Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes

Abstract

Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudo-obstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy” OR “long-term parenteral nutrition”. Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.

Cow's milk allergy non‐responsive to amino acid‐based formula? A successful transplanted patient with immune dysregulation, polyendocrinopathy, enteropathy, and X‐linked syndrome

“…The biopsy was fundamental to distinguish between normal histology (congenital lactase deficiency, congenital sodium diarrhea, neuroendocrine tumors), structural abnormalities (microvillus inclusion disease, integrin deficiency, and immunodeficiencies like IPEX syndrome) and eosinophilic enteropathies 19,21 . Finally, our patient developed severe malnutrition fulfilling the criteria for diagnosis of Intestinal Failure of non‐digestive etiology 22 …”

Complementary and antagonistic effects of combined glucagon‐like peptide‐2 and glucagon‐like peptide‐1 receptor agonist administration on parameters relevant to short bowel syndrome

“…32,34 However, the ability to achieve maximal intestinal growth in association with lower doses of a GLP-2R agonist may prove beneficial in preventing some of the side effects associated with existing GLP-2-based therapies. [1][2][3][4][5] As compared with GLP-2R agonists, which do not affect either GE or GI transit, GLP-1 mimetics potently slow both of these parameters, thereby increasing the time available for nutrient and fluid absorption. 19,22,31,35,36 Unexpectedly, combining the high-dose Ex4 treatment with low-dose h[Gly2]GLP2 prevented the inhibitory effects of Ex4 on GE transit without affecting its effects on GI transit.…”

“…Short bowel syndrome (SBS) is a devastating condition caused by significantly reduced intestinal mass. [1][2][3] Often a result of surgical resection, this disorder results in nutrient and fluid malabsorption alongside lifethreatening complications. Treatments such as fluid support and parenteral nutrition (PN) are therefore necessary to maintain electrolyte, fluid, and nutrient balance in patients.…”

“…Treatments such as fluid support and parenteral nutrition (PN) are therefore necessary to maintain electrolyte, fluid, and nutrient balance in patients. [1][2][3] Although these strategies prevent mortality in most patients, they are not always adequate and are also associated with risks of further complications such as sepsis and liver disease. 4,5 Currently, an analogue of glucagon-like peptide-2 (GLP-2), teduglutide, is the only pharmacological option available to treat SBS.…”

Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview

“…Altogether, PN has pros and cons; it may significantly improve the nutritional status prior to LT, with a beneficial effect on the outcome [ 96 , 98 ], but in some cases, it may aggravate jaundice [ 92 ] and introduce a risk of fatal vascular access-related complications in an already fragile subject [ 99 ]. The home management of enteral/parenteral nutrition is possible after appropriate family education [ 100 , 101 ].…”