Long-term Outcomes of Liver Transplantation for Inborn Errors of Metabolism in Children.

Background: Liver transplantation for inborn errors of metabolism is increasingly common and has historically had positive outcomes. However, this therapeutic modality is not without risks, and patient post-transplant quality of life should be part of the consideration.

Methods: This retrospective, observational cohort study included all pediatric patients receiving liver transplant from 2010 through 2020 at a single center.

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis.

Objective: To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF).

Study Design: Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis.

Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease.

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation.