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.
Highlights
The term “intestinal failure” (IF) was defined originally by Fleming and Remington in 1981 to describe a state of “reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption of food” [1]
We performed a comprehensive search of published literature from January 2010 to the present on the PubMed database by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudoobstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy”
digestive IF (DIF) and non-digestive IF (NDIF) are reported in Tables 2 and 3, respectively; as shown, 66% of all cases of DIF
Summary
The term “intestinal failure” (IF) was defined originally by Fleming and Remington in 1981 to describe a state of “reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption of food” [1]. IF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) [3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24]. Despite the multiplicity of diseases recognized as causes of IF, the pathophysiological mechanisms through which they trigger the gastrointestinal dysfunction are few and are common to DIF and NDIF. 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). In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; through information flow within the network, the Registries could improve IF knowledge and management
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