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A detailed analysis of the current status of intestinal transplantation in Latin America.
Gondolesi, GE, Doeyo, M, Solar-Muñiz, H
Current opinion in organ transplantation. 2020;(2):189-195
Abstract
PURPOSE OF REVIEW Latin America and the Caribbean represent a vast territory, with very different economic and healthcare realities, which result in significant disparities in the management of intestinal failure patients throughout the region. Since 1968, multiple attempts have been done to accomplish a successful intestinal transplant; but it was not until 2004, with the establishment of multidisciplinary programs, that large series with long-term results could be obtained. Currently, three countries (Colombia, Argentina, and Brazil) in the region are actively performing these procedures. RECENT FINDINGS A total number of 135 intestinal transplants have been performed; 11 attempts before 2004, and 124 after that period, 66 transplants were done in Argentina (42 in children), 40 in Colombia, 15 in Brazil (1 child), 2 in Costa Rica and 1 in México; 76% have been isolated, whereas 2 were done with living donors. SUMMARY Publications are still scarce, and compliance to existing registries remains limited. The challenge for the next years is to develop more 'comprehensive units' and extend home parenteral nutrition availability in the rest of the region. Regional cooperation and networking need to be set, in order to achieve regional self-sufficiency and improve long-term results.
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Nutritional Feeding Strategies in Pediatric Intestinal Failure.
Olieman, J, Kastelijn, W
Nutrients. 2020;(1)
Abstract
Intestinal failure is defined as a critical reduction of the gut mass or function, below the minimum needed to absorb nutrients and fluids. The ultimate goal in intestinal failure is to promote bowel adaptation and reach enteral autonomy while a healthy growth and development is maintained. The condition is heterogeneous and complex. Therefore, recommendations for the type and duration of parenteral, enteral, and oral nutrition are variable, with the child's age as an additional key factor. The aim of this review is to provide an overview of nutritional feeding strategies in this heterogeneous population. Different perspectives on nutritional management, nutrition and adaptation, and microbiome and nutrition will be discussed.
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Current status of pediatric intestinal transplantation in the United States.
Venick, RS, Duggan, E, Whatley, J
Current opinion in organ transplantation. 2020;(2):201-207
Abstract
PURPOSE OF REVIEW The present review aims to describe in detail the characteristics, outcomes, and recent trends in the field of pediatric intestinal transplantation in the United States. It will examine the route cause and future implications of these developments. The review will draw from recent publications in the field, the Intestinal Transplant Registry, and contemporary data from large U.S. single centers. RECENT FINDINGS More than 1500 pediatric intestinal transplants have been performed in the United States since 1985, however, over the past decade there have been fewer than 50 transplants/year nationwide. This trend is largely a result of stagnant long-term ITx outcomes and advancements in intestinal rehabilitation programs. Nationally the overall 1-year and 5-year graft survival are 68 and 50% respectively, whereas certain high-volume centers have experienced significantly better results. Sepsis is the leading cause of death following pediatric ITx, whereas rejection is the leading cause of graft loss. Chronic kidney disease and posttransplant lymphoproliferative disorder are significant and relatively prevalent long-term complications. The majority of pediatric ITx recipients receive T-cell depleting induction agents and are on Tacrolimus-based immunosuppression. Most recipient are off parenteral nutrition, but may require supplemental tube feeds. Many pediatric ITx recipients require special education, and in certain domains some report lower health related quality of life. SUMMARY As intestinal rehabilitation has improved in the modern era, the volume of pediatric ITx in the United States has decreased. Although pediatric ITx results have room for improvement nationwide, successful outcomes have been reported at experienced American centers.
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New Insights Into the Indications for Intestinal Transplantation: Consensus in the Year 2019.
Kaufman, SS, Avitzur, Y, Beath, SV, Ceulemans, LJ, Gondolesi, GE, Mazariegos, GV, Pironi, L
Transplantation. 2020;(5):937-946
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Abstract
In 2001, a Statement was published that described indications for intestinal transplantation in patients with intestinal failure expected to require parenteral nutrition indefinitely. Since 2001, advances in the management of intestinal failure including transplantation and patient survival, both on extended parenteral nutrition and after transplantation, have improved, leading to a reduction in the number of intestinal transplants worldwide from a peak of 270 per year in 2008 to 149 per year in 2017. These changes suggest that the original 2001 Statement requires reassessment. All patients with permanent intestinal failure should be managed by dedicated multidisciplinary intestinal rehabilitation teams. Under care of these teams, patients should be considered for intestinal transplantation in the event of progressive intestinal failure-associated liver disease, progressive loss of central vein access, and repeated life-threatening central venous catheter-associated infections requiring critical care. Additional indications for transplantation include large desmoid tumors and other intra-abdominal tumors with reasonable expectation of posttransplant cure, extensive mesenteric vein thrombosis and intestinal infarction, total intestinal aganglionosis, and nonrecoverable congenital secretory diarrhea. Quality of life typically improves after successful intestinal transplantation and may support the decision to proceed with transplantation when other indications are present. However, the requirement for life-long immunosuppression and its associated side effects preclude intestinal transplantation if motivated only by an expectation of improved quality of life. Increasing experience with intestinal transplantation and critical appraisal of transplant outcomes including graft survival and patient quality of life together with potential advances in immunosuppression can be expected to influence transplant practices in the future.
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The Role of a Nutrition Support Team in the Management of Intestinal Failure Patients.
Vlug, LE, Nagelkerke, SCJ, Jonkers-Schuitema, CF, Rings, EHHM, Tabbers, MM
Nutrients. 2020;(1)
Abstract
Parenteral nutrition (PN) is a complex and specialized form of nutrition support that has revolutionized the care for both pediatric and adult patients with acute and chronic intestinal failure (IF). This has led to the development of multidisciplinary teams focused on the management of patients receiving PN: nutrition support teams (NSTs). In this review we aim to discuss the historical aspects of IF management and NST development, and the practice, composition, and effectiveness of multidisciplinary care by NSTs in patients with IF. We also discuss the experience of two IF centers as an example of contemporary NSTs at work. An NST usually consists of at least a physician, nurse, dietitian, and pharmacist. Multidisciplinary care by an NST leads to fewer complications including infection and electrolyte disturbances, and better survival for patients receiving short- and long-term PN. Furthermore, it leads to a decrease in inappropriate prescriptions of short-term PN leading to significant cost reduction. Complex care for patients receiving PN necessitates close collaboration between team members and NSTs from other centers to optimize safety and effectiveness of PN use.
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A detailed analysis of the current status of intestinal transplantation in the middle east.
Nikeghbalian, S, Arasteh, P, Nikoupour, H
Current opinion in organ transplantation. 2020;(2):169-175
Abstract
PURPOSE OF REVIEW Intestinal transplantations are among the most complex transplantations, which are performed in few centers in the world. When patients develop intestinal failure, different treatment modalities including parenteral nutrition, autologous gastrointestinal tract reconstructive surgery, and intestinal transplantations are considered. The Middle East is a region where reports on intestinal failures and intestinal transplantations are mainly lacking. In the present review, we highlighted the status of intestinal transplantations in the Middle East and focused on existing reports from this region. RECENT FINDINGS Very few countries in the Middle East have the facilities for home parenteral nutrition and only two countries including Iran and Turkey perform intestinal transplantations in the region. With advances in intestinal rehabilitation units and development of autologous gastrointestinal tract reconstructive surgery, some centers have been able to reduce the number of patients in need of intestinal transplantations. SUMMARY An overview of the condition of intestinal transplantations in the Middle East shows that the issue of intestinal failure and the treatment facilities still remain an unsolved problem. Although there exists a high need for intestinal transplantation, advances in reconstructive surgeries and the development of parenteral nutrition in this region can significantly reduce the need for intestinal transplantations among patients with intestinal failure.
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[Sitosterolemia (phytosterolemia)].
Lütjohann, D
Der Internist. 2019;(8):871-877
Abstract
Sitosterolemia or phytosterolemia is a rare autosomal recessive hereditary lipid storage disorder. It is caused by homozygous or compound heterozygous mutations in one of the two ABCG5 and ABCG8 genes encoding the intestinal and hepatic heterodimer ABCG5 (sterolin 1)/ABCG8 (sterolin 2) efflux transporters. These mutations lead to intestinal hyperabsorption and reduced hepatic secretion of cholesterol and plant sterols with subsequent accumulation of phytosterols and cholesterol in plasma and deposition in tissue (xanthoma). Phytosterols are found mainly in vegetable oils, margarine, nuts, grains, soybeans and avocados. Patients with sitosterolemia show extreme phenotypic heterogeneity from almost asymptomatic individuals to those with combined severe hypercholesterolemia at a young age, leading to increased atherosclerosis and premature cardiac death. Early abnormalities include hemolytic anemia with stomatocytosis, macrothrombocytopenia and splenomegaly. In addition to strict avoidance of phytosterol-containing foods, the use of the sterol absorption inhibitor ezetimibe, possibly in combination with the bile acid-binding resin cholestyramine, is the most effective treatment option.
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Imaging of adult intestinal failure.
Smith, J, Godfrey, E, Bowden, D, Hickman, K, Sharkey, L, Butler, A, Upponi, S
Clinical radiology. 2019;(8):603-612
Abstract
Intestinal failure is the inability to maintain adequate nutrition or hydration through the gut. It is caused by a diverse range of benign and malignant aetiologies. Imaging takes a central role in the multidisciplinary assessment of patients with intestinal failure.
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Current status of graft-versus-host disease after intestinal transplantation.
Ganoza, A, Mazariegos, GV, Khanna, A
Current opinion in organ transplantation. 2019;(2):199-206
Abstract
PURPOSE OF REVIEW Over the past decades, visceral transplantation has become the standard of care for patients with irreversible intestinal failure who suffer complications of total parenteral nutrition (TPN). Graft-versus-host disease (GVHD) after solid organ transplantation is a rare but often fatal complication with high mortality. GVHD after intestinal transplantation, given the large lymphoid content of the graft, is more frequent compared with other solid organs. It is a complex condition that may have varied clinical presentations. The therapy of GVHD is multifactorial and has evolved with visceral transplantation. RECENT FINDINGS In recent large series of intestinal transplantation performed in centers around the world, GVHD remained an important cause of death (40-70%). Advances in immunology and current treatment options come from the hematopoietic stem-cell transplantation (HSCT) experience given the high prevalence of GVHD in that patient population. Therapeutic options for GVHD are based on disease classification, overall grading, organs involved, and associated symptoms. SUMMARY Graft-versus-host disease (GVHD) is a serious complication that can occur after solid organ and allogenic HSCT. Intestinal or multivisceral transplantation have the highest incidence of GVHD among all solid organ transplants with very high mortality rates. Increased risk of GVHD is present after multivisceral and liver-included transplants compared with isolated intestinal transplant. Visceral transplantation is the all-encompassing term used for transplant that includes small bowel. It includes isolated small bowel transplant, small bowel/pancreas transplant, liver/small bowel/ pancreas transplant, stomach/pancreas/small bowel (modified multivisceral transplant), and stomach/liver/pancreas/small bowel (multivisceral). Each of these may or may not include the colon as part of the allograft. Steroids remain the first line of treatment along with modulation of the primary immunosuppression. Steroid-refractory patients remain a challenge and, to date, no consensus has been achieved for a single agent second-line therapy. Successful outcome depends on early diagnosis and prompt treatment.
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Performance measures for small-bowel endoscopy: A European Society of Gastrointestinal Endoscopy (ESGE) Quality Improvement Initiative.
Spada, C, McNamara, D, Despott, EJ, Adler, S, Cash, BD, Fernández-Urién, I, Ivekovic, H, Keuchel, M, McAlindon, M, Saurin, JC, et al
United European gastroenterology journal. 2019;(5):614-641
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Abstract
The European Society of Gastrointestinal Endoscopy (ESGE) together with the United European Gastroenterology (UEG) recently developed a short list of performance measures for small-bowel endoscopy (i.e. small-bowel capsule endoscopy and device-assisted enteroscopy) with the final goal of providing endoscopy services across Europe with a tool for quality improvement. Six key performance measures both for small-bowel capsule endoscopy and for device-assisted enteroscopy were selected for inclusion, with the intention being that practice at both a service and endoscopist level should be evaluated against them. Other performance measures were considered to be less relevant, based on an assessment of their overall importance, scientific acceptability, and feasibility. Unlike lower and upper gastrointestinal endoscopy, for which performance measures had already been identified, this is the first time small-bowel endoscopy quality measures have been proposed.