Elsevier

The Journal of Pediatrics

Volume 165, Issue 6, December 2014, Pages 1085-1090
The Journal of Pediatrics

Medical Progress
Enteral Nutrition in the Management of Pediatric Intestinal Failure

https://doi.org/10.1016/j.jpeds.2014.08.012Get rights and content

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Definitions

IF occurs when there is a reduction of functional intestinal mass necessary for adequate digestion and absorption for nutrient, fluid, and growth requirements, resulting in the need for intensive nutritional support. The American Gastroenterological Association defines IF as the condition that results “from obstruction, dysmotility, surgical resection, congenital defect, or disease-associated loss of absorption and is characterized by the inability to maintain protein-energy, fluid,

Enteral Feeding in IF

Deprivation of enteral calories, often termed “gut rest” in the setting of surgical or other interventions, causes atrophy of the intestinal mucosa, even in the presence of adequate PN support.7, 8, 9 Upon reintroduction of EN, the surgically or functionally shortened intestine must undergo structural and functional adaptations to best absorb luminal nutrients. The histologic hallmark of this compensatory response is intestinal epithelial cell hyperplasia, including increased villus height and

Micronutrient and Vitamin Deficiencies

Nutrients are differentially absorbed in various locations throughout the small intestine, and therefore, the type of bowel resected will predispose to specific micronutrient and vitamin deficiencies (Table II). For example, a patient with duodenal resection is at risk for iron and folate deficiency, whereas a patient with ileal resection is at risk for a deficiency of vitamin B12 and bile acid malabsorption. Bile acid deficiency may in turn predispose to deficiencies of the fat-soluble

Outcomes of IF

An important challenge in the care of patients with IF is the limited longitudinal data available to guide clinical decisions. The Pediatric Intestinal Failure Consortium is a group of 14 pediatric centers with multidisciplinary intestinal rehabilitation programs. A study of 272 infants with IF reported that the cumulative incidence of sustained enteral autonomy at 3 years was 44%; 26% died and 23% underwent intestinal transplantation.44 Notably, 30% of patients who achieved enteral autonomy

Emerging Therapies

Two recent medical therapies have recently emerged for the treatment of IF: GLP-2 and somatropin (human growth hormone). The endogenous peptide GLP-2 is secreted by intestinal L cells and enhances nutrient absorption and increases mucosal surface area, but has a short half-life due to degradation by the enzyme dipeptidyl peptidase IV.49 Teduglutide is a human recombinant GLP-2 analogue engineered with a single amino acid substitution, resulting in a longer half-life, allowing daily subcutaneous

Discussion

The outlook for children with IF has improved dramatically, with survival improving from 54% in one of the earliest published series59 to 73%-100% more recently.44, 60, 61, 62, 63, 64 Multidisciplinary programs now provide comprehensive and longitudinal IF care and improve survival for pediatric patients with IF.65 Current therapeutic goals in these patients include promotion of intestinal adaptation, optimization of quality of life, limiting of PN-associated morbidity, and eventual transition

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      The level of nutritional support at last follow-up was categorized based on degree of PN dependence: EN (full enteral autonomy), mixed (significant enteral tolerance with less than 75% calories from PN), or PN (greater than 75% caloric intake from PN). All patients underwent a standardized protocol for provision and advancement of enteral feeds using guidelines from a multidisciplinary intestinal rehabilitation program [17]. The route of administration of enteral feeds was transitioned from gastric or jejunal formula to oral formula or oral ad lib diet as appropriate.

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      This increasing population of children highlights the need for new strategies that focus on the achievement of enteral autonomy. Besides early provision of enteral nutrition and supportive therapies during the natural bowel adaptation process,32 one of the currently available strategies to promote enteral autonomy is the use of gastrointestinal hormones, such as glucagon-like peptide (GLP-2), to accelerate and induce adaptation.33-36 Subcutaneous administration of the GLP-2 analog teduglutide increases villous height and crypt depth and reduces the need for PN.36-43

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      Intestinal failure (IF) is defined as a critical reduction of functional gut mass below the minimum needed for the absorption of nutrients and fluids necessary to maintain health and growth [1,2]. IF can result from anatomical resection of the gut length resulting in short bowel syndrome, neuromuscular diseases causing dysmotility of the gastro-intestinal (GI) tract, or diseases of the intestinal epithelium causing chronic malabsorption [3–5]. The insufficient digestion and absorption in these patients make parenteral nutrition (PN) vital [6,7].

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    Funded by the National Institutes of Health (K24 HD 058795 [to C.D.] and T32 DK 7477-30 [to K.G.]). C.D. is a site investigator for the NPS-sponsored trial of teduglutide (registered with ClinicalTrials.gov: NCT01952080). The authors declare no conflicts of interest.

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