RESUMO
Aim: To determine the clinical and biochemical factors associated with biliary atresia. Methods: This retrospective study was carried at the Pediatric Hepatobiliary Clinic, of a tertiary care referral center, from May 2005 to April 2006. Thirty-three infants with neonatal cholestasis were enrolled. All patients were evaluated by detailed history and clinical examination. Patients diagnosed with biliary atresia on intra-operative cholangiogram and liver biopsy underwent the Kasai operation. Clinical and biochemical factors predictive of biliary atresia were determined. Results: Seventeen infants (51.5%) had neonatal hepatitis, (42.4%) biliary atresia and two (6.1%) neonatal sepsis. Clay colored stools was the only clinical feature suggestive of biliary atresia which was seen in 11 biliary atresia children (79%) and was statistically significant (p=0.05). No other biochemical markers were suggestive of biliary atresia, such as alkaline phosphatase (p=0.10) or gamma glutamyl transferase (GGTP) (p=0.64). On follow-up 6 patients (43%) with biliary atresia developed chronic liver disease and two patients (14%) died of their disease, whereas 41% patients with neonatal hepatitis made successful recovery. (p=0.02) Conclusion: Presence of clay colored stools is a predictive marker for biliary atresia and should be used as one of the markers for urgent cholangiogram, since most of the children with biliary atresia go on to develop chronic liver disease.
RESUMO
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.