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Item Liver-Intestine/Multivisceral Perspective: Indications, Patient Selection, and Allocation Policy(Wiley, 2019-11-06) Mangus, Richard S.; Surgery, School of MedicineIntestinal failure (IF) exists when the gastrointestinal tract has insufficient function to maintain hydration and nutrition to support life and to modulate electrolyte and fluid balances. In children, this definition also includes the failure of the intestine to support growth and development. The cause of IF most frequently involves loss of intestine length, so‐called short gut, but may also result from neurological or enterocyte dysfunction that prevents normal peristalsis or absorption.Item Successful Sequential Liver and Isolated Intestine Transplantation for Mitochondrial Neurogastrointestinal Encephalopathy Syndrome: A Case Report(International Scientific Information, 2024-02-27) Kubal, Chandrashekhar A.; Mihaylov, Plamen; Snook, Riley; Soma, Daiki; Saeed, Omer; Rokop, Zachary; Lacerda, Marco; Graham, Brett H.; Mangus, Richard S.; Surgery, School of MedicineBackground: Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) is an autosomal recessive disease caused by thymidine phosphorylase deficiency leading to progressive gastrointestinal dysmotility, cachexia, ptosis, ophthalmoparesis, peripheral neuropathy and leukoencephalopathy. Although liver transplantation corrects thymidine phosphorylase deficiency, intestinal deficiency of the enzyme persists. Retrospective chart review was carried out to obtain clinical, biochemical, and pathological details. Case Report: We present a case of liver and subsequent intestine transplant in a 28-year-old man with MNGIE syndrome with gastrointestinal dysmotility, inability to walk, leukoencephalopathy, ptosis, cachexia, and elevated serum thymidine. To halt progression of neurologic deficit, he first received a left-lobe partial liver transplantation. Although his motor deficit improved, gastrointestinal dysmotility persisted, requiring total parenteral nutrition. After exhaustive intestinal rehabilitation, he was listed for intestine transplantation. Two-and-half years after liver transplantation, he received an intestine transplant. At 4 years after LT and 20 months after the intestine transplant, he remains off parenteral nutrition and is slowly gaining weight. Conclusions: This is the first reported case of mitochondrial neurogastrointestinal encephalomyopathy to undergo successful sequential liver and intestine transplantation.