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Guideline on the management of cholestasis liver diseases(2021)
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摘要: 2015年中华医学会肝病学分会和中华医学会消化病学分会制定了我国第一个胆汁淤积性肝病的专家共识。近年来胆汁淤积性肝病的临床研究提供了新的研究数据和资料。为此, 中华医学会肝病学分会自身免疫性肝病学组组织专家组对近年来的文献证据进行了评估, 制定了本指南。本指南共有胆汁淤积性肝病临床诊治推荐意见22条。本指南的目的是为临床胆汁淤积性肝病诊治提供参考和指导。Abstract: In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued the consensus on the diagnosis and management of cholestatic liver diseases. In the past years, more data have emerged from clinical practice. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 22 recommendations on clinical practice of cholestatic liver diseases. The guidelines aim to provide a working reference for the management of cholestatic liver diseases.
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Key words:
- Cholestatic Liver Disease /
- Diagnosis /
- Therapeutics /
- Guideline
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表 1 推荐意见的证据等级和推荐强度等级
级别 详细说明 证据质量 高(A) 进一步研究不可能改变对该疗效评估结果的可信度 中(B) 进一步研究有可能影响该疗效评估结果的可信度, 且可能改变该评估结果 低或非常低(C) 进一步研究很有可能影响该疗效评估结果的可信度, 且很可能改变该评估结果 推荐强度 强(1) 明确显示干预措施利大于弊或弊大于利 弱(2) 利弊不确定或无论质量高低的证据均显示利弊相当 
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表 2 胆汁淤积性疾病累及基因
胆汁淤积性疾病 累及基因 Alagille综合征 JAG1, NOTCH2 α1抗胰蛋白酶缺乏症 SERPINA1 α-甲酰基-辅酶A消旋酶缺乏症 AMACR 关节挛缩-肾功能不全- 胆汁淤积综合征 VIPAS39, VPS33B 常染色体隐性多囊肾病 PKHD1 胆汁酸螯合障碍 SLC27A5 胆汁酸再吸收障碍 SLC10A1, SLC10A2 胆汁酸受体缺乏 GPBAR1 胆汁酸合成障碍 CYP7A1 胆道闭锁 SLC51B 脑腱性黄瘤症 CYP27A1 胆固醇酯储积病 LIPA 瓜氨酸血症 SLC25A13 先天性胆汁酸合成障碍 ACOX2, AKR1D1, AMACR, CYP7B1, HSD3B7 囊性纤维化 CFTR D-双功能蛋白缺乏 HSD17B Dubin-Johnson综合征 ABCC2 肝外胆汁淤积症 SLC51B 家族性高胆烷血症 BAAT, TJP2 Lucey-Driscoll综合征 UGT1A1 Crigler-Najjar综合征 UGT1A1 肾小管性范可尼综合征3型 EHHADH 胆囊疾病 ABCB4, ABCG8 遗传性果糖不耐症 ALDOB 鱼鳞病、白细胞空泡、脱发和硬化性胆管炎 CLDN1 妊娠期肝内胆汁淤积症 ABCB4, ATP8B1 Joubert综合征 CC2D2A, MKS1, TMEM216, NPHP1 脂质贮存障碍 SCP2 一过性婴儿肝衰竭 TRMU Meckel综合征 CC2D2A, MKS1, NPHP3, TMEM216 线粒体DNA缺失综合征 DGUOK, POLG, MPV17 新生儿硬化性胆管炎 DCDC2 肾消耗病 INVS, NPHP1, NPHP3, NPHP4 Niemann-Pick病 NPC1, NPC2, SMPD1 北美印第安儿童肝硬化 UTP4 过氧化小体病 PEX1, PEX10, PEX11B, PEX12, PEX13, PEX14, PEX16, PEX19, PEX2, PEX26, PEX3, PEX5, PEX6, PEX7 家族性肝内胆汁淤积症 ABCB11, ABCB4, SLC51A, TJP2, ATP8B1, NR1H4, MYO5B 肾囊肿-糖尿病综合征 HNF1B 肾-肝-胰发育不良1型 PHP3 谷固醇血症 ABCG5, ABCG8 Smith-Le mli-Opitz综合征 DHCR7 醛糖转移酶缺乏 TALDO1 1型酪氨酸血症 FAH
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