Autoimmune hepatitis in genetic syndromes:A literature review

查看全文 作　　者：Anna Paola [1]Capra;Emanuele [2]Chiara;Silvana [1]Briuglia

机构地区：[1]Department of Biomedical,Dental,Morphological and Functional Imaging Sciences,University of Messina,Messina 98100,Italy;[2]Department of Clinical Pathology,University of Messina,Messina 98100,Italy

出　　处：《World Journal of Hepatology》索引2021年第13卷第10期,共13页

摘　　要：Genetic syndromes represent relevant and rare diseases.These conditions include a large amount of epidemiological,pathogenetic and clinical features.However,a systematic approach to genetic syndromes is often prevented by the rareness of these diseases.So,although clinical features are usually precisely defined,nowadays more uncommon associations between genetic syndromes and internal medicine related diseases have been insufficiently studied.Autoimmune hepatitis(AIH)is a chronic liver disease caused by loss of tolerance to hepatocyte-specific auto-antigens.Conversely,a better knowledge about specific genetic syndromes in which AIH is more frequent could be important in the clinical management of patients,both for an early diagnosis and for a prompt therapy.Furthermore,a systematic approach could explain if onset,clinical course,and response to treatment of AIH are typical for specific genetic syndromes.We took in consideration all the scientific articles reported in PubMed in the last 10 years,from 2010 to 2020.The purpose of this review is to explore the prevalence of AIH in genetic syndrome,but also to suggest new classification,that could be useful for pathogenetic hypothesis and clinical approach to genetic syndrome.From the 139 publications selected using keywords“autoimmune hepatitis”and“genetic syndrome”,30 papers(21.6%)respected the chosen inclusion criteria,reporting the association between AIH in patients with a genetic syndrome.We have collected in all 47 patients with AIH and genetic syndrome,and with median age of 12.6-year-old.We suggest that when a patient presents a clinical picture of cryptogenic chronic hepatitis,that is unexplained,it is useful to explore differential diagnosis of AIH associated with genetic syndrome.Given the clinical relevance of this topic,further reports are needed to demonstrate our hypothesis and collect new evidence in this field. 关键词：AUTOIMMUNITY HEPATITIS Gene SYNDROME Liver Disease IMMUNITY

相关文献

参考文献（51）
引证文献（4）
耦合文献（43）

参考文献(51)

引证文献(4)

耦合文献(43)

互联网出版许可证新出网证(渝)字10号全国400电话 - 免长途话费

Autoimmune hepatitis in genetic syndromes:A literature review

参考文献(51)

引证文献(4)

耦合文献(43)

用户提示