Dual x-ray absorptiometry monitoring in pediatric short bowel syndrome: an integrative review / Monitorização por absortometria com duplo raio X na síndrome do intestino curto pediátrica: uma revisão integrativa

ABSTRACT Objective: To analyze the bone health of pediatric patients with short bowel syndrome intestinal failure (SBS-IF). Data source: An integrative literature review was performed using the data published in the MEDLINE-PubMed and Scientific Electronic Library Online (SciELO) databases between January 2010 and April 2021, and through a manual search of the reference lists of relevant studies. Studies were included if they assessed bone mineral density by the Dual X-Ray Absorptiometry (DXA) technique, incorporated pediatric patients (up to 20 years of age) with SBS under parenteral nutrition (PN) and were written in English. Eleven primary sources met the inclusion criteria for this study. Data synthesis: Pediatric patients with SBS-IF under long-term parenteral nutrition experienced frequent changes in bone metabolism, leading to osteoporotic fractures and growth failure. These patients have deficiencies in multiple nutrients, such as calcium, magnesium, phosphorus, and vitamin D. Consequently, there are variations in the secretion and regulation of the parathyroid hormone. In addition, the pharmacotechnical limitations related to calcium and phosphorus in the PN solution, use of glucocorticoids, and difficulty performing physical activity are risk factors for the development of metabolic bone disease in pediatric patients with SBS-IF. Conclusions: Low bone mineral density was associated with a high risk of developing osteoporosis, fractures, and growth deficiency in pediatric patients with SBS-IF on PN therapy in the long term.

Objetivo: Analisar a saúde óssea de pacientes pediátricos com síndrome do intestino curto — falência intestinal (SIC-FI). Fontes de dados: Revisão integrativa da literatura usando os dados publicados nas bases de dados Medical Literature Analysis and Retrieval System Online/ United States National Library of Medicine (MEDLINE/PubMed) e Scientific Electronic Library Online (SciELO) entre janeiro de 2010 e abril de 2021 e por meio de busca manual nas listas de referências de estudos relevantes. Foram incluídos estudos em inglês que avaliaram a densidade mineral óssea pela técnica de absorciometria de raio X duplo (DXA), incluíram pacientes pediátricos (até 20 anos de idade) com SIC sob terpia nutricional parenteral. Onze fontes primárias preencheram os critérios de inclusão para este estudo. Síntese dos dados: A pesquisa revelou que pacientes pediátricos com SIC-FI sob nutrição parenteral (NP) de longo prazo tiveram alterações frequentes no metabolismo ósseo, levando a fraturas osteoporóticas e falha de crescimento. Esses pacientes apresentam deficiências de múltiplos nutrientes, como cálcio, magnésio, fósforo e vitamina D. Consequentemente, houve variações na secreção e regulação do hormônio da paratireoide. Além disso, as limitações farmacotécnicas relacionadas ao cálcio e fósforo na solução de NP, o uso de glicocorticoides e dificuldade para realizar atividade física são fatores de risco para o desenvolvimento de doença óssea metabólica em pacientes pediátricos com SIC-FI. Conclusões: A baixa densidade mineral óssea foi associada a um alto risco de desenvolver osteoporose, fraturas e deficiência de crescimento em pacientes pediátricos com SIC-FI sob terapia nutricional parenteral em longo prazo.

Parenteral nutrition: the prison that allows survival in the face of intestinal failure

Objective: Intestinal failure refers to a reduction in intestinal function that necessitates intravenous supplementation of macronutrients, water, or electrolytes due to the intestine's inability to absorb these substances adequately to maintain health and growth. This study aims to explore the experiences and challenges faced by patients enrolled in the intestinal rehabilitation program at Hospital Pablo Tobón Uribe in Medellín. Methodology: This qualitative study adopts a hermeneutic approach and utilizes grounded theory techniques. The sampling process involved both selective and theoretical sampling. A total of 20 semi-structured interviews were conducted, with eight interviews including contributions from family members. The data analysis commenced with open coding, followed by the grouping of codes into descriptive categories. Dimensions and properties were identified within these categories, and analytical categories were subsequently developed through axial and selective coding. This iterative process led to the emergence of the final paradigm matrix. Results: The study revealed that the healthcare system inadequately addresses the needs and expectations of patients with intestinal failure, leading to increased uncertainty about the disease's origin and future prognosis. Intestinal failure and its treatment disrupt various aspects of patients' lives, including personal, family, and work domains. Social stigmatization and rejection are prominent, underscoring the importance of support from family and close individuals in facilitating adaptation and revaluing life. Conclusions: Coping with the challenges of intestinal failure entails embracing the necessity of relying on parenteral nutrition, which is perceived as a prison that paradoxically enables survival.

Objetivo: la insuficiencia intestinal es la reducción de la función intestinal que requiere la suplementación intravenosa de macronutrientes, agua o electrolitos, pues el intestino no logra la absorción mínima para mantener la salud y el crecimiento. El objetivo es comprender el significado que tiene afrontar la condición de insuficiencia intestinal en pacientes que pertenecen al programa de rehabilitación intestinal del Hospital Pablo Tobón Uribe de Medellín. Metodología: estudio cualitativo con enfoque hermenéutico que utilizó técnicas de la teoría fundamentada. El muestreo fue primero selectivo y luego teórico. Se realizó un total de 20 entrevistas semiestructuradas; 8 de las cuales tuvieron el aporte de familiares. El análisis inició por la codificación abierta. Los códigos obtenidos se agruparon en categorías descriptivas, y en ellas se identificaron dimensiones y propiedades que se utilizaron para elaborar categorías analíticas mediante la codificación axial y selectiva que permitió emerger la matriz del paradigma final. Resultados: las necesidades y expectativas de los pacientes con insuficiencia intestinal no son suficientemente atendidas por el sistema de salud, lo que genera mayor incertidumbre sobre el origen de la enfermedad y aún más sobre su futuro. La insuficiencia intestinal y su tratamiento trastornan la vida personal, familiar y laboral. El rechazo social es marcado, por lo que el apoyo familiar y de las personas cercanas es fundamental para lograr la adaptación que les permite revalorar la vida. Conclusiones: afrontar la condición de insuficiencia intestinal representa la experiencia de requerir necesariamente de nutrición parenteral, la cual se percibe como una prisión que paradójicamente permite sobrevivir.

Risk factors forvessel catheter associated infection in children with intestinal failure: a scoping review / 中国实用护理杂志

Objective:To scope review of studies on the risk factors for vessel catheter associated infection in children with intestinal failure was reviewed, so as to provide a reference for clinical medical staff to prevent and control infection.Methods:Based on the framework of scope review method, PubMed, Embase, CINAHL, APA PsycInfo, Web of Science, Scopus, Cochrane Library, China Biomedical Literature Database, China Knowledge Networkand Wanfang database are retrieved. The retrieval period was from the database construction to February 1, 2022. The included literatures were Summarized and analyzed.Results:A total of 28 articles were included, and the risk factors of vessel catheter associated infection in children with intestinal failure were found to include 7 aspects: basic information of children, catheter nursing measures, type of tube-sealing solution, catheter factors, residual intestinal condition of children, nursing level of caregivers, therapeutic factors.Conclusions:The risk factors of vessel catheter associated infection in children with intestinal failure were found to include 7 aspects, which are complex and diverse with distinctive disease characteristics. In the future, medical staff can optimize the pipeline nursing measures; construct the model of cross-specialty health education and improve the nursing level of caregivers to reduce the incidence of vessel catheter associated infection.

The diagnosis and nutritional management of chronic intestinal failure / 中华临床营养杂志

Chronic intestinal failure represents a long-lasting condition of intestinal dysfunction that contributes to inadequate absorption of macronutrients and/or water and electrolytes. It is heterogeneous in clinical manifestation, intervention and prognosis, and usually requires multi-disciplinary management. This review elucidates the state-of-the-art consensus on diagnosis and definition in chronic intestinal failure and exemplifies relevant nutritional management principles with interventions in short bowel syndrome, in hopes of improving the identification and management of chronic intestinal failure in domestic practice.

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.

Fallo intestinal en el paciente crítico: reconocimiento, abordaje multidisciplinario y unidades dedicadas / Intestinal failure in critical patients: recognition, multidisciplinary approach and dedicated units / Insuficiência intestinal no paciente crítico: reconhecimento, abordagem multidisciplinar e unidades dedicadas

Resumen: Introducción: en el paciente crítico el intestino es protagonista de la respuesta al estrés. Los síntomas de insuficiencia intestinal en las unidades de cuidados intensivos no son específicos y no se incluyen en escalas de gravedad comúnmente usadas, sin embargo, el 62% de los pacientes críticos presenta síntomas gastrointestinales al menos una vez al día. Demostrándose la relación existente entre la aparición de problemas gastrointestinales y la mortalidad en pacientes críticos. Objetivo: proporcionar el conocimiento teórico necesario sobre el fallo intestinal como entidad exclusiva, su reconocimiento en el paciente grave, clasificación y enfoque multidisciplinario del tratamiento. Métodos: se realizó una revisión cualitativa y sistemática en los idiomas inglés y español de la literatura publicada y actualizada hasta febrero del 2020. Conclusiones: las alteraciones gastrointestinales son frecuentes en el paciente crítico y un determinante en su mortalidad. Reconocer el fallo y disfunción intestinal permite optimizar el tratamiento, reducir la mortalidad e incidencia de complicaciones, las unidades dedicadas con un equipo de experiencia en el manejo del fallo intestinal agudo constituyen un aspecto clave en este sentido.

Summary: Introduction: in critically ill patients, intestine function expresses the response to stress. Symptoms of intestinal failure in intensive care units are not specific and are not included in commonly used severity scales. However, 62% of critically ill patients present gastrointestinal symptoms at least once a day, demonstrating the relationship between the appearance of gastrointestinal problems and mortality in critical patients. Objective: to provide the necessary theoretical knowledge about intestinal failure as an exclusive entity and how to recognize it in critically ill patients, classification and a multidisciplinary approach to treatment of this condition. Methods: a qualitative and systematic review was carried out of the literature published and updated until February 2020 both in English and in Spanish. Conclusions: gastrointestinal disorders are frequent in critically ill patients and constitute a determining factor in their mortality. Recognizing intestinal failure and dysfunction allows the optimization of treatment, reduces mortality and the incidence of complications. The creation of dedicated units with experienced staff in the management of acute intestinal failure is a key aspect in this regard.

Resumo: Introdução: em pacientes críticos, o intestino é o protagonista da resposta ao estresse. Os sintomas de insuficiência intestinal em unidades de terapia intensiva não são específicos e não estão incluídos nas escalas de gravidade comumente usadas; no entanto, 62% dos pacientes criticamente enfermos apresentam sintomas gastrointestinais pelo menos uma vez ao dia, demonstrando a relação entre o aparecimento de problemas gastrointestinais e mortalidade em pacientes críticos. Objetivo: proporcionar o conhecimento teórico necessário sobre a insuficiência intestinal como entidade exclusiva, seu reconhecimento em pacientes críticos, classificação e abordagem multidisciplinar do tratamento. Métodos: foi realizada uma revisão qualitativa e sistemática nos idiomas inglês e espanhol da literatura publicada e atualizada até fevereiro de 2020. Conclusões: as alterações gastrointestinais são frequentes em pacientes críticos e determinam sua mortalidade. O reconhecimento da insuficiência e disfunção intestinal permite otimizar o tratamento, reduzindo a mortalidade e a incidência de complicações; contar com unidades dedicadas e com equipe experiente no manejo da insuficiência intestinal aguda são um aspecto fundamental nesse sentido.

Exploration of home nutrition support in children with intestinal failure and clinical experience with 10 cases / 中华临床营养杂志

Objective:To explore the application of home nutrition support in children with intestinal failure.Methods:Children with intestinal failure admitted to Division of Pediatric Gastroenterology and Nutrition and Department of Pediatric Surgery in Xinhua Hospital were retrospectively enrolled since January 2009. The details of home nutrition support, nutritional status and home parenteral nutrition (HPN) associated complications were collected.Results:A total of 10 children received HPN support, 7 of whom were with short bowel syndrome (SBS) and the other 3 with pediatric intestinal pseudo-obstruction. The average length of remnant small bowel in 7 SBS children was (36.7±32.4) cm. The average age at HPN onset was (5.4±4.7) years. The average duration of follow-up was (3.1±2.1) years. The average duration of HPN was (619.5±669.1) days after (391.8±340.1) days of parenteral nutrition support in our hospital. All 10 cases started home enteral nutrition (HEN) with tube feeding (3 cases transited to oral feeding during treatment). The average duration of HEN was (536.1±429.6) days. Daily calorie intake was 104.0%±39.0% of the recommended intake according to the guideline, with 46.5%±21.3% via HPN and 57.5%±29.2% via HEN. During follow-up, 3 cases were found with severe malnutrition, 5 with moderate malnutrition and 2 with mild malnutrition. Four children suffered from catheter-related thrombosis and five children were identified with catheter-related blood stream infection. No intestinal failure associated liver disease was observed.Conclusions:HPN is feasible but needs the support of national medical insurance policy. At present, there are still frequent nutritional deficiencies and complications in HPN. Nutrition support team (NST) should provide guidance for more scientific nutrition screening and nutrition management.

Fallo intestinal agudo / Acute intestinal failure / Insuficiência intestinal aguda

RESUMEN El fallo Intestinal agudo es la reducción de la función intestinal bajo el mínimo necesario para absorción de macronutrientes y/o electrolitos. Con el objetivo de brindar a los profesionales de la salud las bases teóricas esenciales que sustenten su autopreparación para la prevención, diagnóstico y tratamiento del fallo intestinal agudo, se realizó una revisión bibliográfica en el Hospital Provincial Docente Doctor León Cuervo Rubio, desde mayo hasta noviembre del 2018, con una sistematización teórica sobre el tema. Se estudiaron 125 artículos de los que se encontraron publicados entre los años 2013 y 2018 en las bases de datos electrónicas. Se elaboró una monografía sobre el fallo intestinal agudo en el paciente crítico, la cual contenía los siguientes núcleos de conocimientos: definición, clasificación, alteraciones gastrointestinales, implicaciones clínicas, diagnóstico y tratamiento. El fallo intestinal agudo en el paciente crítico tiene un importante papel en la aparición de sepsis y fallo multiorgánico.

ABSTRACT Acute Intestinal failure is the reduction of intestinal function under the minimum necessary for absorption of macronutrients and / or electrolytes. In order to provide health professionals with the essential theoretical bases that support their self-preparation for the prevention, diagnosis and treatment of acute intestinal failure, a literature review was carried out at the Provincial Teaching Hospital Doctor León Cuervo Rubio, from May to November of 2018, with a theoretical systematization on the subject. We studied 125 articles that were published between 2013 and 2018 in electronic databases. A monograph on acute intestinal failure was developed in the critical patient, which contained the following knowledge nuclei: definition, classification, gastrointestinal disturbances, clinical implications, diagnosis and treatment. Acute intestinal failure in the critical patient has an important role in the occurrence of sepsis and multiorgan failure.

RESUMO A insuficiência intestinal aguda é a redução da função intestinal abaixo do mínimo necessário para a absorção de macronutrientes e / ou eletrólitos. A fim de fornecer aos profissionais de saúde as bases teóricas essenciais que apoiam sua autopreparação para a prevenção, diagnóstico e tratamento da insuficiência intestinal aguda, foi realizada uma revisão da literatura no Hospital Provincial de Ensino Médico Doutor León Cuervo Rubio, de maio a novembro de 2018, com uma sistematização teórica sobre o assunto. Foram estudados 125 artigos publicados entre 2013 e 2018 em bancos de dados eletrônicos. Foi desenvolvida uma monografia sobre insuficiência intestinal aguda no paciente crítico, que continha os seguintes núcleos de conhecimento: definição, classificação, distúrbios gastrointestinais, implicações clínicas, diagnóstico e tratamento. A insuficiência intestinal aguda no paciente crítico tem um papel importante na ocorrência de sepse e insuficiência de vários órgãos.

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)

Research progress of parenteral nutrition associated liver damage in multiple organ dysfunction syndrome / 中国综合临床

Multiple organ dysfunction syndrome (MODS) patients or intestinal failure due to infection and other factors,intestinal dysfunction can not complete the absorption of nutrients and electrolytes.Progressive malnutrition in severe patients usually develops rapidly.Early parenteral nutrition can significantly improve the prognosis of MODS patients.PN is now widely used in all kinds of severe patients,but parenteral nutrition-associated liver disease (PNALD) caused by parenteral nutrition is prevalent in such patients.The emergence of PNALD increases the difficulty of curing patients' diseases.Many studies have found thatω-3fish oil fat emulsion in nutrient solution is an effective way to prevent and improve PNALD.

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.

Mesenteric Vascular Occlusion: A Therapeutic Challenge - Our Experience Of Five Cases.

Acute mesenteric ischaemia (AMI) is an uncommon event and requires comprehensive clinical care to minimize the morbidities and mortality associated with the condition. SBS is a common occurrence in A M I patients who undergo massive bowel resection due to reduced intestinal absorptive surface area. We report our experience of five patients with AMI, who presented at our surgical emergency within a period of one and half year (Aug 2015 – Dec 2016) with a follow–up of one year and their outcome so as to encourage others in managing such a challenge with more positive mindsets. A mortality rate of 20 %( 1/5) was observed. A structured clinical approach, timely surgical intervention with exteriorisation of anastomosed bowel loop and multidisciplinary post operative management are essential for managing such frail patients to achieve best possible results.

VitaminD deficiency in patients with short bowel syndrome / 医学研究生学报

Vitamin D is typically acquired from sunlight exposure (specifically UVB radiation) and a combination of natural and fortified foods. However, patients with short bowel syndrome are at significantly increased risk of developing suboptimal vitamin D status. Notably, beyond the key role in calcium and phosphate balance and bone structure, vitamin D has been proved to be associated with modulation of the immune system, enhancement of intestinal barrier function, stimulation of intestinal epithelial cell turnover, and regulation of the endocrine. In this review, we summarize the status and biological function of vitamin D in short bowel syndrome.

Multidisciplinary Intestinal Rehabilitation for Short Bowel Syndrome in Adults: Results in a Korean Intestinal Rehabilitation Team

PURPOSE: Intense multidisciplinary team effort is required for the intestinal rehabilitation of patients afflicted with the short bowel syndrome (SBS). These include enteral and parenteral nutrition (PN) support, monitoring of complications related to treatment, and considering further medical or surgical options for intestinal adaptation. METHODS: In the Intestinal Rehabilitation Team (IRT) at the Samsung Medical Center, we have experienced 20 cases of adult SBS requiring multidisciplinary intestinal rehabilitation. This study is a retrospective review of the collected medical records. RESULTS: Of the 20 subjects treated, 12 patients were male and 8 patients were female. At the time of referral to the IRT, the mean age was 51.5 years, and the mean body weight was 50.1 kg, which was 90% of the usual body weight. The diseases or operative managements preceding massive bowel resection were malignancy in 11 cases, cardiac surgery in 2 cases, trauma in 2 cases and one case, each of tuberculosis, corrosive esophagitis, atrial fibrillation, simultaneous pancreas and kidney transplantation, and perforated appendicitis. Of these, there were 14 survivals and 6 mortalities. The fatalities were attributed to progression of disease, intestinal failure-associated liver disease, and sepsis (unrelated to intestinal failure) (2 cases each). Among the 14 surviving patients, 8 patients have been weaned off PN, whereas 6 are still dependent on PN (mean PN dependence 36%). CONCLUSION: This paper reports the results of multidisciplinary intestinal rehabilitation of adult short bowel patients treated at the Samsung Medical Center. Further studies are required to improve survival and enteral tolerance of these patients.

Enfermedad por inclusión microvellositaria como causa de diarrea congénita severa: caso clínico / Microvillous inclusion disease as a cause of severe congenital diarrhea: case report

Las diarreas congénitas son patologías graves de baja frecuencia y alta mortalidad. Se manifiestan durante los primeros días o meses de vida con severa diarrea, generando insuficiencia intestinal y dependencia de nutrición parenteral. Se debe sospechar ante un recién nacido o lactante con pérdidas masivas hidroelectrolíticas, y se diagnostican utilizando parámetros clínicos, endoscópicos, histológicos y eventualmente genéticos. El tratamiento es de soporte, con reposición hidroelectrolítica intensa y nutricional. OBJETIVO: Presentar un caso de diarrea congénita, identificada como Enfermedad por Inclusión Microvellositaria, de presentación neonatal. CASO CLÍNICO: Paciente varón edad actual 3 años, hijo de padres consanguíneos, quien debutó a los 10 días de vida con diarrea secretora severa, requiriendo ingreso a unidad de paciente crítico y nutrición parenteral permanente. Inicialmente además con síndrome de Fanconi, que luego se recupera. Se confirmó la sospecha de Enfermedad de Inclusión Microvellositaria utilizando microscopia óptica, electrónica e inmunohistoquímica. Se obtuvo una favorable evolución utilizando nutrición parenteral total (NPT) a domicilio. CONCLUSIONES: Se presenta el primer caso conocido en Chile de un paciente con diarrea congénita por inclusión microvellositaria manejado y su evolución.

Congenital diarrheas correspond to a severe and low frequency digestive disease, with a high mortality. They start a few days or months after birth, leading to intestinal insufficiency and dependence on parenteral nutrition. It must be highly suspected in newborns or infants with diarrhea and severe electrolyte disorders. The diagnosis is based on clinical, endoscopic, histologic and eventually genetic findings. Treatment is supportive with intensive correction of electrolyte imbalances as well as parenteral nutrition. OBJECTIVE: To present a case report of congenital diarrhea identified as microvillous inclusion disease presenting in the neonatal period. CASE REPORT: Male patient currently 3 years of age, son of consanguineous parents. At 10 days of age presents a severe secretory diarrhea, requiring treatment in a critical care unit and parenteral nutrition. Initially he also presented with Fanconi syndrome, which improved afterwards. The suspicion of congenital microvillous inclusion was confirmed later by optic and electronic microscopy, and inmunohistochemistry. A succesful evolution was later achieved maintaining home parenteral nutrition after discharge. CONCLUSION: We present the first known case in Chile of congenital diarrhea due to microvillous inclusión disease and his evolution.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program / Tratamento da síndrome do intestino encurtado na criança: valor do Programa de Reabilitação Intestinal

Summary The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Resumo A principal causa da falência intestinal aguda é a síndrome do intestino encurtado, decorrente, em geral, de ressecção de extensos segmentos de intestino delgado. Em consequência, os principais sintomas são diarreia aquosa, síndrome de má absorção, desnutrição crônica e óbito, caso o paciente não seja adequadamente tratado. Se o comprimento do intestino remanescente for superior a 30 cm, poderá haver adaptação completa e o paciente poderá ficar livre da nutrição parenteral. O tratamento atualmente preconizado inclui a utilização de nutrição parenteral prolongada e de nutrição enteral, objetivando sempre o ganho ponderal constante, em paralelo a cirurgias que visem ao alongamento do intestino dilatado. Esse conjunto de procedimentos constitui o que se denomina Programa de Reabilitação Intestinal. Essa terapia foi utilizada em 16 crianças, em períodos que variaram de 8 meses a 7 anos e meio, com sobrevida em 75% dos casos. O último recurso utilizado em crianças com ressecção completa do intestino delgado é o transplante intestinal. Até o momento, não há registro de criança brasileira que tenha sobrevivido a esse procedimento, a despeito de sete pacientes terem sido submetidos a ele. Os resultados do Programa de Reabilitação Intestinal nos anima a continuar com esse tipo de tratamento e estimular a criação do programa em outras instituições de atendimento pediátrico.

Nutrition Therapy for Adult Intestinal Failure Patients

Intestinal failure (IF) is a condition, in which the intestinal function or length remaining is below the minimum amount required for the absorption of sufficient nutrients and fluid to maintain normal life. The nutritional supply of IF depends on the anatomical site, length, and function of the remaining bowel. The goals of nutritional therapy for patients with IF are to achieve bowel adaptation to absorb nutrients sufficiently to live a healthy life with the current intestinal condition, and to promote the enteral autonomy to control nutrient digestion, absorption, excretion, and bowel movement. To stabilize and recover the patient's nutrition condition after a huge bowel resection, the intestinal rehabilitation team (IRT) for individual nutritional therapy should be established. IRT carefully monitors the changes in body weight, medication use, patient's symptoms, nutrient deficiency, hydration status, function of the remaining bowel, degree of bowel adaptation, adverse effects due to nutritional therapy, and enteral balance. To achieve intestinal adaptation and enteral autonomy through complicated and difficult nutritional intensive therapy in IF patients, it is essential to manage the patients through multidisciplinary collaboration involving physicians, pharmacists, dietitians, and nurses.

Síndrome de intestino corto, un caso de readaptación intestinal / Short Bowel Syndrome, a Case of Intestinal Rehabilitation / Síndrome de intestino curto, um caso de readaptação intestinal

Presentación del caso: El objetivo de este artículo es presentar la experiencia exitosa del manejo multidisciplinario de una paciente con síndrome de intestino corto y falla intestinal con evolución a la adaptación intestinal. Se trata de una recién nacida prematura con atresia intestinal tipo IV, con múltiples atresias intestinales, quien evolucionó a la falla intestinal y requirió manejo con soporte nutricional parenteral prolongado, múltiples esquemas antibióticos, probióticos, multivitaminas, nutrición enteral con fórmula elemental, hasta lograr su adaptación intestinal y llevar a una dieta normal. La falla intestinal en estos pacientes es un reto para el equipo de salud, ya que no solo implica el manejo quirúrgico de su condición de base, si no del soporte nutricional, equilibrio hidroelectrolítico, disfunción hepática por colestasis, infecciones asociadas, etcétera. Discusión: El síndrome de intestino corto con evolución a la falla intestinal en niños es una condición, cuya prevalencia va en aumento en el ámbito mundial, gracias a los avances en el cuidado intensivo neonatal, cirugía neonatal y en el soporte nutricional de pacientes con condiciones como gastrosquisis, onfalocele y enterocolitis necrotizante. A pesar de las limitaciones del sistema de salud, es posible ofrecer un tratamiento multidisciplinario e integral para llevarlos hasta la adaptación intestinal.

Short bowel syndrome with progression to intestinal failure in children is a condition whose prevalence is increasing worldwide, thanks to advances in neonatal intensive care, neonatal surgery, and nutritional support of patients with conditions such as gastroschisis, omphalocele and enterocolitis. Objective: To present the successful experience of multidisciplinary management of a patient with short bowel syndrome and intestinal failure with progression to intestinal adaptation. Clinical case: A newly born premature with intestinal atresia type IV with multiple intestinal atresia, which evolved to intestinal failure and required management with prolonged parenteral nutritional support, multiple antibiotic schemes, prebiotics, multivitamins, enteral nutrition with elemental formula, in order to achieve their intestinal adaptation and enabling a normal diet. The evolution of these patients' intestinal failure is a challenge for the health team, as it not only involves the surgical management of the patient's condition, but the basic nutritional support, the fluid and electrolyte balance, hepatic dysfunction due to cholestasis associated infections, etc. Conclusion: Despite the limitations of our health care system, it is possible to offer a multidisciplinary and integrated treatment to lead to intestinal adaptation.

Caso clínico: O objetivo é apresentar a experiência de sucesso do manejo multidisciplinar de uma paciente com síndrome de intestino curto e falha intestinal com evolução à adaptação intestinal. Se trata de uma recém nascida prematura com atresia intestinal tipo IV com múltiplas atresias intestinais que evolucionou à falha intestinal e requereu manejo com suporte nutricional parental prolongado, múltiplos esquemas antibióticos, probióticos, multivitaminas, nutrição enteral com fórmula elementar, até conseguir sua adaptação intestinal e levar a uma dieta normal.A falha intestinal nestes pacientes é um desafio para a equipe de saúde, devido a que não só implica o manejo cirúrgico de sua condição de base, mas também do suporte nutricional, equilíbrio hidroeletrolítico, disfunção hepática por colestase, infeções associadas, etc. Discussão: a síndrome de intestino curto com evolução à falha intestinal em crianças é uma condição cuja prevalência vai em aumento a nível mundial, devido aos avanços no cuidado intensivo neonatal, cirurgia neonatal e no suporte nutricional de pacientes com condições como gastrosquise, onfalocele e enterocolite. Apesar das limitações de nosso sistema de saúde, é possível oferecer um tratamento multidisciplinar e integral para leva-los até a adaptação intestinal.