Immunity to poliomyelitis, diphtheria and tetanus in pediatric patients before and after renal or liver transplantation.

Few studies have considered the safety, efficacy and appropriateness of vaccinations in pediatric patients before and after solid organ transplantation. The aim of this study was to evaluate the immune status after primary vaccination to diphtheria, tetanus and poliomyelitis in pediatric patients before and after hepatic transplantation and to poliomyelitis in pediatric patients before and after renal transplantation. All the patients had received a complete primary immunization schedule for diphtheria and tetanus and poliomyelitis.

Hodgkin's disease occurring in a child after liver transplantation.

Here we describe the case of a 14-year-old boy who underwent liver transplantation for post-Kasai biliary atresia when aged 4. Antirejection treatment consisted of prednisone and cyclosporine. At the age of 11 years the patient developed left cervical lymphadenopathy; the biopsy showed classical Hodgkin's disease(HD) of the mixed cellularity (MC) type.

Rescue FK506 early conversion for refractory rejection after pediatric liver transplantation: experience in 20 children.

Tacrolimus (FK506) is an effective and relatively safe novel immunosuppressant able to revert refractory rejection after pediatric liver transplantation (LTx). Between April 1993 and October 1996, 20 pediatric patients were converted to tacrolimus for biopsy-proven, steroid-resistant liver rejection. The mean follow-up was 18 months.

Post-hepatitis primary disease does not influence 6-year survival after liver transplantation beyond 1 year.

Orthotopic liver transplantation (OLT) is used as a definitive treatment for end-stage liver disease and prolonged posttransplant survival has already been reported. The incidence of late mortality and graft morbidity is, however, not well defined and the role of primary viral disease in the long-term follow-up results is not clear. Data of posttransplant follow-up in 213 patients, 156 adults and 57 children, who survived at least 1 year were reviewed in order to define causes of graft dysfunction, graft loss and death.

Steatocystoma multiplex and leuconychia in a child with Alagille syndrome.

Alagille syndrome is a rare autosomal dominant developmental disorder, characterized by congenital paucity of interlobular bile ducts, peculiar facies, posterior embryotoxon, bone abnormalities, and peripheral pulmonary artery stenosis. Cutaneous involvement in this disorder is quite rare and the association of Alagille syndrome with multiple comedones and cysts has been described only once. Here, we report the clinical and histological findings of a 7-year-old patient affected by Alagille syndrome who presented multiple cystic lesions and comedones reminiscent of steatocystoma multiplex and a congenital total true leuconychia.

Factors influencing malnutrition in children waiting for liver transplants.

Nutrition deficiencies are common in children with chronic liver disease. To determine whether age, hepatic dysfunction, or energy intake influences this malnutrition, we evaluated the nutritional status of 49 children aged 2.5 mo to 13 y (mean: 35 mo; median: 12 mo).

Expression of lysosomal acid lipase mutants detected in three patients with cholesteryl ester storage disease.

Lysosomal acid lipase (LAL) gene mutations were identified in three patients with cholesteryl ester storage disease (CESD). Direct sequencing of genomic DNA revealed that: patient 1 was a compound heterozygote for a P181L mutation and an A to G3' splice site substitution that causes skipping of exon 7, with a loss of 49 amino acids from LAL (delta 205-253); patient 2 was a compound heterozygote for a G66V mutation and a 5' splice site mutation (G to A) that leads to skipping of exon 8 (delta 254-277); and patient 3 was a compound heterozygote for a L273S mutation and an unidentified null allele. Furthermore, patients 2 and 3 showed a novel G-2A polymorphism that could be detected by an Xbal restriction fragment length polymorphism.

Paediatric liver transplantation in Italy.

The paediatric liver transplantation programme was started, at our institution, in 1988 and since then 75 children have received 79 transplants, which corresponds to about 85% of all the paediatric liver transplantations performed in Italy. The main indications to transplantation were biliary atresia, familiar cholangiopathies and metabolic diseases. The patients came from all the regions of Italy, except for 2 of them who came from abroad.

Hepatitis C virus genotypes and reinfection of the graft during long-term follow-up in 35 liver transplant recipients.

To understand the clinical outcome of hepatitis C virus (HCV) recurrence, data from 35 liver transplant recipients who survived more than 6 months were reviewed. The presence of HCV-RNA was evaluated and genotyping was performed. On the basis of alanine aminotransferase (ALT) levels, patients were sorted into four groups.

Early metabolic treatment after liver transplant: amino acid tolerance.

Objective: We investigated the amino acid (AA) tolerance during Total Parenteral Nutrition (TPN) in adult patients undergone liver transplant (LTX).

Design: The treatment (Glucose and AA), induced on the 2nd postoperative day, was later maintained with 27 kcal/kg Ideal Body Weight (IBW) as glucose and 0.12 (12 patients: protocol #1), 0.