关键词: Bile acid Cholecardia syndrome Complication Gallbladder and biliary diseases Pathogenesis Ursodeoxycholic acid

Mesh : Female Pregnancy Humans Alagille Syndrome / complications diagnosis genetics Cholestasis, Intrahepatic / complications drug therapy Ursodeoxycholic Acid / therapeutic use Pregnancy Complications

来  源:   DOI:10.1016/j.phrs.2023.107006

Abstract:
Gallbladder and biliary diseases (GBDs) are one of the most common digestive diseases. The connections between GBDs and several organs other than the liver have gradually surfaced accompanied by the changes in people\'s diet structure and the continuous improvement of medical diagnosis technology. Among them, cholecardia syndrome that takes the heart as the important target of GBDs complications has been paid close attention. However, there are still no systematic report about its corresponding clinical manifestations and pathogenesis. This review summarized recent reported types of cholecardia syndrome and found that arrhythmia, myocardial injury, acute coronary syndrome and heart failure are common in the general population. Besides, the clinical diagnosis rate of intrahepatic cholestasis of pregnancy (ICP) and Alagille syndrome associated with gene mutation is also increasing. Accordingly, the underlying pathogenesis including abnormal secretion of bile acid, gene mutation, translocation and deletion (JAG1, NOTCH2, ABCG5/8 and CYP7A1), nerve reflex and autonomic neuropathy were further revealed. Finally, the potential treatment measures and clinical medication represented by ursodeoxycholic acid were summarized to provide assistance for clinical diagnosis and treatment.
摘要:
胆囊和胆道疾病(GBD)是最常见的消化系统疾病之一。随着人们饮食结构的改变和医学诊断技术的不断进步,GBD与肝脏以外的其他器官之间的联系逐渐浮出水面。其中,以心脏为重要靶点的胆囊疾病综合征引起了人们的密切关注。然而,目前还没有关于其相应的临床表现和发病机制的系统报道。这篇综述总结了最近报道的胆囊综合征的类型,发现心律失常,心肌损伤,急性冠脉综合征和心力衰竭在普通人群中很常见。此外,妊娠期肝内胆汁淤积症(ICP)和Alagille综合征相关基因突变的临床诊断率也在不断提高。因此,潜在的发病机制包括胆汁酸分泌异常,基因突变,易位和缺失(JAG1,NOTCH2,ABCG5/8和CYP7A1),神经反射和自主神经病变进一步显示。最后,总结了以熊去氧胆酸为代表的潜在治疗措施和临床用药,为临床诊断和治疗提供帮助。
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