Determination of risk factors for Epstein-Barr virus-associated posttransplant lymphoproliferative disorder in pediatric liver transplant recipients using objective case ascertainment.

Background: Previous studies have suggested an increased risk of Epstein-Barr virus-associated posttransplant lymphoproliferative disorder (EBV-PTLD) in patients receiving tacrolimus for immunosuppression. We hypothesized that after correction for confounding variables, immunosuppression with tacrolimus is not associated with an increased risk of EBV-PTLD.

Methods: Potential cases of EBV-PTLD, identified by chart review, were independently ascertained by three clinicians and defined using published criteria.

Bone mineral density in long-term survivors following pediatric liver transplantation.

We sought to estimate the prevalence of reduced bone mass, defined by lumbar spine bone mineral density (LS-BMD) (z-score < -2.0), and to determine the factors associated with LS-BMD after liver transplantation in children and adolescents. LS-BMD z-scores were measured in a sample of subjects who had undergone liver transplantation in childhood or adolescence using dual energy x-ray absorptiometry (DXA).

Health-related quality of life in pediatric liver transplant recipients: A single-center study.

The goals of the present study were (1) to measure health-related 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.

Long-term outcome after liver transplantation in children.

Children (defined as under 18 yr of age) account for approximately 12.5% of all liver transplants in the United States. Even though the annual number of liver transplantation procedures remains relatively constant, the population of long-term survivors of liver transplantation has grown.

Effect of treatment with prostaglandin E1 and N-acetylcysteine on pediatric liver transplant recipients: a single-center study.

Prostaglandin E1 (PGE1) and N-acetylcysteine (NAC) have been used as single agents to decrease reperfusion injury and improve outcome after solid-organ transplantation (Tx). We hypothesized that combined treatment with NAC and PGE1 would be safe and reduce reperfusion injury. We therefore carried out a pilot study to assess the safety of this drug combination and gain information regarding the efficacy of treating pediatric liver transplant recipients with NAC and PGE1.

Long-term outcome after partial external biliary diversion for intractable pruritus in patients with intrahepatic cholestasis.

Background: Chronic intrahepatic cholestasis is associated with severe pruritus that is often refractory to maximal medical management and leads to significantly impaired quality of life. The hypothesis in this study was that partial external biliary diversion (PEBD) can substantially improve intractable pruritus secondary to intrahepatic cholestasis with subsequent improvement of functional quality of life.

Methods: Parents' and/or patients' clinical rating of pruritus, growth percentiles, biochemical parameters, and liver biopsies performed before and after surgery were compared in a retrospective medical record review.

Zonal regulation of gene expression during liver regeneration of urokinase transgenic mice.

Liver gene transcription plays a fundamental role in the hepatic reparative response to injury. However, little is known about the functional relationship of gene expression between diseased and regenerative compartments following a liver injury. To address the hypothesis that the control of gene expression and the cellular proliferative response are specific to diseased and regenerative liver compartments independently, we assessed the expression of liver growth modulators, hepatocyte proliferation, and apoptosis in transgenic livers overexpressing the urokinase-type plasminogen activator (uPA).

Biliary atresia--surgical management and treatment options as they relate to outcome.

Results show that the use of sequential surgical treatment, employing Kasai portoenterostomy in infancy, followed by selective liver transplantation for children with progressive hepatic deterioration yields improved overall survival. All children with successful Kasai portoenterostomy procedures who do not require OLT are survivors. Using newer transplant techniques, the 5-year survival rate for children who receive transplants with a primary diagnosis of biliary atresia was 82%.

Liver transplantation in the pediatric population: indications and monitoring.

Advances in immunosuppressive therapy and surgical technique have brought liver transplantation to an effective therapeutic option for children with end-stage liver disease. The indications and contraindications for transplantation, recipient selection, ethical and financial considerations, and quality of life after transplantation are outlined in this review.