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Long-term outcome following pediatric liver transplantation for metabolic disorders

Terrell Stevenson1, Maria T Millan, Karen Wayman

  • 1Department of Surgery, Stanford University, Stanford, CA, USA.

Pediatric Transplantation
|August 13, 2009
PubMed

Insights

Pediatric liver transplantation offers excellent survival and improved growth for children with metabolic disorders. While psychomotor development enhances, cognitive skills remain in the low-normal range post-transplant.

Area of Science:

  • Pediatric Hepatology
  • Metabolic Disorders
  • Transplantation Immunology

Background:

  • Metabolic disorders can lead to severe liver dysfunction in children.
  • Liver transplantation is a potential treatment, but long-term outcomes require evaluation.

Purpose of the Study:

  • To assess long-term survival, growth, and neurodevelopmental outcomes in children with metabolic disorders following liver transplantation.

Main Methods:

  • Retrospective chart review of 54 children with metabolic disorders evaluated between 1989-2005.
  • Analysis of presenting symptoms, transplant details, survival rates, metabolic parameters, and developmental assessments.

Main Results:

  • 33 children underwent liver transplantation (12 with combined liver-kidney).
  • Excellent patient survival (100%) and allograft survival (92-100%) at a median 3.6-year follow-up.
  • Improved weight Z scores and normal BMI; significant psychomotor development gains (p < 0.01), but mental skills remained low-normal.

Conclusions:

  • Liver or combined liver-kidney transplantation is associated with excellent long-term survival in pediatric metabolic disorders.
  • Transplantation improves growth and psychomotor development, though cognitive function may lag.
  • Outcomes comparable to biliary atresia patients for psychomotor development, but lower for mental skills.

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