Splanchnic transplantation. An approach to the infant dependent on parenteral nutrition who develops irreversible liver disease

JAMA. 1989 Mar 10;261(10):1458-62. doi: 10.1001/jama.261.10.1458.

Abstract

Two infants with short-bowel syndrome and liver failure associated with obligatory parenteral nutrition received a composite allograft that consisted of en bloc liver, stomach, duodenum, pancreas, jejunum, and ileum. Solutions to the fatal complications in the first case resulted in a functioning composite splanchnic system in the second case. Despite a number of early complications, the small intestine and liver developed near-normal function until a monoclonal, malignant, B-cell lymphoproliferative disorder appeared. The analysis of these two cases supports three summary observations: the operative procedure can be safely performed in a metabolically compromised infant; intestinal allograft rejection, in this model, is controllable with existing immunosuppressive drugs; and this procedure appears to be associated with a uniquely high incidence of lymphoma. Since transplantation is a feasible solution to this devastating infantile disease, further development of this therapy must incorporate means of preventing lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Cyclosporins / administration & dosage
  • Cyclosporins / pharmacokinetics
  • Herpesvirus 4, Human
  • Humans
  • Infant
  • Liver Diseases / etiology
  • Liver Diseases / surgery*
  • Lymphoproliferative Disorders / immunology*
  • Lymphoproliferative Disorders / pathology
  • Male
  • Parenteral Nutrition*
  • Postoperative Complications* / pathology
  • Short Bowel Syndrome / etiology
  • Short Bowel Syndrome / surgery
  • Viscera / transplantation*

Substances

  • Cyclosporins