Abstract
Background and Objectives: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.Methods: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.Results: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.Conclusion: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.
Highlights
Children with chronic liver disease (CLD) could present with jaundice, itching, abdominal distension, pale stools and dark urine.[1]
The study conducted by Hanif et al.[3] showed that in their group of children of CLD, viral hepatitis B followed by Wilson disease were the commonest diseases
Another study performed in Pakistan revealed the hepatitis C and glycogen storage disease were the top two diseases in the list of their group of children.[6]
Summary
Children with chronic liver disease (CLD) could present with jaundice, itching, abdominal distension, pale stools and dark urine.[1] Due to lack of resources and expertise in the field of Paediatric Hepatology especially in the developing world such as Pakistan, it is challenging to investigate and treat such diseases in children in a timely fashion. Both morbidity and mortality increase in children. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality
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