Biliary atresia (BA) is the end result of a destructive, rhosis. Until recently, BA was uniformly fatal; liver inflammatory process that affects intra-and extrahe-transplantation has altered the inevitability of that patic bile ducts, leading to fibrosis and obliteration of outcome, but at a ''cost'' to the patient, who must face the biliary tract with the development of biliary cirrho-the consequences of living with a transplanted organ, sis. It is the commonest cause of chronic cholestasis in and to society in real dollars. Our understanding of the infants and children, and therefore is the most frequent etiology and pathogenesis of BA has remained virtually indication for liver transplantation in this age group. unchanged for two decades. This disorder is of interest The disease occurs worldwide, affecting an estimated 1 to all individuals involved in basic and clinical studies basis for BA; 2) the optimal timely and precise methods of discrimination from other causes of neonatal cholestasis; 3) the natural history of BA; 4) the optimal use Biliary atresia (BA), the most serious digestive disand timing of medical and surgical interventions (porease affecting infants, is an idiopathic, localized, comtoenterostomy and transplantation); and 5) the effect plete obliteration or discontinuity of the hepatic or comof preexisting liver disease or surgical complications mon bile ducts at any point from the porta hepatis (cholangitis, sepsis, peritonitis) on the outcome of to the duodenum. 1,2 Obstruction of bile flow leads to transplantation. Because a small number of patients cholestasis, progressive fibrosis, and, ultimately, cirare seen in individual centers and patients are not managed in a uniform manner between centers, an accurate assessment of these issues is difficult. ThereAbbreviations: BA, biliary atresia; HPE, hepatoportoenterostomy; BASE, fore, a collaborative, multicenter study is needed; a hybiliary atresia splenic malformation syndrome; CDC, chenodeoxycholic acid;pothesis potentially subject to study in such a project CMV, cytomegalovirus. is that BA is the phenotype of several underlying disor- nature of the disorder has perplexed physicians for over Cincinnati,

No abstract

The goals of the present study were (1) to measure healthrelated quality of life (HRQOL) in pediatric liver transplantation (LT), (2) to identify demographic and clinical factors that correlate with HRQOL, and (3) to compare two instruments that have been used to measure HRQOL in children and adolescents. We conducted a single-center cross-sectional study of 77 pediatric LT recipients ages 5 to 18 years, all of whom had had LT at least 6 months previously. We used the Child Health Questionnaire Parent Form 50 (CHQPF50) and the PedsQL4.0 to determine measured dimensions of physical and psychosocial health from the parents' perspective. Individual scale scores range from 0 to 100, with higher scores reflecting better health. Data on demographics, clinical status at transplantation, posttransplantation clinical course, and graft function were collected to identify predictors of posttransplantation HRQOL. Fifty-three percent of the liver transplant recipients had biliary atresia, 78% were white, and 61% were female. The mean age at LT was 3.8 ؎ 3.6 years, and the range of time since LT was 1 to 15 years. HRQOL in pediatric liver transplant recipients was lower than that reported for healthy children but similar to that for children with other chronic illness. Age at transplantation, the time elapsed since transplantation, hospitalizations within the previous year, maternal education, and race were significant predictors of physical health. Age at transplantation and maternal education predicted psychosocial function. HRQOL was decreased in a population of pediatric liver transplant recipients compared with the general population and similar to that for children with chronic illness. Prospective longitudinal studies will permit us to define predictors of HRQOL at different periods of time after transplantation. The information gained from this study will help us to better define expectations and the clinical course after liver transplantation to patients and their families. (Liver Transpl 2003; 9:62-71.)

This low-dose chemotherapy regimen is effective for children with EBV-positive, nonfulminant PTLD who have experienced treatment failure with front-line therapy, and this study represents the largest series of PTLD patients treated prospectively with a uniform chemotherapy regimen.