Paediatric autoimmune liver diseases: A descriptive study of patients from Saudi Arabia

被引:3
作者
Saadah, Omar I. [1 ,4 ]
Khayat, Ammar [2 ,4 ]
Alsufyani, Hadeel A. [3 ]
Bin-Taleb, Yagoub [4 ]
机构
[1] King Abdulaziz Univ, Fac Med, Dept Paediat, Jeddah, Saudi Arabia
[2] Umm Al Qura Univ, Coll Med, Dept Paediat, Mecca, Saudi Arabia
[3] King Abdulaziz Univ, Fac Med, Dept Med Physiol, Jeddah, Saudi Arabia
[4] King Abdulaziz Univ Hosp, Dept Paediat, Paediat Gastroenterol Unit, Jeddah, Saudi Arabia
关键词
Autoimmune liver disease; Autoimmune hepatitis; Saudi Arabia; Autoimmune sclerosing cholangitis; children; PRIMARY SCLEROSING CHOLANGITIS; HEPATITIS; CHILDREN; CHILDHOOD;
D O I
10.1016/j.ajg.2021.05.009
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Background and study aims: Autoimmune liver diseases (ALDs) are a clinico-pathologic spectrum of disorders that share some similarities. They are formally classified as autoimmune hepatitis (AIH), isolated autoimmune sclerosing cholangitis (ASC), and the overlap syndrome of these. We describe the clinical, biochemical, and outcomes data of a cohort of autoimmune ALDs patients in a tertiary care centre. Patients and methods: This is a cross-sectional study conducted at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Data were collected in 2007-2018. All cases were 18 years old or younger at the time of diagnosis. The data collection comprised clinical, laboratory, imaging, treatment, and longitudinal follow-up data. Results: Twenty-five patients were identified; 14 (56%) were females. Twenty-one patients (84%) had AIH-1,1 patient (4%) had AIH-2, and 3 patients (12%) had autoimmune sclerosing cholangitis (ASC). An insidious course was found in 21 (84%) cases. Acute hepatitis and fulminant hepatic failure was found to be very rare. Eight cases (32%) had cirrhosis at diagnosis. A total of 20 cases (80%) had complete remission following therapy. The median follow-up period was 45 months. There was no mortality, and only one patient was referred for transplant. Thus, the transplant-free survival was 96%. Conclusions: Our study showed predominance of AIH-1 over AIH-2. Response to treatment in our cohort was found to be similar to the results found in some other key studies. Prognosis and transplant-free survival is better than many published paediatric studies. (c) 2021 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:146 / 150
页数:5
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