Metabolic liver diseases in childhood: Indian scenario.

The present survey was undertaken to ascertain the spectrum of childhood metabolic liver diseases (MLD) and diagnostic methods available in teaching medical institutions in India. Out of 17 medical colleges approached in different parts of the country, pediatricians from 11 institutions agreed to participate. Six colleges had organised pediatric gastroenterology & hepatology services (category I) and five were not having such services (category II). The participants provided information regarding the number of chronic liver disease patients seen at their centres during the past 5-10 years with their etiological diagnosis, the proportion of metabolic liver diseases in these children and the methods used to label etiology. Patients with acute hepatitis B infection and neonatal cholestasis were excluded. In the past 1-9 years, (38-236) and (4-57) children with chronic liver disease (CLD) were seen in category I and category II centres respectively. Chronic hepatitis B and C and metabolic liver diseases were being diagnosed in most of the centres, though in category I other causes of chronic liver diseases were also reported. Metabolic liver diseases constituted 8 to 43% of the reported CLD in category I colleges and 0-46% in the other pediatric centres. Most major categories of metabolic liver diseases were seen at various centres. Indian Childhood Cirrhosis (ICC) was very infrequently reported even in the large hospitals and Wilson's disease was the most frequently diagnosed metabolic liver disease. Reference diagnostic tests for most of the metabolic liver disorders were not accessible to majority of participating institutions. Metabolic liver diseases constitute a significant proportion of childhood chronic liver diseases in our country. There is an important need to set up reference laboratories in the country to facilitate diagnostic work up of metabolic liver diseases at affordable costs.