Consenso colombiano de Falla Intestinal Crónica en Síndrome de Intestino Corto / Colombian Consensus on Chronic Intestinal Failure in Short Bowel Syndrome

Introducción. Los términos falla intestinal crónica, síndrome de intestino corto (SIC) y nutrición parenteral total son muy frecuentes en la práctica clínica cotidiana.El objetivo de esta guía fue establecer un marco de referencia de práctica clínica basado en el mejor de nivel de evidencia en pacientes con falla intestinal crónica secundaria a síndrome de intestino corto. Métodos. Se estableció un grupo de expertos interdisciplinarios en el manejo de la falla intestinal crónica quienes, previa revisión de la literatura escogida, se reunieron de manera virtual acogiendo el método Delphi para discutir una serie de preguntas seleccionadas, enfocadas en el contexto terapéutico de la falla intestinal crónica asociada al síndrome de intestino corto. Resultados. La recomendación del grupo de expertos colombianos es que se aconseje a los pacientes con SIC consumir dietas regulares de alimentos integrales que genere hiperfagia para compensar la malabsorción. Las necesidades proteicas y energéticas dependen de las características individuales de cada paciente; la adecuación del régimen debe ser evaluada a través de pruebas clínicas, antropométricas y parámetros bioquímicos. Se sugiere, especialmente a corto plazo después de la resección intestinal, el uso de análogos de somatostatina para pacientes con yeyunostomía de alto gasto en quienes el manejo de líquidos y electrolitos es problemático. En pacientes con SIC, que son candidatos a tratamiento con enterohormonas, Teduglutida es la primera opción. Conclusión. Existen recomendaciones en el manejo integral de la rehabilitación intestinal respaldadas ampliamente por este consenso y es importante el reconocimiento de alternativas terapéuticos enmarcadas en el principio de buenas prácticas clínicas.

Introduction. The terms chronic intestinal failure, short bowel syndrome (SBS), and total parenteral nutrition are very common in daily clinical practice. The objective of this guideline was to establish a reference framework for clinical practice based on the best level of evidence in patients with chronic intestinal failure secondary to short bowel syndrome. Methods. A group of interdisciplinary experts in the management of chronic intestinal failure was established who, after reviewing the selected literature, met virtually using the Delphi method to discuss a series of selected questions, focused on the therapeutic context of chronic intestinal failure associated with short bowel syndrome. Results. The recommendation of the Colombian expert group is that patients with SBS be advised to consume regular diets of whole foods that generate hyperphagia to compensate malabsorption. Protein and energy needs depend on the individual characteristics of each patient; the adequacy of the regimen must be evaluated through clinical, anthropometric tests and biochemical parameters. The use of somatostatin analogue is suggested, especially in the short term after bowel resection, for patients with high-output jejunostomy in whom fluid and electrolyte management is problematic. In SBS, who are candidates for enterohormonal therapy, Teduglutide is the first choice. Conclusion. There are recommendations on the comprehensive management of intestinal rehabilitation that are widely supported by this consensus and it is important to recognize therapeutic alternatives framed in the principle of good clinical practice.

Profilaxia com lock de etanol em cateter venoso central de longa permanência em crianças com disfunção intestinal grave: relato de seis casos / Ethanol lock prophylaxis in long-stay central venous catheters in children with severe intestinal dysfunction: a report of six cases

Resumo O objetivo deste estudo foi relatar o uso de lock de etanol na profilaxia infecciosa de cateteres venosos de longa permanência em recém-nascidos com disfunção intestinal grave e dependentes de nutrição parenteral total e prolongada, internados em um Centro de Terapia Intensiva Neonatal (nível terciário) entre 2015 e 2020. Das 914 admissões, seis (0,65%) recém-nascidos preencheram os critérios de inclusão. A mediana da idade da passagem do cateter foi de 121,5 dias, sendo dois cateteres PowerPicc (PICC Power Sinergy™, São Paulo), um cateter Groshong (Groshong™ Central Venous Catheter BD, São Paulo) e três cateteres de silicone, todos tunelizados. O tempo de permanência apresentou mediana de 182,5 dias. Cinco pacientes apresentaram pelo menos um episódio de infecção associada ao cateter venoso central, sendo isolados agentes Gram-positivos, negativos e fungos. A mediana de dias de internação foi de 555, e a mortalidade, 33,3%. O lock de etanol não apresentou efeitos colaterais e foi relativamente eficaz na prevenção de infecções relacionadas ao cateter venoso central.

Abstract The aim of this study was to report on use of ethanol lock in long-term catheters in newborns with severe intestinal dysfunction, dependent on total and prolonged parenteral nutrition, in a Neonatal Intensive Care Center (tertiary level), between 2015 and 2020. Six infants (0.65%) out of the 914 admitted during the period met the inclusion criteria. The median age at catheter placement was 121.5 days. Two Powerpicc (PICC Power Sinergy™, São Paulo), one Groshong (Groshong™ Central Venous Catheter BD, São Paulo), and three silicone catheters were used, all tunneled, and the median dwell duration was 182.5 days. Four patients had at least one episode of infection related to the central venous catheter, and Gram-positive, Gram-negative, and fungal agents were isolated. The median length of hospital stay was 555 days and mortality was 33.3%. The ethanol lock did not cause any side effects and was relatively effective in preventing infections related to the central venous catheter.

Progress in intestinal adaptation after enterectomy / 中华胃肠外科杂志

Intestinal adaptation is a spontaneous compensation of the remanent bowel after extensive enterectomy, which improves the absorption capacity of the remanent bowel to energy, fluid and other nutrients. Intestinal adaptation mainly occurs within 2 years after enterectomy, including morphological changes, hyperfunction and hyperphagia. Intestinal adaptation is the key factor for patients with short bowel syndrome to weaning off parenteral nutrition dependence and mainly influenced by length of remanent bowel, type of surgery and colon continuity. In addition, multiple factors including enteral feeding, glucagon-like peptide 2 (GLP-2), growth hormone, gut microbiota and its metabolites regulate intestinal adaptation via multi-biological pathways, such as proliferation and differentiation of stem cell, apoptosis, angiogenesis, nutrients transport related protein expression, gut endocrine etc. Phase III clinical trials have verified the safety and efficacy of teduglutide (long-acting GLP-2) and somatropin (recombinant human growth hormone) in improving intestinal adaptation, and both have been approved for clinical use. We aim to review the current knowledge about characteristics, mechanism, evaluation methods, key factors, clinical strategies of intestinal adaptation.

Rehabilitación del intestino utilizando un análogo semisintético del péptido 2 similar al glucagón. Primera experiencia con teduglutide en Uruguay / Intestinal rehabilitation using semisynthetic glucagon-like peptide-2 analogue. First experience with teduglutide in Uruguay / Reabilitação do intestino usando um análogo semi-sintético do peptídeo 2 semelhante ao glucagon. Primeira experiência com teduglutide no Uruguai

Resumen: La falla intestinal crónica (FIC) o tipo III es una condición invalidante, y la nutrición parenteral crónica (NPC) domiciliaria es el tratamiento que permite a estos pacientes mantenerse con vida. Sin embargo, solamente uno de cada tres países latinoamericanos cuentan con ese recurso, y sus complicaciones no son infrecuentes. Estas complicaciones son las principales indicaciones para trasplante intestinal, un procedimiento que en la mayoría de los países de ingresos medios no se ha desarrollado y no ha presentado los resultados esperados. En los últimos años, la rehabilitación intestinal a nivel mundial ha mejorado sustancialmente con el uso de análogos semisintéticos del péptido 2 similares al glucagón, existiendo cada vez mayor evidencia que demuestra la posibilidad de rehabilitación intestinal e independencia de la NPC con este fármaco, incluso en pacientes con anatomía desfavorable. Estos resultados han permitido mejorar la supervivencia y la calidad de vida de pacientes con FIC y, en muchas ocasiones, prescindir del trasplante. El paciente del caso que presentamos es el primero en recibir esta terapéutica en nuestro país. En este artículo analizamos la respuesta precoz favorable al tratamiento y sus perspectivas a futuro.

Abstract: Long-term home parenteral nutrition (HPN) is a life-saving treatment for patients with chronic intestinal failure, an invalidating condition. However, only 1 out of 3 countries can rely on this treatment and complications associated to chronic parenteral nutrition are rather frequent. The latter constitute the main indication for intestinal transplantion, a procedure that in most middle-income countries has not yet developed and has not shown the expected outcome. In recent years, intestinal rehabilitation has significantly improved at the global level with the use of GLP2, based on the growing evidence that proves the possibility of intestinal rehabilitation and independence from parenteral nutrition with Teduglutide, even in the case of patients with unfavorable anatomy. These results have caused a positive impact on survival and the quality of life of patients with chronic renal failure, and they can often abstain from transplant. The patient of the case study is the first one who received this therapy in our country and this article analyses his favorable early response to treatment and future perspectives.

Resumo: A insuficiência intestinal crônica (CIF) ou tipo III é uma condição incapacitante e a nutrição parenteral crônica (NPC) domiciliar é o tratamento que permite a sobrevida desses pacientes. No entanto, apenas 1 em cada 3 países latino-americanos dispõe desse recurso e as complicações da NPC não são raras. Essas complicações são as principais indicações para o transplante intestinal, procedimento que na maioria dos países de renda média não foi desenvolvido ou não apresentou os resultados esperados. Nos últimos anos, a reabilitação intestinal em todo o mundo tem melhorado substancialmente com o uso de sGLP2, com um número cada vez maior de evidências que mostram a possibilidade de reabilitação intestinal e independência da NPC, mesmo em pacientes com anatomia desfavorável. Esses resultados têm possibilitado prolongar a sobrevida e melhorar a qualidade de vida dos pacientes com CIF e, em muitos casos, dispensar o transplante. O paciente do caso que apresentamos é o primeiro a receber essa terapia em nosso país. Neste artigo, analisamos a resposta favorável ao tratamento precoce e suas perspectivas futuras.

Guía clínica para el manejo de la falla intestinal secundaria a síndrome de intestino corto en pediatría / Clinical guidelines for the managment of intestinal failure secondary to pediatric short bowel syndrome

La falla intestinal secundaria a síndrome de intestino corto en pediatría es una entidad poco frecuente, de alta morbimortalidad. Requiere de un equipo interdisciplinario para su abordaje, lo cual ha demostrado que disminuye la morbimortalidad y aumenta la posibilidad de que los pacientes logren la autonomía intestinal. Existe una falta de evidencia científica en diferentes abordajes de la patología. Consideramos necesario el desarrollo de esta Guía para el Manejo Clínico construida sobre la base de la metodología Delphi modificada, en la Asociación Argentina de Nutrición Enteral y Parenteral, por 16 expertos que se reunieron para discutir y consensuar los principales aspectos de tratamiento clínico. Se analizaron 4 aspectos: definiciones y epidemiología; nutrición enteral, nutrición parenteral; tratamientos farmacológicos y quirúrgicos,y criterios de derivación a centros de alta complejidad. Sin duda este documento será de utilidad para los pacientes, los profesionales y las instituciones, así como para los diferentes financiadores del sistema de salud.

Intestinal failure secondary to short bowel syndrome in pediatrics, is a rare condition with high morbimortality. A follow up multidisciplinary team is necessary to minimize complications and optimize the intestinal rehabilitation. There are no gold standard guidelines for the management of this group of complex patients. The development of clinical guidelines may contribute for an adequate management of patients with intestinal failure and short bowel syndrome. This Clinical Guideline for the Management was developed by 16 experts based on modified Delphi methodology. The meetings were held at the Argentinian Association of Enteral and Parenteral Nutrition (Asociación Argentina de Nutrición Enteral y Parenteral); the topics analyzed were definitions, epidemiology, enteral and parenteral nutrition, pharmacological and surgical treatments, and criteria for referring patients to intestinal rehabilitation centers. The document is aimed to provide basic scientific knowledge for medical institutions, health providers, healthcare providers, patients and families.

Mechanism of gut-microbiota-liver axis in the pathogenesis of intestinal failure-associated liver disease / 中华胃肠外科杂志

Intestinal failure (IF) is defined as the critical reduction of functional intestines below the minimum needed to absorb nutrients and fluids, so that intravenous supplementation with parenteral nutrition (PN) is required to maintain health and/or growth. Although the benefits are evident, patients receiving PN can suffer from serious cholestasis due to lack of enteral feeding and small intestinal bacterial overgrowth (SIBO). One such complication that may arise is intestinal failure-associated liver disease (IFALD). Evidences from recent studies suggest that alterations in the intestinal microbiota, as well as intraluminal bile acid driven signaling, may play a critical role in both hepatic and intestinal injury. Since Marshall first proposed the concept of the gut-liver axis in 1998, the role of gut-liver axis disorders in the development of IFALD has received considerable attention. The conversation between gut and liver is the key to maintain liver metabolism and intestinal homeostasis, which influences each other and is reciprocal causation. However, as a "forgotten organ" , intestinal microbiota on the pathogenesis of IFALD has not been well reflected. As such, we propose, for the first time, the concept of gut-microbiota-liver axis to emphasize the importance of intestinal microbiota in the interaction of gut-liver axis. Analysis and research on gut-microbiota-liver axis will be of great significance for understanding the pathogenesis of IFALD and improving the prevention and treatment measures.

The effect of probiotics on ıntestinal motility in an experimental short bowel model

Abstract Purpose To investigate the effect of probiotics on spontaneous contractions of smooth muscle isolated from jejunum and ileum of rat model. Methods Four rat groups were created (n=8, in each) including control (Group 1), control+probiotic (Group 2), short bowel (Group 3), and short bowel+probiotic (Group 4). Groups 1 and 2 underwent sham operation, Groups 3 and 4 underwent massive bowel resection. Bifidobacterium Lactis was administered in Groups 2 and 4 daily (P.O.) for three weeks. On postoperative week 3, rats were sacrificed, and jejunum and ileum smooth muscle were isolated for organ bath. Muscle contraction changes were analyzed before and after addition of antagonists. Results Short bowel group exhibited increased amplitude and frequency of spontaneous contractions. The addition of probiotics significantly decreased enhanced amplitude and frequency of bowel contraction in short bowel group and returned to control values. L-NNA increased amplitude and frequency of contractions in all groups. While indomethacin and nimesulide increased the amplitude in all groups, the frequency was only increased in jejunum. Hexamethonium and tetrodotoxin did not change the contraction characteristics in all groups. Conclusion We suggest that early use of probiotics may significantly regulate bowel motility, and accordingly improve absorption of nutrients in short bowel syndrome.

Falla intestinal. Artículo de revisión / Intestinal failure Review article

La falla intestinal (FI) se define como la disminución de la función del intestino por debajo de lo mínimo necesario para la absorción de los macronutrientes y / o agua y electrolitos, de tal manera que se requiere de la suplementación intravenosa (SIV) para mantener la salud y el crecimiento. Desde el punto de vista funcional se clasifica en tres tipos. FI tipo I: condición aguda, de corto duración y generalmente auto limitada, FI tipo II: estado agudo prolongado, a menudo en pacientes metabólicamente inestables, que requieren cuidado multidisciplinario y SIV durante períodos de una semana o meses, acompañada de complicaciones sépticas, metabólicas y nutricionales y FI tipo III: condición crónica, en pacientes metabólicamente estables, que requieren SIV durante meses o años. Su manejo requiere de terapia nutricional y en casos seleccionados cirugía autóloga de reconstrucción(AU)

Intestinal failure (FI) is defined as the decrease in intestinal function below the minimum necessary for the absorption of macronutrients and / or water and electrolytes, in such a way that intravenous supplementation (IVS) is required to maintain health and growth. From a functional point of view, it is classified into three types. FI type I: acute condition, of short duration and generally self-limited, FI type II: prolonged acute state, often in metabolically unstable patients, requiring multidisciplinary care and SIV for periods of a week or months, accompanied by septic, metabolic and nutrition and FI type III: chronic condition, in metabolically stable patients, who require SIV for months or years. Its management requires nutritional therapy and in selected cases autologous reconstruction surgery(AU)

Dependiendo de la nutrición parenteral para sobrevivir. Falla intestinal crónica por síndrome de intestino corto. Comunicación de un caso y revisión de la literatura / Depending on parenteral nutrition to survive. Chronic intestinal failure due to short bowel syndrome.A case report and literature review / Dependendo da nutrição parenteral para sobreviver. Falência intestinal crónica por síndrome de intestino curto. Relato de caso e revisão da literatura

Cuando la falla intestinal ocurre luego de una resección intestinal masiva se denomina síndrome de intestino corto. Es una entidad rara, con un espectro clínico que va desde una disfunción leve y reversible hasta una condición grave e incapacitante. Presenta una elevada morbimortalidad, altos costos de atención médica e importante impacto en la calidad de vida. El 50% de los pacientes con síndrome de intestino corto no se adaptan espontáneamente ni mejoran la absorción por el intestino remanente, por lo que deben recurrir de por vida a la nutrición parenteral. La terapia nutricional especializada y el tratamiento hormonal focalizado en el intestino son pilares en la rehabilitación promoviendo la adaptación intestinal, logrando independencia de la nutrición parenteral y evitando el trasplante intestinal. Actualmente, teduglutide es el tratamiento de referencia en estos pacientes, pero hasta el momento inaccesible en Uruguay por su elevado costo. Presentamos el único caso en nuestro país de un paciente de 23 años portador de falla intestinal crónica por síndrome de intestino corto que depende de la nutrición parenteral desde hace 17 años y presenta complicaciones graves asociadas a esta técnica.

Short bowel syndrome consists of intestinal failure after a massive intestinal resection. It is an unusual condition, ranging from a mild and reversible malfunction to a severe condition causing inability. Short bowel syndrome has high morbimortality, high medical costs and a significant impact on quality of life. 50% of patients with short bowel syndrome do not spontaneously adapt and neither does absorption improve by the remaining intestine, thus they depend on parenteral nutrition for the rest of their lives. Specialized nutritional therapy and focalized hormone therapy in the intestine are pillars in rehabilitation. They promote intestine adaptation and achieve independence from parenteral nutrition and avoid intestinal transplant. Today, Teduglutide is the reference treatment for these patients, although it is still not available in Uruguay due to its high cost. The study presents a 23 year old patient carrier of chronic intestinal failure due to short bowel syndrome who has depended on parenteral nutrition for 17 years and presents severe complications associated to this technique.

Quando a falência intestinal ocorre depois de uma ressecção intestinal massiva é chamada Síndrome de intestino curto. É uma entidade rara, com um espectro clínico que pode manifestar-se por uma disfunção leve e reversível até um quadro grave e incapacitante. Apresenta alta morbimortalidade, altos custos de atenção médica e grande impacto sobre a qualidade de vida do paciente. 50% dos pacientes com síndrome de intestino curto não se adaptam espontaneamente nem conseguem uma melhoria da absorção pelo intestino restante sendo obrigados a utilizar nutrição parenteral de forma permanente. A terapia nutricional especializada e o tratamento hormonal focado no intestino são as bases da reabilitação promovendo a adaptação intestinal, diminuindo a dependência da nutrição parenteral e evitando o transplante intestinal. Atualmente o Teduglutide é o tratamento de referência para estes pacientes, porém devido ao seu elevado custo não é acessível aos pacientes no Uruguai. Apresentamos o único caso no país, de um paciente de 23 anos portador de falência intestinal crônica por síndrome de intestino curto que depende de nutrição parenteral há 17 anos e apresenta complicações graves associadas a esta técnica.

A new experimental model of intrinsic denervation in ileum from wistar rats through intramural microinjections of benzalkonium chloride / Um novo modelo experimental de desnervação intrínseca em íleo de ratos wistar através de micro injeções intramurais de cloreto de benzalcônio

Extensive literature is available about the intrinsic denervation of segments of the digestive tube through the application of CB in the serosa of the viscera. However, this technique has some disadvantages like causing peritonitis, flanges and high mortality, limiting its use in humans. The aim of the present study was to evaluate the feasibility of benzalkonium chloride (CB) to induce intrinsic chemical denervation, through applications of CB in the intramural ileum of wistar rats, as well as deepen the knowledge about the evolution of neuronal injury caused in the process. We used 40 rats, divided into two groups (control-GC and benzalkonium-GB) of 20 animals each, divided into four sub-groups according to the time of postoperative assessment of 24, 48 hours, 30 and 90 days. The animals were submitted to intramural microinjections of sterile saline solution 0.9% (GC) or benzalkonium chloride (GB) in ileal portion, and subsequent histopathological analysis and immunohistochemistry for evaluation of neuronal injury. A significant decrease (p<0.05) was found of the neuronal myenteric count over time in groups, GB3, GB4 and GB2. The specific positive immunolabeling for H2AX and Caspase-3 confirmed the results obtained in the histopathological evaluation, denoting the ignition of irreversible cell injury in 24 hours, evolving into neuronal apoptosis in 48 hours after application of the CB 0.3%. Under the conditions in which this work was conducted, it can be concluded that the application of CB 0.3% by means of microinjections intramural in the ileal wall is able to induce intrinsic chemical denervation of the diverticulum of wistar rats and that the main mechanism of neuronal death is induction of apoptosis.(AU)

Existe vasta literatura sobre a desnervação intrínseca de segmentos do tubo digestório através da aplicação de CB na serosa da víscera. Entretanto, essa técnica tem a desvantagem de causar peritonite, formação de bridas e alta mortalidade, não sendo factível para eventuais utilizações em humanos. O objetivo do presente estudo foi avaliar a viabilidade do Cloreto de benzalcônio (CB) induzir desnervação química intrínseca, por meio de aplicações intramurais em íleo de ratos wistar, além de aprofundar o conhecimento sobre a evolução da lesão neuronal causada neste processo. Foram utilizados 40 ratos, distribuídos em dois grupos (controle- GC e benzalcônio- GB) de 20 animais cada, subdivididos em quatro subgrupos de acordo com o tempo de avaliação pós-operatória de 24, 48 horas, 30 e 90 dias. Os animais foram submetidos à microinjeções intramurais de solução salina estéril 0,9% (GC) ou de cloreto de benzalcônio (GB) em porção ileal, e posterior análise histopatológica e imuno-histoquímica, para avaliação da lesão neuronal. Houve diminuição significativa (p<0,05) na contagem neuronal mientérica ao longo do tempo nos grupos GB2, GB3 e GB4. A imunomarcação específica positiva para H2AX e Caspase-3 confirmou os resultados obtidos na avaliação histopatológica, denotando início da lesão celular irreversível em 24 horas, evoluindo para apoptose neuronal em 48 horas após a aplicação do CB 0,3%. Nas condições em que este trabalho foi conduzido, é possível concluir que a aplicação de CB 0,3% por meio de microinjeções intramurais na parede ileal é capaz de induzir desnervação química intrínseca da porção ileal de ratos wistar e que o principal mecanismo de morte neuronal é a indução de apoptose.(AU)

Nutritional Management of a Patient with a High-Output Stoma after Extensive Small Bowel Resection to Treat Crohn's Disease

For patients with short bowel syndrome who undergo ileostomy, nutritional management is essential to prevent complications associated with a high-output stoma (HOS). We report a practical example of ostomic, medical nutrition therapy provided by an intensive nutritional support team (NST). A 42-year-old male with a history of Crohn's disease visited Seoul National University Hospital for treatment of mechanical ileus. He underwent loop ileostomy after extensive small bowel resection. As his remaining small bowel was only 160 cm in length, the stomal output was about 3,000 mL/day and his body weight fell from 52.4 to 40.3 kg. Given his clinical condition, continuous tube feeding for 24 h was used to promote adaptation of the remnant bowel. Thereafter, an oral diet was initiated and multiple, nutritional educational sessions were offered by dietitians. Constant infusion therapy was prescribed and included in the discharge plan. Two months after discharge, his body weight had increased to 46.6 kg and his hydration status was appropriately maintained. This case suggests that the critical features of medical nutritional therapy for ostomy management are frequent assessments of fluid balance, weight history, and laboratory data and after nutritional interventions.

Therapeutic experience of type Ⅲ-b congenital intestinal atresia / 浙江大学学报·医学版

OBJECTIVE@#To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA).@*METHODS@#The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively.@*RESULTS@#Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory.@*CONCLUSIONS@#For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.

The effect of ileocecal valve removal in a model of short bowel syndrome / Efeito da remoção da válvula ileocecal em modelo de síndrome do intestino curto

ABSTRACT Background : Short bowel syndrome is a harmful condition that needs experimental research. Aim: To assess the impact of the ileocecal valve removal in a model of short bowel syndrome, in order to investigate the evolution of the colon under this circumstance. Method: Fifteen Wistar rats were equitable divided into: Control (Sham), Group I (70% enterectomy preserving ileocecal valve) and Group II (70% enterectomy excluding ileocecal valve). After enterectomy was performed jejunoileal or jejunocecal anastomosis and sacrificed the animals on 30th postoperative day for histomorphometric study of the colon. During this period, was observed the clinical evolution of the animals weekly including body weight measurement. Results: Group I and II presented progressive loss of weight. In Group I was observed diarrhea, perineal hyperemia and purple color of the colon during autopsy. Histomorphometry assay showed hypertrophy and hyperplasia of colon mucosa in Group I. In Group II the colon wall was thicker due to hypertrophy and muscular hyperplasia, and in mucosa vascular proliferation and inflammatory infiltrate were intense. Conclusion : This short bowel syndrome model is relevant and achieve 100% of survival. Animal's weight loss was not altered by the presence or exclusion of the ileocecal valve. Animals with 70% of small bowel removal and presence of the ileocecal valve attained a better clinical evolution and histological colon adaptation than those without ileocecal valve.

RESUMO Racional: Síndrome do intestino curto é condição clínica crítica e que precisa de pesquisa experimental. Objetivo: Avaliar o impacto da remoção da válvula ileocecal em um modelo de síndrome do intestino curto para investigar o comportamento do cólon nesta circunstância. Método: Quinze ratos Wistar foram divididos em três grupos de cinco: Controle (Sham), grupo I (enterectomia de 70% com preservação da válvula ileocecal), e grupo II (70% enterectomia de 70% excluindo a válvula ileocecal). Após a enterectomia foi restabelecido o trânsito com anastomose jejunoileal no grupo I e jejunocecal no grupo II. Os animais foram sacrificados no 30º dia do pós-operatório para histomorfometria do cólon. Durante este período, observou-se a evolução clínica semanal, incluindo a medição do peso corporal. Resultados: Grupos I e II apresentaram perda progressiva de peso. No grupo I houve diarreia, períneo hiperemiado e cor violácea do cólon durante a autópsia. A histomorfometria mostrou hipertrofia e hiperplasia da mucosa do cólon no grupo I. No grupo II a parede do cólon estava mais espessa devido à hipertrofia e hiperplasia das camadas muscular e mucosa onde a proliferação vascular e infiltração inflamatória foi intensa. Conclusão: Este modelo é factível e atingiu 100% de sobrevida. A perda de peso não foi alterada pela presença ou exclusão da válvula ileocecal. Animais com remoção de 70% do intestino delgado e presença da válvula ileocecal apresentaram melhor evolução clínica e adaptação histológica do cólon que os sem válvula ileocecal.

Biomarkers for intestinal failure in short bowel syndrome: A new era in GI rehabilitation? / Biomarcadores para la insuficiencia intestinal en síndrome de intestino corto: ¿Una nueva era en la rehabilitación gastrointestinal?

Damage control and gastrointestinal surgery have come a long way from the first reported case of an enterocutaneous fistula to advances in Intestinal transplant and vacuum assisted therapy. Everything we have known in between such as intestinal resections, enteral/parenteral nutrition, delayed abdominal wall closure and intestinal reconstruction have all lead to an exponential increase in our knowledge of gastrointestinal surgery. One area that still remains a significant challenge and clinical dilemma to the general surgeon is intestinal failure in short bowel syndrome. Not only does the anatomical complexity of short bowel syndrome offer difficulties in the definite reconstruction, but also the accompanying intestinal failure increases patient morbidity and mortality. There are no current algorithms or systematic approaches to these daunting clinical scenarios and although surgery has come a long way, there is still room for determining optimal approaches. Therefore, it is critical to keep researching new ways to treat these patients. A relatively new horizon in managing intestinal failure in short bowel syndrome is the use of biomarkers. Here we present a short review on the possible future treatment. The aim of this paper is to provide a pathway for future research into the treatment of this complex area of general surgery

La cirugía gastrointestinal y de control de daños ha tenido un recorrido amplio desde el primer caso reportado de fístula entero-cutánea, hasta llegar al uso de presión subatmosférica para el cierre asistido y el trasplante intestinal. Todos los avances propuestos en el intermedio, como las resecciones intestinales, los planes de nutrición entérica y parenteral, el cierre postergado de la pared abdominal y la reconstrucción intestinal, han llevado a un aumento exponencial del conocimiento de la cirugía gastrointestinal. A pesar de esto, hay un área que permanece como un reto significativo y un dilema clínico para el cirujano general: la falla intestinal en el síndrome de intestino corto. En esta, su complejidad anatómica presenta dificultades a la hora de su reconstrucción, y su alteración funcional aumenta la morbimortalidad del paciente. Así como sucede en la mayoría de las fallas específicas de órganos, esta se caracteriza por cambios en los marcadores séricos que ya han sido bien descritos en la literatura médica. En la falla cardiaca hay elevación del péptido natriurético auricular; en la falla renal, elevación de la creatinina sérica; en la falla hepática, elevación de las transaminasas, y así sucesivamente. Estos marcadores no solo indican la gravedad de la situación, sino que se relacionan con la suficiencia del órgano en cuanto a su función y su mejoría con la rehabilitación. Ahora, ¿cuáles son los marcadores del sistema gastrointestinal? Recientemente, la seriedad de la falla intestinal y su solución han sido objeto de la observación clínica y sintomática con el fin de determinar la orientación de la rehabilitación intestinal y el momento ideal para el inicio de la vía oral. En los últimos años han surgido biomarcadores pertinentes al estudio del sistema digestivo. En esta revisión se discuten los aspectos relacionados con el presente y el futuro de los marcadores serológicos intestinales en el síndrome de intestino corto

Botulinum toxin as a treatment for short bowel syndrome in rats

Abstract Purpose: The denervation of the intestine with benzalkonium chloride (BAC) reduces mortality and improves weight gain in rats with short bowel syndrome (SBS). Nevertheless, translating these promising findings from bench to bedside is not feasible because BAC promotes peritonitis and irreversible denervation which may be followed by an uncontrolled dilatation of the viscera. The use of botulinum toxin (BT) instead of BAC to achieve the denervation of the remaining small intestine in SBS could be an interesting option because it leads to a mild and transient denervation of the intestine. Methods: Here we evaluated the effects of the ileal denervation with BT in rats with SBS by verifying the body weight variation and intestinal morphological parameters. Four groups with 6 animals each were submitted to enterectomy with an ileal injection of saline (group E) or BT (group EBT). Control groups were submitted to simulated surgery with an ileal injection of BT (group BT) or saline (group C - control). Results: We observed that the treatment of the remaining ileum with BT completely reversed the weight loss associated to extensive small bowel resection. Conclusion: This may provide a new promising approach to the surgical treatment of SBS.

Thiamine Deficiency in a Child with Short Bowel Syndrome and Review / 대한소아소화기영양학회지

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.

Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management / 대한소아소화기영양학회지

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting “nutritional failure” which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

Hypothyroidism associated with short bowel syndrome in children: a report of six cases

SUMMARY Short bowel syndrome (SBS) is the leading cause of intestinal failure in children, a condition of absence of sufficient bowel to meet the nutritional and metabolic needs of a growing individual. The treatment of patients in this situation is based on the association of parenteral and enteral nutrition for prolonged periods of time until intestinal rehabilitation occurs with complete enteral nutrition autonomy. Six consecutive cases of children with SBS (residual intestinal length of 5 cm to 75 cm) were managed with this program and were diagnosed with associated hypothyroidism during the treatment (ages at the diagnosis 5 months to 12 years). All patients were successfully treated with oral hormone reposition therapy and in one patient, the replacement was performed via rectal enemas due to a complete absence of small bowel. Although iodine deficiency associated to long-term parenteral nutrition is a well-known condition, this is the first report in the literature about an expressive number of patients with hypothyroidism detected in patients with SBS during the prolonged treatment for intestinal rehabilitation.

Are there differences in the growth adaptation processes of growing and mature organism models of short bowel syndrome?

OBJECTIVES: The purpose of this study was to present an experimental model of short bowel syndrome (SBS) in weaning rats and to compare the adaptative mechanisms of the remaining bowel in weaning rats and adult animals by means of morphometric, histologic and molecular methods. METHODS: Twenty-four weaning rats were divided into 3 groups of 8 animals, one control group and two short bowel groups (euthanasia after 4 and 21 days), and were compared with similar adult groups. Morphometric evaluations of the animals and histopathological and molecular studies of the remaining bowel were performed. RESULTS: The weight of young rats increased after enterectomy, whereas that of adult rats decreased after enterectomy (p<0.0001). The ratio of intestinal length/body weight was significantly higher in weaning rats than in adults (p<0.002), showing that intestinal growth was more intense in weaning rats. Intestinal resection promoted increased thickness of the small bowel lamina propria (p=0.001) and reduced thickness of the colon lamina propria (p=0.04) in weaning rats relative to those in adults. In addition, intestinal resection promoted increased expression of the Bcl-xl gene (antiapoptotic) in adult animals compared with that in weaning rats (p=0.001). CONCLUSION: Morphometric, histological and molecular differences were shown in the adaptation processes of growing and mature organisms.