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Paul W. Wales - One of the best experts on this subject based on the ideXlab platform.
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exogenous glucagon like peptide 2 improves outcomes of intestinal adaptation in a distal intestinal resection neonatal piglet model of Short Bowel Syndrome
Pediatric Research, 2014Co-Authors: Megha Suri, Patricia L Brubaker, David L Sigalet, Justine M. Turner, Pamela R. Wizzard, Patrick N. Nation, R. O. Ball, Paul B. Pencharz, Paul W. WalesAbstract:Exogenous glucagon-like peptide-2 improves outcomes of intestinal adaptation in a distal-intestinal resection neonatal piglet model of Short Bowel Syndrome
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novel neonatal piglet models of surgical Short Bowel Syndrome with intestinal failure
Journal of Pediatric Gastroenterology and Nutrition, 2011Co-Authors: Justine M. Turner, Pamela R. Wizzard, Patrick N. Nation, R. O. Ball, Paul W. Wales, Christine Pendlebury, Consolato Sergi, Paul B. PencharzAbstract:OBJECTIVES: Short Bowel Syndrome occurring after surgery for acquired or congenital intestinal abnormalities causes considerable neonatal morbidity and mortality. Animal models are a valuable research tool for this problem; however, few successful neonatal models have been developed and most do not include distal intestinal resection as seen commonly in human babies. We report novel piglet models addressing these gaps. SUBJECTS AND METHODS: Neonatal piglets (1-6 days) underwent venous and gastric catheter insertion and 75% intestinal resection. Group 1 (n = 6) had midintestinal resection with jejunoileal anastomosis; group 2 (n = 5) had distal intestinal resection with jejunocolic anastomosis; group 3 (n = 5) were sham controls; and group 4 (n = 5) were sow reared. Postoperatively, groups 1 to 3 piglets commenced parenteral nutrition (PN), and enteral nutrition was introduced and advanced using a standard regimen. Data collection included days on PN, weight gain, fat absorption, small intestine lengthening, and Bowel/liver histology. RESULTS: Group 2 piglets had more days on PN (P = 0.008), less weight gain (P = 0.027), and greater malabsorption (P = 0.012). They did not show small intestine lengthening and had more cholestatic liver disease. Group 1 piglets had histological evident intestinal adaptation and 1.5-fold intestinal lengthening (P = 0.001). CONCLUSIONS: These novel piglet models of Short Bowel Syndrome are the first to represent the full clinical spectrum of intestinal failure as observed in human neonates. By considering the impact of different Short Bowel anatomy on potential for adaptation and growth, these animal models are a significant advance. They permit evaluation of new therapies to promote intestinal adaptation and reduce complications, such as cholestasis.
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does the colon play a role in intestinal adaptation in infants with Short Bowel Syndrome a multiple variable analysis
Journal of Pediatric Surgery, 2010Co-Authors: Ivan Diamond, Nicole De Silva, Mariechantal Struijs, Paul W. WalesAbstract:Abstract Purpose We sought to examine in a multiple variable model the impact of residual colonic length on time to intestinal adaptation in a cohort of infants with Short Bowel Syndrome. Method Infants with a surgical diagnosis of Short Bowel Syndrome who underwent operation 90 days or younger were included in this analysis. Univariate Cox proportional hazards models for time to full-enteral feeds were developed. Predictors significant at the .2 level were entered into a stepwise multiple variable Cox proportional hazards model. Results A total of 106 infants were included in the cohort (70 adapted). Predictors meeting the criteria for the multiple variable model were as follows: multidisciplinary management ( P = .045), Serial Transverse Enteroplasty Procedure ( P = .057), percent small Bowel ( P P P = .001), number of septic ( P P Conclusions The colon does not seem to play a significant role in intestinal adaptation. However, in addition to highlighting the importance of residual small Bowel length, our model highlights the benefit of multidisciplinary intestinal rehabilitation and reduction of septic complications in achieving intestinal adaptation.
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Short Bowel Syndrome epidemiology and etiology
Seminars in Pediatric Surgery, 2010Co-Authors: Paul W. Wales, Emily R ChristisonlagayAbstract:Pediatric Short Bowel Syndrome (SBS) is most commonly caused by congenital or acquired conditions of the newborn. SBS is associated with an inability of the Bowel to adequately absorb water and nutrients in sufficient quantities to meet caloric, fluid, and electrolyte demands, thus necessitating dependence on parenteral nutrition (PN). It is this dependence on PN, that is responsible for the majority of morbidity and mortality associated with SBS, including central venous catheter infections and PN-induced cholestatic liver dysfunction. There are very few estimates of SBS incidence and mortality in the literature. The epidemiology of SBS is reviewed and the limitations of the published literature are discussed.
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changing the paradigm omegaven for the treatment of liver failure in pediatric Short Bowel Syndrome
Journal of Pediatric Gastroenterology and Nutrition, 2009Co-Authors: Ivan R Diamond, Paul B. Pencharz, Jae H Kim, Anca Sterescu, Paul W. WalesAbstract:ABSTRACTBackground:Parenteral omega-3 fatty acids, such as Omegaven, may benefit patients with pediatric Short Bowel Syndrome (SBS) who develop parenteral nutrition–associated liver disease (PNALD).Patients and Methods:Retrospective cohort describing the outcome of all 12 children with SBS and advan
Jon A. Vanderhoof - One of the best experts on this subject based on the ideXlab platform.
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enteric microbial flora bacterial overgrowth and Short Bowel Syndrome
Clinical Gastroenterology and Hepatology, 2006Co-Authors: John K. Dibaise, Rosemary J Young, Jon A. VanderhoofAbstract:Small intestinal bacterial overgrowth (SIBO) occurs commonly in Short-Bowel Syndrome (SBS) and, in some instances, may result in significant problems. SIBO is characterized by a variety of signs and symptoms resulting from nutrient malabsorption caused by an increased number and/or type of bacteria in the small intestine. The anatomic and physiologic changes that occur in SBS together with medications commonly used in these patients facilitate the development of SIBO. Because many aspects related to SIBO in the SBS population remain poorly understood, it was our aim to review the current understanding of the gut flora and issues related to SIBO occurring in SBS.
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Intestinal Rehabilitation and the Short Bowel Syndrome: Part 2
The American journal of gastroenterology, 2004Co-Authors: John K. Dibaise, Rosemary J Young, Jon A. VanderhoofAbstract:The management of patients with intestinal failure due to Short Bowel Syndrome is complex, requiring a comprehensive approach that frequently necessitates long-term, if not life-long, use of parenteral nutrition. Despite tremendous advances in the provision of parenteral nutrition over the past three decades, which have allowed significant improvements in the survival and quality of life of these patients, this mode of nutritional support carries with it significant risks to the patient, is very costly, and ultimately, does not attempt to improve the function of the remaining Bowel. Intestinal rehabilitation refers to the process of restoring enteral autonomy, and thus, allowing freedom from parenteral nutrition, usually by means of dietary, medical, and occasionally, surgical strategies. While recent investigations have focused on the use of trophic substances to increase the absorptive function of the remaining gut, whether intestinal rehabilitation occurs as a consequence of enhanced Bowel adaptation or is simply a result of an optimized, comprehensive approach to the care of these patients remains unclear. In Part 1 of this review, we provided an overview of Short Bowel Syndrome and pathophysiological considerations related to the remaining Bowel anatomy in these patients. We also reviewed intestinal adaptation and factors that may enhance the adaptive process, focusing on evidence derived from animal studies. In Part 2, relevant data on the development of intestinal adaptation in humans are reviewed as is the general management of Short Bowel Syndrome. Lastly, the potential benefits of a multidisciplinary intestinal rehabilitation program in the care of these patients are also discussed.
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treatment strategies for small Bowel bacterial overgrowth in Short Bowel Syndrome
Journal of Pediatric Gastroenterology and Nutrition, 1998Co-Authors: Jon A. Vanderhoof, Rosemary J Young, Nancy D Murray, Stuart S KaufmanAbstract:Background: Small Bowel bacterial overgrowth is a common complication of Short Bowel Syndrome, and although it is often controlled with antimicrobial therapy, alternative strategies may occasionally be needed. Methods: Six patients with bacterial overgrowth are described, who did not respond to antimicrobial therapy and required additional medical or surgical measures to control the overgrowth. Results: Recalcitrant bacterial overgrowth was successfully treated with periodic small Bowel irrigation with a balanced hypertonic electrolyte solution, colonic flushes, encouraging frequent stooling, intestinal lengthening procedure, or probiotic therapy with Lactobacillus plantarum 299V and Lactobacillus GG. Conclusions: Small Bowel bacterial overgrowth should be aggressively evaluated in patients with Short Bowel Syndrome who are not progressing in a normal manner. Inadequate or incomplete response to antibiotic therapy is common, and several additional treatment possibilities are available.
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influence of bacterial overgrowth and intestinal inflammation on duration of parenteral nutrition in children with Short Bowel Syndrome
The Journal of Pediatrics, 1997Co-Authors: Stuart S Kaufman, Rosemary J Young, Nancy D Murray, Candace A Loseke, James V Lupo, Lewis W Pinch, Jon A. VanderhoofAbstract:Objectives Massive intestinal resection results in Short Bowel Syndrome and necessitatesprolonged parenteral feeding. The purpose of this work was to assess the impact of late complications of Short Bowel Syndrome, including intestinal bacterial overgrowth and enterocolitis, on the duration of parenteral nutrition (PN) in comparison with factors evident in the neonatal period. Methods Retrospective chart review. Results Of 49 children, 42 were weaned from parenteral nutrition after a treatment course of 17 ±14 months. In these 42, postresection small intestinal length equaled 81 ±65 cm; 45% had an ileocecal valve. Small intestinal length in the seven children who were PN dependent was 31 ±30 cm (p
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Short-Bowel Syndrome in children and adults
Gastroenterology, 1997Co-Authors: Jon A. Vanderhoof, Alan Norman LangnasAbstract:Short-Bowel Syndrome is the malabsorptive state that follows extensive resection of the small intestine. Potential long-term survival without parenteral nutrition heavily depends on stimulation of the process of intestinal adaptation, through which the remaining small intestine gradually increases its absorptive capacity. This process is heavily nutrient dependent, and aggressive use of enteral nutrition is required to stimulate its completion. A combination of osmotic sensitivities, nutrient malabsorption, Bowel dilatation and dysmotility, and changes in bacterial flora influence the symptoms and the management of this disorder. Chronic complications include parenteral nutrition-induced liver disease, nutrient deficiency states, and, frequently, small Bowel bacterial overgrowth. Intestinal transplantation has been successfully developed in some centers in the United States, and preliminary experience suggest a long-term survival of 50%-75%, better in patients receiving an isolated intestinal transplant than a combined liver/Bowel transplant. The ultimate role of intestinal transplantation is still undergoing evaluation.
Palle Jeppesen - One of the best experts on this subject based on the ideXlab platform.
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long term teduglutide for the treatment of patients with intestinal failure associated with Short Bowel Syndrome
Clinical and translational gastroenterology, 2016Co-Authors: Lauren K. Schwartz, Stephen J Okeefe, Nader N Youssef, Ulrich-frank Pape, Ken Fujioka, S M Gabe, Georg Lamprecht, Benjamin Li, Palle JeppesenAbstract:Long-Term Teduglutide for the Treatment of Patients With Intestinal Failure Associated With Short Bowel Syndrome
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gut hormones in the treatment of Short Bowel Syndrome and intestinal failure
Current Opinion in Endocrinology Diabetes and Obesity, 2015Co-Authors: Palle JeppesenAbstract:PURPOSE OF REVIEW: The approval of teduglutide, a recombinant analog of human glucagon-like peptide (GLP) 2, by the US Food and Drug Administration (Gattex) and the European Medicines Agency (Revestive) has illustrated the potential of selected gut hormones as treatments in patients with Short-Bowel Syndrome and intestinal failure. Gut hormones may improve the structural and functional intestinal adaptation following intestinal resection by decreasing a rapid gastric emptying and hypersecretion, by increasing the intestinal blood flow, and by promoting intestinal growth. This review summarizes the findings from phase 2 and 3 teduglutide studies, and pilot studies employing GLP-1 and agonists for this orphan condition. RECENT FINDINGS: In a 3-week, phase 2, metabolic balance study, teduglutide increased the intestinal wet weight absorption by approximately 700 g/day and reduced fecal energy losses by approximately 0.8 MJ/day (∼200 Kcal/day). In two subsequent 24-week, phase 3 studies, teduglutide reduced the need for parenteral support in the same magnitude. Adverse events were mainly of gastrointestinal origin and consistent with the known mechanism of action of teduglutide. Pilot studies suggest that GLP-1 may be less potent. Synergistic effects may be seen by co-treatment with GLP-2. SUMMARY: Gut hormones promote intestinal adaptation and absorption, decreasing fecal losses, thereby decreasing or even eliminating the need for parenteral support. This will aid the intestinal rehabilitation in these severely disabled Short-Bowel Syndrome patients.
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spectrum of Short Bowel Syndrome in adults intestinal insufficiency to intestinal failure
Journal of Parenteral and Enteral Nutrition, 2014Co-Authors: Palle JeppesenAbstract:Short Bowel Syndrome (SBS) refers to the malabsorptive state caused by physical or functional loss of portions of the small intestine, most commonly following extensive intestinal resection. Such resections hinder absorption of adequate amounts of macronutrients, micronutrients, electrolytes, and water, resulting in malnutrition, diarrhea, and dehydration. Clinical features of SBS vary along a continuum, depending on the extent and anatomy of intestine lost and the ability of the patient and the remaining intestine to compensate for the loss. The impact of SBS can be extensive, leading to diminished health-related quality of life because of its many physical and psychological effects on patients. SBS is associated with decreased survival; risk factors for SBS-related mortality include very Short remnant small Bowel, end-jejunal remnant anatomy, and arterial mesenteric infarction as primary cause. Although parenteral nutrition and/or intravenous fluid (PN/IV) is a life-saving measure for many patients with...
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acute effects of the glucagon like peptide 2 analogue teduglutide on intestinal adaptation in Short Bowel Syndrome
Journal of Pediatric Gastroenterology and Nutrition, 2014Co-Authors: T Thymann, Palle Jeppesen, Barbara J Stoll, Lars Mecklenburg, Douglas G Burrin, Andreas Vegge, Niels Qvist, Thomas Eriksen, Per T SangildAbstract:: Neonatal Short Bowel Syndrome following massive gut resection is associated with malabsorption of nutrients. The intestinotrophic factor glucagon-like peptide 2 (GLP-2) improves gut function in adult patients with Short Bowel Syndrome, but its effect in pediatric patients remains unknown. Our objective was to test the efficacy of the long-acting synthetic human GLP-2 analogue, teduglutide (ALX-0600), in a neonatal piglet jejunostomy model. Two-day-old pigs were subjected to resection of 50% of the small intestine (distal part), and the remnant intestine was exteriorized on the abdominal wall as a jejunostomy. All pigs were given total parenteral nutrition for 7 days and a single daily injection of the following doses of teduglutide: 0.01 (n = 6), 0.02 (n = 6), 0.1 (n = 5), or 0.2 mg · kg · day (n = 6), and compared with placebo (n = 9). Body weight increment was similar for all 4 teduglutide groups but higher than placebo (P < 0.05). There was a dose-dependent increase in weight per length of the remnant intestine (P < 0.01) and fractional protein synthesis rate in the intestine was increased in the 0.2 mg · kg · day group versus placebo (P < 0.001); however, functional and structural endpoints including activity of digestive enzymes, absorption of enteral nutrients, and immunohistochemistry (Ki67, villin, FABP2, ChgA, and GLP-2R) were not affected by the treatment. Teduglutide induces trophicity on the remnant intestine but has limited acute effects on functional endpoints. Significant effects of teduglutide on gut function may require a longer adaptation period and/or a more frequent administration of the peptide. In perspective, GLP-2 or its analogues may be relevant to improve intestinal adaptation in pediatric patients with Short Bowel Syndrome.
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glucagon like peptide 2 stimulates mucosal microcirculation measured by laser doppler flowmetry in end jejunostomy Short Bowel Syndrome patients
Regulatory Peptides, 2013Co-Authors: P Hoyerup, Per M Hellstrom, Peter T Schmidt, Christopher F Brandt, C Askovhansen, P B Mortensen, Palle JeppesenAbstract:Abstract Background In animal and human studies glucagon-like peptide-2 (GLP-2) has been shown to increase blood flow in the superior mesenteric artery and the portal vein. This study describes the effect of GLP-2 measured directly on the intestinal mucosal blood flow by laser Doppler flowmetry (LDF) in end-jejunostomy Short Bowel Syndrome (SBS) patients. Methods In five SBS patients with end-jejunostomy a specially designed laser Doppler probe was inserted into the stoma nipple, and blood flow measured directly on the jejunal mucosa for 105 min in relation to no treatment, systemic saline infusion, topical adrenaline application and a subcutaneous injection of 800 μg native GLP-2. Results The GLP-2 injection increased jejunal mucosal blood flow by 79 ± 37% compared to conditions, where no treatment was given (p Conclusions GLP-2 raises jejunal microcirculation in SBS patients with end-jejunostomy. This may explain the redness and increase in the end-jejunostomy nipple size imminently after commencing GLP-2 injections. The potential beneficial effects of this GLP-2-mediated increase of blood flow in the mesenteric bed should be investigated in clinical conditions other than the Short Bowel Syndrome.
Tom Jaksic - One of the best experts on this subject based on the ideXlab platform.
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necrotizing enterocolitis is associated with earlier achievement of enteral autonomy in children with Short Bowel Syndrome
Journal of Pediatric Surgery, 2016Co-Authors: Eric A Sparks, Biren P Modi, Christopher Duggan, Bram P Raphael, Faraz A Khan, Jeremy G Fisher, Brenna S Fullerton, Amber M Hall, Tom JaksicAbstract:Purpose Necrotizing enterocolitis (NEC) remains one of the most common underlying diagnoses of Short Bowel Syndrome (SBS) in children. The relationship between the etiology of SBS and ultimate enteral autonomy has not been well studied. This investigation sought to evaluate the rate of achievement of enteral autonomy in SBS patients with and without NEC.
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neonatal Short Bowel Syndrome
Seminars in Fetal & Neonatal Medicine, 2011Co-Authors: Ivan M Gutierrez, Kuang Horng Kang, Tom JaksicAbstract:Neonatal Short Bowel Syndrome is a disease with a high morbidity and mortality. The management of these patients is complex and requires a multidisciplinary approach. Recent advances in medical and surgical treatment options have improved outcomes. The following review highlights salient points in the management of this challenging patient population.
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cisapride improves enteral tolerance in pediatric Short Bowel Syndrome with dysmotility
Journal of Pediatric Gastroenterology and Nutrition, 2011Co-Authors: Bram P Raphael, Tom Jaksic, Samuel Nurko, Hongyu Jiang, Kristen Hart, Daniel Kamin, Christopher DugganAbstract:Background and Objectives: Gastrointestinal dysmotility is common in pediatric Short-Bowel Syndrome, leading to prolonged parenteral nutrition dependence. There is limited literature regarding the safety and efficacy of cisapride for this indication. The aim of the study was to describe the safety and efficacy of cisapride for enteral intolerance in pediatric Short-Bowel Syndrome. Methods: Open-labeled pilot study in a limited access program for cisapride. Indications were Short-Bowel Syndrome with underlying dysmotility and difficulty advancing enteral feeds despite standard therapies and without evidence of anatomic obstruction. Patients received cisapride 0.1 to 0.2 mg/kg per dose for 3 to 4 doses per day. We collected electrocardiogram, nutrition, and anthropometric data prospectively at study visits. Results: Ten patients with mean (SD) age of 30.3 (30.5) months were enrolled in our multidisciplinary pediatric intestinal rehabilitation program. Median (interquartile range [IQR]) duration of follow-up was 8.7 (3.1–14.3) months. Median (IQR) residual Bowel length was 102 (85–130) cm. Median (IQR) citrulline level was 14.5 (10.5–31.3) μmol/L. Diagnoses included isolated gastroschisis (n = 3), gastroschisis with intestinal atresia (n = 4), necrotizing enterocolitis (n = 2), and long-segment Hirschsprung disease (n = 1). Six subjects had at least 1 prior Bowel-lengthening procedure. Median (IQR) change in percentage enteral energy intake was 19.9% (15.4%–29.8%) during follow-up (P = 0.01). Seven patients improved in enteral tolerance during treatment and 2 were weaned completely from parenteral nutrition. Complications during therapy were prolonged corrected QT interval (n = 2), gastrointestinal bleeding (n = 2), D-lactic acidosis (n = 1), and death due to presumed sepsis (n = 1). Longitudinal analysis (general estimating equation model) showed a strong positive association between cisapride duration and improved enteral tolerance. Mean percentage of enteral intake increased by 2.9% for every month of cisapride treatment (P Conclusions: Cisapride is a potentially useful therapy in patients with pediatric Short-Bowel Syndrome with gastrointestinal dysmotility. We observed modest improvement in feeding tolerance where prior treatments failed; however, patients treated with cisapride require careful cardiac monitoring because corrected QT prolongation occurred in 20% of our cohort.
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improved survival in a multidisciplinary Short Bowel Syndrome program
Journal of Pediatric Surgery, 2008Co-Authors: Biren P Modi, Tom Jaksic, Monica Langer, Avery Y Ching, Clarissa Valim, Stephen D Waterford, Julie Iglesias, Debora Duro, Christopher DugganAbstract:Purpose Pediatric Short Bowel Syndrome (SBS) remains a management challenge with significant mortality. In 1999, we initiated a multidisciplinary pediatric intestinal rehabilitation program. The purpose of this study was to determine if the multidisciplinary approach was associated with improved survival in this patient population.
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serial transverse enteroplasty enhances intestinal function in a model of Short Bowel Syndrome
Annals of Surgery, 2006Co-Authors: Robert W Chang, Patrick J Javid, Steven Andreoli, Heung Bae Kim, Dario O Fauza, Tom JaksicAbstract:Objective/Summary Background Data: Serial transverse enteroplasty (STEP) is a new intestinal lengthening procedure that has been shown to clinically increase Bowel length. This study examined the impact of the STEP procedure upon intestinal function in a model of Short Bowel Syndrome. Methods: Young pigs (n 10) had a reversed segment of Bowel interposed to induce Bowel dilatation. Five pigs underwent a 90% Bowel resection with a STEP procedure on the remaining dilated Bowel while 5 served as controls and had a 90% Bowel resection without a STEP procedure. Determinations of nutritional status, absorptive capacity, and bacterial overgrowth were conducted 6 weeks after resection. Statistical comparisons were made by 2-sample t test (significance at P 0.05). Results: The STEP procedure lengthened the Bowel from 105.2 7.7 cm to 152.2 8.3 cm (P 0.01). The STEP animals showed improved weight retention compared with controls (mean, 0.5% 1.8% body weight versus 17.6% 1.5%, P 0.001). Intestinal carbohydrate absorption, as measured by D-Xylose absorption and fat absorptive capacity as measured by serum vitamin D and triglyceride levels, were increased in the STEP group versus controls. Serum citrulline, a marker of intestinal mucosal mass, was significantly elevated in the STEP pigs compared with controls. None of the STEP animals but 4 of 5 control animals were noted to have gram-negative bacterial overgrowth in the proximal Bowel. Conclusions: STEP improves weight retention, nutritional status, intestinal absorptive capacity, and serum citrulline levels in a porcine Short Bowel model. A salutary effect upon bacterial overgrowth was also noted. These data support the use of this operation in Short Bowel Syndrome.
Paul B. Pencharz - One of the best experts on this subject based on the ideXlab platform.
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exogenous glucagon like peptide 2 improves outcomes of intestinal adaptation in a distal intestinal resection neonatal piglet model of Short Bowel Syndrome
Pediatric Research, 2014Co-Authors: Megha Suri, Patricia L Brubaker, David L Sigalet, Justine M. Turner, Pamela R. Wizzard, Patrick N. Nation, R. O. Ball, Paul B. Pencharz, Paul W. WalesAbstract:Exogenous glucagon-like peptide-2 improves outcomes of intestinal adaptation in a distal-intestinal resection neonatal piglet model of Short Bowel Syndrome
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novel neonatal piglet models of surgical Short Bowel Syndrome with intestinal failure
Journal of Pediatric Gastroenterology and Nutrition, 2011Co-Authors: Justine M. Turner, Pamela R. Wizzard, Patrick N. Nation, R. O. Ball, Paul W. Wales, Christine Pendlebury, Consolato Sergi, Paul B. PencharzAbstract:OBJECTIVES: Short Bowel Syndrome occurring after surgery for acquired or congenital intestinal abnormalities causes considerable neonatal morbidity and mortality. Animal models are a valuable research tool for this problem; however, few successful neonatal models have been developed and most do not include distal intestinal resection as seen commonly in human babies. We report novel piglet models addressing these gaps. SUBJECTS AND METHODS: Neonatal piglets (1-6 days) underwent venous and gastric catheter insertion and 75% intestinal resection. Group 1 (n = 6) had midintestinal resection with jejunoileal anastomosis; group 2 (n = 5) had distal intestinal resection with jejunocolic anastomosis; group 3 (n = 5) were sham controls; and group 4 (n = 5) were sow reared. Postoperatively, groups 1 to 3 piglets commenced parenteral nutrition (PN), and enteral nutrition was introduced and advanced using a standard regimen. Data collection included days on PN, weight gain, fat absorption, small intestine lengthening, and Bowel/liver histology. RESULTS: Group 2 piglets had more days on PN (P = 0.008), less weight gain (P = 0.027), and greater malabsorption (P = 0.012). They did not show small intestine lengthening and had more cholestatic liver disease. Group 1 piglets had histological evident intestinal adaptation and 1.5-fold intestinal lengthening (P = 0.001). CONCLUSIONS: These novel piglet models of Short Bowel Syndrome are the first to represent the full clinical spectrum of intestinal failure as observed in human neonates. By considering the impact of different Short Bowel anatomy on potential for adaptation and growth, these animal models are a significant advance. They permit evaluation of new therapies to promote intestinal adaptation and reduce complications, such as cholestasis.
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changing the paradigm omegaven for the treatment of liver failure in pediatric Short Bowel Syndrome
Journal of Pediatric Gastroenterology and Nutrition, 2009Co-Authors: Ivan R Diamond, Paul B. Pencharz, Jae H Kim, Anca Sterescu, Paul W. WalesAbstract:ABSTRACTBackground:Parenteral omega-3 fatty acids, such as Omegaven, may benefit patients with pediatric Short Bowel Syndrome (SBS) who develop parenteral nutrition–associated liver disease (PNALD).Patients and Methods:Retrospective cohort describing the outcome of all 12 children with SBS and advan
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neonatal Short Bowel Syndrome outcomes after the establishment of the first canadian multidisciplinary intestinal rehabilitation program preliminary experience
Journal of Pediatric Surgery, 2007Co-Authors: Ivan Diamond, Paul B. Pencharz, Nicole De Silva, Jae H Kim, Paul W. WalesAbstract:Abstract Background A multidisciplinary intestinal rehabilitation team has been in place at our institution for 3 years. Our goal was to compare the preliminary outcomes of neonates with Short Bowel Syndrome before with those after the establishment of our formalized intestinal failure program (Group for the Improvement of Intestinal Function and Treatment [GIFT]). Methods We conducted a retrospective analysis of our intestinal failure registry comparing preGIFT (1997-1999) with GIFT (2003-2005) outcomes. Results Fifty-four patients (mean gestational age=33.5 weeks) were included in the GIFT cohort, and 40 patients (mean gestational age=30.7 weeks) formed the preGIFT cohort. Overall mortality rates (33.3% vs 37.5%, P = .84) were equivalent in the 2 cohorts, although fewer patients died of liver failure after the establishment of the GIFT. Among those with liver failure, the mortality in the preGIFT group was 9/10 as compared with that of 6/13 in the GIFT group ( P = .03). The decrease in liver-related deaths was partly attributable to earlier referral for and increased survival to transplant (4 for the GIFT group vs 0 for the preGIFT group). Conclusions Analysis of the preliminary outcomes of the GIFT program suggests that the natural history of neonatal Short Bowel Syndrome remains unaltered to date despite a coordinated approach to care. However, improved communication and integration with the transplant service have resulted in earlier assessment, increased rates of transplantation, and decreased mortality from liver failure.
