Pediatric Chronic Liver Diseases: A Clinicopathological Study from a Tertiary Care Center

Authors

1 Department of Pediatrics, Faculty of Medicine, Sohag University, Egypt.

2 Department of Pathology, Faculty of Medicine, Sohag University, Egypt.

3 Department of Pathology, Faculty of Medicine, Cairo University, Egypt.

Abstract

Background
Chronic liver diseases (CLD) in children represent a growing health problem with significant morbidity and mortality. This study aimed to define the clinicopathological pattern of pediatric CLD in Sohag University Hospital, Sohag,Upper Egypt.
Materials and Methods
A total of 151children with CLD were included in a prospective hospital-based study from June 2014 to May 2018. Cases of acute liver illness or hepatic focal lesions were excluded. All patients were subjected to detailed history and thorough physical examination. Abdominal ultrasonography, CBC, liver function tests, viral serology, evaluation of autoantibodies for autoimmune hepatitis, and liver core biopsies were performed for all children.
Results
Pediatric CLD comprised 1.6% of total admissions in pediatric department. Neonatal cholestasis disorders (NCD), and metabolic liver disorders (MLD) were the leading causes of CLD (41.05% and 35.1%, respectively). NCD comprised neonatal hepatitis (25.1%), extrahepatic biliary atresia (13.2%), and paucity of interlobular bile ducts (2.7%). MLD included glycogen storage disease (26.5%), undetermined inborn error of metabolism (5.3%), Gaucher's disease (2.0%), and Niemann Pick disease (1.3%). Other causes of CLD comprised autoimmune hepatitis (8.6%), congenital hepatic fibrosis (5.9%), non-alcoholic fatty liver disease (4.0%), chronic hepatitis C infection (2.7%), and Budd Chiari disease (0.6%). On follow-up of 89 cases, stationary clinical course, clinical improvement, and clinical deterioration were seen in 52.8%, 34.8%, and 12.3% of them, respectively.
Conclusion
The rate of CLD is growing in Upper Egypt and is mainly caused by neonatal cholestasis and metabolic liver disorders. In general, the outcome of children is favorable and comparable to other countries.

Keywords