J.Jpn. Surg. Soc.. 119(6): 658-664, 2018

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Feature topic

CURRENT STATUS OF PEDIATRIC INTESTINAL TRANSPLANTATION

Intestinal failure (IF) is a rare, debilitating condition that presents both acute and chronic medical management challenges, needing specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs (IRPs) are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation (IT). The management of IF has improved dramatically in the past few decades with advances in IRPs. However, patients with severe irreversible IF still experience numerous complications. Children with IF are especially at risk of developing IF-associated liver disease (IFALD). IT has become an effective therapy to improve their prognosis and quality of life (QOL) but is often reserved for patients with the most severe type of IF who develop life-threatening complications. End-stage IFALD leads to combined liver-intestinal transplant or multivisceral transplantation, but these procedures are difficult to perform in Japan, where liver-containing grafts from pediatric deceased donors are rarely available even after the revised Organ Transplant Act went into effect in 2010. Further advances in IRPs with multidisciplinary teams and improvement of long-term outcomes after IT are required to decrease mortality and improve the QOL of children with severe IF.

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