Marvin Ament

Although liver transplantation is now accepted as the ideal therapy for end-stage liver disease, relatively few centers have gained a large experience in children, and good results have been elusive. Technical difficulty and a high incidence of graft failure are among the obstacles to success. At the University of California at Los Angeles, 39% of our liver transplants are in the patients who are younger than 18 years. We have analyzed our experience with 103 patients to emphasize factors important to a favorable outcome with the procedure. One hundred twenty-three transplants were performed in 103 children (mean age, 5.2 years; 48% younger than 3 years). No reduced-size grafts were used. Scrupulous attention to technical details of the vascular reconstruction, including frequent use of the supraceliac aorta of the recipient and interrupted suture techniques, ensured construction of sound hepatic artery and portal vein anastomoses at the first operation. Preoperative exchange transfusions were used if the prothrombin time was prolonged beyond 7 seconds, resulting in an average blood loss of only 3.3 volumes. Cyclosporine dosage was maintained in the high therapeutic range for the first 4 weeks, and anti-T-cell antibody (OKT3) was used for rejection (38%). Amphotericin prophylaxis was used for biliary atresia patients with multiple previous operations. Eighty-two of one hundred three patients (80%) are alive. There were no intraoperative deaths. Actuarial survival rates at 6 months, 1 year, and 5 years are 80%, 79%, and 77%, respectively. Survival of patients who underwent transplantation at age less than 1 year is 65% versus 85% at age more than 1 year (p = 0.08). Retransplantation was performed in 19 patients (18%), with a survival rate of 58%. Hepatic artery thrombosis, the most frequent technical complication, occurred in only 16 patients (13%). Survival rates of ABO identical-match versus nonidentical-match grafts were 96% and 60%, respectively (p = 0.02). Graft survival was only 47% if more than one steroid cycle was needed, compared to 75% survival with OKT3 treatment. Despite impairment of renal function (glomerular filtration rate [GFR] less than 80 cc/kg/min) in 54% of patients and hypertension requiring therapy in 27%, 90% of the children demonstrated enhancement of growth, development, and functional status. The following conclusions were made. (1) Pediatric liver transplantation is the treatment of choice for all types of end-stage liver disease and should be considered early. (2) Factors that enhance survival include technical precision, aggressive retransplantation, antifungal chemoprophylaxis and therapy, and judicious immunosuppression with use of OKT3 for rejection.(ABSTRACT TRUNCATED AT 400 WORDS)

This chapter contains sections titled: History The GIVEN M2A system Localization Suspected blood indicator Indication for use Diagnostic yield Contraindications to capsule endoscopy Pacemaker safety Bowel preparation Promotility agents Endoscopic assistance Complications Outcome of capsule endoscopy Pediatric patients

Total parenteral nutrition (TPN) has made survival beyond infancy possible for many infants who have sustained small intestinal loss as a result of gastroschisis or omphalocele. The length and quality of life in these patients have often been limited by the development of late sequelae secondary both to the protracted use of TPN and the long-term complications of a shortened gut. This study was undertaken to determine what factors influence the morbidity and mortality of short-bowel syndrome (SBS) due to gastroschisis or omphalocele. A retrospective chart review of 850 infants who received TPN from January 1977 through December 1999 was carried out. All infants were treated at one academic medical center; those who had received > or =3 months of TPN were further segregated and their diagnosis, surgical procedures, length of bowel, ability to wean from TPN, follow-up weight and height, and developmental progress were recorded. Seventeen children were identified with SBS and either gastroschisis or omphalocele. Tight primary or secondary closure of the abdominal wall was believed to be a major cause of bowel necrosis and SBS in at least ten of the 17 patients. Overall survival was 76 per cent (13/17); survival was correlated with length of remaining bowel and was 86 per cent in patients having more than 15 cm of small bowel remaining but only 33 per cent in patients with less than 15 cm of small bowel remaining (P = 0.05). A longer length of residual small bowel resulted in a significantly shorter duration of TPN with a mean duration of 1.0 year for survivors having >38 cm and 10.0 years for survivors with <38 cm of bowel remaining (P = 0.03). Hepatic dysfunction with progressive failure resulting from TPN was related to death in three of the four nonsurvivors. The presence or absence of an ileocecal valve appeared unrelated both to the success of TPN weaning and to the length of time on TPN (P > 0.2). Eight of the 13 survivors have no ileocecal valve; five have undergone >50 per cent colonic resection. Nine of the survivors have adapted to enteral feedings (mean 36 +/- 60 months) during which time weaning from TPN occurred. The mean age of survivors is 7.9 +/- 5.1 years. Near-normal weights (defined as exceeding the fifth percentile for weight) were achieved for 92 per cent of the patients (12/13) with 46 per cent of the patients (6/13) exceeding the 50th percentile. Near-normal heights (exceeding the fifth percentile) were achieved for 77 per cent of the patients (10/13) with 15 per cent of the patients (2/13) exceeding the 50th percentile. Quality of life was measured on the basis of return to public school: nine of ten school-age survivors attend school and eight are normal without signs of developmental delay. Tight abdominal closure of gastroschisis or omphalocele may cause bowel necrosis and SBS. TPN has improved the long-term survival and quality of life in infants with SBS.