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1.
Nihon Shokakibyo Gakkai Zasshi ; 121(10): 835-841, 2024.
Artigo em Japonês | MEDLINE | ID: mdl-39384396

RESUMO

A 29-year-old man presented to the hospital with complaints of abdominal pain and vomiting. Abdominal computed tomography revealed intestinal malrotation, a whirl sign of the small intestine, and occlusion of the superior mesenteric artery and vein. Due to dilation of the small intestine and poor contrast enhancement of the intestinal wall, he was diagnosed with strangulating small bowel obstruction with intestinal necrosis. Emergency surgery was performed owing to the diagnosis of small bowel volvulus with intestinal malrotation, which necessitated massive small bowel resection. The remnant small intestine was approximately 100cm in length. The patient developed short bowel syndrome postoperatively but eventually resumed a normal diet.


Assuntos
Volvo Intestinal , Intestino Delgado , Complicações Pós-Operatórias , Síndrome do Intestino Curto , Humanos , Volvo Intestinal/cirurgia , Volvo Intestinal/diagnóstico por imagem , Volvo Intestinal/etiologia , Masculino , Adulto , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/etiologia , Síndrome do Intestino Curto/complicações , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/cirurgia , Intestino Delgado/anormalidades , Complicações Pós-Operatórias/etiologia
2.
J Pediatr Gastroenterol Nutr ; 79(3): 705-715, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39046027

RESUMO

BACKGROUND: Infants with small bowel stomas (SBstoma) frequently struggle with absorption and rely on parenteral nutrition (PN). Intestinal absorption is difficult to predict based solely on intestinal anatomy. The purpose of this study was to characterize the microbiota and metabolic by-products within stoma effluent and correlate with clinical features and intestinal absorption. METHODS: Prospective cohort study collecting stoma samples from neonates with SBstoma (N = 23) or colostomy control (N = 6) at initial enteral feed (first sample) and before stoma closure (last sample). Gut bacteriome (16S ribosomal RNA [rRNA] sequencing), short-chain fatty acids (SCFAs) and bile acids (BAs) were characterized along with volume and energy content of a 48 h collection via bomb calorimetry (last sample). Hierarchical clustering and linear regression were used to compare the bacteriome and BAs/SCFAs, to bowel length, PN, and growth. RESULTS: Infants with ≤50% small bowel lost more fluid on average than those with >50% and controls (22, 18, 16 mL/kg/day, p = 0.013), but had similar energy losses (7, 10, 9 kcal/kg/day, p = 0.147). Infants growing poorly had enrichment of Proteobacteria compared to infants growing well (90% vs. 15%, p = 0.004). An increase in the ratio of secondary BAs within the small bowel over time, correlated with poor prognostic factors (≤50% small bowel, >50% of calories from PN, and poor growth). CONCLUSION: Infants with SBstoma and poor growth have a unique bacteriome community and those with poor enteral tolerance have metabolic differences compared to infants with improved absorption.


Assuntos
Microbioma Gastrointestinal , Intestino Delgado , Humanos , Estudos Prospectivos , Masculino , Feminino , Recém-Nascido , Intestino Delgado/microbiologia , Intestino Delgado/metabolismo , Lactente , Síndrome do Intestino Curto/microbiologia , Síndrome do Intestino Curto/metabolismo , Síndrome do Intestino Curto/cirurgia , Absorção Intestinal , Estomas Cirúrgicos/microbiologia , Nutrição Parenteral , Ácidos e Sais Biliares/metabolismo , Ácidos Graxos Voláteis/metabolismo , Ácidos Graxos Voláteis/análise , Colostomia
3.
Gastroenterol Clin North Am ; 53(3): 481-491, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39068009

RESUMO

This comprehensive review focuses on advances in surgical techniques and in vivo animal models for treating short bowel syndrome (SBS) with intestinal organoids. Notably, this review discusses a novel method involving the replacement of the epithelium of large intestinal tissue with small intestinal organoids, which improves function and prognosis when grafted back into the small intestine. This study not only underscores the importance of integrating organoid technology and surgical techniques to improve the outcomes of patients with SBS but also acknowledges the challenges that lie ahead, including achieving functional organoids with peristaltic movement and vascularization.


Assuntos
Organoides , Síndrome do Intestino Curto , Síndrome do Intestino Curto/cirurgia , Humanos , Organoides/transplante , Animais , Colo/cirurgia , Modelos Animais de Doenças , Intestino Delgado/transplante , Mucosa Intestinal/transplante
4.
Clin Nutr ESPEN ; 62: 247-252, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38857151

RESUMO

AIMS: To report the results and successes of intestinal transplantation (ITx) in the most active European centres, to emphasize that, although it is a difficult procedure, it should remain a therapeutic option for children with total, definitive and complicated intestinal failure when intestinal rehabilitation fails. METHODS: We retrospectively collected data about all patients less than 18 receiving an ITx from 2010 to 2022 in 8 centres, and outcomes in July 2022. RESULTS: ITx was performed in 155 patients, median age 6.9 years, in 45% for short bowel syndromes, 22% congenital enteropathies, 25% motility disorders, and 15% re-transplantations. Indications were multiple in most patients, intestinal failure-associated liver disease in half. The graft was in 70% liver-containing. At last follow up 64% were alive, weaned from parenteral nutrition, for 7.9 years; 27% had died and the graft was removed in 8%, mostly early after ITx. DISCUSSION: ITx, despite its difficulties, can give a future to children with complicated intestinal failure. It should be considered among the therapeutic options offered to patients with a predicted survival rate lower than that after ITx. Patients should be early discussed within multidisciplinary teams in ITx centres, to avoid severe complications impacting the results of ITx, or even to avoid ITx.


Assuntos
Intestinos , Humanos , Estudos Retrospectivos , Criança , Masculino , Feminino , Intestinos/transplante , Pré-Escolar , Lactente , Resultado do Tratamento , Adolescente , Insuficiência Intestinal , Síndrome do Intestino Curto/cirurgia , Enteropatias/cirurgia , Europa (Continente) , Nutrição Parenteral
5.
J Pediatr Gastroenterol Nutr ; 79(2): 278-289, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38828781

RESUMO

OBJECTIVES: To review recent evaluations of pediatric patients with intestinal failure (IF) for intestinal transplantation (ITx), waiting list decisions, and outcomes of patients listed and not listed for ITx at our center. METHODS: Retrospective chart review of 97 patients evaluated for ITx from January 2014 to December 2021 including data from referring institutions and protocol laboratory testing, body imaging, endoscopy, and liver biopsy in selected cases. Survival analysis used Kaplan-Meier estimates and Cox proportional hazards regression. RESULTS: Patients were referred almost entirely from outside institutions, one-third because of intestinal failure-associated liver disease (IFALD), two-thirds because of repeated infective and non-IFALD complications under minimally successful intestinal rehabilitation, and a single patient because of lost central vein access. The majority had short bowel syndrome (SBS). Waiting list placement was offered to 67 (69%) patients, 40 of whom for IFALD. The IFALD group was generally younger and more likely to have SBS, have received more parenteral nutrition, have demonstrated more evidence of chronic inflammation and have inferior kidney function compared to those offered ITx for non-IFALD complications and those not listed. ITx was performed in 53 patients. Superior postevaluation survival was independently associated with higher serum creatinine (hazard ratio [HR] 15.410, p = 014), whereas inferior postevaluation survival was associated with ITx (HR 0.515, p = 0.035) and higher serum fibrinogen (HR 0.994, p = 0.005). CONCLUSIONS: Despite recent improvements in IF management, IFALD remains a prominent reason for ITx referral. Complications of IF inherent to ITx candidacy influence postevaluation and post-ITx survival.


Assuntos
Intestinos , Listas de Espera , Humanos , Estudos Retrospectivos , Masculino , Feminino , Criança , Pré-Escolar , Lactente , Intestinos/transplante , Adolescente , Insuficiência Intestinal , Síndrome do Intestino Curto/cirurgia , Hepatopatias/cirurgia
6.
Nutrients ; 16(10)2024 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-38794694

RESUMO

BACKGROUND: Although parenteral nutrition (PN) significantly improves mortality rates in pediatric short bowel syndrome (SBS), long-term PN has many possible complications and impacts quality of life. Bowel lengthening procedures (BLPs) increase the contact surface of food and the intestinal mucosa and enable the better absorption of nutrients and liquids, possibly leading to a PN decrease. METHODS: We retrospectively reviewed the data of patients with short bowel syndrome who underwent BLPs in the period from January 2016 to January 2022. Overall, eight patients, four male, five born prematurely, underwent BLPs. RESULTS: There was a significant decrease in the percentage of total caloric intake provided via PN and PN volume after the BLPs. The more evident results were seen 6 months after the procedure and at the last follow-up, which was, on average, 31 months after the procedure. Two patients were weaned off PN after their BLPs. Patients remained well nourished during the follow-up. CONCLUSIONS: The BLP led to a significant decrease in PN needs and an increase in the food intake; however, significant changes happened more than 6 months after the procedure.


Assuntos
Nutrição Parenteral , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/terapia , Masculino , Feminino , Estudos Retrospectivos , Resultado do Tratamento , Lactente , Pré-Escolar , Criança , Estado Nutricional , Ingestão de Energia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Qualidade de Vida
7.
Pediatr Transplant ; 28(3): e14756, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38623905

RESUMO

INTRODUCTION: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. OBJECTIVES: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. METHODS: The review is based on the authors' experience supported by an in-depth review of the published literature. RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. DISCUSSION: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.


Assuntos
Enteropatias , Insuficiência Intestinal , Síndrome do Intestino Curto , Transplantes , Criança , Lactente , Humanos , Intestinos , Intestino Delgado , Nutrição Parenteral , Enteropatias/cirurgia , Síndrome do Intestino Curto/cirurgia
8.
Pediatr Surg Int ; 40(1): 79, 2024 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-38492075

RESUMO

BACKGROUND: Intestinal Failure, parenteral nutrition (PN) dependence, and subsequent liver disease are the most challenging and life-threatening complications of short bowel syndrome experienced by patients with total intestinal aganglionosis. Skipped Aganglionic Lengthening Transposition (SALT) showed to be a promising procedure to overcome such problems. We herein report the results of two patients who underwent SALT at the Umberto Bosio Center for Digestive Diseases. PATIENTS AND METHODS: Between November 2019 and July 2022, 2 patients with total intestinal aganglionosis underwent SALT as autologous intestinal lengthening procedure. Perioperative data and long-term outcomes are reported. Patient #1-A 18 month-old male (PN dependant) with 30 cm of ganglionated bowel at birth experienced a 35% increase of intestinal length after SALT (from 43 to 58 cm) thanks to three 5 cm interposed aganglionic loops. Postoperative course was uneventful and he was totally weaned by PN after 28 months postoperatively. He is without PN only receiving enteric feeding 53 months after the procedure. Patient #2-A 11 year-old female (PN dependant) with 100 cm of ganglionated jejunum underwent SALT at 11 years and experienced a 19% increase of bowel length thanks to four 5 to 7 cm interposed aganglionic loops. Postoperatively she required excision of two out of the four loops due to severe strictures and inadequate perfusion with a subsequent overall 10% increase of length after SALT. Of note, she improved significantly with a progressive reduction of PN that has been stopped after 18 months. CONCLUSION: Skipped aganglionic lengthening transposition (SALT) seems to be very effective in improving nutrients absorption in patients with total intestinal aganglionosis by increasing absorptive bowel surface and decelerating intestinal flow for a longer and more effective contact of enteric material with ileal mucosa. Provided these impressive results are confirmed in the very long-term, SALT could become a valid alternative for the treatment of patients with total intestinal aganglionosis carrying at birth at least 20 to 30 cm of ganglionated jejunum.


Assuntos
Doença de Hirschsprung , Insuficiência Intestinal , Síndrome do Intestino Curto , Criança , Feminino , Humanos , Lactente , Masculino , Intestino Delgado , Intestinos/cirurgia , Síndrome do Intestino Curto/cirurgia , Resultado do Tratamento
9.
Chirurgie (Heidelb) ; 95(5): 375-381, 2024 May.
Artigo em Alemão | MEDLINE | ID: mdl-38427034

RESUMO

BACKGROUND: Intestinal transplantation (ITx) is the only causal treatment for complicated chronic intestinal failure after mesenteric ischemia and impending failure of parenteral supplementation. Isolated or combined ITx with the inclusion of the intestine is associated with demanding immunological, perioperative and infection associated challenges. AIM: The characterization of chronic intestinal failure, the indications, transplant survival, transplantation techniques and success rates. MATERIAL AND METHODS: Collection, summary and critical appraisal of international guidelines, the guidelines of the German Medical Chamber, and the international literature. RESULTS: The first successful ITx were performed in 1987 and 1988 at the University of Kiel Germany and the University of Pittsburgh, USA. The number of ITx rose continuously but in phases from the end of the 1990s to over 200 per year but has currently decreased to 100-150 per year due to optimized intestinal rehabilitation. While the 1­year and 3­year transplant survival rates were 30% and 20% before 1991, they increased in phases up to 60% and 50%, respectively, after 1995 and have now achieved almost 80% and 70%, respectively. CONCLUSION: The substantial improvement in the results of ITx can be partly explained by progress in operative techniques, intensive care medicine and a better understanding of mucosal immunity; however, optimized strategies in immunosuppression as well as prevention of infectious diseases and malignancies have also made decisive contributions.


Assuntos
Enteropatias , Insuficiência Intestinal , Isquemia Mesentérica , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/complicações , Isquemia Mesentérica/cirurgia , Isquemia Mesentérica/complicações , Intestinos/cirurgia , Enteropatias/complicações , Enteropatias/cirurgia , Doença Crônica
10.
Clin Nutr ; 43(5): 1043-1050, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38554476

RESUMO

BACKGROUND & AIMS: Acid-base disturbances are common in short bowel (SB) patients due to increased intestinal bicarbonate loss. However, the resulting systemic acid load has not been quantified. Base excess is used to monitor metabolic acid-base disturbances but inadequately reflects the acid load. Our aim was to investigate the systemic acid/base load in SB-patients to obtain quantitative estimates to guide the composition of parenteral support. METHODS: We calculated total acid load in SB patients by summing 24-h urinary net acid excretion (NAE) and the provision of base equivalents in parenteral support. We then compared differences among anatomical SB-types: jejunostomy (SB-J), jejunocolostomy (SB-JC), and jejunoileostomy (SB-JIC). 47 urine samples from 34 SB patients were analyzed for bicarbonate (HCO3-), ammonium (NH4+), and titratable acid (TA) concentrations. NAE was calculated as (TA + NH4+) - HCO3-. Mixed-effects repeated-measures models were used to statistically examine differences between SB-types and associations with parenteral nutrition and NAE. A healthy cohort served as control. RESULTS: In comparison to SB-J, SB-JC patients had a 4.1 mmoL/l lower base excess (95% CI: -6.3 to -1.8) and an 84.5 mmol/day higher total acid load (CI: 41.3 to 127.7). There were no significant differences between SB-JIC and SB-J regarding base excess, NAE, or total acid load. Higher amounts of infused acetate, sodium, and chloride, but not the acetate/chloride ratio, were associated with lower NAE and higher base excess. CONCLUSIONS: Due to increased colonic bicarbonate loss, patients with SB-JC have a ∼4.4-fold higher acid load than healthy controls. The ion transport mechanisms mediating this bicarbonate loss from the remaining colon need further experimental investigation. NAE could be a useful tool to adjust base infusion in SB.


Assuntos
Bicarbonatos , Colo , Nutrição Parenteral , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Nutrição Parenteral/métodos , Colo/cirurgia , Colo/metabolismo , Adulto , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/terapia , Anastomose Cirúrgica , Desequilíbrio Ácido-Base , Idoso , Equilíbrio Ácido-Base
11.
Bol Med Hosp Infant Mex ; 81(1): 23-30, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38503324

RESUMO

BACKGROUND: Short bowel syndrome (SBS) is one of the most frequent causes of intestinal failure, needing parenteral nutrition to maintain an energy-protein and water-electrolyte balance. At the Hospital Infantil de México Federico Gómez (HIMFG), the formation of two stomas is a technique used for intestinal rehabilitation, where the use of residue through the bypass technique (BT) helps to maintain gastrointestinal functionality, water-electrolyte, and nutritional stability. This study aimed to describe the technique of using intestinal residue through BT as a treatment strategy in intestinal rehabilitation and its effect on the biochemical and nutritional status of pediatric patients with SBS. METHODS: An analytical and retrospective cross-sectional study was performed in patients hospitalized at HIMFG with SBS who underwent BT during their hospital stay between 2019 and 2020 and then followed up for 8 weeks. RESULTS: A total of 10 patients were included in this study, with a mean age of 24 months; 50% were female. BT was able to reduce the inflammatory process in the liver caused by the continuous use of parenteral nutrition; enteral caloric intake increased from 25.32 kcal/kg/day to 72.94 kcal/kg/day, but it was insufficient to improve their nutritional status. CONCLUSIONS: BT is a safe and effective alternative in intestinal rehabilitation in patients with SBS to stimulate trophism and intestinal functionality, allowing a progression of enteral feeding and a decrease in the hepatic inflammatory process that occurs in these patients with prolonged parenteral nutrition.


INTRODUCCIÓN: El síndrome de intestino corto (SIC) es una de las causas más frecuentes de insuficiencia intestinal que requiere del uso de nutrición parenteral para mantener un balance energético-proteico e hidroelectrolítico. En el Hospital Infantil de México Federico Gómez (HIMFG) la formación de dos estomas es una técnica empleada para la rehabilitación intestinal, donde con el aprovechamiento de residuo mediante la técnica de puenteo (TP) se ayuda a mantener la funcionalidad gastrointestinal, equilibrio hidro-electrolítico y estabilidad nutricional. El objetivo de este estudio fue describir la técnica del aprovechamiento de residuo intestinal mediante TP como estrategia de tratamiento en la rehabilitación intestinal y su efecto en el estado bioquímico y nutricional de pacientes pediátricos con SIC. MÉTODOS: Se llevó a cabo un estudio transversal analítico y retrospectivo en pacientes hospitalizados en el HIMFG con SIC en quienes se realizó la TP durante su estancia intrahospitalaria entre 2019 y 2020. RESULTADOS: Se incluyeron 10 pacientes en este estudio, con una edad promedio de 24 meses, y el 50% de sexo femenino. La TP logró disminuir el proceso inflamatorio hepático ocasionado por el uso continuo de nutrición parenteral; la ingesta calórica por vía enteral incrementó de 25.32 kcal/kg/día a 72.94 kcal/kg/día, pero fue insuficiente para mejorar el estado nutricional. CONCLUSIONES: La TP es una alternativa segura y efectiva en la rehabilitación intestinal en pacientes con SIC para estimular el trofismo y funcionalidad intestinal, permitiendo una progresión de la alimentación enteral y disminución del proceso inflamatorio hepático que se presentan en estos pacientes con nutrición parenteral prolongada.


Assuntos
Síndrome do Intestino Curto , Humanos , Criança , Feminino , Pré-Escolar , Masculino , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/etiologia , Estudos Retrospectivos , Estudos Transversais , Derivação Jejunoileal/efeitos adversos , México
12.
Colorectal Dis ; 26(2): 243-257, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38177086

RESUMO

AIM: The gastrointestinal bile acid (BA)/microbiota axis has emerged as a potential mediator of health and disease, particularly in relation to pathologies such as inflammatory bowel disease (IBD) and colorectal cancer. Whilst it presents an exciting new avenue for therapies, it has not yet been characterized in surgical resection of the ileum, where BA reabsorption occurs. The identification of BA/microbiota signatures may provide future therapies with perioperative personalized medicine. In this work we conduct a systematic review with the aim of investigating the microbiome and BA changes that are associated with resection of the ileum. METHOD: The databases included were MEDLINE, EMBASE, Web of Science and Cochrane libraries. The outcomes of interest were faecal microbiome and BA signatures after ileal resection. RESULTS: Of the initial 3106 articles, three studies met the inclusion/exclusion criteria for data extraction. A total of 257 patients (46% surgery, 54% nonsurgery controls) were included in the three studies. Two studies included patients with short bowel syndrome and the other included patients with IBD. Large-scale microbiota changes were reported. In general, alpha diversity had decreased amongst patients with ileal surgery. Phylum-level changes included decreased Bacteroidetes and increased Proteobacteria and Fusobacteria in patients with an intestinal resection. Surgery was associated with increased total faecal BAs, cholic acid and chenodeoxycholic acid. There were decreases in deoxycholic acid and glycine and taurine conjugated bile salts. Integrated BA and microbiota data identified correlations with several bacterial families and BA. CONCLUSION: The BA/microbiota axis is still a novel area with minimal observational data in surgery. Further mechanistic research is necessary to further explore this and identify its role in improving perioperative outcomes.


Assuntos
Ácidos e Sais Biliares , Fezes , Microbioma Gastrointestinal , Íleo , Doenças Inflamatórias Intestinais , Humanos , Ácidos e Sais Biliares/metabolismo , Microbioma Gastrointestinal/fisiologia , Íleo/cirurgia , Íleo/microbiologia , Fezes/microbiologia , Doenças Inflamatórias Intestinais/cirurgia , Doenças Inflamatórias Intestinais/microbiologia , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/microbiologia , Síndrome do Intestino Curto/metabolismo , Feminino , Masculino , Adulto , Pessoa de Meia-Idade
13.
JPEN J Parenter Enteral Nutr ; 47(8): 1028-1037, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37726175

RESUMO

BACKGROUND: Enteral drug therapy is challenging in short bowel syndrome with intestinal failure (SBS-IF) because of unpredictable absorption. SEFA-6179 is an enterally administered medium-chain fatty acid analogue under development for intestinal failure-associated liver disease. We investigate the pharmacokinetics of two SEFA-6179 formulations in two large-animal models of SBS-IF, including a new pseudojejunostomy model. METHODS: Twenty Yucatan minipigs were obtained. Half underwent pre-resection pharmacokinetic study with single-dose SEFA-6179 administration. All minipigs then underwent 90% jejunoileal resection, with either a jejunoileal anastomosis or bypass of the intraperitoneal colon with anastomosis just proximal to the rectum (pseudojejunostomy). On postoperative day 3, a single-dose pharmacokinetic study was performed. RESULTS: Both SBS-IF models were well tolerated. Compared with the jejunoileal anastomosis minipigs, pseudojejunostomy minipigs had a more severe malabsorptive phenotype with weight loss by postoperative day 4 (+0.1 vs -0.9 kg, P = 0.03) and liquid diarrhea (Bristol 5 vs Bristol 7, P = 0.0007). Compared with pre-resection minipigs, both jejunoileal and pseudojejunostomy minipigs had lower total plasma exposure of SEFA-6179 measured by area under the curve (jejunoileal: 37% less, P = 0.049; pseudojejunostomy: 74% less, P = 0.0001). Peak plasma concentration was also lower in the pseudojejunostomy group compared with pre-resection (65% less, P = 0.04), but not lower in the jejunoileal group (P = 0.47). CONCLUSION: In two SBS-IF minipig models, SEFA-6179 had substantially decreased absorption compared with pre-resection minipigs. Dose optimization for different intestinal anatomy and function may be required. We describe a new SBS-IF pseudojejunostomy model that may improve the translation of preclinical research to patients with SBS-IF who have enterostomies.


Assuntos
Enteropatias , Insuficiência Intestinal , Síndrome do Intestino Curto , Animais , Humanos , Suínos , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/tratamento farmacológico , Porco Miniatura , Intestinos , Ácidos Graxos , Modelos Animais de Doenças
14.
Pediatr Surg Int ; 39(1): 276, 2023 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-37755555

RESUMO

INTRODUCTION: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung's disease. Here, we report the results of the Japanese ITx registry. METHODS: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. RESULTS: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. CONCLUSION: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good.


Assuntos
Enteropatias , Síndrome do Intestino Curto , Criança , Humanos , Japão , Qualidade de Vida , Síndrome do Intestino Curto/cirurgia , Estudos Retrospectivos , Intestinos , Enteropatias/cirurgia , Doadores Vivos
15.
J Pediatr Gastroenterol Nutr ; 77(6): 788-795, 2023 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-37705420

RESUMO

OBJECTIVES: Anastomotic ulceration (AU) is a rare but life-threatening complication of pediatric short bowel syndrome (SBS). AUs may be challenging to detect and refractory to treatment. This study aimed to identify features associated with symptomatic bleeding AUs in children with SBS and factors that may impact resolution of bleeding. The relationship between dietary changes and symptomatic anastomotic hemorrhage was also explored. METHODS: We conducted a retrospective chart review of 381 patients cared for in the Intestinal Rehabilitation Program at our center from 2013 to 2022. Patients with symptomatic AUs were identified based on at least 1 endoscopic procedure showing AUs and evidence of clinically significant gastrointestinal bleeding. We collected patient demographics, clinical characteristics, dietary history, radiologic imaging, and histopathology. We used descriptive statistics to identify patterns of presentation. RESULTS: AUs were identified in 22 patients who were followed for a median duration of 2.9 years after anastomotic ulcer identification. AUs uniformly evolved years after the initial anastomosis (median 3.2 years). Characteristics included bowel stricture (4/22), small bowel-colon anastomosis (19/22), partial colectomy (17/22), and an increase in whole foods fraction (12/18). Bleeding resolved with operative intervention in the majority with anastomotic stricture (3/4). Recurrent bleeding was common in those without stricture (13/18). In a subset of patients without stricture, whole food reduction was associated with improvement or resolution of bleeding (5/6). CONCLUSIONS: We observed a higher proportion of patients with AUs who responded to surgical intervention in the subset of children with definitive anastomotic strictures versus those without, suggesting that careful characterization of intestinal anatomy may be critical to predicting response to therapy. We also observed that bleeding from AU typically first manifested within 1 year of a shift from elemental or hydrolyzed enteral formula to a whole food-based diet (including commercial blenderized feeds), which may indicate that components of the enteral diet play a role in the pathogenesis of AU. Further studies are needed to validate these hypotheses.


Assuntos
Obstrução Intestinal , Síndrome do Intestino Curto , Humanos , Criança , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/cirurgia , Estudos Retrospectivos , Constrição Patológica/etiologia , Seguimentos , Úlcera/etiologia , Úlcera/cirurgia , Anastomose Cirúrgica/efeitos adversos , Obstrução Intestinal/etiologia , Resultado do Tratamento
16.
JPEN J Parenter Enteral Nutr ; 47(8): 1047-1055, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37573479

RESUMO

INTRODUCTION: The International Intestinal Failure Registry (IIFR) is an international consortium to study intestinal failure (IF) outcomes in a large contemporary pediatric cohort. We aimed to identify predictors of early (1-year) enteral autonomy. METHODS: We included IIFR pilot phase patients. IF was defined by a parenteral nutrition need for at least 60 days due to a primary gastrointestinal etiology. The primary outcome was time to enteral autonomy achievement. We built a mixed-effects Weibull accelerated failure time model with random effects by center to analyze variables associated with enteral autonomy achievement with a primary outcome of time ratio (TR). RESULTS: We included 189 patients (82% with short bowel syndrome) representing 11 international centers. Cumulative incidence of early enteral autonomy was 51.6%, and death was 6.5%. In multivariable analysis, ostomy presence (TR, 2.63; 95% CI, 1.41-4.90) was associated with increased time to enteral autonomy achievement, and Asian/Indian (TR, 0.28; 95% CI, 0.10-0.81) and Pacific Islander race (TR, 0.34; 95% CI, 0.13-0.90) were associated with decreased time to enteral autonomy achievement. In a second model in the subset with measured percentage of bowel length remaining, ostomy presence (TR, 4.21; 95% CI, 1.90-9.33) was associated with increased time to enteral autonomy achievement, whereas greater percentage of bowel remaining (TR, 0.96; 95% CI, 0.94-0.98) was associated with decreased time to enteral autonomy achievement. CONCLUSIONS: Minimizing bowel resection at initial surgery and establishing bowel continuity by ostomy reversal can effectively decrease the time to early enteral autonomy achievement in children with IF.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Humanos , Criança , Estudos Retrospectivos , Intestino Delgado/cirurgia , Intestinos , Síndrome do Intestino Curto/cirurgia
17.
Expert Rev Gastroenterol Hepatol ; 17(7): 677-690, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37264646

RESUMO

INTRODUCTION: Advances in the management of intestinal failure have led to a reduction in the number of intestinal transplants. The number of bowel transplants has been mainly stable even though a slight increase has been observed in the last 5 years. AREAS COVERED: Standard indication includes patients with a reasonable life expectancy. Recent progress can be deduced by the increased number of intestine transplants in adults: this is due to the continuous improvement of 1-year graft survival worldwide (without differences in 3- and 5-year) associated with better abdominal wall closure techniques. This review aims to provide an update on new indications and changes in trends of pediatric and adult intestine transplantation. This analysis, which stretches through the past 5 years, is based on a collection of related manuscripts from PubMed. EXPERT COMMENTARY: Intestinal transplants should be solely intended for a group of individuals for whom indications for transplantation are clear and both medical and surgical rehabilitations have failed. Nevertheless, many protocols developed over the years have not yet solved the key question represented by the over-immunosuppression. Novel indications and recent progress in the bowel transplant field, minimal yet consistent, represent a pathway to be followed.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Síndrome do Intestino Curto , Adulto , Humanos , Criança , Intestino Delgado/transplante , Intestinos/transplante , Terapia de Imunossupressão/métodos , Síndrome do Intestino Curto/cirurgia
18.
Nutrients ; 15(12)2023 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-37375644

RESUMO

Liraglutide, a glucagon-like peptide-1 agonist, has been shown to have beneficial effects on fecal output in short bowel syndrome (SBS) by small human studies. Its potential effects early after gut resection are not known. In this pilot observational study, we described the 1- and 6-month liraglutide effects in 19 adult patients with a new SBS diagnosis within 1 month after surgical resection. Stomal/fecal and urinary outcomes, serum/urinary electrolytes, and body composition were assessed. Both within-group differences and between-group comparisons with 20 SBS patients refusing liraglutide treatment were evaluated. The main liraglutide-related side effect was mild nausea, except in one patient, who experienced severe nausea/vomiting. The median ostomy/fecal output was significantly reduced by -550 mL/day after 6 months of treatment (vs. -200 mL/day in untreated, p = 0.04). The number of patients reaching a ≥20% output reduction was 10/19 (52.6%) treated vs. 3/20 (15.0%) untreated patients (p = 0.013) at 1 month and 12/19 (63.2%) vs. 6/20 (30.0%) (p = 0.038) at 6 months, respectively. Participants with a clinically relevant output reduction at 6 months had a significantly lower baseline weight and BMI. Energy parenteral supply significantly decreased, while infused volumes, oral energy, and fluid intakes slightly decreased, though not significantly. This pilot study supports liraglutide benefits in ostomy/fecal output early after surgical gut resection in SBS patients, particularly in those with lower baseline weight values.


Assuntos
Liraglutida , Síndrome do Intestino Curto , Adulto , Humanos , Liraglutida/efeitos adversos , Projetos Piloto , Síndrome do Intestino Curto/tratamento farmacológico , Síndrome do Intestino Curto/cirurgia , Peso Corporal , Náusea/tratamento farmacológico , Hipoglicemiantes/uso terapêutico
19.
Semin Pediatr Surg ; 32(3): 151314, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37276784

RESUMO

Massive intestinal resection is a regrettably necessary but life-saving intervention for progressive or fulminant necrotizing enterocolitis (NEC). However, the resultant short bowel syndrome (SBS) poses its own array of challenges and complications. Within hours of such an abrupt loss of intestinal length, the intestine begins to adapt. Our ability to understand this process of intestinal adaptation has proven critical in our ability to clinically treat the challenging problem of short bowel syndrome. This review first highlights key data relating to intestinal adaptation including structural and functional changes, biochemical regulation, and other factors affecting the magnitude of intestinal adaptation responses. We then focus on intestinal rehabilitation as it relates to strategies to enhance intestinal adaptation while meeting nutritional needs and preventing complications of parenteral nutrition.


Assuntos
Enterocolite Necrosante , Síndrome do Intestino Curto , Recém-Nascido , Humanos , Síndrome do Intestino Curto/cirurgia , Intestinos , Nutrição Parenteral , Adaptação Fisiológica , Enterocolite Necrosante/cirurgia
20.
Nursing ; 53(6): 29-35, 2023 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-37212815

RESUMO

ABSTRACT: Short bowel syndrome is a rare condition that can lead to intestinal failure. It occurs as a congenital condition or due to massive small bowel resection. This article provides an overview of this condition in adult patients, including diagnosis and treatment in inpatient and outpatient settings.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Humanos , Adulto , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/cirurgia
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