The multivisceral liver-intestine-pancreas-stomach allograft was first described by Starzl nearly 50 years ago. Since then, over 1000 children have received small bowel transplantation (SBTx), alone or with the liver and other organs, for refractory short gut syndrome (SGS) because of a variety of congenital conditions. In 2001, SBTx was approved as definitive therapy for SGS by Medicare. Currently, 1- and 5-year graft survival routinely exceeds 90% and 80%, respectively. The expected outcomes also include freedom from parenteral nutrition, normalization of growth parameters, and quality of life. However, recurrent rejection, complications of high-dose immunosuppression, or chronic rejection, which is more likely to occur after SBTx without a liver graft, account for differences between early and late survival. Future efforts aimed at overcoming such challenges include preventing SBTx through early referral to comprehensive SGS management programs and understanding why the liver protects the small bowel allograft from rejection. Finally, inflammatory mechanisms, which predispose the highly immunogenic small bowel allograft to a protracted risk of resistant rejection must be elucidated, in order to ensure durable success.
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