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The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries. The mean (standard deviation [SD]) age at enrollment was 55.4 (15.46) years, and 60.2% of patients were women. Crohn's disease was the most common cause of major intestinal resection in both ever-treated (34.1%) and never-treated patients (20.4%). A similar proportion of ever-treated and never-treated patients had a prior history of colorectal polyps (2.7% vs. 3.6%), whereas proportionally fewer ever-treated patients reported a history of colorectal cancer (1.8% vs. 6.2%) or any malignancy (17.7% vs. 30.0%) than never-treated patients. Never-treated patients received a numerically greater mean (SD) volume of parenteral nutrition and/or intravenous fluids than ever-treated patients (12.4 [8.02] vs. 10.1 [6.64] L/week). Ever-treated patients received a mean teduglutide dosage of 0.05 mg/kg/day. This is the first report of patient baseline characteristics from the SBS Registry, and the largest cohort of patients with SBS-IF to date. Overall, ever-treated and never-treated patients had similar baseline characteristics. Differences between treatment groups may reflect variations in patient selection and degree of monitoring.

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We describe cases of intestinal failure wherein inpatient admission was critical toward enteral autonomy. We performed a retrospective chart review of 6 children with long-term parenteral nutrition dependence who were weaned from parenteral nutrition after admission. Admissions included feeding and medication titration, interdisciplinary care, and a home parenteral nutrition team consultation.

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PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.

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OBJECTIVE: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. METHODS: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). RESULTS: Twenty-eight children were included in the study group, median (IQR) age was 11 (8-27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8-24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. CONCLUSION: Children with IF using prolonged PN showed lower W/A and H/A compared to the control group, but without significant difference between the PhA of children with IF compared to controls.

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Intestine remains the least frequently transplanted solid organ, although the survival and quality-of-life benefits of transplant to individuals with irreversible intestinal failure have been well demonstrated. The trend seen over the past 15 years of fewer listings and fewer transplants appears to be continuing, most noticeably in infants, children, and adolescents. There were only 146 additions to the intestine waiting list in 2022, and the proportion of adult candidates continues to increase, so that now 61% of the intestine waiting list are adult candidates. There has been little change in the distribution by sex, race and ethnicity, or primary diagnosis on the waiting list, or for those receiving transplant. The transplant rate for adults has decreased to 55.6 transplants per 100 patient-years, but the pediatric transplant rate remains relatively stable at 22.8 transplants per 100 patient-years. The decrease in transplant rates for adults is primarily the result of falling rates for those listed for combined intestine-liver, and this is reflected in the pretransplant mortality rates, which are twice as high for candidates in need of both organs compared with those listed for intestine alone. Overall, intestine transplant numbers decreased to a total of 82 intestine transplants in 2022, only one above the lowest ever value of 81 in 2019. No major changes were seen in the immunosuppression protocols, with most recipients having induction therapy and tacrolimus-based maintenance. Graft failure rates appear to have improved at 1, 3, and 5 years for intestine without liver, but this is not seen for combined intestine-liver. Graft and patient survival are better for pediatric recipients compared with adult recipients for both liver-inclusive and liver-exclusive transplant. Rates of posttransplant lymphoproliferative disorder are higher for recipients of intestine without liver.

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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.

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The understanding of the mechanisms for the development of ascites has evolved over the years, involving the liver, peritoneum, heart, and kidneys as key responsible for its formation. In this article, we review the pathophysiology of ascites formation, introducing the role of the intestine as a major responsible for ascites production through "a game changer" case.

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Abstract 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.

Resumen 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.

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OBJECTIVE: To evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS). STUDY DESIGN: We used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients <18 years with SBS hospitalized between January 1, 2006, and October 1, 2015. We analyzed readmissions with recurrent event analysis and analyzed LOS with linear mixed effects modeling. We used a conceptual model to guide our multivariable analyses, adjusting for race, ethnicity, and insurance status. RESULTS: We included 4289 children with 16 347 hospitalizations from 43 institutions. Fifty-seven percent of the children were male, 21% were Black, 19% were Hispanic, and 67% had public insurance. In univariable analysis, children from low-income neighborhoods had a 38% increased risk for all-cause hospitalizations (rate ratio [RR] 1.38, 95% CI 1.10-1.72, P = .01), an 83% increased risk for CLABSI hospitalizations (RR 1.83, 95% CI 1.37-2.44, P < .001), and increased hospital LOS (ß 0.15, 95% CI 0.01-0.29, P = .04). In multivariable analysis, the association between low-income neighborhoods and elevated risk for CLABSI hospitalizations persisted (RR 1.70, 95% CI 1.23-2.35, P < .01, respectively). CONCLUSIONS: Children with SBS from low-income neighborhoods are at increased risk for hospitalizations due to CLABSI. Examination of specific household- and neighborhood-level factors contributing to this disparity may inform equity-based interventions.

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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.

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RESUMEN Antecedentes: la insuficiencia intestinal, en su grado más grave, se conoce como fallo intestinal crónico (FIC). Las últimas décadas han sido testigo de la incorporación, evolución y perfeccionamiento de tratamientos que, en su conjunto, se denominan rehabilitación intestinal y abarcan tanto procedimientos quirúrgicos como tratamientos médicos que, llevados a cabo por equipos multi e interdisciplinarios, alcanzan altas tasas de éxito. Objetivo: describir los resultados de 16 años en el tratamiento de pacientes con FIC secundario a síndrome de intestino corto (SIC), y la evolución del papel de la cirugía, la rehabilitación médica y el trasplante. Material y métodos: análisis retrospectivo de una base de datos prospectiva de pacientes con fallo intestinal crónico secundario a SIC entre febrero de 2006 y marzo de 2022. Resultados: se incluyeron 492 pacientes (368 adultos-A y 124 pediátricos-P). Grupo A: 111 pacientes recibieron cirugía de reconstrucción autóloga del tracto gastrointestinal (CRATGI), 16 péptido semisintético similar al glucagón de tipo 2 (sGLP-2); el 83% logró la rehabilitación, con un 77% de supervivencia a 10 años; el 6,8% (17 pacientes) requirió trasplante intestinal (TxI), con un 89% de independencia de la nutrición parenteral (NP) al año y supervivencia post-TxI del 29% a los 10 años. Grupo B: 18 recibieron CRATGI; 9, enteroplastia serial transversa (STEP); y 6, sGLP-2; el 52% se rehabilitó, con una supervivencia del 69% a los 10 años; 28 pacientes recibieron TxI, con 69% de independencia de nutrición parenteral (NP) al año y supervivencia del 39% a los 10 años. Conclusión: los resultados presentados resaltan el papel central de la cirugía y la rehabilitación médica para alcanzar la suficiencia intestinal.

ABSTRACT Background: Severe intestinal insufficiency is known as chronic intestinal failure (CIF). In recent decades, medical treatments and surgical procedures have been incorporated, developed and improved under the name intestinal rehabilitation. When performed by multiand interdisciplinary teams, these treatments have high success rates. Objective: The aim of present study is to describe the 16-year outcomes in the management of patients with CIF secondary to short bowel syndrome (SBS) and the role of surgery, medical rehabilitation, and transplantation. Material and methods: We conducted a retrospective analysis on a prospective database of patients treated with chronic intestinal failure due to SBS between February 2006 and March 2022. Results: A total of 492 patients (368 adults (Group A) and 124 pediatric patients (Group B)] were included. Group A: 111 patients underwent autologous gastrointestinal reconstruction surgery (AGIRS), 16 were treated with semisynthetic glucagon-like peptide-2 (sGLP2); 83% achieved rehabilitation, with 77% survival at 10 years; 6.8% (17 patients) required intestinal transplantation (ITx), with 89% independence from parenteral nutrition (PN) at 1 year and post-ITx survival of 29% at 10 years. Group B: 18 patients underwent AGIRS; 9 underwent serial transverse enteroplasty (STEP); and 6 received sGLP2; 52% were rehabilitated, with 69% survival at 10 years; 28 patients received ITx, with 69% independence from PN at 1 year and 39% survival at 10 years. Conclusion: These results highlight the central role of surgery and medical rehabilitation in the recovery of intestinal function.

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Type II intestinal failure (IF-II) is a condition in which the gastrointestinal tract is compromised. Liver complications may occur because of the pathology and/or prolonged use of parenteral nutrition (PN); oxidative stress has been implicated as one of the causes. Lipid emulsions containing n-3 polyunsaturated fatty acids (PUFAs) have been proposed for the treatment. We aimed to evaluate the effect of 7-day n-3 PUFA supplementation on oxidative stress in IF-II patients receiving PN. This was a randomized, controlled, double-blinded, pilot trial of adult patients with IF-II, receiving either conventional PN (control) or PN enriched with n-3 PUFAs (intervention). Twenty patients were included (14 men, 49 ± 16.9 years), with the ANCOVA analysis the glucose (p = 0.003), and direct bilirubin (p = 0.001) levels reduced; whereas the high-density lipoprotein cholesterol (HDL-C) increased (p = 0.017). In the random-effect linear regression analysis, a reduction (p < 0.0001) in the malondialdehyde (MDA) level was found in the intervention group when the covariables age, HDL-C level, and alanine aminotransferase activity were considered. After 1 week of PN supplementation with n-3 PUFAs, the marker levels of some oxidative stress, blood lipids, and hepatic biomarkers improved in patients with IF-II.

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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.

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There has been just over 30 years of experience in clinical intestine transplant. A rise in demand until 2007 with improving transplant outcomes preceded a subsequent fall in demand due, at least in part, to improvements in pretransplant care of patients with intestinal failure. Over the past 10 to 12 years, there has been no suggestion of an increase in demand and, particularly for adult transplant, there may be a continued trend toward fewer additions to the waiting list and fewer transplants, especially in those needing combined intestine-liver transplant. In addition, over the same period there has been no noticeable improvement in graft survival, with 1- and 5-year graft failure rates averaging 21.6% and 52.5%, respectively, for intestine-alone transplants and 28.6% and 47.2%, respectively, for combined intestine-liver allografts.

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BACKGROUND: Most data on anemia in children with intestinal failure (IF) have been obtained from studies in which the data were collected at a single point in time. We aimed to identify the frequency of anemia and factors associated with hemoglobin levels in children with IF during their course of home parenteral nutrition. METHODS: We performed a longitudinal cohort study of patients with IF followed up at a pediatric intestinal rehabilitation center. Outcome variables were hemoglobin levels and prevalence of anemia during the follow-up period. The exposure variables were age, duration of parenteral nutrition, chronic disease, and serum concentrations of C-reactive protein, iron, copper, selenium, vitamins A, D, B12 , and folic acid. RESULTS: Twenty-five children with a median time of receiving parenteral nutrition of 40.7 months were included. A median (and interquartile range) of 40.7 (25.2-58) hemoglobin measurements were performed per patient. Mean (SD) hemoglobin was 10.7 (1.8) g/dL at baseline and 11.6 (0.9) g/dL in the last observation (paired t test, P = 0.07); 32% of patients had mean hemoglobin values below the lower limit for age. In a multivariable predictive model, having C-reactive protein >1 mg/dL was associated with a decrease of 0.57 g/dL in hemoglobin (95% CI, -0.90 to -0.24, P = 0.01), and an increase of 1 mg/L in vitamin A concentration was associated with the increase of 0.93 g/dL in Hb level (95% CI, 0.24-1.61; P = 0.008). CONCLUSION: Anemia affects almost one-third of children with IF and its frequency decreases during the follow-up period. Hemoglobin levels are associated with inflammatory response and serum micronutrient concentrations.

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OBJECTIVE: To measure the time that caregivers spend on tasks related to providing care to their child with intestinal failure receiving home parenteral nutrition (PN). STUDY DESIGN: We conducted an exploratory cross-sectional study of caregivers of children with intestinal failure receiving long-term PN followed by our intestinal rehabilitation program. Caregivers completed a daily diary of care-related tasks. Data were analyzed using descriptive statistics. Exploratory models were completed to evaluate factors that influenced the amount of time that caregivers spent providing care. SAS University Edition 2018 (SAS Institute, Cary, NC) was used for data analysis with a P value of less than .05 considered significant. RESULTS: Thirty-four caregivers of children with intestinal failure consented with response rates of 85%. The mean age of the primary caregiver was 37 ± 7.9 years of age with 97% being the child's mother. The median PN exposure was 1239 days (IQR, 432-3012). Caregivers reported a median of 29.2 hours per week (IQR, 20.8-45.7 hours per week) of direct medical care. The majority of time was spent on providing PN and care of the central venous catheter (6.1 hours; IQR, 5.2-8.8). CONCLUSIONS: Caregivers of children with intestinal failure receiving long-term PN provide a significant amount of care to ensure their child remains healthy at home. The most significant amounts of time were spent on the administration of the PN and care of the central venous catheter.

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BACKGROUND: Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease. METHODS: A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed. RESULTS: Of the 61 enrolled patients, 56 with available follow-up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan-Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year. CONCLUSION: The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers.

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OBJECTIVE: To identify prenatal and neonatal predictors of short bowel syndrome-related intestinal failure (SBS-IF) in gastroschisis. STUDY DESIGN: This retrospective study included all patients with gastroschisis born between 2000 and 2017 who were enrolled in our home parenteral nutrition program, and all patients with gastroschisis born in our institution who survived 2 weeks, during the same time period. Prenatal ultrasound features, neonatal status, anatomic features, oral feeding, and parenteral nutrition dependency were analyzed. RESULTS: Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6 months (oral feeding group). The mean follow-up was 7.9 years (IQR, 1.6-17.5 years) and 5.0 years (IQR, 0.1-18.2 years), respectively. Both bowel matting (OR, 14.23; 1.07-16.7; P = .039) and secondarily diagnosed atresia or stenosis (OR, 17.78; 3.13-100.98; P = .001) were independent postnatal predictors of SBS-IF. Eighteen children (51% of the SBS-IF group) were still dependent on artificial nutrition at the last follow-up. patients with SBS-IF who achieved full oral feeding had a median residual small-bowel length of 74 cm (IQR, 51-160 cm) vs 44 cm (IQR, 10-105 cm) for those still dependent on artificial nutrition (P = .02). An initial residual small bowel length of more than 50 cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%. CONCLUSIONS: Bowel matting, complex gastroschisis, and secondary intestinal obstruction were associated with SBS-IF in gastroschisis. For patients with SBS-IF, a small bowel length of more than 50 cm was predictive of secondary nutritional autonomy.

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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.

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.

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