]).PFIC3临床上可表现为反复瘙痒、黄疸、白陶土样便、肝脾肿大及胃肠道出血等,常在成年前进展为肝硬化和肝衰竭~([1-3]).PFIC3是进行性家族性肝内胆汁淤积症的一种亚型,与进行性家族性肝内胆汁淤积症1型和2型的主要区别在于血清γ-谷氨酰转肽酶(GGT)升高及肝组织病理表现为明显的小胆管增生~([4])。" @default.
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- W2437007167 date "2010-03-01" @default.
- W2437007167 modified "2023-09-24" @default.
- W2437007167 title "Progressive familial intrahepatic cholestasis type 3" @default.
- W2437007167 doi "https://doi.org/10.3760/cma.j.issn.1007-3418.2010.03.023" @default.
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