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An Approach to the Infant Dependent on Parenteral Nutrition Who Develops Irreversible Liver Disease

James W. Williams, MD; Howard N. Sankary, MD; Preston F. Foster, MD; Jerome Lowe, MD; Gilbert M. Goldman, MD

JAMA. 1989;261(10):1458-1462.

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.

(JAMA. 1989;261:1458-1462)

Author Affiliations
From the Department of General Surgery, Section of Transplantation (Drs Williams, Sankary, and Foster), and Departments of Pathology (Dr Lowe) and Pediatrics (Dr Goldman), Rush-Presbyterian-St Luke's Medical Center, Chicago, III.

Footnotes
Reprint requests to Section of Transplantation, Rush-Presbyterian—St Luke's Medical Center, 1753 W Congress Pkwy, Chicago, IL 60612 (Dr Williams).

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