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Clinical Profile of chronic hepatobiliary disorders in children: experience from tertiary referral centre in western India
Ira Shah 1, Susmita Bhatnagar2
Department of Pediatric Medicine1 and Pediatric Surgery2
Hepatobiliary Clinic, B. J. Wadia Hospital for Children,
Parel, Mumbai-400012

Corresponding Author
: Dr. Ira Shah


Aim: To determine etiological spectrum as well as clinical profile of chronic hepatobiliary disorders in children. 

Methods: 45 children with chronic hepatobiliary disorders were evaluated in the study.out of 105 children with liver diseases referred to the clinic. . All underwent detailed history and clinical examination. Clinical and laboratory features as well as causes of chronic hepatobiliary disorders were studied. 

Results:- The common causes were biliary atresia in 11 (25%) patients, neonatal hepatitis and Wilson’s disease in 6 (13%) patients each, glycogen storage disorder (GSD) and idiopathic hepatitis in 5 patients (11%) each, Hepatitis B in 2 (5%), Hepatitis C in 1 (2%), Hepatitis B and C in 1 (2%), Caroli’s disease in 2 (5%), autoimmune hepatitis in 2 (5%); sclerosing cholangitis, viral hemophagocytosis and thalassemia major in 1 (2%) patient each. Common clinical presentations were jaundice in 32 (71%), dark urine in 19 (42%), fever in 13 (29%), failure to thrive in 7 (16%), splenomegaly in 21 (47%) and hepatomegaly in 32 (71%). Also children with neonatal cholestasis presented in 1st year of life, those with idiopathic liver disease and GSD presented within 1st 5 years of life and those with Wilson’s disease. Autoimmune hepatitis, Caroli’s disease presented between 5-10 years of age and viral hepatitis was seen in 2nd decade of life (p <0.001). 

Conclusion: - Commonest cause of chronic hepatobiliary disorders in children is neonatal hepatitis. Metabolic liver disease usually presents in 1st 5 years of life whereas chronic viral hepatitis has a presentation in adolescence.