Abstract
Although the development of parenteral nutrition has improved outcomes of intestinal failure, parenteral nutrition still often leads to numerous life-threatening complications. Intestinal failure-associated liver disease (IFALD), which can be progressive and fatal in children, is one of the most serious complications. Intestinal transplantation (Itx) represents a definitive treatment for patients with irreversible intestinal failure. By 2013, a total of 2887 intestinal transplants had been performed worldwide, 55% of which were in patients under 18 years old. In Japan, 60% of all recipients were under 18 years old. Because of recent advances in the management of Itx, short-term outcomes following transplantation have improved; however, the long-term outcomes remain insufficient. Furthermore, some patients with severe IFALD require liver-intestinal transplantation, a procedure that remains practically unavailable in Japan. In this article, we provide an overview of the process and the problems of pediatric Itx, with special focus on the situation in Japan.
