In This Issue 1 Nutrition Options in Short-Bowel Syndrome SPRING 2017 Division of Gastroenterology, 3 Gastric Carcinoids with Duodenal Ulcers Hepatology, and Nutrition 4 Living Donor Liver Transplant (LDLT) 6 PancreasFest 2017 / Honors and Awards 7 Pittsburgh Gut Club 8 What Is This? Nutrition Options in Short-Bowel Syndrome By David G. Binion, MD, and Zachary Zator, MD Intestinal transplantation is an option for select patients with short-bowel syndrome- associated intestinal failure (SBS-IF) who fail or do not tolerate nutritional rehabilitation. There are a range of factors to consider in the nutritional management of patients before and after intestinal transplantation. SBS-IF can be defined as the inability to maintain proper nutritional balance — including of proteins, electrolytes, macronutrients, micronutrients, and fluids — while adhering to a conventional diet in the face of an anatomically or functionally limited gut surface. The ideal management of patients with SBS-IF involves a multidisciplinary team of gastro enterologists, nurses, dietitians, pharmacists, and surgeons. Pharmacotherapeutic agents aimed at minimizing fluid losses have been routinely employed to support these patients. For instance, antidiarrheal agents, such as loperamide or diphenoxylate, are used alongside proton pump inhibitors. Somatostatin analogs, like octreotide, inhibit gastrointestinal secretions from the stomach, pancreas, and intestines and have been proven beneficial in the past. However, their role can be limited, as somatostatin can actually inhibit enteral protein synthesis. In recent years, attention has turned beyond mere supportive care to potentially therapeutic pharmacologic agents, such as teduglutide, a human recombinant GLP-2 analog, which was approved in 2012. Glucagon-like peptide-2 (GLP-2) is secreted in response to luminal nutrients reaching the distal ileum and colon, and promotes the growth of intestinal mucosa by increasing mesenteric blood flow, decreasing gastric acid secretion, and enhancing crypt cell growth Continued on Page 2 Accreditation Statement: The University of Pittsburgh School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. The University of Pittsburgh School of Medicine designates this enduring material for a maximum of 0.5 AMA PRA Category 1 Credits™. Each physician should only claim credit commensurate with the extent of their participation in the activity. Other health care professionals are awarded .05 continuing education units (CEU), which are equivalent to 0.5 contact hours. Disclosures: Dr. Binion reports grants and research support from Janssen Biotech, Merck, and UCB Pharma, and he also serves as a consultant for Janssen Biotech, AbbVie, UCB Pharma, and Synthetic Biologics. All other contributing authors and editors of this publication report no relationships with proprietary entities producing health care goods and services. digest Instructions: To take the CME evaluation and receive credit, please visit UPMCPhysicianResources.com/GI and click on UPMC Digest Spring 2017. Affiliated with the University of Pittsburgh School of Medicine, UPMC is ranked among the nation’s best hospitals by U.S. News & World Report. 2 DIGEST — SPRING 2017 Nutrition Options in Short-Bowel Syndrome (Continued from Page 1) while inhibiting apoptosis. However, the endogenous form of GLP-2 common deficiencies seen in one pediatric study after transition is rapidly degraded in vivo. As a recombinant analog of GLP-2, to enteral nutrition were iron (94.7%), magnesium (90.5%), teduglutide has a significantly longer half-life and acts by binding to zinc (50%), vitamin D (66.7%), and vitamin A (40%).4 Another GLP-2 receptors and potentiating its effects. In phase III randomized, study found that almost all patients (96%) were deficient in the 5 placebo-controlled trials, the active form of vitamin B6 within 30 days of transplantation. As a volume of parenteral support result, proactive supplementation with both a multivitamin and that patients required was micronutrient-specific formulations is considered necessary significantly reduced in those in the posttransplant population. 1,2 patients receiving teduglutide. Proper nutritional management of patients before and after The medication is fairly well intestinal transplantation involves a directed, multidisciplinary tolerated, with abdominal pain approach aimed at defining, monitoring, and eliminating deficiencies (30%), minor injection-site in individual patients. With the help of supportive care techniques reactions (22%), nausea (18%), and novel pharmacologic agents like teduglutide, patients with short and headaches (16%) being the most commonly reported adverse bowel syndrome-associated intestinal failure can be managed effects. Given the nature of the drug, theoretic concerns exist related successfully. If intestinal transplantation is required, careful attention to tumor promotion, and colonoscopy is recommended six months must be paid to not only caloric requirements, but also to hydration before and one year after drug initiation. status and micronutrient and macronutrient homeostasis. When medical management of SBS-IF fails, intestinal transplan- tation is pursued in select patients. The optimal strategy to manage Dr. Binion is a professor of medicine with the Division of nutritional needs in these complex postoperative patients is Gastroenterology, Hepatology, and Nutrition. He serves as a co-director and translational research leader for the IBD not entirely known. Moreover, requirements vary depending on Center and directs the Division’s Nutrition Support Program. the length of time since transplantation. Right after transplant, patients generally need higher caloric intake to maintain a healthy nutritional state. Yet at three months after transplantation, the enteral graft generally absorbs carbohydrates and other forms of energy quite well. In one pediatric study, a ratio of energy intake to Dr. Zator is a year III gastroenterology fellow with the Division of Gastroenterology, Hepatology, and Nutrition, resting energy expenditure of 1.34 ± 0.18 was needed for patients where he also serves as the chief GI fellow. on full parenteral nutrition (PN), compared with a ratio of 2.15 ± 0.27 for full enteral intake.3 Long-term data from a different study suggests that these escalated enteral requirements may decrease over time. Ensuring that patients meet these needs while tran- sitioning from parenteral to enteral nutrition is critical to nutritional homeostasis. From a practical perspective, it is important to References recognize that the enteral energy needed to maintain adequate 1 Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B, O’Keefe SJ. nutrition is highest early after transplant, but likely returns closer Randomised Placebo-controlled Trial of Teduglutide in Reducing Parenteral to the population norms over time. These early nutritional Nutrition and/or Intravenous Fluid Requirements in Patients With Short Bowel demands can be met through increased caloric intake. Syndrome. Gut. 2011; 60(7): 902-14. 2 Outside of caloric requirements in the posttransplant population, O’Keefe SJ, Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B. Safety and Efficacy of Teduglutide After 52 Weeks of Treatment in Patients With attention must be paid to maintaining adequate hydration. Nearly all Short Bowel Intestinal Failure. Clin Gastroenterol Hepatol. 2013; 11(7): 815-23. patients who undergo intestinal transplantation are managed with an 3 Ordonez F, Barbot-Trystram L, Lacaille F, Chardot C, Ganousse S, Petit LM, ileostomy for at least the initial period after transplant. The ileostomy Colomb-Jung V, Dalodier E, Salomon J, Talbotec C, Campanozzi A, Ruemmele F, facilitates endoscopic biopsies to monitor for rejection but also Révillon Y, Sauvat F, Kapel N, Goulet O. Intestinal Absorption Rate in Children bypasses the absorptive surface of the colon. In general, ostomy After Small Intestinal Transplantation. Am J Clin Nutr. 2013; 97(4): 743-9. output greater than 40 mL/kg/d is considered increased. In this 4 Ubesie AC, Cole CR, Nathan JD, Tiao GM, Alonso MH, Mezoff AG, Henderson scenario, underlying infection (viral, bacterial) should be investigated, CJ, Kocoshis SA. Micronutrient Deficiencies in Pediatric and Young Adult accompanied by supportive care with fluid supplementation. Intestinal Transplant Patients. Pediatr Transplant. 2013; 17(7): 638-45. 5 Matarese LE, Dvorchik I, Costa G, Bond GJ, Koritsky DA, Ferraris RP, Touger- Following intestinal transplantation, patients are at risk for Decker R, O’Sullivan-Maillet JK, Abu-Elmagd KM. Pyridoxal-5’-Phosphate specific micronutrient deficiencies, and careful monitoring and Deficiency After Intestinal and Multivisceral Transplantation. Am J Clin Nutr. treatment are essential to avoid complications. The most 2009; 89(1): 204-9. Affiliated with the University of Pittsburgh School of Medicine, UPMC is ranked among the nation’s best hospitals by U.S. News & World Report. UPMC Division of Gastroenterology, Hepatology, and Nutrition 3 Gastric Carcinoids with Duodenal Ulcers: A Hint for Diagnosis of Multiple Endocrine Neoplasia Type I (MEN1) By Cynthia Cherfane, MD CASE PRESENTATION A 65-year-old man with a history of coronary artery disease was admitted to the hospital for non-ST segment elevation myocardial infarction (NSTEMI). He underwent percutaneous coronary intervention and was started on aspirin