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.

Pediatric parenteral nutrition in India.

Nutritional support to patients in neonatal and pediatric intensive care units is critical not only to minimize negative nitrogen balance but also to promote growth and development. Continuous technological and logistical advances in the Western countries have improved the efficacy and reduced the complications of parenteral nutrition (PN) to the extent that despite the constraints of cost and infrastructure, PN is now fast growing in India. Although widespread availability is very much desired, it is important that the technique is developed with considerable expertise and used judiciously with full knowledge of its indications, limitations, dangers and benefits. Indications for PN include surgical conditions (short gut syndrome), very low birth weight infants (particularly with necrotizing enterocolitis and surgical anomalies), malabsorption syndromes, conditions requiring bowel rest (acute pancreatitis, severe ulcerative colitis and necrotizing enterocolitis) and several non-gastrointestinal indications (end stage liver disease, renal failure, multiple trauma and extensive burns). Provision of PN is associated with significant and sometimes life threatening complications. The possible complications are technical (thrombosis, perforation of vein, thrombophlebitis), infections, metabolic disturbances, hepatobiliary stenosis, cholestasis, fibrosis, cirrhosis or cholelithiasis and bone related complications like osteopenia and fractures. Meticulous monitoring is necessary not only to detect complications but also to document clinical benefit.

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.

Haemophilus influenzae type b vaccine in India: need and timing, immunogenecity and tolerance.

OBJECTIVE: (i) To assess the natural immunity and susceptibility to Haemophilus influenzae type b (Hib) infections in children in India. (ii) To study the immunogenecity and tolerance of Hib vaccine (ACTHIB) in young infants. DESIGNS: (i) Cross sectional study. (ii) Prospective trial. SETTING: Well baby and immunization clinics. METHODS: (i) PRP antibody titers against Hib estimated in 172 healthy infants and children aged 1 month to 10 years. (ii) Antibody titres estimated before and after ACTHIB vaccine given with primary immunization (age group 6 to 8 weeks) in 50 babies. RESULTS: (i) Naturally protective levels of Hib antibodies found in less than 20% of infants under one year, but in over 80% above 4 years. (ii) Seroconversion after ACTHIB vaccination was 100% with very high protective levels. There were no significant adverse reactions. CONCLUSIONS: ACTHIB vaccine proved to be safe and highly immunogenic. As susceptibility to Hib is highest in the first year of life, the vaccine should be recommended in the primary immunization schedule (combined with DPT). The very high titers achieved suggest the possibility of decreasing the number of doses or the amount of antigen to reduce the prevalent high cost.

Cost effective treatment of osteogenic sarcoma of the extremities.

In an ongoing trial at our institute 10 patients of high grade osteogenic sarcoma of the extremities have been treated with preoperative chemotherapy including ifosfamide 2 mg/M2/day i.v. for 5 days, doxorubicin 20 mg/M2/day i.v. for 3 days and cisplatinum 120 mg/M2 i.v. on day 1 at 3-4 weeks interval for 2 courses followed by surgery. One patient refused surgery and further treatment. Pathological study of the 9 surgical specimens showed grade IV necrosis in 5, grade III necrosis in 2 and grade I & II necrosis in 2. Overall response rate (Grade III & IV) was 87.5%. The patients showing Grade III/IV response received a further 3 cycles of the same chemotherapy postoperatively. The patient who refused surgery is still alive at 30 months. Our followup ranges from 4-34 months. All patients developed myelosuppression and one patient died after 4th course of chemotherapy due to septicemia. We expect grade IV response to preoperative chemotherapy will be translated into longer disease free survival. Protocols followed in western countries are not practicable in Asian countries. Hence this new combination has been developed without compromising response rate.

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.

Ciprofloxacin in typhoid fever.

The study covers 78 children with typhoid fever who were hospitalized in April & May 1990. Serious complications were present in 32% (toxemia 22%, ileus 25% and myocarditis 8%). Blood cultures were positive in 30 of 49 tested. Others were diagnosed by positive Widal test. In vitro cultures of S. typhi were resistant to chloramphenicol (90%), ampicillin (93%) and co-trimoxazole (97%). However all were highly sensitive to ciprofloxacin and moderately sensitive to cephalexin and gentamycin. Ciprofloxacin alone or in combination was given in 73 of the 78 children and found to be remarkably effective in controlling the disease and preventing relapse. No serious side effects were noted. The cohort is being followed up for possible long term adverse effects.