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ACLF patients in our population had high short-term mortality rates with majority of deaths in alcoholics. Alcohol intake and bacterial infections were mainly responsible for AD in our study.

BackgroundAlagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case. We believe that we report what could be the first case of Alagille syndrome presenting with café au lait spots, as no such published case report could be found in the literature.Case presentationWe report an unusual case of childhood cholestatic jaundice with neonatal onset of jaundice. A 10-year-old boy from the Indian subcontinent presented with obstructive jaundice from early infancy. He also had recurrent fractures of his upper limb bones, intermittent bleeding from his nose, productive cough, decreased night vision, hyperpigmented spots over his skin, and progressive enlargement of his abdomen. Histological examination of a liver biopsy specimen revealed a paucity of bile ducts and changes suggestive of chronic liver disease. Our patient was diagnosed with Alagille syndrome and managed conservatively but died 1 year after the final diagnosis.ConclusionsThis particular syndromic form of paucity of bile duct disorder has been rarely reported in the Indian literature so far. Our case is notable because the child had café au lait spots and very early onset of chronic liver disease, which is quite rare in Alagille syndrome. We believe this to be the first case report on Alagille syndrome manifesting with café au lait syndrome and such early onset of chronic liver disease.

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The patients attending the OPD and their attendants were subjected to a questionnaire about different aspects of hepatitis B. Binary logistic regression analysis (SPSS 16) was employed to assess the statistical importance of the observations. Results: In all, 682 individuals (65% patients, 35% nonpatients) were studied; 78% were males while 22% were females. Majority were in the age group of 31-40 years. 65% hailed from rural area; 65% were poor. About half of the subjects attended state run medical centers for medical attention; only 17% preferred ORIGINAL ARTICLE 20 medical colleges. Awareness about the disease and the vaccine among the subjects was 38% and 32%, respectively. 50% of those who were aware had no knowledge about route of transmission, infectivity, or importance of vaccination. Educated individuals were more aware about hepatitis B vaccine (P < 0.05). Those who read newspaper and listened to radio were more aware about hepatitis B (P < 0.05), and its vaccine (P < 0.05). The percentage of vaccination was 20% among study subjects, but in 30%, their children were vaccinated. The common reason for non-vaccination was lack of awareness (50%); of them, 60% blamed government/doctors/ media for their ignorance. Majority (56%) received the vaccine from government hospitals or health centers. Only (10%) obtained vaccination from private centers. Reasons cited for non-vaccination included ignorance (50%), carelessness (12%), high cost (10%), and nonavailability (6%). Source of information regarding hepatitis B included television (75%), newspapers (55%), and radio (26%).Conclusions: Only about one-third of the population in coastal Eastern India are aware about hepatitis B and its vaccine. Less than a third of the population are vaccinated for hepatitis B. The educated, especially those who read newspapers and listened to radio, were more aware about the disease/vaccine. The government health agencies and physicians should work together to educate the masses about hepatitis B and its vaccine.