Short bowel syndrome related intestinal failure outcomes in Latin America: Insights from the RESTORE Registry.

BACKGROUND: Short bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry. METHODS: A prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed. RESULTS: From May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 ± 18 years, 48% were female, and the mean follow-up was 22.6 ± 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 ± 55 cm. The complications were as follows: infections: 0.4/1000 catheter-days; thrombosis: 0.24/1000 catheter-days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow-up, 0.9% underwent transplant, and 45.6% continued follow-up. For the children, the mean age 48 ± 52 months, 48% were female, 52% were premature. The mean follow-up was 17.2 ± 5.6 months; the mean remaining intestinal length was 38 ± 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter-days; thrombosis: 2.22/1000 catheter-day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow-up. CONCLUSION: The RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry.

Current Status of Chronic Intestinal Failure Management in Adults.

BACKGROUND: Chronic intestinal failure (CIF) is a heterogeneous disease that affects pediatric and adult populations worldwide and requires complex multidisciplinary management. In recent years, many advances in intravenous supplementation support, surgical techniques, pharmacological management, and intestinal transplants have been published. Based on these advances, international societies have published multiple recommendations and guidelines for the management of these patients. The purpose of this paper is to show the differences that currently exist between the recommendations (ideal life) and the experiences published by different programs around the world. METHODS: A review of the literature in PubMed from 1980 to 2024 was carried out using the following terms: intestinal failure, CIF, home parenteral nutrition, short bowel syndrome, chronic intestinal pseudo-obstruction, intestinal transplant, enterohormones, and glucagon-like peptide-2. CONCLUSIONS: There is a difference between what is recommended in the guidelines and consensus and what is applied in real life. Most of the world's countries are not able to offer all of the steps needed to treat this pathology. The development of cooperative networks between countries is necessary to ensure access to comprehensive treatment for most patients on all continents, but especially in low-income countries.

Baseline Characteristics of Adult Patients Treated and Never Treated with Teduglutide in a Multinational Short Bowel Syndrome and Intestinal Failure Registry.

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.

Is neurodevelopment impaired in Brazilian children with intestinal failure on prolonged parenteral nutrition? A single center study.

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.

Bioelectrical impedance phase angle and nutritional status in children with intestinal failure on prolonged parenteral nutrition.

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.

Iodine status in children with intestinal failure.

OBJECTIVES: Children on long-term parenteral nutrition (PN) are at high risk of iodine deficiency (ID). However, most available information comes from cross-sectional studies. We investigated the iodine status, associated factors, and prevalence of hypothyroidism in children with intestinal failure (IF) who were followed up longitudinally. METHODS: This was a cohort study of children with IF monitored for urine iodine concentration (UIC), iodine intake, serum selenium concentration, and thyroid function in an intestinal rehabilitation program. The outcome variable ID was defined as a UIC value < 100 µg/L. Adjusted generalized estimating equations were used to assess the effects of the exposure variables on the UIC. RESULTS: Twenty-four patients aged 62.7 (39.1; 79.7) months who received PN for 46.5 (21.5) months were included. The average energy supply was 81.2 kcal/kg/day, 77.6% of which was provided by PN. An average of 5.2 UIC measurements per patient were performed. ID prevalence decreased from baseline (83.3%) to the last assessment (45.8%). Three patients had hypothyroidism secondary to iodine and selenium combined severe deficiency. Iodine intake from enteral or oral nutritional formulas was positively associated with UIC (ß = 0.71 [0.35, 1.07]; p < 0.001). Meeting approximately 80% of the estimated average requirement for iodine from nutritional formulas resulted in a greater probability of normal UIC values. CONCLUSION: ID is highly prevalent in children with IF who receive long-term PN and its frequency decreases with iodine intake from nutritional formulas. Severe combined iodine and selenium deficiencies are associated with the development of hypothyroidism in these patients.

Inflammation and micronutrient deficiency as major risk factors for anemia in children with intestinal failure: A longitudinal cohort study.

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.

High Rate of Venous Thromboembolism in Severe Pediatric Intestinal Failure.

OBJECTIVE: To quantify the rate of venous thromboembolism (VTE) in patients with pediatric intestinal failure and identify associated risk factors. STUDY DESIGN: We performed a retrospective cohort study in pediatric patients (<21 years old) with severe pediatric intestinal failure (≥90 consecutive days of parenteral nutrition) secondary to short bowel syndrome who were treated from 2014 to 2021 at an interdisciplinary intestinal rehabilitation program. The primary outcome was the incidence of VTE. Multivariable regression was performed to identify independent clinical predictors of VTE. RESULTS: A total of 263 patients (59.7% male) met the criteria for inclusion. The cumulative incidence of VTE was 28.1%, with a rate of 0.32 VTEs per 1000 catheter-days. On univariate analysis, the number of catheter days, number of catheters, and history of central line-associated blood stream infection were associated with VTE. On multivariable logistic regression, a higher number of catheters was an independent risk factor for VTE (aOR, 1.17; 95% CI, 1.06-1.29). Additionally, earlier gestational age was a risk factor for VTE such that every week decrease in gestational age conferred a 9% increased risk of VTE (aOR, 1.09; 95% CI, 1.02-1.16). CONCLUSIONS: In this retrospective study, 28.1% of patients with severe pediatric intestinal failure developed VTE; the number of catheters and early gestational age were noted to be independent risk factors for VTE. This high incidence of VTE highlights the need to investigate VTE in pediatric intestinal failure prospectively, including the potential benefit of prophylactic anticoagulation.

[Pediatric Intestinal Failure, 10 years of experience from a specialized unit]. / Insuficiencia Intestinal Pediátrica, 10 años de experiencia en una Unidad de Cuidados Especializados.

Intestinal Failure (IF) includes the loss of functional intestinal mass and the requirement of long term Parenteral Nutrition (PN) to achieve the development and growth in childhood. OBJECTIVE: To evaluate the experience in a specialized unit for pediatric patients with IF, describing the clinical cha racteristics of those admitted from November 2009 to December 2019. PATIENTS AND METHOD: Retros pective and descriptive review from clinical records of 24 cases that matched the inclusion criteria. The following variables were recorded: gender, neonatal history, origin unit, patient age and anthropome tric diagnosis at admission to the unit, cause of IF, hospital stay, anthropometric data and parenteral nutrition dependency at discharge. In those patients with a diagnosis of short bowel syndrome (SBS), the cause of intestinal resection and the characteristics of the intestinal remnant were identified: ana tomical classification, remnant length (defining ultra-short as < 25 cm), presence of ileocecal valve, and characteristics of the colon. RESULTS: The median age at admission was 7.8 months. Seventeen cases were preterm. Regarding IF etiology, 10 patients presented SBS, 6 patients with Intestinal Neuromus cular Disease (INMD), 7 children with SBS associated with INMD, and 1 case of intestinal lymphan giectasia. Within the SBS etiologies found in this group, intestinal atresia (8 cases) and necrotizing enterocolitis (9 patients) were the main causes with a similar proportion. Eight patients had no ileo cecal valve. According to anatomical classification, 1 case was Type I, 8 were Type II, and 8 were Type III. Related to bowel length, 3 were ultrashort, besides being Type II; in those with > 40 cm of bowel length, 7 were Type III. Overall average hospital stay was 456.4 days. Enteral autonomy was achieved in 16 patients and 8 cases required home parenteral nutrition. CONCLUSIONS: IF requires life support, PN and prolonged hospital stay. The principal etiology of IF is SBS caused by congenital intestinal atresia and necrotizing enterocolitis. Nevertheless, the high frequency of INMD could be attributed to the local protocol analysis. Most of our patients had poor prognosis factors, however, the management by a specialized team allowed the achievement of enteral autonomy in 66.7% of cases.

Carrying the Burden: Informal Care Requirements by Caregivers of Children with Intestinal Failure Receiving Home Parenteral Nutrition.

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.

First registry of adult patients with chronic intestinal failure due to short bowel syndrome in Argentina: The RESTORE project.

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.

Predicting Factors of Protracted Intestinal Failure in Children with Gastroschisis.

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.

Use of Intravenous Soybean and Fish Oil Emulsions in Pediatric Intestinal Failure-Associated Liver Disease: A Multicenter Integrated Analysis Report on Extrahepatic Adverse Events.

OBJECTIVE: To compare extrahepatic adverse events during fish oil lipid emulsion (FOLE) or soybean oil lipid emulsion (SOLE) treatment in children with intestinal failure-associated liver disease (IFALD). STUDY DESIGN: In this multicenter integrated analysis, bleeding, bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), infections, and signs of lipid emulsion intolerance were compared between FOLE recipients (1 g/kg/d) (n = 189) and historical controls who received SOLE (≤3 g/kg/d) (n = 73). RESULTS: When compared with SOLE recipients, FOLE recipients had a lower gestational age (30.5 vs 33.0 weeks; P = .0350) and higher baseline direct bilirubin (DB) (5.8 vs 3.0 mg/dL; P < .0001). FOLE recipients had a decreased incidence of bleeding (P < .0001), BPD (P < .001), ROP (P < .0156), bacterial and fungal infections (P < .0001), and lipid intolerance signs (P < .02 for all). Patients with bleeding vs patients without bleeding had higher baseline DB; the ORs for baseline DB (by mg/dL) and treatment (FOLE vs SOLE) were 1.20 (95% CI: 1.10, 1.31; P ≤ .0001) and 0.22 (95% CI: 0.11, 0.46; P ≤ .0001), respectively. In preterm infants, a higher BPD (P < .0001) and ROP incidence (P = .0071) was observed in SOLE recipients vs FOLE recipients. CONCLUSIONS: Children with IFALD who received FOLE had fewer extrahepatic adverse events, including a decreased incidence of bleeding, preterm comorbidities, and lipid intolerance signs compared with children with IFALD who received SOLE. TRIAL REGISTRATION CLINICALTRIALS.GOV: NCT00910104 and NCT00738101.

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)

Cirugía de elongación intestinal tipo "Step", en paciente con síndrome de intestino corto y enfermedad de Crohn. Primer caso realizado en Venezuela / Intestinal elongation surgery (step procedure) in a patient with short bowel syndrome and crohn disease. First case done in Venezuela

En el síndrome de intestino corto-insuficiencia intestinal, uno de los principales factores pronóstico, es la longitud intestinal residual, por lo que estrategias quirúrgicas que permitan aumentar la longitud cobran relevar importancia en el proceso de adaptación y eventual autonomía intestinal.Caso clínico: Paciente femenina, portadora de enfermedad de Crohn, con antecedentes de resección masiva de intestino por cuadro hemorrágico, se evidencia en el estudio radiológico moderada dilatación de asas delgadas, por lo que se practica cirugía de elongación intestinal tipo STEP, pudiendo aumentar la longitud intestinal de 32cm a 54cm. Resultados:Tiempo operatorio 270 min, período de seguimiento 10 meses, recuperación nutricional de 14,48 IMC a 22,5 IMC,con un esquema nutricional actual de 3 veces a la semana de 12 horas de administración(AU)

In the short bowel syndrome-intestinal failure, one of the most important key factors is the intestinal measure, so the surgical strategy searching to improve intestinal length is very important in order to facilitate the intestinal adaptation process. Clinic presentation: Female patient, with Crohn disease, previou surgery: massive intestinal resection due to hemorrhage. On intestinal X ray was observed mild dilated jejunum. It was performed intestinal lengthening surgery (STEP procedure). Intestinal length previous surgery 32cm, after procedure 54cm. Results: Operative time 27 min, follow up 10 months, nutritional recover IMC 14,48 to 22,5 Kg/m2 , nutritional therapy: 3 days/week TPN(AU)