Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre
Iqtadar Seerat1, Eitzaz Ud Din Khan2, Muhammad Atique3
1Dr Iqtadar Seerat, MRCPCH, FRCPCH. Consultant and Head of Department of Paediatric Gastroenterology & Hepatology, Pakistan Kidney and Liver Institute & Research Centre, Lahore, Pakistan.
Insights
Autoimmune hepatitis is the most frequent pediatric chronic liver disease in Pakistan, excluding viral causes. Prompt diagnosis and treatment at specialized centers improve outcomes for children with liver conditions.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Internal Medicine
Background:
- Chronic liver disease (CLD) in children presents diverse symptoms, posing challenges in resource-limited settings.
- Paediatric hepatology services in Pakistan face limitations in timely diagnosis and treatment of CLD.
- This study focuses on CLD spectrum beyond Hepatitis B & C virus infections in children.
Purpose of the Study:
- To determine the spectrum of pediatric chronic liver diseases in Pakistan, excluding viral hepatitis.
- To analyze the outcomes of non-viral CLD in children.
- To identify common causes and diagnostic approaches for CLD in Pakistani children.
Main Methods:
- Retrospective descriptive study conducted at a tertiary care center in Lahore, Pakistan.
- Included 32 children (≤15 years) with CLD, excluding Hepatitis B & C virus infections.
- Data collected from electronic medical records; diagnosis aided by ultrasound, biopsy, MRCP, and genetic testing.
Main Results:
- Autoimmune hepatitis was the most common cause (34.3%), followed by progressive familial intrahepatic cholestasis type-2 (21.8%).
- Other identified causes included post-Kasai for biliary atresia (12.5%), glycogen storage disease type-1 (15.6%), Wilson disease (9.3%), and primary sclerosing cholangitis (6.2%).
- Diagnostic modalities included liver ultrasound, biopsy, MRCP, and genetic testing.
Conclusions:
- Autoimmune hepatitis is the leading non-viral chronic liver disease in Pakistani children.
- A systematic diagnostic approach and timely referrals to specialized centers are crucial for optimal patient outcomes.
- Early intervention and expert management can significantly reduce morbidity and mortality in pediatric CLD.
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.
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