Transjugular Intrahepatic Portosystemic Shunt (TIPSS) for Budd Chiari Syndrome.

We report a four year old boy who presented with liver failure secondary to antithrombin III deficiency related Budd Chiari syndrome. He was treated with TIPSS (transjugular intrahepatic porto systemic shunt) which reversed the encephalopathy, normalised the liver function and improved growth, pre-empting the need for a liver transplantation. This is the first reported case of TIPSS in a child with a fulminant presentation of Budd-Chiari Syndrome.

Fulminant hepatic failure: etiology, viral markers and outcome.

OBJECTIVE: To investigate the etiology and outcome of fulminant hepatic failure (FHF) in children. SETTING: Hospital based descriptive. METHODS: 36 children (22 males and 14 females) presenting with FHF over a period of one year were investigated. The ages ranged from 1.5 to 9 years. FHF was defined as occurrence of encephalopathy within eight weeks of onset of jaundice with no evidence of pre-existing liver disease. Detailed history, clinical examination, routine biochemical parameters and relevant diagnostic tests were carried out. Viral markers studied were anti HAV-IgM, HBsAg, anti HBc-IgM, anti-HCV and anti HEV-IgM. RESULTS: A viral etiology could be established in 22 children (61.1%). Hepatitis A (n = 12), Hepatitis B (n = 3), Hepatitis A and B (n = 2), and Hepatitis A and E (n = 4). Two children had enteric fever (1 with associated HEV), 2 children had Wilson's disease, 1 child had Indian Childhood Cirrhosis (ICC) and 2 children had drug induced hepatitis. Etiological diagnosis was not possible in 8 children (22%). Fourteen children (39%) died. Poor outcome was associated with spontaneous bleeding, raised prothrombin time, lower transaminases and higher bilirubin on admission. CONCLUSION: Viral hepatitis is the commonest cause of FHF in children. HAV alone or in combination is responsible for upto 50% of all FHF in children. Chronic liver disease can also present as FHF. Etiological diagnosis is not possible to upto one-fourth of all cases.

Catch up growth and its determinants in low birth weight babies: a study using Z scores.

Two hundred and forty seven low birthweight (LBW) survivors of our Neonatal Intensive Care Unit and 164 normal birthweight controls were followed up longitudinally from birth to 4 years and their growth trends (weight, height, head circumference) were expressed as mean Z scores in 500 g birthweight categories. Whereas LBW's demonstrated rapid growth in the first 6 months of life, followed by generally parallel trends with some tendency to rise, controls showed distinct growth faltering especially after one year. Only 30.8% of LBWs (and 49% of controls) were within the designated catch up levels for weight by age 4 years. The corresponding number for catch up of height and head circumference in LBW's was 22.8% and 26.5%, respectively. On multiple regression analysis, the most important determinants of catch up (at 4 years) in LBW's were weight at 1 year (beta = 0.51), height at 1 year (beta = 0.31) and mother's weight (beta = 0.04). Thus, Z scores enabled the demonstration of changing growth trends, simultaneous comparisons with local controls and international standards and comparison within indices. Growth charts incorporating Z score should be made available in a simplified manner for use in the community.