920 results on '"Short bowel syndrome"'
Search Results
2. [Ladd's syndrome in older children].
- Author
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Svarich VG, Kagantsov IM, and Svarich VA
- Subjects
- Humans, Child, Abdominal Pain, Radiography, Vomiting, Intestines surgery, Short Bowel Syndrome
- Abstract
Most often, Ladd's syndrome clinically manifests within the first month after birth. There are few reports devoted to diagnosis of this syndrome in patients aged 6-10 years. We present 2 children with Ladd's syndrome. The disease manifested at the age of 14 years in both patients. One child was diagnosed before complications that made it possible to carry out successful surgical treatment with subsequent recovery. The second child had necrosis of rotated small bowel. This child died in long-term postoperative period due to short bowel syndrome despite adequate treatment. In some cases, Ladd's syndrome manifests in puberty. Contrast-enhanced X-ray examination of gastrointestinal tract is indicated in children for periodic abdominal pain associated or not associated with vomiting.
- Published
- 2022
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3. Therapeutic potential of an intestinotrophic hormone, glucagon-like peptide 2, for treatment of type 2 short bowel syndrome rats with intestinal bacterial and fungal dysbiosis.
- Author
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Hu X, Cheng W, Fan S, Huang Y, Chen X, Jiang Z, and Wang J
- Subjects
- Actinobacteria genetics, Actinobacteria isolation & purification, Animals, Colon surgery, Colostomy, Discriminant Analysis, Disease Models, Animal, Dysbiosis, Fungi genetics, Fungi isolation & purification, Glucagon-Like Peptide 2 therapeutic use, Least-Squares Analysis, Male, Principal Component Analysis, RNA, Ribosomal, 16S analysis, RNA, Ribosomal, 16S metabolism, Rats, Rats, Sprague-Dawley, Short Bowel Syndrome drug therapy, Short Bowel Syndrome microbiology, Gastrointestinal Microbiome drug effects, Glucagon-Like Peptide 2 pharmacology, Intestines microbiology, Short Bowel Syndrome pathology
- Abstract
Background: Previous studies showed that type 2 short bowel syndrome (SBS) rats were accompanied by severe intestinal bacterial dysbiosis. Limited data are available for intestinal fungal dysbiosis. Moreover, no effective therapeutic drugs are available for these microbiota dysbiosis. The aims of our study were to investigate the therapeutic potential of glucagon-like peptide 2 (GLP-2) for these microbiota dysbiosis in type 2 SBS rats., Methods: 8-week-old male SD rats which underwent 80% small bowel resection, ileocecum resection, partial colon resection and jejunocolostomy, were treated with saline (SBS group, n = 5) or GLP-2 (GLP2.SBS group, n = 5). The Sham group rats which underwent transection and re-anastomosis were given a saline placebo (Sham group, n = 5). 16S rRNA and ITS sequencing were applied to evaluate the colonic bacterial and fungal composition at 22 days after surgery, respectively., Results: The relative abundance of Actinobacteria, Firmicutes and proinflammatory Proteobacteria increased significantly in SBS group rats, while the relative abundance of Bacteroidetes, Verrucomicrobia and Tenericutes decreased remarkably. GLP-2 treatment significantly decreased Proteus and increased Clostridium relative to the saline treated SBS rats. The diversity of intestinal fungi was significantly increased in SBS rats, accompanied with some fungi abnormally increased and some resident fungi (e.g., Penicillium) significantly decreased. GLP-2 treatment significantly decreased Debaryomyces and Meyerozyma, and increased Penicillium. Moreover, GLP-2 partially restored the bacteria-fungi interkingdom interaction network of SBS rats., Conclusion: Our study confirms the bacterial and fungal dysbiosis in type 2 SBS rats, and GLP-2 partially ameliorated these microbiota dysbiosis.
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- 2021
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4. In Vivo Transplantation of Human Intestinal Organoids Enhances Select Tight Junction Gene Expression.
- Author
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Boyle MA, Sequeira DJ, McNeill EP, Criss ZK 2nd, Shroyer NF, and Speer AL
- Subjects
- Animals, Cell Culture Techniques methods, Cell Line, Gene Expression Regulation, Human Embryonic Stem Cells, Humans, Male, Mice, Models, Animal, Tight Junctions metabolism, Intestines cytology, Organoids transplantation, Short Bowel Syndrome surgery, Tight Junction Proteins metabolism, Tissue Engineering
- Abstract
Background: Short bowel syndrome is a potentially fatal condition with inadequate management options. Tissue-engineered small intestine (TESI) is a promising solution, but confirmation of TESI function will be crucial before human application. We sought to define intestinal epithelial barrier function in human intestinal organoid (HIO)-derived TESI., Materials and Methods: HIOs were generated in vitro from human embryonic stem cells. After 1 mo, HIOs were collected for analysis or transplanted into the kidney capsule of immunocompromised mice. Transplanted HIOs (tHIOs) were harvested for analysis at 4 or 8 wk. Reverse transcription quantitative polymerase chain reaction and immunofluorescent staining were performed for tight junction components: claudin 3 (CLDN3), claudin 15 (CLDN15), occludin (OCLN), and zonula occludens-1, or tight junction protein-1 (TJP1/ZO-1)., Results: Four-week-old tHIOs demonstrated significantly (P < 0.05) higher levels of CLDN15 (6x), OCLN (4x), and TJP1/ZO-1 (3x) normalized to GAPDH than in vitro HIOs. Eight-week-old tHIOs demonstrated significantly (P < 0.05) higher expression levels of CLDN3 (26x), CLDN15 (29x), OCLN (4x), and TJP1/ZO-1 (5x) than in vitro HIOs. There was no significant difference in expression of these tight junction components between 4- and 8-week-old tHIOs. Immunofluorescent staining revealed the presence of claudin 3, claudin 15, occludin, and zonula occludens-1 in both in vitro HIOs and tHIOs; however, the morphology appeared more mature in tHIOs., Conclusions: In vitro HIOs have lower levels of tight junction mRNA, and tight junction proteins appear morphologically immature. Transplantation facilitates maturation of the HIOs and enhances select tight junction gene expression., (Copyright © 2020 Elsevier Inc. All rights reserved.)
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- 2021
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5. [Interdisciplinary endoscopy in children].
- Author
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Tafazzoli K, Kohl M, Kirstein M, and Wünsch L
- Subjects
- Child, Humans, Infant, Endoscopy, Intestines
- Abstract
Introduction: Endoscopic diagnostics and interventions in children require a high level of expertise from different fields. The small dimensions, the vulnerability of the patients and the rarity of the diseases and problems as well as the necessity for the most modern endoscopic technology can only be mastered by an adequately constructed team., Methods: We describe the typical indications, personnel and technical requirements and make suggestions for process organization. The necessity for an interdisciplinary approach is described using three illustrative examples., Result: No single specialty alone can cope with the manifold challenges of pediatric endoscopy. The organization should therefore favor low-threshold collaborations., Outlook: Further development of techniques is needed especially in the field of premature infant care and children with intestinal failure and motility disorders.
- Published
- 2021
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6. Optimizing Nutritional Strategies to Prevent Necrotizing Enterocolitis and Growth Failure after Bowel Resection.
- Author
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Moschino L, Duci M, Fascetti Leon F, Bonadies L, Priante E, Baraldi E, and Verlato G
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- Enterocolitis, Necrotizing complications, Enterocolitis, Necrotizing diagnosis, Enterocolitis, Necrotizing surgery, Humans, Infant, Infant, Newborn, Infant, Premature, Infant, Premature, Diseases diagnosis, Infant, Premature, Diseases surgery, Short Bowel Syndrome etiology, Short Bowel Syndrome prevention & control, Enterocolitis, Necrotizing prevention & control, Failure to Thrive prevention & control, Infant Nutritional Physiological Phenomena, Infant, Premature, Diseases prevention & control, Intestines surgery
- Abstract
Necrotizing enterocolitis (NEC), the first cause of short bowel syndrome (SBS) in the neonate, is a serious neonatal gastrointestinal disease with an incidence of up to 11% in preterm newborns less than 1500 g of birth weight. The rate of severe NEC requiring surgery remains high, and it is estimated between 20-50%. Newborns who develop SBS need prolonged parenteral nutrition (PN), experience nutrient deficiency, failure to thrive and are at risk of neurodevelopmental impairment. Prevention of NEC is therefore mandatory to avoid SBS and its associated morbidities. In this regard, nutritional practices seem to play a key role in early life. Individualized medical and surgical therapies, as well as intestinal rehabilitation programs, are fundamental in the achievement of enteral autonomy in infants with acquired SBS. In this descriptive review, we describe the most recent evidence on nutritional practices to prevent NEC, the available tools to early detect it, the surgical management to limit bowel resection and the best nutrition to sustain growth and intestinal function.
- Published
- 2021
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7. Prognostic factors in acute mesenteric ischemia and evaluation with multiple logistic regression analysis effecting morbidity and mortality.
- Author
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Ozturk S, Unver M, Ozdemir M, Bozbıyık O, Turk Y, Firat O, and Calıskan C
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- Aged, Female, Humans, Intestines blood supply, Male, Mesenteric Ischemia diagnosis, Mesenteric Ischemia pathology, Prognosis, Retrospective Studies, Risk Assessment, Risk Factors, Intestines pathology, Intestines surgery, Mesenteric Ischemia mortality, Mesenteric Ischemia surgery
- Abstract
<b>Background:</b> Acute mesenteric ischaemia (AMI) is a catastrophic abdominal emergency characterized by sudden critical interruption to the intestinal blood flow which commonly leads to bowel infarction and death. AMI still has a poor prognosis with an in-hospital mortality rate of 50-69 %. This high mortality rate is related to the delay in diagnosis which is often diffucult and overlooked. Early intervention is crucial and the potential for intestinal viability. <br><b>Methods:</b> The charts of 140 patients who were hospitalazed with AMI between May 1997 and August 2013 in Ege University Faculty of Medicine, department of general surgery were retrospectively reviewed. Demographical and clinical features of patients determining the best predictors which effect on morbidity and mortality were evaluated by Multiple Logistic Regression analysis by Enter method after adjustment for all possible confounding factors. <br><b>Results:</b> After Multiple LR analysis by Enter method after adjustment for all possible confounding factors affecting morbidity; shock, exploration and stay in hospital were statistically significant. Age, cardiac comorbidities, ASA scores, the time delay between onset of acute abdominal pain to surgery, the presence of acidosis and shock, the involved organs (small bowel and both), type of surgery and medical treatment and small bowel length under 100 cm were statistically significant on mortality. <br><b>Conclusion:</b> Risk factors related to mortality and morbidity have been poorly analyzed due to lack of prospective studies and smaller number of patients. Early diagnosis generally depends on clinical awareness and suspicion. Age and time of delay between onset of acute abdominal pain to surgery longer than 24 hours are the most important parameters that predict the mortality for patients presenting with shock and acidosis.
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- 2020
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8. Remnant Intestinal Length Defines Intestinal Adaptation and Hepatic Steatosis: Two Zebrafish Models.
- Author
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Maselli KM, Gee K, and Grikscheit TC
- Subjects
- Animals, Digestive System Surgical Procedures adverse effects, Disease Models, Animal, Fatty Liver etiology, Humans, Intestines physiopathology, Male, Short Bowel Syndrome etiology, Short Bowel Syndrome physiopathology, Zebrafish, Digestive System Surgical Procedures methods, Fatty Liver epidemiology, Intestinal Diseases surgery, Intestines surgery, Short Bowel Syndrome prevention & control
- Abstract
Background: Short bowel syndrome (SBS) is a condition that results from inadequate intestinal absorptive capacity, usually after the loss of functional intestine. We have previously developed a severe model of SBS in zebrafish that demonstrated increased intestinal adaptation (IA) and epithelial proliferation in SBS zebrafish. However, many children with SBS do not have this extreme intestinal loss. Therefore, in this study, we developed a variation of this model to evaluate the effects of increasing intestinal length on IA and the complications of SBS., Materials and Methods: After Institutional Animal Care and Use Committee approval, adult male zebrafish were assigned to three groups: sham (n = 30), S1-SBS (n = 30), and S3-SBS (n = 30). Sham surgery included ventral laparotomy alone. S1-SBS surgery consisted of laparotomy with creation of a proximal stoma at S1 (jejunostomy equivalent) and ligation at S4. S3-SBS surgery had stoma creation at S3 (ileostomy equivalent) and the same ligation. Fish were harvested at 14 d. Markers of IA were measured from proximal intestinal segments, and the liver was analyzed for development of hepatic steatosis., Results: At 14 d, S3-SBS fish lost less weight than S1-SBS and had increased markers of IA compared with sham fish, which were decreased compared with S1-SBS fish. S3-SBS fish had decreased proximal intestinal inflammation compared with S1-SBS fish. S1-SBS fish developed extensive hepatic steatosis. Although S3-SBS fish have increased hepatic steatosis compared with sham fish, it is decreased compared with S1-SBS., Conclusions: Longer remnant intestine decreases the extent of IA, inflammation, and hepatic steatosis in a zebrafish model of SBS., (Copyright © 2020 Elsevier Inc. All rights reserved.)
- Published
- 2020
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9. Hyperspectral Imaging (HSI) in Acute Mesenteric Ischemia to Detect Intestinal Perfusion Deficits.
- Author
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Mehdorn M, Köhler H, Rabe SM, Niebisch S, Lyros O, Chalopin C, Gockel I, and Jansen-Winkeln B
- Subjects
- Aged, Aged, 80 and over, Colectomy, Coloring Agents, Female, Humans, Indocyanine Green, Intestine, Small surgery, Male, Mesenteric Ischemia mortality, Middle Aged, Optical Imaging, Postoperative Complications, Prospective Studies, Short Bowel Syndrome etiology, Diagnostic Imaging methods, Intestines blood supply, Mesenteric Ischemia diagnostic imaging, Mesenteric Ischemia surgery
- Abstract
Background: Acute mesenteric ischemia is a life-threatening acute condition, which requires an interdisciplinary approach, including vascular recanalization and surgical treatment. Visual evaluation of intestinal perfusion might be misleading, and therefore, additional tools are necessary to reliably be able to resect the ischemic intestine. Hyperspectral imaging (HSI) has been shown to be feasible and safe for real-time assessment of tissue perfusion in visceral surgery but has never been used in cases of acute mesenteric ischemia. Therefore, we applied HSI in acute mesenteric ischemia to evaluate it for potential aid in the objectively discriminating ischemic and well-perfused intestine during explorative laparotomy., Methods: We recorded HSI measurements in 11 cases of acute mesenteric ischemia during explorative laparotomy. We evaluated the recorded images for macroscopic visual perfusion quality and divided it into three groups. Of those three groups, we calculated and compared the HSI indexes of tissue saturation, near-infrared perfusion index, organ hemoglobin index, and tissue water index, as well as the reflectance spectra., Results: We found significant differences in tissue saturation (0.7% versus 0.45%; P = 0.002) and near-infrared perfusion index (0.58 versus 0.23; P < 0.001) in poorly perfused intestinal segments compared with the viable intestine. Furthermore, we could detect an increasing peak at 630 nm of the reflectance spectra in less viable tissues, indicating a maximum in necrotic tissues. We attributed this peak to an increase in met-hemoglobin content in necrotic tissues, which is supported by the increase in the HSI organ hemoglobin index., Conclusions: HSI is able to discriminate tissue perfusion in acute mesenteric ischemia reliably and therefore might be helpful for resection. In addition, HSI gives information on tissue viability via reflectance spectra., (Copyright © 2020. Published by Elsevier Inc.)
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- 2020
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10. Intravenous supplementation type and volume are associated with 1-year outcome and major complications in patients with chronic intestinal failure.
- Author
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Pironi L, Steiger E, Joly F, Wanten GJA, Chambrier C, Aimasso U, Sasdelli AS, Szczepanek K, Jukes A, Theilla M, Kunecki M, Daniels J, Serlie MJ, Cooper SC, Poullenot F, Rasmussen HH, Compher CW, Crivelli A, Hughes SJ, Santarpia L, Guglielmi FW, Rotovnik Kozjek N, Ellegard L, Schneider SM, Matras P, Forbes A, Wyer N, Zmarzly A, Taus M, O'Callaghan M, Osland E, Thibault R, Cuerda C, Jones L, Chapman B, Sahin P, Virgili NM, Lee ADW, Orlandoni P, Matysiak K, Di Caro S, Doitchinova-Simeonova M, Masconale L, Spaggiari C, Garde C, Serralde-Zúñiga AE, Olveira G, Krznaric Z, Petrina Jáuregui E, Zugasti Murillo A, Suárez-Llanos JP, Nardi E, Van Gossum A, and Lal S
- Subjects
- Administration, Intravenous methods, Adult, Catheter-Related Infections complications, Chronic Disease, Drug Dosage Calculations, Female, Humans, Intestinal Absorption, Liver Failure complications, Male, Pharmaceutical Solutions administration & dosage, Severity of Illness Index, Fat Emulsions, Intravenous administration & dosage, Fluid Therapy methods, Intestinal Diseases etiology, Intestinal Diseases physiopathology, Intestinal Diseases therapy, Intestines physiopathology, Parenteral Nutrition, Home adverse effects, Parenteral Nutrition, Home methods
- Abstract
Background and Aim: No marker to categorise the severity of chronic intestinal failure (CIF) has been developed. A 1-year international survey was carried out to investigate whether the European Society for Clinical Nutrition and Metabolism clinical classification of CIF, based on the type and volume of the intravenous supplementation (IVS), could be an indicator of CIF severity., Methods: At baseline, participating home parenteral nutrition (HPN) centres enrolled all adults with ongoing CIF due to non-malignant disease; demographic data, body mass index, CIF mechanism, underlying disease, HPN duration and IVS category were recorded for each patient. The type of IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorised as <1, 1-2, 2-3 and >3 L/day. The severity of CIF was determined by patient outcome (still on HPN, weaned from HPN, deceased) and the occurrence of major HPN/CIF-related complications: intestinal failure-associated liver disease (IFALD), catheter-related venous thrombosis and catheter-related bloodstream infection (CRBSI)., Results: Fifty-one HPN centres included 2194 patients. The analysis showed that both IVS type and volume were independently associated with the odds of weaning from HPN (significantly higher for PN <1 L/day than for FE and all PN >1 L/day), patients' death (lower for FE, p=0.079), presence of IFALD cholestasis/liver failure and occurrence of CRBSI (significantly higher for PN 2-3 and PN >3 L/day)., Conclusions: The type and volume of IVS required by patients with CIF could be indicators to categorise the severity of CIF in both clinical practice and research protocols., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2020
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11. Treating short bowel syndrome with pharmacotherapy.
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Hollanda Martins Da Rocha M, Lee ADW, Marin MLM, Faintuch S, Mishaly A, and Faintuch J
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- Adult, Animals, Humans, Off-Label Use, Short Bowel Syndrome drug therapy, Short Bowel Syndrome surgery, Antidiarrheals therapeutic use, Glucagon-Like Peptide 2 therapeutic use, Intestines physiopathology, Parenteral Nutrition, Home methods, Short Bowel Syndrome therapy
- Abstract
Introduction: Short bowel syndrome (SBS) has traditionally been regarded as a rapidly fatal medical catastrophe. The advent of pharmacological options directly targeting disease pathophysiology justified this review., Areas Covered: Since the 1970s, home parenteral nutrition has reduced mortality, converting SBS into a chronic and disabling compensated and occasionally curable illness. Off-label antidiarrheal drugs and related products, though having minimal scientific evidence of efficacy, represent the standard-of-care and are here reviewed. Trophic intestinal hormones, including GLP-2 and its analogs, have great promise for alleviating malabsorption, the most important symptom within a nonsurgical, routine outpatient framework. Current indications involve adults with massive intestinal losses (fecal wet weight >1500 g/day). Surgical options such as intestinal lengthening or transplantation are also addressed although these options are considerably more aggressive and have stricter indications., Expert Opinion: GLP-2 analogs are the first candidates from a pioneering pharmacotherapic family within the SBS framework, namely disease-modifying, absorption-restoring agents. This family of drugs, potentially applicable in all contexts of severe intestinal loss, could become the therapeutic benchmark of the near future.
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- 2020
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12. [Autologous reconstructive surgery and intestinal rehabilitation in the management of short bowel syndrome].
- Author
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Urbán D, Kőnig R, and Cserni T
- Subjects
- Humans, Transplantation, Autologous, Intestines transplantation, Plastic Surgery Procedures methods, Short Bowel Syndrome surgery
- Abstract
Based on the latest definition, short bowel syndrome is defined as intestinal failure due to the loss of significant small bowel length or function, when the homeostasis and growth can only be maintained with intravenous supplementation of fluid, electrolytes and macronutrients. The natural adaptation of the short bowel can only compensate for the loss up to a certain level. According to this, we differentiate (1) acute, (2) prolonged and (3) chronic types of intestinal failure/short bowel syndrome. The most common causes are necrotising enterocolits, intestinal malrotation and volvulus, gastroschisis and ileal atresia. The management of type 3 short bowel syndrome has evolved significantly during the last decades, due to the multidisciplinary approach, hence the survival and quality of life of the patients have improved and transplantation is rarely necessary. Our aim was to review the most important considerations of intestinal rehabilitation, like management of increased gastrin secretion, high output stoma, decreased transit time, central venous lines, enteral and parenteral nutrition and the enhancement of the natural adaptation. We reviewed the former and the latest options of the autologous intestinal reconstructive surgery (AIRS) like the reversed segment, small bowel interposition, ileocaecal valve replacement, bowel lengthening and tailoring (LILT, STEP and SILT), controlled bowel expansion and the latest results with distraction enterogenesis and tissue engineering. Orv Hetil. 2020; 161(7): 243-251.
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- 2020
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13. Impact of Teduglutide on Quality of Life Among Patients With Short Bowel Syndrome and Intestinal Failure.
- Author
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Chen K, Mu F, Xie J, Kelkar SS, Olivier C, Signorovitch J, and Jeppesen PB
- Subjects
- Adult, Female, Humans, Male, Middle Aged, Gastrointestinal Agents therapeutic use, Intestines physiopathology, Peptides therapeutic use, Quality of Life, Short Bowel Syndrome drug therapy
- Abstract
Background: Teduglutide reduces or eliminates parenteral support (PS) dependency in patients with short bowel syndrome (SBS). Recent post hoc analyses demonstrated that effects are correlated with baseline PS volume. We assessed the SBS-related quality-of-life (QoL) impact of teduglutide, particularly whether improvements are greater among subgroups achieving more PS volume reduction., Methods: Using phase 3 trial data of teduglutide in patients with SBS (NCT00798967), change in Short Bowel Syndrome-Quality of Life (SBS-QoL) scores from baseline were compared between teduglutide vs placebo in the overall population and subgroups classified by baseline PS volume requirement, disease etiology, and bowel anatomy. Generalized estimating equation models were fitted to assess impact of teduglutide on SBS-related QoL using data from all visits, adjusted for baseline characteristics., Results: Of 86 patients, 43 each were randomized to teduglutide or placebo (mean age: 51 vs 50 years, respectively). In adjusted analyses, teduglutide had a nonsignificant reduction (improvement) of -8.6 points (95% CI: 2.6 to -19.8) in SBS-QoL sum score from baseline to Week-24 vs placebo. The impact of teduglutide varied by subgroup. Patients treated with teduglutide experienced significantly greater reductions in SBS-QoL sum score at Week-24 vs placebo in 2 subgroups, ie, the third (highest) tertile baseline PS volume (-27.3, 95% CI: -50.8 to -3.7) and inflammatory bowel disease (IBD; -29.6, 95% CI: -46.3 to -12.9). Results were similar for SBS-QoL subscale and item scores., Conclusions: The impact of teduglutide treatment on SBS-related QoL vs placebo varied among subgroups and was significant and most pronounced among patients with highest baseline PS volume requirement or IBD., (© 2019 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals, Inc. on behalf of American Society for Parenteral and Enteral Nutrition.)
- Published
- 2020
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14. Inflammatory Bowel Disease and Short Bowel Syndrome.
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Fuglestad MA and Thompson JS
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- Colitis, Ulcerative complications, Colitis, Ulcerative diagnosis, Colitis, Ulcerative surgery, Conservative Treatment methods, Crohn Disease complications, Crohn Disease diagnosis, Crohn Disease surgery, Female, Humans, Inflammatory Bowel Diseases complications, Inflammatory Bowel Diseases pathology, Male, Prognosis, Recovery of Function, Reoperation methods, Risk Assessment, Short Bowel Syndrome physiopathology, Inflammatory Bowel Diseases surgery, Intestines transplantation, Short Bowel Syndrome etiology, Short Bowel Syndrome therapy
- Abstract
Short bowel syndrome / intestinal failure (SBS/IF) is a rare and debilitating disease process that mandates a multidisciplinary approach in its management. Inflammatory bowel disease (IBD), in particular Crohn's disease (CD), predisposes patients to development of SBS/IF. This review discusses SBS/IF from the perspective of IBD, with an emphasis on prevention and treatment in the setting of CD. The aims of this review are to emphasize the unique treatment goals of the newly diagnosed SBS/IF patient, and highlight the role of both medical and surgical therapies in the management of IBD-related SBS/IF, including intestinal transplantation., (Copyright © 2019 Elsevier Inc. All rights reserved.)
- Published
- 2019
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15. Pediatric intestinal transplantation: Analysis of the intestinal transplant registry.
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Raghu VK, Beaumont JL, Everly MJ, Venick RS, Lacaille F, and Mazariegos GV
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- Child, Preschool, Databases, Factual, Female, Graft Survival, Humans, Infant, Male, Retrospective Studies, Treatment Outcome, Intestines transplantation, Registries
- Abstract
The ITR serves as an international database for centers around the world to contribute to current knowledge about intestinal transplant outcomes. Led by the IRTA and managed by the Terasaki Research Institute, the ITR collects data annually and uses these data to generate reports that guide management strategies and policy statements. The aim of this manuscript was to analyze outcomes specific to pediatric intestinal transplantation. Outcome data for children transplanted from 1985 to 2017 were analyzed and predictive factors assessed. A total of 2010 children received 2080 intestine containing allografts during this period. Overall, 1-year and 5-year patient and graft survival were 72.7%/66.1% and 57.2/48.8%, respectively. One-year conditional survival was most strongly associated with being a first-time transplant recipient and liver-inclusive grafts. Patient survival was most strongly associated with elective status of transplantation as compared with hospitalized status. Enteral autonomy following transplantation has continued to improve by era with colonic inclusion demonstrating additional incremental improvement in enteral autonomy and freedom from intravenous fluid. While PTLD and technical complications contribute less to graft loss than in earlier eras, rejection remains the largest contributor to long-term graft loss. Re-transplantation is linked with significantly worse conditional graft survival, and sepsis remains the largest contributor to patient death. Newer data elements are focusing on impact of donor variables, donor and recipient tissue typing, and impact of the development of de novo antibodies., (© 2019 Wiley Periodicals, Inc.)
- Published
- 2019
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16. Bench to Bedside: Approaches for Engineered Intestine, Esophagus, and Colon.
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Levin D
- Subjects
- Humans, Intestinal Mucosa, Stem Cells, Tissue Scaffolds, Colon, Esophagus, Intestines, Tissue Engineering methods
- Abstract
The generation of tissue engineered organs from autologous cells will allow replacement of diseased or absent organs without the need for immunosuppression. Common steps of tissue engineering include isolation of pluripotent or multipotent stem cells, preparation of synthetic or biologic scaffold, and implantation into a host to support the proliferation of engineered tissue. Some organs have been successfully transplanted in human patients; gastrointestinal tract tissues are nearing clinical introduction. The state of the science has progressed rapidly and providers and researchers alike must take appropriate steps to ensure strict adherence to ethical standards before introduction to human therapy., (Copyright © 2019 Elsevier Inc. All rights reserved.)
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- 2019
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17. Successful management of extensive bowel resection without intestinal continuity: a case report.
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Mohri K, Takeuchi E, Miyake H, Nagai H, Yoshioka Y, Okuno M, and Yuasa N
- Subjects
- Adult, Aortic Dissection, Colostomy, Humans, Male, Postoperative Complications, Intestinal Obstruction, Intestines pathology, Intestines surgery
- Abstract
Patients with intestinal ischemia associated with acute aortic dissection often require emergent bowel resection, which results in serious complications. We present a case of successful surgical management of extensive bowel necrosis caused by acute aortic dissection. A 42-year-old man underwent emergent subtotal resection of the small intestine, right colectomy, tube gastrostomy, and transverse colostomy; however, intestinal continuity was not restored. He developed two major postoperative complications: unconsciousness due to metabolic alkalosis caused by massive discharge from the gastrostomy and jaundice due to bile salt depletion caused by disruption of the enterohepatic circulation. His serum bilirubin levels decreased after the infusion of gastric discharge through gastrostomy into the transverse colon through the colostomy; thereafter, a second operation was performed to restore gastrointestinal continuity. Overall, patients undergoing massive bowel resection without intestinal continuity require careful management of electrolytes and bile salt., Competing Interests: None.
- Published
- 2019
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18. Early Predictors of Enteral Autonomy in Pediatric Intestinal Failure Resulting From Short Bowel Syndrome: Development of a Disease Severity Scoring Tool.
- Author
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Belza C, Fitzgerald K, de Silva N, Avitzur Y, and Wales PW
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- Adaptation, Physiological, Cohort Studies, Female, Gestational Age, Humans, Infant, Infant, Newborn, Intestinal Diseases etiology, Kaplan-Meier Estimate, Male, Retrospective Studies, Short Bowel Syndrome complications, Treatment Outcome, Intestinal Diseases physiopathology, Intestines physiopathology, Parenteral Nutrition, Severity of Illness Index, Short Bowel Syndrome physiopathology, Short Bowel Syndrome therapy
- Abstract
Introduction: Patients with short bowel syndrome (SBS) are dependent on parenteral nutrition (PN) while their bowel attempts to compensate. Our objective was to create an SBS disease severity score to predict the probability of achieving enteral autonomy (EA)., Methods: A retrospective cohort study of children with SBS managed by our Intestinal Rehabilitation Program was completed. Data abstracted included demographic, bowel anatomy, and outcome variables including conjugated bilirubin (CB) and enteral nutrition (EN) tolerated 6 months postoperatively. Univariate analysis and Cox proportional hazards (CPH) model were performed. A score was created based on weighting of coefficients. An α-value of < 0.05 was considered significant., Results: One hundred thirty-nine patients were analyzed (61% males). Ninety-five (68%) achieved EA. Patients possessing >50% residual small bowel (hazard ratio [HR] 2.68 [95% confidence interval {CI} 1.60-4.49], P < 0.001), ileocecal valve intact (HR 0.61 [95% CI 0.37-1.01], P < 0.055), and >50% enteral tolerance at 6 months (HR 5.70 [95% CI 2.77-11.74] P < 0.001) were positively associated with EA. CB >34 µmol/L (2 mg/dL) was negatively associated with EA (HR 0.42 [95% CI0.27-0.66], P < 0.001). A severity score was created by weighting CPH parameter estimates (small bowel length >50%, ileocecal valve intact, CB <34 µmol/L, and EN >50% for a maximum score of 8), and disease severity strata were developed (severe [25.7% EA], moderate [52.9% EA], and mild [97.1% EA])., Conclusion: We propose a pediatric SBS disease severity score that predicts probability of EA. The score allows prognostication of individual patients and could assist research by adjusting outcome reporting or stratifying recruitment., (© 2019 American Society for Parenteral and Enteral Nutrition.)
- Published
- 2019
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19. Development of Intestinal Scaffolds that Mimic Native Mammalian Intestinal Tissue.
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Ladd MR, Costello CM, Gosztyla C, Werts AD, Johnson B, Fulton WB, Martin LY, Redfield EJ, Crawford B, Panaparambil R, Sodhi CP, March JC, and Hackam DJ
- Subjects
- Animals, Biocompatible Materials chemistry, Decanoates chemistry, Glycerol analogs & derivatives, Glycerol chemistry, Immunohistochemistry, Male, Mice, Mice, Inbred C57BL, Microscopy, Electron, Scanning, Polymers chemistry, Swine, Intestines cytology, Tissue Engineering methods, Tissue Scaffolds chemistry
- Abstract
Impact Statement: This study is significant because it demonstrates an attempt to design a scaffold specifically for small intestine using a novel fabrication method, resulting in an architecture that resembles intestinal villi. In addition, we use the versatile polymer poly(glycerol sebacate) (PGS) for artificial intestine, which has tunable mechanical and degradation properties that can be harnessed for further fine-tuning of scaffold design. Moreover, the utilization of PGS allows for future development of growth factor and drug delivery from the scaffolds to promote artificial intestine formation.
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- 2019
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20. Restoring gut physiology in short bowel patients: from bench to clinical application of autologous intestinal reconstructive procedures.
- Author
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Lauro A, Coletta R, and Morabito A
- Subjects
- Biomedical Technology, Humans, Plastic Surgery Procedures, Technology Transfer, Transplantation, Autologous, Intestine, Small physiopathology, Intestine, Small transplantation, Intestines physiopathology, Intestines transplantation, Short Bowel Syndrome physiopathology, Short Bowel Syndrome surgery
- Abstract
Introduction : Short bowel syndrome represents the leading etiology that causes intestinal failure both in children and adults. Total parenteral nutrition support has dramatically improved the prognosis for these patients but, if related irreversible complications occur, the alternative is represented by surgery and/or transplantation. Areas covered : Autologous gastrointestinal reconstructive procedures are a feasible, alternative approach with good long-term outcome data inexperienced surgical centers. Expert opinion : Ongoing innovative efforts have driven the surgical options for successful autologous reconstructive surgery: bowel elongation/tapering techniques (LILT, STEP, and the new SILT) together with the 'reversed bowel segment' procedure are now recognized procedures and all must be tailored to the individual patient needs to obtain the optimal result in terms of enteral autonomy. Background laboratory experimentation with new procedures e.g. options for bowel dilation techniques and distraction-induced enterogenesis, may provide additional management and treatment modalities.
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- 2019
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21. Chronic rejection after intestinal transplantation: A systematic review of experimental models.
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Kitamura K, Buchholz BM, Abu-Elmagd K, Kalff JC, Schäfer N, and von Websky MW
- Subjects
- Animals, Disease Models, Animal, Graft Rejection diagnosis, Graft Rejection etiology, Graft Rejection therapy, Intestines transplantation, Organ Transplantation adverse effects
- Abstract
Intestinal transplantation (ITX) constitutes a salvage treatment for irreversible intestinal failure and failure of parenteral nutrition. Chronic rejection (CR) remains the key obstacle for long-term intestinal graft survival but the pathomechanisms are incompletely understood. This study systematically reviews experimental models addressing CR after ITX in order to summarize current knowledge on CR pathogenesis and identify promising experimental strategies. A systematic literature search was conducted in line with the PRISMA guidelines, and 68 out of 677 articles qualified for the final analysis. The average methodological quality of the studies was suboptimal with 7 out of 11 points as assessed by a modified Oxford Centre for Evidence-Based Medicine score. Histology of the chronically rejected graft was almost universally integrated as outcome parameter but we found significant heterogeneity in utilized transplant techniques, organ preservation, immunosuppression and time points of CR-assessment. Several studies identified cellular and humoral immunologic mechanisms in chronic intestinal rejection. Yet, neither preventive nor therapeutic strategies against CR have been successfully introduced into human intestinal transplantation highlighting the persistent need for optimized experimental models. In this review, we aim to improve the translational value of forthcoming investigations on CR by discussing the experimental status quo and potential innovative approaches., (Copyright © 2019. Published by Elsevier Inc.)
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- 2019
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22. Milk Fat Globule Membrane Inhibits NLRP3 Inflammasome Activation and Enhances Intestinal Barrier Function in a Rat Model of Short Bowel.
- Author
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Li Y, Wu J, Niu Y, Chen H, Tang Q, Zhong Y, Lambers TT, and Cai W
- Subjects
- Animals, Disease Models, Animal, Intestinal Mucosa drug effects, Intestinal Mucosa physiology, Intestines physiology, Lipid Droplets, Male, Rats, Rats, Sprague-Dawley, Glycolipids pharmacology, Glycoproteins pharmacology, Inflammasomes drug effects, Intestines drug effects, NLR Family, Pyrin Domain-Containing 3 Protein drug effects, Short Bowel Syndrome physiopathology
- Abstract
Background: The milk fat globule membrane (MFGM) contains various bioactive components which have been shown to maintain gut barrier integrity. This study aimed to evaluate the protective effects of MFGM on intestinal barrier function and its possible mechanisms in a rat model of short bowel syndrome (SBS)., Materials and Methods: Five-week-old male Sprague-Dawley rats were divided into 3 groups (n = 8 per group), consisting of Sham group and rats submitted to massive small-bowel resection then supplemented with either water (SBS) or 1.5g/kg/d MFGM (SBS+MFGM) by daily gavage. Rats were sacrificed on day 15 postoperation. Intestinal adaptation, gut permeability, bacterial translocation (BT), expression of tight junction proteins, mucin 1 (MUC1), and nucleotide-binding oligomerization domain leucine-rich repeat and pyrin domain-containing protein 3 (NLRP3) pathway in the ileum were evaluated., Results: Both SBS+MFGM and SBS groups exhibited lower body weight and higher ileum villus height than Sham group, but no difference was detected between each other. SBS group had significantly higher intestinal permeability and BT rate than other groups (P < .05). Compared with SBS rats, SBS+MFGM group showed higher expression of tight junction proteins and MUC1, lower expression of NLRP3 and caspase-1 in the ileum, as well as lower interleukin (IL)-1β but higher IL-18 levels in ileum tissue., Conclusions: Supplementation of MFGM helps to modulate NLRP3 inflammasome activation and enhances gut barrier integrity in rats after massive small-bowel resection, which provides experimental support for potential applications of MGFM in intestinal barrier dysfunction, although further studies are needed., (© 2018 American Society for Parenteral and Enteral Nutrition.)
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- 2019
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23. Autologous intestinal reconstruction: a single institution study of the serial transverse enteroplasty (STEP) and the longitudinal intestinal lengthening and tailoring (LILT).
- Author
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Shah AA, Petrosyan M, Franklin AL, Chahine AA, Torres C, and Sandler AD
- Subjects
- Child, Child, Preschool, Female, Humans, Infant, Length of Stay, Male, Parenteral Nutrition, Retrospective Studies, Serum Albumin analysis, Digestive System Surgical Procedures methods, Intestines surgery, Short Bowel Syndrome surgery
- Abstract
Purpose: To review the effectiveness of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) operations in a cohort of patients with short bowel syndrome (SBS)., Methods: We conducted a retrospective analysis of children with SBS treated at our institution from 2004 until 2014. Children aged 0 days to 18 years with SBS who underwent autologous intestinal reconstruction were included in the study., Results: Twenty-two SBS patients underwent 31 different lengthening procedures (LP). Seventeen patients underwent their primary lengthening procedures at our institution: 9 (53%) patients underwent a LILT, 7 (41%) underwent a STEP and 1 (6%) had a simultaneous LILT and STEP procedure. 12/22 patients had a second STEP, two had a third STEP and one patient had an intestinal transplantation after the LP. Median intestinal length at the time of surgery was 25 cm (range 12-90 cm). There was no difference in gain of intestinal length after LILT vs. STEP (p = 0.74). Length of stay and initiation of feeds were similar. Serum albumin increased after autologous bowel lengthening (p < 0.001). 50% were weaned off parenteral nutrition (PN) (5/9 of the LILT, 1/7 of the STEP, 1/1 of the combined LILT/STEP). There were no surgical complications or deaths., Conclusion: In patients with SBS, LILT and STEP procedures are effective for autologous intestinal reconstruction and enable intestinal rehabilitation.
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- 2019
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24. Plasma citrulline is not a biomarker for intestinal adaptation in short bowel syndrome, studied in piglets: a model for human neonates.
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Lansing M, Turner JM, Wizzard P, Lavallee CM, Lim DW, Muto M, Nation PN, Pencharz PB, Ball RO, and Wales PW
- Subjects
- Anastomosis, Surgical, Animals, Animals, Newborn, Biomarkers blood, Colon surgery, Ileum surgery, Intestine, Small pathology, Jejunum surgery, Models, Animal, Short Bowel Syndrome physiopathology, Swine, Adaptation, Physiological, Citrulline blood, Intestines physiopathology, Short Bowel Syndrome surgery
- Abstract
Background: There are no in vivo methods to measure adaptation in neonatal short bowel syndrome (SBS). We evaluated citrulline (Cit) levels in neonatal piglet surgical models of SBS., Methods: Piglets underwent 75% mid-intestinal resection with jejunoileal anastomosis (JI), 75% distal resection of ileum with jejunocolic anastomosis (JC) or sham surgery. Jugular and gastric catheters were inserted for parenteral and enteral nutrition. On D7, small intestine length and weight were measured, jejunum collected for histopathology and Cit level determined., Results: JI (n = 5) compared to JC (n = 5) had increased small intestinal length (JC - 17.5 cm; JI +22.0 cm; p = 0.02) and mass (JC 43.1 mg/cm/kg; JI 51.3 mg/cm/kg; p = 0.02), while Cit did not differ (JI 801.0 µM; JC 677.7 µM; p = 0.90). Including non-resected shams (n = 4), Cit correlated with length (R
2 = 0.48; p = 0.006), but not for SBS alone (R2 = 0.11; p = 0.4), mass (R2 = 0.05; p = 0.5). A second experiment compared change in Cit levels from baseline to D7. Levels declined in sham (n = 8) and JC (n = 10) (sham - 110.1 µM; JC - 56.6 µM; p = 0.17), regardless of intestinal lengthening (sham 29.9 cm; JC - 10.4 cm; p = 0.002)., Conclusion: Citrulline levels predict large differences in intestinal length and 'identify' SBS. However, citrulline cannot discriminate between adaptation in JI and JC, nor predict intestinal lengthening.- Published
- 2019
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25. Emerging treatments for short bowel syndrome in adult patients.
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Billiauws L and Joly F
- Subjects
- Adult, Animals, Gastrointestinal Agents adverse effects, Humans, Intestinal Absorption drug effects, Intestines physiopathology, Nutritional Status drug effects, Recovery of Function, Short Bowel Syndrome diagnosis, Short Bowel Syndrome physiopathology, Treatment Outcome, Gastrointestinal Agents therapeutic use, Intestines drug effects, Parenteral Nutrition, Home adverse effects, Short Bowel Syndrome therapy
- Abstract
Introduction: Short bowel syndrome (SBS) is the major cause of chronic intestinal failure (IF), defined as 'the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth'. Areas covered: Spontaneous intestinal adaptation, including increased hormonal secretion, development of hyperphagia and gut microbiota dysbiosis, occurs 2 years after resection, improving intestinal absorption and decreasing PN dependency. Hormonal treatments, promoting intestinal hyperadaptation, have been proposed in patients with SBS with chronic IF. Clinical studies showed teduglutide to increase urine production and reduce the need for parenteral support volume in these patients. According to the latest ESPEN Guidelines, if a growth factor treatment is considered, the GPL2 analog, teduglutide, should be the first-choice treatment. Expert opinion: These therapies underline the importance of patient monitoring at home and the complexity for HPN adaptation. A multidisciplinary approach should be a gold standard.
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- 2019
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26. Short bowel syndrome in children and adults: from rehabilitation to transplantation.
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Lauro A and Lacaille F
- Subjects
- Adult, Age Factors, Child, Child, Preschool, Fecal Microbiota Transplantation, Gastrointestinal Microbiome, Humans, Infant, Infant, Newborn, Intestinal Absorption, Intestines microbiology, Intestines physiopathology, Nutritional Status, Recovery of Function, Risk Factors, Short Bowel Syndrome epidemiology, Short Bowel Syndrome physiopathology, Treatment Outcome, Intestines transplantation, Parenteral Nutrition adverse effects, Short Bowel Syndrome rehabilitation, Short Bowel Syndrome surgery
- Abstract
Introduction: Short bowel syndrome (SBS) is a dramatic clinical condition in both children and adults; the residual bowel length is not sufficient to avoid intestinal failure, with subsequent malnutrition and growth retardation, and intravenous support is required to provide the nutrients normally coming from the intestine. Apart from the primary disease, the medical status can be worsened by complications of intestinal failure: if there are irreversible, the prognosis is poor unless a successful intestinal rehabilitation is achieved. Areas covered: The rescue of the remnant small bowel requires a multidisciplinary expertise to achieve digestive autonomy. The use of intestinal trophic factors has shown encouraging results in improving the intestinal adaptation process. Whenever the residual bowel length is inadequate, in a well-selected population weaning parenteral nutrition (PN) off could be attempted by surgery through lengthening procedures. A further subset of patients, with total and irreversible intestinal failure and severe complications on PN, may have an indication to intestinal transplantation. This procedure is still affected by poor long-term results. Expert commentary: Novel approaches developed through a multidisciplinary team work, such as manipulation of microbiota or tissue bioengineering, should be added to current therapies to treat successfully SBS.
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- 2019
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27. Intestinal lengthening via multiple in-continuity springs.
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Dubrovsky G, Huynh N, Thomas AL, Shekherdimian S, and Dunn JC
- Subjects
- Alloys pharmacology, Animals, Digestive System Surgical Procedures instrumentation, Swine, Digestive System Surgical Procedures methods, Intestines surgery, Short Bowel Syndrome surgery, Tissue Expansion Devices
- Abstract
Background: Short bowel syndrome is a debilitating condition with few effective treatments. Spring-mediated distraction enterogenesis can be used to lengthen intestine. The purpose of this study is to determine whether multiple springs in series can safely increase the total amount of lengthening., Methods: Juvenile mini-Yucatan pigs each received three nitinol springs placed within their jejunum. Plication was used to narrow the intestine around each spring to secure them. Compressed springs were used in the experimental group, while uncompressed springs were used in the control group. The intestine was examined 3 weeks later for lengthening and histologic changes., Results: All pigs tolerated diets postoperatively with continued weight gain, and no dilation or obstruction of the intestine was observed. Segments of intestine that contained compressed springs had a significant increase in length from 2.5 cm to 3.9 ± 0.2 cm per spring, compared to segments containing control springs that showed no change (p < 0.001)., Conclusions: Intestinal plication can be safely used to secure multiple springs in series to achieve intestinal lengthening without compromising intestinal function. Using several springs at once allows for a greater amount of total lengthening. This is a promising model that has potential in the treatment of short bowel syndrome., (Copyright © 2018 Elsevier Inc. All rights reserved.)
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- 2019
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28. Children With Intestinal Failure Undergoing Intestinal Rehabilitation Are at Risk for Essential Fatty Acid Deficiency.
- Author
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Gunnar R, Lumia M, Pakarinen M, and Merras-Salmio L
- Subjects
- Child, Child, Preschool, Deficiency Diseases epidemiology, Enteral Nutrition, Ethylenediamines blood, Fat Emulsions, Intravenous, Fatty Acids, Essential blood, Female, Humans, Infant, Infant, Newborn, Intestinal Diseases blood, Intestinal Diseases complications, Intestine, Small pathology, Lipids blood, Lipids deficiency, Male, Pediatrics, Prevalence, Risk Factors, Short Bowel Syndrome, Trientine blood, Deficiency Diseases etiology, Fatty Acids, Essential deficiency, Intestinal Diseases therapy, Intestines pathology, Lipids administration & dosage, Nutritional Status, Parenteral Nutrition adverse effects
- Abstract
Background: Essential fatty acid (EFA) status may be compromised during the intestinal failure (IF) rehabilitation. Parenteral lipid restriction is used to treat intestinal failure associated liver disease (IFALD), while the enteral fatty acid (FA) absorption remains limited. We analyzed the FA status among pediatric IF and intestinal insufficiency patients., Methods: We evaluated 49 patients aged 0-18 years attending our nationwide IF referral center. Their serum FA fractions were determined and examined against previous nutrition, parenteral lipid emulsion, and intestinal anatomy data. The patients were divided into 3 subgroups according to their dependence on parenteral nutrition (PN): full enteral (EN) (n = 33), supplemental PN (n = 14) or predominantly PN (n = 20). Trien:tetraen ratio (TTR) ≥0.2 was considered diagnostic for essential fatty acid deficiency (EFAD) and increased risk was suspected if TTR exceeded 0.1., Results: We identified 8 (16%) patients with elevated TTR ≥0.1; in 3 of them the ratio exceeded 0.2. Five of these children belonged to supplemental PN group. This group carried the highest incidence of elevated TTR (P = 0.0016), with median TTR at 0.06 (interquartile range 0.03-0.09) and two-thirds of the analyzed TTR ≥0.5. Increased EFAD risk was associated with young age (P = 0.0291), current PN with low parenteral lipid content (P = 0.0003), and short remaining small bowel (P = 0.0013)., Conclusions: IF children with supplemental PN carry the highest overall risk for EFAD. Young age, current PN, and short remaining small bowel also increase the risk for EFAD., (© 2018 American Society for Parenteral and Enteral Nutrition.)
- Published
- 2018
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29. Survey of Nutrition Management Practices in Centers for Pediatric Intestinal Rehabilitation.
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Nucci AM, Ellsworth K, Michalski A, Nagel E, and Wessel J
- Subjects
- Cholestasis etiology, Cholestasis prevention & control, Humans, Infant, Liver, Liver Diseases etiology, Liver Diseases prevention & control, Nutritionists, Short Bowel Syndrome rehabilitation, Surveys and Questionnaires, United States, Dietetics, Enteral Nutrition, Intestines, Parenteral Nutrition, Pediatrics, Short Bowel Syndrome therapy
- Abstract
Background: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized., Methods: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section. The survey included 24 open-ended questions related to PN and enteral feeding strategies, nutrition management of PN-associated liver disease, and laboratory monitoring., Results: Dietitians from 14 centers completed the survey. Management components for patients at risk for cholestasis were consistent and included fat minimization, trace element modification, avoiding PN overfeeding, and providing EN. Parenteral amino acid solutions designed for infants/young children are used in patients <1 or 2 years of age. Trace minerals are dosed individually in 10 of 14 centers. Eleven centers prescribe a continuous infusion of breast milk or elemental formula 1-2 weeks after resection while 3 centers determine the formula type by the extent of resection. Most (86%) centers do not have a protocol for initiating oral/motor therapy. Laboratory panel composition varied widely by center. The selection and frequency of use depended on clinical variables, including cholestatic status, exclusive vs partial PN dependence, postrepletion verification vs routine monitoring, intestinal anatomy, and acuity of care., Conclusion: EN and PN management strategies are relatively consistent among U.S. centers. Collaborative initiatives are necessary to define better practices and establish laboratory monitoring guidelines., (© 2017 American Society for Parenteral and Enteral Nutrition.)
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- 2018
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30. Reduction of Parenteral Nutrition and Hydration Support and Safety With Long-Term Teduglutide Treatment in Patients With Short Bowel Syndrome-Associated Intestinal Failure: STEPS-3 Study.
- Author
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Seidner DL, Fujioka K, Boullata JI, Iyer K, Lee HM, and Ziegler TR
- Subjects
- Adult, Aged, Female, Humans, Intestinal Diseases, Male, Middle Aged, Short Bowel Syndrome therapy, Time Factors, Treatment Outcome, Fluid Therapy, Gastrointestinal Agents therapeutic use, Intestines, Parenteral Nutrition, Peptides therapeutic use, Short Bowel Syndrome drug therapy
- Abstract
Background: Patients with intestinal failure associated with short bowel syndrome (SBS-IF) require parenteral support (PS) to maintain fluid balance or nutrition. Teduglutide (TED) reduced PS requirements in patients with SBS-IF in the randomized, placebo (PBO)-controlled STEPS study (NCT00798967) and its 2-year, open-label extension, STEPS-2 (NCT00930644)., Methods: STEPS-3 (NCT01560403), a 1-year, open-label extension study in patients with SBS-IF who completed STEPS-2, further monitored the safety and efficacy of TED (0.05 mg/kg/day). Baseline was the start of TED treatment, in either STEPS or STEPS-2. At the end of STEPS-3, patients treated with TED in both STEPS and STEPS-2 (TED-TED) received TED for ≤42 months, and patients treated with TED only in STEPS-2 (no TED treatment [NT]/PBO-TED) received TED for ≤36 months., Results: Fourteen patients enrolled (TED-TED, n = 5; NT/PBO-TED, n = 9) and 13 completed STEPS-3. At the last dosing visit, mean (SD) PS was reduced from baseline by 9.8 (14.4 [50%]) and 3.9 (2.8 [48%]) L/week in TED-TED and NT/PBO-TED, respectively. Mean (SD) PS infusions decreased by 3.0 (4.6) and 2.1 (2.2) days per week from baseline in TED-TED and NT/PBO-TED, respectively. Two patients achieved PS independence; 2 additional patients who achieved independence in STEPS-2 maintained enteral autonomy throughout STEPS-3. All patients reported ≥1 treatment-emergent adverse event (TEAE); 3 patients had TEAEs that were reported as treatment related. No patient had a treatment-related treatment-emergent serious AE., Conclusions: Long-term TED treatment yielded a safety profile consistent with previous studies, sustained efficacy, and a further decline in PS requirements., (© 2018 The Authors. Nutrition in Clinical Practice published by Wiley Periodicals, Inc. on behalf of American Society for Parenteral and Enteral Nutrition.)
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- 2018
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31. Autologous intestinal reconstruction surgery.
- Author
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Ramos-Gonzalez G and Kim HB
- Subjects
- Humans, Intestines surgery, Transplantation, Autologous, Treatment Outcome, Digestive System Surgical Procedures methods, Intestines transplantation, Plastic Surgery Procedures methods, Short Bowel Syndrome surgery
- Abstract
The management of short bowel syndrome has mainly been focused on intestinal rehabilitation as part of multidisciplinary team approach in specialized centers. While some patients go through a process of bowel adaptation that allows them to reach enteral autonomy, others reach a plateau before this and require prolonged parenteral nutrition and/or intestinal transplantation. Various autologous intestinal reconstruction procedures centered on bowel tapering have been described to increase functional intestinal area and help gain enteral autonomy. This review discusses the surgical techniques, advantages, limitations, and general outcomes of each procedure., (Copyright © 2018. Published by Elsevier Inc.)
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- 2018
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32. Soluble Fiber Use in Pediatric Short Bowel Syndrome: A Survey on Prevailing Practices.
- Author
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Harvie ML, Norris MAT, and Sevilla WMA
- Subjects
- Defecation drug effects, Dietary Carbohydrates adverse effects, Dietary Fiber adverse effects, Dietary Supplements, Humans, Infant, Nurses, Nutritionists, Pectins therapeutic use, Physicians, Short Bowel Syndrome therapy, Surveys and Questionnaires, Dietary Carbohydrates therapeutic use, Dietary Fiber therapeutic use, Enteral Nutrition, Intestines, Pediatrics
- Abstract
Background: In pediatric short bowel syndrome (SBS), adding fiber to enteral feedings is 1 treatment method to manage increased stool output. However, there are no standardized recommendations on the use of fiber in this setting, including type, dosage, titration strategies, etc. OBJECTIVE: The aim of this study is to determine current prevailing practices on the use of fiber in the treatment of chronic high stool output in the pediatric SBS population., Methods: An anonymous electronic survey with 13 questions was sent through health professional electronic mailing lists. The survey was completed by healthcare professionals including physicians (primary care, subspecialists, and surgeons), nurse practitioners, and registered dietitians., Results: A total of 94 responses were received. The most common supplemental fiber used was pectin (62.8%). The 2 major factors considered when initiating fiber therapy were consistency of stool (74.5%) and volume of stool output (85.1%). The major factor that determined discontinuation of fiber was abdominal distention (67%). A majority of providers waited 2 weeks or less to see improvement following fiber initiation before discontinuing it., Conclusions: The goal of the survey was to gather more information with regard to fiber use in the management of SBS patients. The data collected can be used to provide future direction on determining best practices for fiber use in SBS patients., (© 2018 American Society for Parenteral and Enteral Nutrition.)
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- 2018
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33. Intestinal microbiota in short bowel syndrome.
- Author
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Piper HG
- Subjects
- Humans, Prebiotics, Probiotics therapeutic use, Short Bowel Syndrome therapy, Gastrointestinal Microbiome, Intestines microbiology, Short Bowel Syndrome microbiology
- Abstract
Children with short bowel syndrome have significant changes to their intestinal microbiota after intestinal loss. The purpose of this article is to understand the potential implications of these changes on gut function, hepatic cholestasis and overall nutrition. Possible therapies to restore the commensal bacterial community in these patients will also be reviewed., (Copyright © 2018. Published by Elsevier Inc.)
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- 2018
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34. Intestinal Failure and Rehabilitation.
- Author
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Buchman AL
- Subjects
- Adaptation, Physiological drug effects, Algorithms, Diarrhea drug therapy, Diarrhea etiology, Diet, Humans, Intestinal Diseases physiopathology, Postoperative Complications therapy, Intestinal Absorption, Intestinal Diseases diet therapy, Intestinal Diseases rehabilitation, Intestines surgery, Parenteral Nutrition
- Abstract
The rendering of proper care for the patient with intestinal failure requires the provider to have a functional understanding of digestion and absorption, nutrient requirements, and intestinal adaptation. Inherent in those concepts is that not only is nutritional absorption compromised, but medication absorption is as well. The principles of the management of home parenteral nutrition must be mastered and then proper and controlled weaning of parenteral nutrition may be commenced by use of dietary and pharmacologic means with appropriate clinical outcome measures followed. This complicated management requires a team experienced in both medical and surgical management of intestinal failure., (Copyright © 2018 Elsevier Inc. All rights reserved.)
- Published
- 2018
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35. Systematic Review and Meta-Analysis of the Utilization of Ethanol Locks in Pediatric Patients With Intestinal Failure.
- Author
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Rahhal R, Abu-El-Haija MA, Fei L, Ebach D, Orkin S, Kiscaden E, and Cole CR
- Subjects
- Catheterization, Central Venous adverse effects, Child, Humans, Infant, Catheter-Related Infections prevention & control, Catheterization, Central Venous methods, Catheters, Indwelling adverse effects, Central Venous Catheters adverse effects, Ethanol, Intestinal Diseases complications, Intestines
- Abstract
Background: Intestinal failure is a chronic condition related to loss of bowel length and/or function, resulting in dependence on central venous catheters for fluids and nutrition. Catheter use can be associated with significant complications, including catheter-related bloodstream infections (CRBSIs), which can lead to loss of vascular access, advancing intestinal failure associated-liver disease and death. Our objective was to evaluate the effectiveness and safety of ethanol locks as compared with standard heparin locks in pediatric intestinal failure., Methods: Databases, including MEDLINE and EMBASE, were searched until March 2017. Titles and abstracts were reviewed independently and relevant articles reassessed by full-text review. The main outcome was the rate of CRBSIs, while secondary outcomes were catheter replacement and repair., Results: Nine observational studies were included. The mean difference in rate of CRBSIs was 6.27 per 1000 catheter days (95% CI, 4.89-7.66) favoring ethanol locks, with a 63% overall reduction in infection rate. The mean difference in catheter replacement rate (per 1000 catheter days) was 4.56 (95% Cl, 2.68-6.43) favoring ethanol locks. The overall effect on catheter repair rate (per 1000 catheter days) was -1.67 (95% CI, -2.30 to -1.05), indicating lower repair rate with heparin locks., Conclusion: Sufficient evidence was noted showing that ethanol locks reduced CRBSIs and catheter replacements. Our findings raise questions about the effect of the ethanol lock on catheter integrity based on the noted increase in repair rate. This requires further prospective evaluation and may support selective application of ethanol locks to patients with documented CRBSIs., (© 2017 American Society for Parenteral and Enteral Nutrition.)
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- 2018
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36. Postoperative Enteral Nutrition Guidelines Reduce the Risk of Intestinal Failure-Associated Liver Disease in Surgical Infants.
- Author
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Shores DR, Alaish SM, Aucott SW, Bullard JE, Haney C, Tymann H, Nonyane BAS, and Schwarz KB
- Subjects
- Enteral Nutrition adverse effects, Enteral Nutrition methods, Female, Humans, Incidence, Infant, Infant, Newborn, Intestinal Diseases epidemiology, Intestinal Diseases etiology, Liver Diseases epidemiology, Liver Diseases etiology, Logistic Models, Male, Postoperative Care methods, Postoperative Complications epidemiology, Postoperative Complications etiology, Practice Guidelines as Topic, Prospective Studies, Retrospective Studies, Risk Factors, Severity of Illness Index, Treatment Outcome, Enteral Nutrition standards, Intestinal Diseases prevention & control, Intestines surgery, Liver Diseases prevention & control, Postoperative Care standards, Postoperative Complications prevention & control
- Abstract
Objective: To assess the effectiveness of postoperative feeding guidelines in reducing the incidence and severity of intestinal failure-associated liver disease (IFALD) among infants., Study Design: Two cohorts of infants <6 months old undergoing intestinal surgery were compared: preguideline (retrospective data from 2007 to 2013; n = 83) and postguideline (prospective data from 2013 to 2016; n = 81). The guidelines included greater initial enteral nutrition volumes of 20 mL/kg/d and daily feeding advancement if tolerated. The primary outcomes were incidence of IFALD (peak direct bilirubin [DB] >2 mg/dL) and severity (DB >5 mg/dL for moderate-severe). Multiple logistic regression was used to determine the odds of developing IFALD. Other outcomes were time to reach 50% and 100% goal calories from enteral nutrition and the incidence of necrotizing enterocolitis after feeding., Results: The incidence of IFALD decreased from 71% to 51% (P = .031), and median peak DB decreased from 5.7 to 2.4 mg/dL (P = .001). After adjusting for diagnosis and prematurity, the odds of developing IFALD of any severity were reduced by 60% (OR 0.40, 95% CI 0.20-0.85), and the odds of developing moderate-to-severe IFALD were reduced by 72% (OR 0.28, 95% CI 0.13-0.58) with guideline use. Time to reach 50% enteral nutrition decreased from a median of 10 to 6 days (P = .020) and time to reach 100% enteral nutrition decreased from 35 to 21 days (P = .035) with guideline use. The incidence of necrotizing enterocolitis after initiating enteral nutrition did not change (5% vs 9%, P = .346)., Conclusions: Implementation of feeding guidelines reduced time to reach feeding goals, significantly reducing IFALD incidence and severity., (Copyright © 2017 Elsevier Inc. All rights reserved.)
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- 2018
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37. Fasting and Postprandial Plasma Citrulline and the Correlation to Intestinal Function Evaluated by 72-Hour Metabolic Balance Studies in Short Bowel Jejunostomy Patients With Intestinal Failure.
- Author
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Fjermestad H, Hvistendahl M, and Jeppesen PB
- Subjects
- Adult, Aged, Fasting, Female, Humans, Male, Middle Aged, Time Factors, Citrulline blood, Intestines physiopathology, Jejunostomy, Postprandial Period, Short Bowel Syndrome blood, Short Bowel Syndrome surgery
- Abstract
Background: Fasting plasma citrulline (p-citrulline) is a marker of functional enterocyte mass. However, the optimal timing of measurement in relation to meals has yet to be clarified. Furthermore, p-citrulline has been proposed to be a surrogate marker for small bowel length and intestinal absorption parameters in short bowel syndrome patients with intestinal failure (SBS-IF)., Materials and Methods: Eight patients with SBS-IF and 8 healthy controls (HCs) were given a standardized mixed test meal, and p-citrulline was measured 15 minutes before and 60, 120, and 180 minutes after completion of the meal. The patients with SBS-IF had their intestinal absorption of wet weight, energy, macronutrients, and electrolytes measured in relation to 72-hour metabolic balance studies. We investigated the possible correlations between p-citrulline and short bowel length, absorptive parameters, and the dependence on parenteral support (PS)., Results: In the patients with SBS-IF, we found a 12% (P = .041) reduction in postprandial citrulline levels after 180 minutes. In the HCs, there was a 13% postprandial reduction at 60 minutes (P = .018). No significant correlations between fasting p-citrulline and bowel length, bowel absorptive function, or the dependence on PS were found. Even when excluding 2 patients in whom the intestinal absorption was adjacent to the intestinal insufficiency borderlines, these correlations were not significant., Conclusion: Based on findings in this small study, the optimal timing of p-citrulline measurement is on fasting samples. However, p-citrulline seems insufficiently discriminative to serve as a valid biomarker of bowel length, bowel absorptive function, or dependence on PS in patients with SBS-IF., (© 2017 American Society for Parenteral and Enteral Nutrition.)
- Published
- 2018
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38. Tissular growth factors profile after teduglutide administration on an animal model of intestinal anastomosis.
- Author
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Costa BP, Gonçalves AC, Abrantes AM, Alves R, Matafome P, Seiça R, Sarmento-Ribeiro AB, Botelho MF, and Castro-Sousa F
- Subjects
- Animals, Gene Expression Regulation drug effects, Ileum metabolism, Ileum surgery, Intercellular Signaling Peptides and Proteins biosynthesis, Intercellular Signaling Peptides and Proteins genetics, Male, Rats, Rats, Wistar, Short Bowel Syndrome, Anastomosis, Surgical, Gastrointestinal Agents pharmacology, Intercellular Signaling Peptides and Proteins metabolism, Intestines drug effects, Intestines surgery, Peptides pharmacology
- Abstract
Background: Teduglutide is an enterotrophic analogue of glucagon-like peptide-2, with an indirect and poorly understood mechanism of action, approved for the rehabilitation of short-bowel syndrome. This study aims to analyze the response of tissue growth factors to surgical injury and teduglutide administration on an animal model of intestinal anastomosis., Methods: Wistar rats (n = 59) were distributed into four groups: "ileal resection" or "laparotomy", each one subdivided into "postoperative teduglutide administration" or "no treatment"; and sacrificed at the third or the seventh day, with ileal sample harvesting. Gene expression of insulin-like growth factor 1 (Igf1), vascular endothelial growth factor a (Vegfa), transforming growth factor β1 (Tgfβ1), connective tissue growth factor (Ctgf), fibroblast growth factor 2 (Fgf2), fibroblast growth factor 7 (Fgf7), epidermal growth factor (Egf), heparin-binding epidermal-like growth factor (Hbegf), platelet-derived growth factor b (Pdgfb) and glucagon-like peptide 2 receptor (Glp2r)was studied by real-time polymerase chain reaction., Results: Upregulation of Fgf7, Fgf2, Egf, Vegfaand Glp2rat the third day and of Pdgfat the seventh day was verified in the perianastomotic segment. Teduglutide administration was associated with higher fold-change of relative gene expression of Vegfa(3.6 ± 1.3 vs.1.9 ± 2.0, p = 0.0001), Hbegf(2.2 ± 2.3 vs. 1.1 ± 0.9, p = 0.001), Igf1(1.6 ± 7.6 vs. 0.9 ± 0.7, p = 0.002) and Ctgf(1.1 ± 2.1 vs. 0.6 ± 2.0, p = 0.013); and lower fold-change of Tgfβ1, Fgf7and Glp2r., Conclusions: Those results underscore the recognized role of Igf1and Hbegfas molecular mediators of the effects of teduglutide and suggest that other humoral factors, like Vegfand Ctgf, may also be relevant in the perioperative context. Induction of Vegfa, Igf1and Ctgfgene expressions might indicate a favorable influence of teduglutide on the intestinal anastomotic healing.
- Published
- 2018
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39. Surgical Anatomy Does Not Affect the Progression of Intestinal Failure-Associated Liver Disease in Neonatal Piglets.
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Lavallee CM, Wizzard PR, Lansing M, Vine DF, Nation PN, Yap JY, Willing BP, Wales PW, and Turner JM
- Subjects
- Animals, Animals, Newborn, Disease Models, Animal, Disease Progression, Female, Liver Diseases pathology, Male, Short Bowel Syndrome surgery, Swine, Anastomosis, Surgical methods, Intestines pathology, Intestines surgery, Liver Diseases etiology, Short Bowel Syndrome complications, Short Bowel Syndrome pathology
- Abstract
Background: Intestinal failure-associated liver disease (IFALD) causes significant morbidity in neonates with short bowel syndrome (SBS) dependent on parenteral nutrition (PN). Resected ileum, with loss of the ileocecal valve (ICV), is the most common anatomy in SBS, yet its impact on IFALD has not been adequately studied., Methods: Neonatal piglets were randomized to 75% intestinal resection with jejunocolic anastomosis (JC, n = 12), 75% resection with jejunoileal anastomosis and intact ICV (JI, n = 13), PN-fed sham (sham, n = 14), or sow-fed control (SF, n = 8). Surgical and sham piglets received 100% PN for 14 days before bile flow was measured and blood chemistry, liver pathology, jejunal permeability, and bacterial translocation were assessed., Results: Bile flow was lower for PN-fed compared with SF (P = .002) but not different between the PN-fed groups. Total bilirubin (P = .03) and liver pathology (P < .001) were greater in PN-fed than SF groups but not different between PN-fed groups. Serum bile acids were increased in sham (P = .01) but not different between SBS groups. PN-fed piglets with sepsis had lower bile flow (P = .001) and increased bilirubin (P = .04). Neither jejunal permeability nor bacterial translocation were different between JC, JI, or sham groups., Conclusion: Contrary to our hypothesis, the remnant anatomy does not appear to worsen the progression of IFALD. However, the role of sepsis in IFALD should be further explored, in addition to other mechanisms, including PN factors, host immune responses, and intestinal bacterial dysbiosis., (© 2017 American Society for Parenteral and Enteral Nutrition.)
- Published
- 2018
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40. Marked stem/progenitor cell expansion occurs early after murine ileostomy: a new model.
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Fowler KL, Wieck MM, Hilton AE, Hou X, Schlieve CR, and Grikscheit TC
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- Adaptation, Physiological, Animals, Female, Male, Mice, Adult Stem Cells physiology, Ileostomy, Intestines cytology, Models, Animal, Short Bowel Syndrome
- Abstract
Background: Improving treatment for short bowel syndrome requires a better understanding of how intestinal adaptation is affected by factors like mechanoluminal stimulation. We hypothesized that in mice, luminal diversion via an ileostomy would drive adaptive changes similar to those seen in human intestine after diversion while offering the opportunity to study the immediate events after resection that precede intestinal adaptation., Materials and Methods: With Institutional Animal Care and Use Committee approval, a distal ileostomy with a long distal Hartman's was created in 9- to 14-week-old C57/B6 mice (n = 8). Control mice only had a midline laparotomy without stoma formation (n = 5). A rim of tissue from the proximal stoma was resected as a historical control for the proximal segment. Postoperatively, mice received a high-protein liquid diet and water ad libitum. On day 3, tissue from both the proximal and distal limbs were collected for histologic and RNA analysis. Morphometric measures, immunofluorescent antigen detection, and RNA expression were compared with Student paired t-tests with a P value < 0.05 considered significant., Results: At 3 d, survival for mice with an ileostomy was 87% and average weight loss was 12.5% of initial weight compared to 6.05% for control mice. Compared to the distal limb, the proximal limb in mice with an ileostomy demonstrated significantly taller villi with deeper and wider crypts. The proximal limb also had decreased expression of intestinal stem cell markers lgr5, bmi1, sox9, and ascl2. Fewer goblet and enteroendocrine cells per hemivillus were also noted in the proximal limb. In control mice, none of these measures were significant between proximal and distal ileum except for villus height., Conclusions: This new murine ileostomy model allows study of intestinal adaptation without intestinal anastomosis, which can be technically challenging and morbid., (Copyright © 2017 Elsevier Inc. All rights reserved.)
- Published
- 2017
- Full Text
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41. Parenteral therapy and complications in patients with intestinal failure in a regional unit.
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Lorentsen R, Munck LK, and Wildt S
- Subjects
- Adult, Aged, Aged, 80 and over, Denmark, Female, Humans, Incidence, Inflammatory Bowel Diseases complications, Male, Middle Aged, Neoplasms complications, Retrospective Studies, Short Bowel Syndrome etiology, Short Bowel Syndrome physiopathology, Surgical Procedures, Operative adverse effects, Thrombosis epidemiology, Catheter-Related Infections epidemiology, Central Venous Catheters adverse effects, Intestines physiopathology, Parenteral Nutrition, Short Bowel Syndrome therapy
- Abstract
Objective: To describe a cohort of patients with intestinal failure (IF) and tunnelled catheters in a regional IF unit, treatment and catheter-related complication rates, and to compare the quality of care with previously published results from specialised IF centres in Denmark., Methods: A retrospective chart review of an adult IF patient cohort receiving parenteral therapy through tunnelled catheters in a regional IF unit from 2005 to 2014. Demographics, indication, type and frequency of parenteral therapy, dwell time, cause of removal and complications were recorded., Results: Parenteral therapy was provided to 78 patients with a median age of 64 (25-86) years. Numbers increased from seven patients in 2005 to 40 in 2014. The cause of IF was surgical complications (33%), cancer (28%), inflammatory bowel disease (IBD, 15%) and other causes (24%). The median duration of parenteral therapy was 453 days (range: 16-3651 days). One hundred and forty-two tunnelled catheters were inserted. The incidence of catheter-related blood stream infection (CRBSI) was 1.51/1000 days (95% CI: 1.20-1.90) and the incidence of thrombosis was 0.10/1000 days (0.04-0.25). Seventy-two episodes of CRBSI occurred with 89 microorganisms cultured, the most common being coagulase-negative Staphylococcus (n = 25, 28%)., Conclusion: The rate of CRBSI did not differ from larger centres in Denmark but the rate of thrombotic events was higher than expected. Parenteral therapy can safely and effectively be offered to patients with IF in smaller centres.
- Published
- 2017
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42. The use of metabolic balance studies in the objective discrimination between intestinal insufficiency and intestinal failure.
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Prahm AP, Brandt CF, Askov-Hansen C, Mortensen PB, and Jeppesen PB
- Subjects
- Adult, Diagnosis, Differential, Dietary Fats metabolism, Disease Progression, Female, Humans, Intestinal Diseases etiology, Intestinal Diseases metabolism, Intestinal Diseases therapy, Malabsorption Syndromes etiology, Malabsorption Syndromes metabolism, Malabsorption Syndromes therapy, Male, Middle Aged, Parenteral Nutrition, Retrospective Studies, Short Bowel Syndrome complications, Short Bowel Syndrome metabolism, Short Bowel Syndrome therapy, Basal Metabolism, Energy Intake, Homeostasis, Intestinal Absorption, Intestinal Diseases diagnosis, Intestinal Mucosa metabolism, Intestines pathology
- Abstract
Background : In research settings that use metabolic balance studies (MBSs) of stable adult patients with short bowel syndrome, intestinal failure (IF) and dependence on parenteral support (PS) have been defined objectively as energy absorption <84% of calculated basal metabolic rate (BMR), wet weight (WW) absorption <23 g · kg body weight
-1 · d-1 , or both. Objective: This study aimed to explore and validate these borderlines in the clinical setting. Design: Intestinal absorption was measured from April 2003 to March 2015 in 175 consecutive patients with intestinal insufficiency (INS) in 96-h MBSs. They had not received PS 3 mo before referral. Results: To avoid the need for PS, the minimum absorptive requirements were energy absorption of ≥81% of BMR and WW absorption of ≥21 g · kg body weight-1 · d-1 , which were equivalent to findings in research settings (differences of 3.6% and 8.7%; P = 0.65 and 0.60, respectively). Oral failure defined as energy intake <130% of calculated BMR or WW intake <40 g · kg body weight-1 · d-1 was seen in 71% and 82% of the 10% of patients with the lowest energy absorption and WW absorption, respectively. Conclusions: In clinical settings, the borderlines between INS and IF were not significantly different from those in research settings, even in an unselected patient population in which oral failure was also a predominant cause of nutritional dyshomeostasis. MBSs may be recommended to identify the individual patient in the spectrum from INS to IF, to objectivize the cause of nutritional dyshomeostasis (oral failure, malabsorption, or both), and to quantify the effects of treatment., (© 2017 American Society for Nutrition.)- Published
- 2017
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43. Enteral nutrition in pediatric intestinal failure: does initial feeding impact on intestinal adaptation?
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Capriati T, Nobili V, Stronati L, Cucchiara S, Laureti F, Liguori A, Tyndall E, and Diamanti A
- Subjects
- Adaptation, Physiological, Adolescent, Age Factors, Child, Child, Preschool, Humans, Infant, Infant, Newborn, Short Bowel Syndrome diagnosis, Short Bowel Syndrome physiopathology, Treatment Outcome, Enteral Nutrition adverse effects, Intestines physiopathology, Short Bowel Syndrome therapy
- Abstract
Introduction: Primary IF can be due to impaired gut length or impaired gut function; short bowel syndrome (SBS) is the leading cause of IF. In IF patients complete enteral starvation should be avoided whenever possible and enteral/oral nutrition (EN/ON) should be employed at the maximum tolerated amount in each phase of the clinical evolution of IF. Intraluminal nutrients have stimulatory effects on epithelial cells and on trophism that enhance intestinal adaptation. Areas covered: Evidence for nutritional interventions in pediatric IF is limited and of poor quality. Clinical practice in SBS feeding are more 'experience-based' rather than 'evidence-based' and this dearth of clinical evidence is partly due to the rarity of this condition. This review updates knowledge concerning the impact of the initial diet with EN/ON in neonatal onset SBS in the process of bowel adaption. Expert commentary: Human milk resulted the preferred starting diet and it is generally combined with amino-acids (AAs) in Northern America and with hydrolyzed proteins (HFs) in Europe; polymeric diet is rarely employed. HFs were not more effective than AAs in promoting intestinal adaptation.
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- 2017
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44. Alterations in intestinal microbiota relate to intestinal failure-associated liver disease and central line infections.
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Wang P, Wang Y, Lu L, Yan W, Tao Y, Zhou K, Jia J, and Cai W
- Subjects
- Bacteria isolation & purification, Bacterial Infections metabolism, Bacterial Infections microbiology, Enzyme-Linked Immunosorbent Assay, Feces chemistry, Feces microbiology, Female, Humans, Infant, Intestinal Mucosa metabolism, Leukocyte L1 Antigen Complex metabolism, Liver Diseases metabolism, Liver Diseases microbiology, Male, Mass Spectrometry, Short Bowel Syndrome etiology, Short Bowel Syndrome metabolism, Bacteria genetics, Bacterial Infections complications, DNA, Bacterial analysis, Gastrointestinal Microbiome, Intestines microbiology, Liver Diseases complications, Short Bowel Syndrome microbiology
- Abstract
Background: The gut microbiota plays a vital role in modulating the metabolic and immune functions of the intestines. We aimed to analyze the dysbiosis of microbiota in infants with short bowel syndrome (SBS) with different complications., Procedure: We included 26 fecal samples from 18 infants with SBS during parenteral nutrition. The samples were categorized into three groups: asymptomatic, parenteral nutrition-associated liver disease (PNALD), and central line-associated bloodstream infection (CLABSI). Seven healthy infants were enrolled as controls. Fecal microbiota, secretory IgA, calprotectin, bile acids, and short chain fatty acids were detected., Results: The bacterial diversity of the Asymptomatic and Control Groups was significantly higher than that in the PNALD and CLABSI Groups. Proteobacteria was the most pronounced phylum in the PNALD and CLABSI Groups. Decreased acetate was observed in all SBS samples; however, fecal secretory IgA and calprotectin and the proportion of primary and secondary bile acids did not differ from those in healthy controls., Conclusions: Marked alterations of the intestinal microbiota with decreased level of acetate were shown in SBS patients compared with healthy controls. Over-abundance of Proteobacteria (especially Enterobacteriaceae) was found in the samples from the PNALD and CLABSI Groups., Level of Evidence: Prognosis Study, Level I., (Copyright © 2017 Elsevier Inc. All rights reserved.)
- Published
- 2017
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45. Differential Effects on Intestinal Adaptation Following Exogenous Glucagon-Like Peptide 2 Therapy With and Without Enteral Nutrition in Neonatal Short Bowel Syndrome [Formula: see text].
- Author
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Lim DW, Diané A, Muto M, Vine DF, Nation PN, Wizzard PR, Sigalet DL, Bigam DL, Pencharz PB, Turner JM, and Wales PW
- Subjects
- Adaptation, Physiological, Animals, Dietary Fats metabolism, Disease Models, Animal, Glucagon-Like Peptide 2 administration & dosage, Humans, Intestinal Absorption, Intestines pathology, Intestines surgery, Male, Parenteral Nutrition, Short Bowel Syndrome pathology, Short Bowel Syndrome physiopathology, Sus scrofa, Animals, Newborn, Enteral Nutrition, Glucagon-Like Peptide 2 therapeutic use, Intestines physiopathology, Short Bowel Syndrome therapy
- Abstract
Background: We aim to study the efficacy of exogenously administered glucagon-like peptide 2 (GLP-2) on intestinal adaptation in 2 preclinical models of neonatal short bowel syndrome (SBS) according to remnant intestinal anatomy, with and without ileum. Furthermore, we aim to determine if this adaptive effect was potentiated with enteral nutrition (EN)., Methods: Neonatal piglets were block-randomized to 75% mid-intestinal (JI group, retains ileum) or distal-intestinal (JC group, has no ileum) resection or no resection (sham control) and GLP-2 treatment (11 nmol/kg/d) or saline control for 7 days. Piglets received nutrition support, either 100% parenteral nutrition (PN; 0% EN, n = 32 in total) or 80% PN + 40% EN (n = 28 in total). Adaptation was assessed by morphological and histological changes, as well as RT quantitative polymerase chain reaction of nutrient transporters and tight junctional proteins and fat absorption. Data are analyzed by 3-way analysis of variance (ANOVA) and 2-way ANOVA per EN level., Results: GLP-2 treatment lengthened villi, deepened crypts, and improved intestinal weight in the remnant intestine of JC piglets. EN was a more potent adaptive stimulus for JI piglets. Small intestinal lengthening occurred only in the JI group, when given EN. There was no difference in total fat absorption and messenger RNA expression of nutrient transporters and tight junctional proteins., Conclusions: GLP-2 administration augmented structural adaptation in JC piglets with distal intestinal resection. Given JI anatomy, further stimulation by GLP-2 treatment over innate adaptation and stimulation by EN was modest and restricted to ileum. The differential effect of GLP-2 in neonatal SBS, depending on remnant anatomy, has important implications for clinical translation and planning of clinical trials.
- Published
- 2017
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46. Generation of intestinal surface: an absorbing tale.
- Author
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Walton KD, Freddo AM, Wang S, and Gumucio DL
- Subjects
- Animals, Humans, Microvilli metabolism, Models, Biological, Morphogenesis, Signal Transduction, Intestinal Absorption, Intestines growth & development
- Abstract
The vertebrate small intestine requires an enormous surface area to effectively absorb nutrients from food. Morphological adaptations required to establish this extensive surface include generation of an extremely long tube and convolution of the absorptive surface of the tube into villi and microvilli. In this Review, we discuss recent findings regarding the morphogenetic and molecular processes required for intestinal tube elongation and surface convolution, examine shared and unique aspects of these processes in different species, relate these processes to known human maladies that compromise absorptive function and highlight important questions for future research., (© 2016. Published by The Company of Biologists Ltd.)
- Published
- 2016
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47. The human milk oligosaccharide 2'-fucosyllactose augments the adaptive response to extensive intestinal.
- Author
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Mezoff EA, Hawkins JA, Ollberding NJ, Karns R, Morrow AL, and Helmrath MA
- Subjects
- Animals, Cecum surgery, Diet, Digestive System Surgical Procedures, Energy Metabolism drug effects, Humans, Ileum surgery, Male, Mice, Mice, Inbred C57BL, Microbiota, RNA, Ribosomal, 16S biosynthesis, Trisaccharides chemistry, Weight Gain drug effects, Adaptation, Physiological drug effects, Intestines drug effects, Intestines surgery, Milk, Human chemistry, Short Bowel Syndrome drug therapy, Trisaccharides pharmacology
- Abstract
Intestinal resection resulting in short bowel syndrome (SBS) carries a heavy burden of long-term morbidity, mortality, and cost of care, which can be attenuated with strategies that improve intestinal adaptation. SBS infants fed human milk, compared with formula, have more rapid intestinal adaptation. We tested the hypothesis that the major noncaloric human milk oligosaccharide 2'-fucosyllactose (2'-FL) contributes to the adaptive response after intestinal resection. Using a previously described murine model of intestinal adaptation, we demonstrated increased weight gain from 21 to 56 days (P < 0.001) and crypt depth at 56 days (P < 0.0095) with 2'-FL supplementation after ileocecal resection. Furthermore, 2'-FL increased small bowel luminal content microbial alpha diversity following resection (P < 0.005) and stimulated a bloom in organisms of the genus Parabacteroides (log2-fold = 4.1, P = 0.035). Finally, transcriptional analysis of the intestine revealed enriched ontologies and pathways related to antimicrobial peptides, metabolism, and energy processing. We conclude that 2'-FL supplementation following ileocecal resection increases weight gain, energy availability through microbial community modulation, and histological changes consistent with improved adaptation., (Copyright © 2016 the American Physiological Society.)
- Published
- 2016
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48. Modified Spiral Intestinal Lengthening and Tailoring for Short Bowel Syndrome.
- Author
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Mehrabi V, Mehrabi A, Jamshidi SH, Pedram MS, Sabagh MS, Jaberansari N, Fonouni HR, Sharifi AH, Malekzadeh R, and Frongia G
- Subjects
- Animals, Dogs, Feasibility Studies, Female, Plastic Surgery Procedures, Swine, Digestive System Surgical Procedures methods, Intestines surgery, Short Bowel Syndrome surgery
- Abstract
Background: The spiral intestinal lengthening and tailoring (SILT) procedure is a new surgical technique for autologous intestinal reconstruction in patients with short bowel syndrome. The aim of this work is to present a first description of a modified SILT technique by which the mucosal layer is left intact to possibly reduce the severe postoperative complications of intestinal leakage and abdominal abscess formation., Materials and Methods: The modified SILT technique was performed on a 10-cm-long intestinal segment in 2 pigs to determine the technical feasibility. Thereafter, the short-term clinical feasibility was monitored clinically in 2 dogs by gastrointestinal X-ray series at postoperative day 4 and by relaparotomy postoperative day 10., Results: It was technically feasible to lengthen the intestinal segment from 10 cm to 20 cm and tailoring it from 3 cm to 1.7 cm in diameter, while leaving the intestinal mucosal layer intact. The postoperative course was uneventful for both dogs. The gastrointestinal X-ray series showed an inconspicuous intestinal transit time without any signs of stricture, perforation, or leakage. In the relaparotomy, the initially achieved lengthening and tailoring extents were preserved and the operated intestinal segment was well perfused with no early signs of necrosis, stenosis, or leakage., Conclusions: Leaving the mucosal layer intact during SILT is technically and clinically feasible in the short term in a large animal model. Further studies are needed to fully assess the impact of this technical modification on the long-term outcome of larger series., (© The Author(s) 2015.)
- Published
- 2016
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49. Adult zebrafish intestine resection: a novel model of short bowel syndrome, adaptation, and intestinal stem cell regeneration.
- Author
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Schall KA, Holoyda KA, Grant CN, Levin DE, Torres ER, Maxwell A, Pollack HA, Moats RA, Frey MR, Darehzereshki A, Al Alam D, Lien C, and Grikscheit TC
- Subjects
- Animals, Antimetabolites pharmacology, Bromodeoxyuridine pharmacology, Cell Proliferation, Digestive System Surgical Procedures methods, Disease Models, Animal, Humans, Insulin-Like Growth Factor I metabolism, Intestinal Mucosa pathology, Male, Stem Cells physiology, Weight Loss, Zebrafish, Zebrafish Proteins metabolism, Adaptation, Biological physiology, Intestines pathology, Intestines physiopathology, Intestines surgery, Short Bowel Syndrome metabolism
- Abstract
Loss of significant intestinal length from congenital anomaly or disease may lead to short bowel syndrome (SBS); intestinal failure may be partially offset by a gain in epithelial surface area, termed adaptation. Current in vivo models of SBS are costly and technically challenging. Operative times and survival rates have slowed extension to transgenic models. We created a new reproducible in vivo model of SBS in zebrafish, a tractable vertebrate model, to facilitate investigation of the mechanisms of intestinal adaptation. Proximal intestinal diversion at segment 1 (S1, equivalent to jejunum) was performed in adult male zebrafish. SBS fish emptied distal intestinal contents via stoma as in the human disease. After 2 wk, S1 was dilated compared with controls and villus ridges had increased complexity, contributing to greater villus epithelial perimeter. The number of intervillus pockets, the intestinal stem cell zone of the zebrafish increased and contained a higher number of bromodeoxyuridine (BrdU)-labeled cells after 2 wk of SBS. Egf receptor and a subset of its ligands, also drivers of adaptation, were upregulated in SBS fish. Igf has been reported as a driver of intestinal adaptation in other animal models, and SBS fish exposed to a pharmacological inhibitor of the Igf receptor failed to demonstrate signs of intestinal adaptation, such as increased inner epithelial perimeter and BrdU incorporation. We describe a technically feasible model of human SBS in the zebrafish, a faster and less expensive tool to investigate intestinal stem cell plasticity as well as the mechanisms that drive intestinal adaptation., (Copyright © 2015 the American Physiological Society.)
- Published
- 2015
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50. Increased Anti-Flagellin and Anti-Lipopolysaccharide Immunoglobulins in Pediatric Intestinal Failure: Associations With Fever and Central Line-Associated Bloodstream Infections.
- Author
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Galloway DP, Troutt ML, Kocoshis SA, Gewirtz AT, Ziegler TR, and Cole CR
- Subjects
- Anti-Bacterial Agents therapeutic use, Biomarkers, Catheter-Related Infections complications, Catheter-Related Infections drug therapy, Catheter-Related Infections immunology, Catheterization, Central Venous, Catheters, Child, Preschool, Female, Fever etiology, Fever immunology, Gram-Negative Bacteria, Humans, Infant, Male, Ohio, Short Bowel Syndrome immunology, Short Bowel Syndrome microbiology, Antibodies, Anti-Idiotypic blood, Catheter-Related Infections diagnosis, Fever diagnosis, Flagellin immunology, Intestines microbiology, Intestines pathology, Lipopolysaccharides immunology, Short Bowel Syndrome complications
- Abstract
Background: Central line-associated bloodstream infections (CLABSIs) pose a significant challenge in the lives of patients with intestinal failure (IF). We hypothesized that plasma immunoglobulins against flagellin (FLiC) and lipopolysaccharide (LPS) would be able to differentiate CLABSIs from nonbacterial febrile episodes and that levels would increase with infection and decline following appropriate antibiotic treatment., Materials and Methods: Patients with IF, due to short bowel syndrome, between the ages of 3 months and 4 years of age, were recruited at Cincinnati Children's Hospital Medical Center. Anti-FLiC and anti-LPS plasma antibody levels were measured in 13 children with IF at baseline, during febrile events, and also following treatment with antibiotics. These were also measured in 11 healthy children without IF who were recruited as controls., Results: Plasma anti-FLiC IgA levels increased during febrile episodes in all patients with IF (baseline mean of 1.10 vs febrile episode mean of 1.32 optical density units, respectively; P = .046). Neither plasma anti-FLiC nor anti-LPS IgA or IgG levels distinguished CLABSI from nonbacterial febrile episodes compared with baseline levels. Compared with controls, patients with IF had significantly higher plasma levels of anti-FLiC and anti-LPS IgA at baseline., Conclusion: Plasma anti-FLiC IgA antibody levels rise during febrile episodes but do not differentiate between nonbacterial febrile illnesses and CLABSIs in pediatric IF. However, the upregulation of these antibodies in IF suggests the baseline systemic presence of Gram-negative bacterial products., (© 2014 American Society for Parenteral and Enteral Nutrition.)
- Published
- 2015
- Full Text
- View/download PDF
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