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A 12-year-old immunized boy, 3rd issue of consanguineous parents, presented with jaundice for the last 4 months and gradual abdominal distension for last 2 months. Mother also mentioned the swelling of both ankles for the same duration. He had anorexia, nausea and generalized weakness. There was no history of previous jaundice, blood transfusion, surgical procedure, history of taking offending drugs, no family history of liver disease, deterioration of school performance or neuropsychiatric manifestations, bleeding manifestations, behavioral abnormality, altered consciousness or convulsion.

A 10-year-old immunized girl, 6th issue of consanguineous parents, presented with the complaints of jaundice for the last 2 years and deterioration of school performance for the same duration. She also had generalized itching for the last 6 months. She had no history of altered sleep pattern, any gastrointestinal bleeding, surgical or dental procedures, history of blood and blood products transfusion, taking any offending drugs, sib death or family history of such type of illness.

A 6-month-old boy of non-consanguineous parents admitted to the Department of Pediatric Gastroenterology with the complaints of progressive jaundice, dark urine and generalized pruritus for one month. The boy was well up to five months of age. Then he developed jaundice which was progressive in nature with intermittent pale colored stool along with dark urine. His mother also complaints for generalized pruritus which was severe in intensity (disturbing sleep and daily activities) without any diurnal variations. There was no history of sib death or family history of a similar type of illness.