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

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 786
Author(s):  
Antonella Diamanti ◽  
Giacomo Calvitti ◽  
Diego Martinelli ◽  
Emma Santariga ◽  
Teresa Capriati ◽  
...  

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.

Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis

Short-bowel syndrome 98Excessive diarrhoea 100Motility disorders 101Mucosal disorders 102The term intestinal failure (IF) refers to a functionally impaired gastrointestinal tract unable to maintain biochemical homeostasis and support normal growth. Short-bowel syndrome (SBS) is a common cause of IF and usually defined as a severe reduction in functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for growth. Other causes of IF include mucosal abnormalities giving rise to protracted diarrhoea, and neuromuscular disorders resulting in chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS). See ...


2018 ◽  
Vol 31 (02) ◽  
pp. 099-107 ◽  
Author(s):  
Khalil El-Chammas ◽  
Manu Sood

AbstractChronic intestinal pseudo-obstruction (CIP) is defined by either continuous or intermittent symptoms of bowel obstruction in the absence of fixed lumen excluding lesion. CIP includes a heterogeneous group of disorders which result either from diseases affecting the enteric neurons and smooth muscle lining or those involving the autonomic innervation of the bowel. Symptoms associated with CIP are nonspecific, which can sometimes contribute to the delay in recognizing the condition and making the correct diagnosis. The diagnostic workup should include imaging and manometry studies and, occasionally, full-thickness bowel biopsies for histopathological examination may be required. Multidisciplinary team approach for the management of these patients is recommended, and the team members should include a gastroenterologist, surgeon, chronic pain specialist, clinical nutritionist, and a psychologist. The treatment goals should include optimizing the nutritional status and preventing or delaying the development of intestinal failure. The majority of the patients require enteral or parenteral nutrition support, and chronic pain is a common and distressing symptom. Small bowel transplantation may be required if patients develop liver complications due to parenteral nutrition, have difficult central line access, or have poor quality of life and worsening pain despite aggressive medical management.


2020 ◽  
pp. 3025-3031
Author(s):  
Alexander Gimson

A wide range of miscellaneous disorders can affect the bowel. Those discussed in this chapter include cystic disorders (colitis cystica profunda and pneumatosis cystoides intestinalis), protein-losing enteropathy, vascular disorders of the intestine (spontaneous intramural haemorrhage and ortoenteric fistulas), intestinal pseudo-obstruction (acute colonic pseudo-obstruction, chronic intestinal pseudo-obstruction), various disorders of the colon and rectum (including microscopic, collagenous, and lymphocytic colitis, malakoplakia, solitary rectal ulcer syndrome, and stercoral ulcers), complications of parenteral nutrition and intestinal failure, peritoneal disorders, and endometriosis.


2012 ◽  
Vol 142 (5) ◽  
pp. S-613-S-614 ◽  
Author(s):  
Christopher F. Brandt ◽  
Louise Bangsgaard ◽  
Tine Jess ◽  
Michael Staun ◽  
Lone Tjellesen ◽  
...  

2021 ◽  
Vol 46 ◽  
pp. S654
Author(s):  
A.S. Sasdelli ◽  
M. Guidetti ◽  
A. Musio ◽  
G.A. Mari ◽  
C. Battaiola ◽  
...  

2006 ◽  
Vol 65 (3) ◽  
pp. 217-221 ◽  
Author(s):  
Tracey Johnson ◽  
Elaine Sexton

Managing infants, children and adolescents, ranging from premature infants to 18-year-old adolescents, on parenteral nutrition (PN) is a challenge. The ability of children to withstand starvation is limited and, unlike adults, children require nutrition for growth. PN in children is often required secondary to a congenital bowel problem rather than because of an acquired condition. Conditions requiring PN include motility disorders, congenital disorders of the intestinal epithelium and short-bowel syndrome (SBS). Intestinal failure may be temporary and children with SBS may be weaned from PN. However, other children require permanent PN. There are no comprehensive guidelines for the nutritional requirements of children and adolescents requiring PN. Practice in individual centres is based on clinical experience rather than clinical trials. Requirements are assessed on an individual basis according to age, nutritional status and clinical condition. These requirements need regular review to ensure that they remain appropriate for the changing age and weight of the child. Assessments of intakes use different methods, e.g. reference tables and predictive equations. Complications of PN include infection, accidental damage to, or removal of, the line and cholestatic liver disease. Home parenteral nutrition (HPN) is associated with fewer line infections and allows continuation of nutritional support in a more normal environment, encouraging normal development and participation in family activities. However, having a child at home on HPN is associated with physical and psychological stresses. A feeling of depression, loneliness and social isolation is common amongst children and their families. Home-care services are essential to supporting children at home and should be tailored to, and sensitive to, the individual needs of each family.


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