Search

Our aim was to analyze the outcomes in children with short‐bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure–associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007–2018. We retrospectively reviewed charts of 135 patients with SBS‐PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2–4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty‐eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%–38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy‐three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of <100,000/μl(median 50,500/μl) and median CB of 21 mg/dl, seven achieved CB normalization and had improved platelet count. Overall survival was 97% (censored 96.3%). We demonstrate high transplant‐free survival and enteral autonomy rates among children with SBS‐IFALD relying on low‐dose soybean lipid emulsion. [ABSTRACT FROM AUTHOR]

The glucagon‐like peptide‐2 (GLP‐2) analogue teduglutide is a medical treatment option for patients with short‐bowel syndrome–associated chronic intestinal failure. Because studies in mice have shown that GLP‐2 analogues may promote the growth of colonic neoplasms, surveillance colonoscopies before and during teduglutide therapy were recommended. The occurrence of small‐intestinal neoplasms has not been reported so far, except for a recent report about de novo development of hamartomatous duodenal polyps. We report a case of de novo development of small‐intestinal premalignant adenomatous polyps in both bulbar duodenum and distal jejunum in a patient treated with teduglutide for 41 months. Therefore, additional endoscopic surveillance of the upper gastrointestinal tract may be advised during teduglutide therapy for early detection and removal of potential small‐bowel adenomas. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) is a term applied when intestinal function becomes insufficient to allow adequate absorption of fluid, electrolytes, or nutrients required for normal growth and survival. The development of parenteral nutrition (PN) as a treatment modality has greatly improved the outcome for children with IF and has contributed to improved outcomes in neonates and children from intensive care settings. Home parenteral nutrition (HPN) is central to the care of patients when IF is expected to last for more than 3–6 months. Normal growth and long-term survival is now expected for most children and young people on HPN when only 10 years ago, the prognosis for survival was poor. Adolescents with IF now successfully transfer to adult services, many of which will have been dependent on PN since early infancy. This article reviews the current indications for PN and the recent advances in PN that have contributed to better outcomes. A guideline on Paediatric Parenteral Nutrition of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the European Society for Clinical Nutrition and Metabolism (ESPEN) supported by the European Society of Paediatric Research (ESPR) was published in 2010. This has been recently updated (ESPGHAN/ESPEN/ESPR/CSPEN guidelines on pediatric parenteral nutrition, 2018) with up-to-date evidence for health professionals working with infants, children and adolescents receiving PN. This update includes details the use of Taurolock™ to prevent central venous catheter (CVC) related sepsis, and the role of new multicomponent lipid emulsions. Recommendations have been given with regard to the use of in-line filtration and photoprotection of PN fluids and advice on energy, fluid, electrolyte, macro-and micronutrient intake has been updated. [ABSTRACT FROM AUTHOR]

Introduction:  Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.Materials and Methods:  Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994-2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).Results:  In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0-38.0); median birth weight was 2,440 g (IQR: 1,700-2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0-92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.Conclusion:  We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF. [ABSTRACT FROM AUTHOR]

Background and Aims: Intestinal failure (IF) is a serious and common complication of short bowel syndrome with patients depending on parenteral nutrition (PN) support. Effective nutrition management requires an accurate estimation of the patient's basal metabolic rate (BMR) to avoid underfeeding or overfeeding. However, indirect calorimetry, considered the gold standard for BMR assessment, is a time- and resource-consuming procedure. Consequently, several equations for prediction of BMR have been developed in different settings, but their accuracy in patients with IF are yet to be investigated. We evaluated the accuracy of predicted BMR in clinically stable patients with IF dependent on home parenteral nutrition (HPN).Methods: In total, 103 patients with IF were included. We used indirect calorimetry for assessment of BMR and calculated predicted BMR using different equations based on anthropometric and/or bioelectrical impedance parameters. The accuracy of predicted BMR was evaluated using Bland-Altman analysis with measured BMR as the gold standard.Results: The average measured BMR was 1272 ± 245 kcal/d. The most accurate estimations of BMR were obtained using the Harris-Benedict equation (mean bias, 14 kcal/d [ P = .28]; limits of agreement [LoA], -238 to 266 kcal/d) and the Johnstone equation (mean bias, -16 kcal/d [ P = .24]; LoA, -285 to 253 kcal/d). For both equations, 67% of patients had a predicted BMR from 90%-110% All other equations demonstrated a statistically and clinically significant difference between measured and predicted BMR.Conclusions: The Harris-Benedict and Johnstone equations reliably predict BMR in two-thirds of clinically stable patients with IF on HPN. [ABSTRACT FROM AUTHOR]

Background: In phase III clinical studies, treatment with teduglutide was associated with clinically meaningful reductions (≥20% from baseline) in parenteral support (PS; parenteral nutrition and/or intravenous fluids) requirements in adult patients with intestinal failure associated with short bowel syndrome (SBS-IF). This analysis reports clinical characteristics of patients who achieved complete independence from PS during teduglutide treatment.Materials and Methods: Post hoc analysis of adult patients who achieved complete PS independence during treatment with teduglutide 0.05 mg/kg/d. Data were pooled from 5 teduglutide clinical trials (2 phase III placebo-controlled trials [NCT00081458 and NCT00798967] and their respective extension studies [NCT00172185, NCT00930644, NCT01560403]). Descriptive statistics were used; no between-group comparisons were performed because of the small sample size and lack of comparator.Results: Of 134 patients, 16 gained oral or enteral autonomy after a median of 5 years of PS dependence and 89 weeks of teduglutide treatment. Demographic and baseline disease characteristics varied among patients (median age, 55 years; 50% men; median baseline PS volume, 5.1 L/wk; median residual small intestine length, 52.5 cm). Most patients who achieved PS independence had colon-in-continuity; however, there was no significant difference in the frequency of PS independence among patients who maintained colon-in-continuity vs those who did not.Conclusion: Findings from this post hoc analysis suggest that oral or enteral autonomy is possible for some patients with SBS-IF who are treated with teduglutide, regardless of baseline characteristics and despite long-term PS dependence. [ABSTRACT FROM AUTHOR]

Background/aims: The objective of this study was to describe a clinically well-defined, single-center, intestinal failure (IF) cohort based on a template of definitions and classifications endorsed by the European Society for Clinical Nutrition and Metabolism (ESPEN).Methods: A cross-sectional, retrospective, adult IF cohort, receiving parenteral support (PS), was extracted from the Copenhagen IF database at the tertiary IF center, Copenhagen University Hospital, Rigshospitalet, Denmark.Results: Rigshospitalet provided PS to 188 adult patients with IF on December 31, 2011. Six patients received only fluids and electrolytes, while 97% required parenteral energy (17 ± 12 kcal/kg/d). Although 92% of the cohort had undergone intestinal resection, only 53% were classified as patients with short bowel syndrome (SBS) according to the pathophysiological classification. In the remaining cohort, patients were distributed as 5% with intestinal fistula, 12% with intestinal dysmotility, 5% with mechanical obstruction, and 14% with mucosal diseases. Twelve percent had a combination of pathophysiological causes. The patients with SBS (n = 100) were subdivided according to bowel anatomy into group 1 (jejuno/ileostomy, n = 82), group 2 (jejuno-colonic-anastomosis, n = 16), and group 3 (jejuno-ileo-colonic-anastomosis, n = 2). When evaluating the cohort requirements for PS using the ESPEN chronic IF classification based on the need for fluid volume and energy, 53% of the patients with IF were distributed in the maximum categories.Conclusion: The orphan condition of IF with its large patient heterogeneity mandates establishment of uniform definitions and a harmonization of classifications. As illustrated, the ESPEN-endorsed definitions and classifications are well designed and may serve as a common uniform template to facilitate both intra- and intercenter comparisons between reference centers and thus outcome results. [ABSTRACT FROM AUTHOR]

Background: institutions with multidisciplinary teams have shown improvements in patient outcomes with intestinal failure. Multidisciplinary approach allows an integral management and effective communication between families and care teams.Objective: describe the multidisciplinary management and outcome in pediatric patients with intestinal failure.Methods: retrospective study in patients 18 years old or less, with intestinal failure and Total Parenteral Nutrition (TPN) required. Simple frequencies and percentages were used for qualitative variables, and central tendency and dispersion measures were used for quantitative variables.Results: 33 patients with a median follow up of 281 days were evaluated. The median duration of the TPN was 68 days and the mean of catheter-related infections was 2.26 per patient. In 31 patients oral or enteral nutrition was provided, starting in 61.3% of cases through tube and continuous infusion. As concomitant treatment 72.7% of children received ursodeoxycholic acid, 67.7%, cholestyramine 57.6% loperamide, 48.5% antibiotics and 36.4% probiotic. The families of 24 patients were evaluated by social work professionals. Intestinal autonomy was achieved in 69.7% of cases, 72.7% of them showed an improvement in the score z of weight and showed an end albumin significantly higher than the initial (p value: 0.012).Conclusions: the management of patients with intestinal failure is a challenge for health institutions and require care based on a standardized protocol and a multidisciplinary group. [ABSTRACT FROM AUTHOR]

Abstract: Short bowel syndrome (SBS) is a reduction in functioning bowel length which is most often a result of surgical resection. Risk factors in the neonatal period include necrotising enterocolitis, small bowel atresia and gastroschisis. With increasing survival of preterm infants there is an increase in incidence. Management is dependent on the use of parenteral nutrition to maintain fluid and electrolyte homeostasis and promote growth and development with the longer term aim being to promote intestinal adaptation to achieve partial or complete enteral autonomy. In this review we discuss the incidence, aetiology, pathophysiology, medical and surgical treatments and outcome. [Copyright &y& Elsevier]

Extensive resection of the intestine impairs its absorptive capacity and results in short bowel syndrome when the nutritional equilibrium is compromised. The remnant intestine adapts structurally to compensate, but nutritional autonomy cannot be achieved in patients with intestinal failure, requiring intravenous fluids and parenteral nutrition (PN) for sustenance of life. PN is expensive and associated with serious complications. Efforts to minimize or eliminate the need for PN heralded research focusing on the therapeutic utility of intrinsic gut factors involved in the postresection adaptation process. With the breakthrough recognition of the intestinotrophic properties of glucagon-like peptide-2, teduglutide, a recombinant analogue of glucagon-like peptide-2, is being investigated as a promising hope to mitigate the requirement of PN. Clinical studies to date have demonstrated a desirable benefit-to-risk profile in regards to its safety and efficacy. If approved for marketing, it will be the first of its class in short bowel syndrome management, offering an innovative therapeutic modality for this debilitating condition. [ABSTRACT FROM AUTHOR]

Abstract: Background: Catheter-related bloodstream infections (CRBSI) cause morbidity and mortality in patients with intestinal failure dependent on parenteral nutrition. Ethanol lock of central venous catheters may decrease CRBSI, but limited pediatric data are available. Methods: Home parenteral nutrition patients with at least one previous CRBSI were initiated on a 70% ethanol lock protocol for a minimum of 4 hours. Infection rates (per 1000 catheter days) before and after initiation of ethanol locks were compared using a paired t test. Results: Ten patients (4 girls; median age, 44 months [range, 31-129 months]) began ethanol lock therapy after a total of 91 CRBSIs (37 gram-positive, 30 gram-negative, and 24 fungal) with a mean of 10.2 ± 6.2 per 1000 catheter days. Patients received ethanol lock for an average of 227 ± 64 days with only 3 CRBSI occurring (CRBSI rate of 0.9 ± 1.8 per 1000 catheter days [P = .005]). Central venous catheter replacements decreased from 5.6 per 1000 days to 0.3 per 1000 days posttherapy (P = .038). Ethanol lock was discontinued in 2 of 10 patients because of catheter thrombosis. Conclusion: Preliminary results demonstrate a significant decrease in CRBSI with a 70% ethanol lock protocol. Catheter thrombosis may be a limitation that needs to be addressed. With such a dramatic therapeutic effect, a randomized trial is feasible and should be performed. [Copyright &y& Elsevier]

Introduction: Current medical management of short bowel syndrome (SBS) involves the use of lifelong parenteral nutrition (PN). Glucagon-like peptide-2 (GLP-2), an important intestinotrophic growth factor has been shown to increase intestinal absorption in SBS through augmentation of post-resection intestinal adaptation. This may lead to the reduction of PN dependence in patients with SBS. Areas covered in review: Advancing research of GLP-2 physiology has spurred the growing understanding of the diverse effects of GLP-2. The development of the degradation resistant GLP-2 analog, teduglutide (GattexTM, NPS Pharmaceuticals, Bedminster, NJ), has allowed its exploration as a therapeutic agent in a variety of clinical settings. Recent multicenter, placebocontrolled studies of GLP-2 in SBS patients demonstrate meaningful reductions in PN requirements with good safety profiles. The reparative and immunomodulatory effects of teduglutide may also be beneficial in patients with inflammatory bowel disease (IBD). Safety concerns about possible carcinogenic properties during long-term use require ongoing evaluation. Summary: GLP-2 appears to offer a novel adjuvant treatment modality for SBS. Promise for its use in other clinical settings like IBD has been shown in small pilot studies. [ABSTRACT FROM AUTHOR]