Paediatric autoimmune liver diseases: A descriptive study of patients from Saudi Arabia |
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| Authors: | Omar I. Saadah Ammar Khayat Hadeel A. Alsufyani Yagoub Bin-Taleb |
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| Affiliation: | 1. Department of Paediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia;2. Department of Paediatrics, College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia;3. Department of Medical Physiology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia;4. Paediatric Gastroenterology Unit, Department of Paediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia;1. Department of Gastroenterology, The Second Hospital, Shanxi Medical University, Taiyuan, Shanxi 030001, PR China;2. Endoscopy Center, Affiliated Tumor Hospital of Shanxi Medical University, Taiyuan, Shanxi 030001, PR China;1. Clinical Pathology, Faculty of Medicine, Beni-Suef University, Beni-Suef, Egypt;2. Endemic Medicine, Faculty of Medicine, Cairo University, Cairo, Egypt;1. Endemic Medicine and Hepatogastroenterology Department, Faculty of Medicine, Cairo University, Cairo, Egypt;2. Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt;1. Division of Gastroenterology, American University of Beirut Medical Center, P.O. Box 11-0236/16-B, Beirut, Lebanon;2. Department of Family Medicine, American University of Beirut Medical Center, PO Box: 11-0236, Riad El Sol, 1107 2020 Beirut, Lebanon;3. Department of Pathology & Laboratory Medicine, American University of Beirut Medical Center, PO Box 11-0236, Riad El Solh 11072020, Beirut, Lebanon;1. Department of Gastroenterology, Shenzhen Hospital of Southern Medical University, Shenzhen, Guangdong 518100, China;2. Department of Liver Disease Center, Shenzhen Hospital of Southern Medical University, Shenzhen, Guangdong 518100, China;3. Information Management Section, Bethune International Peace Hospital, Shijiazhuang City, Hebei Province 050000, China;1. Department of Pathology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia;2. Department of Pathology, Faculty of Medicine, Minia University, Al-Minia, Egypt;3. Department of Pathology, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia |
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| Abstract: | Background and study aimsAutoimmune 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 methodsThis 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.ResultsTwenty-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%.ConclusionsOur 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. |
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| Keywords: | Autoimmune liver disease Autoimmune hepatitis Autoimmune sclerosing cholangitis, children Saudi Arabia |
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