Seerat Iqtadar, Khan Eitzaz Ud Din, Atique Muhammad, Aujla Usman Iqbal
Dr Iqtadar Seerat, MRCPCH, FRCPCH. Consultant and Head of Department of Paediatric Gastroenterology & Hepatology, Pakistan Kidney and Liver Institute & Research Centre, Lahore, Pakistan.
Dr. Eitzaz UD Din Khan, FCPS. Consultant & Head of Department of Anaesthesia, Pakistan Kidney and Liver Institute & Research Centre, Lahore, Pakistan.
Pak J Med Sci. 2021 May-Jun;37(3):680-683. doi: 10.12669/pjms.37.3.3862.
Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children.
This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined.
Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing.
Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality.
儿童慢性肝病（CLD）症状表现多样。在巴基斯坦等发展中国家，儿科肝病领域资源有限，这给及时调查和治疗慢性肝病儿童带来了巨大挑战。本研究旨在确定儿童慢性乙型和丙型肝炎病毒（HBV&HCV）肝病以外的慢性肝病谱及转归。
本回顾性描述性研究在巴基斯坦拉合尔的巴基斯坦肾脏与肝脏研究所及研究中心的儿科胃肠病学与肝病科进行。研究时间为2019年8月至2020年1月。在此期间共诊治了162例慢性肝病儿童。其中130例慢性HBV和HCV儿童被排除在本研究之外。纳入了32例15岁及以下的慢性肝病儿童。转诊来自该国不同地区的初级和二级医疗保健中心。数据从医院电子病历数据库收集，然后录入电子表格进行分析。采用t检验进行统计分析并确定p值。
在32例儿童中，自身免疫性肝炎（n = 11；34.3%）是慢性肝病转诊的最常见原因，其次是2型进行性家族性肝内胆汁淤积症（n = 7；21.8%）、先天性胆道闭锁Kasai术后（n = 4；12.5%）、1型糖原贮积病（n = 5；15.6%）、威尔逊病（n = 3；9.3%）和原发性硬化性胆管炎（n = 2；6.2%）。诊断主要借助肝脏超声、肝活检、磁共振胰胆管造影和基因检测来确立。
自身免疫性肝炎是最常见的慢性肝病。我们的系统方法，除了全面的检查外，有助于我们进行诊断并随后启动适当治疗，从而取得更优的治疗效果。建议在有资源和专业知识的三级中心进行及时转诊，以降低患者的发病率和死亡率。
