Short Bowel Syndrome in Children: Current Practice and Future Perspectives

Igor Sukhotnik, MD (Editor)
The Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Laboratory of Intestinal Adaptation and Recovery, Dept. of Pediatric Surgery, Bnai Zion Medical Center, Haifa, Israel

Series: Digestive Diseases – Research and Clinical Developments
BISAC: MED031000

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Short bowel syndrome (SBS) is defined as intestinal failure following a loss of intestinal length which causes marked maldigestion and malabsorption of dietary nutrients and induces major fecal issues, loss of energy, nitrogen, and fat. Intestinal failure and SBS continue to be important clinical problems due to their high mortality and morbidity rates, as well as their devastating socioeconomic effects. Although intestinal transplantation has emerged as a feasible alternative in the treatment of children with SBS in the last two decades, intestinal adaptation remains the only chance for survival in a subset of these patients. Intestinal adaptation is defined as a process of progressive recovery from intestinal failure following bowel resection.

In this book, the etiology of SBS, its pathophysiology, and the cellular and molecular mechanisms of intestinal adaptation are reviewed. The most common complications of SBS, including intestinal failure associated liver disease and sepsis, are outlined with strategies to reduce them. The medical management and nutritional support of a patient with short bowel syndrome is complex and requires a multidisciplinary approach with the surgeon, gastroenterologist, pharmacist and dietitian working together. Using a multivariate analysis approach, the one factor which contributed most significantly to this marked improvement in survival was the implementation of a comprehensive multi-specialty care team. Other groups have reported similar improved survival rates ranging from 87% to 100% in recent years; although the precise factor which has driven this marked improvement is not precisely known.

The multidisciplinary teams created to manage the complexities of this population have shown improved outcomes. A review of the pharmacologic agents and growth factors that have been studied experimentally and administered clinically for the management of short bowel syndrome is presented. The mechanisms of action of peptide growth factors in intestinal cell proliferation as well as the effects of these factors on intestinal re-growth in an animal model of short bowel syndrome are discussed. Surgical procedures to promote intestinal adaptation and intestinal lengthening have largely changed the available options for non-transplant interventions. The potential role of tissue engineering for SBS that go beyond the “simple” generation of the tubular small intestine is discussed. Such knowledge will likely provide the basis for further advances in the treatment of patients with short bowel syndrome and suggest new therapeutic strategies to maintain gut integrity, eliminate the dependence on total parenteral nutrition, and avoid the need for intestinal transplantation.

(Imprint: Nova Biomedical )

Chapter 1. Short Bowel Syndrome: Definition, Incidence, Historical Overview and Etiology
(I. Sukhotnik, Department of Pediatric Surgery, Bnai Zion Medical Center, Technion-Israel Institute of Technology, The Ruth & Bruce Rappaport Faculty of Medicine, Haifa, Israel)

Chapter 2. Survival Outcomes of Pediatric Intestinal Failure Patients and the Cost of Comprehensive Care
(Matthew W. Ralls, Meghan A. Arnold and Daniel H. Teitelbaum, C.S. Mott Children’s Hospital, University of Michigan Hospital and Health Systems, Ann Arbor, Michigan, USA)

Chapter 3. Short Bowel Syndrome: Pathophysiology and Intestinal Adaptation
(I. Sukhotnik, Department of Pediatric Surgery, Bnai Zion Medical Center, Technion-Israel Institute of Technology, The Ruth & Bruce Rappaport Faculty of Medicine, Haifa, Israel)

Chapter 4. Mortality and Morbidity of Short Bowel Syndrome
(A.G. Coran and I. Sukhotnik, University of Michigan, Section of Pediatric Surgery, Mott Children’s Hospital, Ann Arbor, Michigan, USA and others)

Chapter 5. Intestinal Failure-Associated Liver Disease
(Ron Shaoul, Pediatric Gastroenterology Unit, Rambam Medical Center, Technion, Faculty of Medicine, Haifa, Israel)

Chapter 6. Management of Short Bowel Syndrome
(Rima Sohotnik and Ori Segol, Dept. of Gastroenterology, Carmel Medical Center, Technion-Israel Institute of Technology, The Ruth & Bruce Rappaport Faculty of Medicine, Haifa, Israel)

Chapter 7. Novel Agents in the Treatment of Intestinal Failure: Humoral Factors
(Bhani Chawla, Yongjia Feng and Daniel H. Teitelbaum, C.S. Mott Children’s Hospital, University of Michigan Hospital and Health Systems Ann Arbor, Michigan, USA)

Chapter 8. The World Experience of Pediatric Intestinal Failure Program: Successful Outcomes from Intestinal Rehabilitation
(Amnon Rofe and I. Sukhotnik, Departments of Medical Management and
Pediatric Surgery, Bnai Zion Medical Center, Technion-Israel Institute of Technology, The Ruth and Bruce Rappaport Faculty of Medicine, Haifa, Israel)

Chapter 9. Bowel-Preserving Surgery for Short Bowel Syndrome
(Yechiel Sweed, Department of Pediatric Surgery, Nahariya Medical Center, Nahariya and Faculty of Medicine in the Galilee, Bar Ilan University, Safed, Israel)

Chapter 10. Intestine Transplantation in Children
(K. Thacker and Y. Avitzur, Division of Gastroenterology, Hepatology and Nutrition, and Transplant Centre, Hospital for Sick Children, Toronto, Canada)

Chapter 11. Tissue Engineering and Regenerative Medicine Research Perspectives for Intestinal Failure
(Amulya K. Saxena, Department of Pediatric- and Adolescent Surgery, Head of Unit for Experimental Fetal Surgery and Tissue Engineering, Medical University of Graz, Graz, Austria)

Index

"Short bowel syndrome (SBS) in infants and children continues to be a major cause of morbidity and mortality in spite of all the advances that have occurred in the last thirty years in the management of congenital and acquired abnormalities of the gastrointestinal tract. This is the case currently in spite of major advances in nutritional support, both enteral and parenteralnutrition, intestinal transplantation and advanced therapies for support of the failing liver. The management of patients with short bowel syndrome is complex, requiring a comprehensive approach that frequently necessitates long-term use of parenteral nutrition." READ MORE... - Hua Yang, M.D., Ph.D., Professor and Chairman, Department of General Surgery, Xinqiao Hospital, Third Military Medical University

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