Permanent intestinal failure.
BACKGROUND
Intestinal failure (IF) requires the use of parenteral nutrition as long as it persists and may be in case of persistence an indication for intestinal transplantation.


METHODS
Literature search was performed both electronically and manually.


CONCLUSIONS
Biological evaluation of IF is becoming possible with the use of plasma citrulline as a marker of intestinal mass. Short bowel syndrome (SBS) is the leading cause of intestinal failure in infants while few epidemiological data are available to date. Data on morbidity and mortality in pediatric patients with SBS are very limited while long-term outcome seems to be improving. Other causes of intestinal failure include neuro muscular intestinal disease and congenital disease of enterocyte development. The management of IF should include therapies adapted to each type and stage of IF based on a multidisciplinary approach, in centers involving pediatric surgery, pediatric gastroenterology, parenteral nutrition expertise, home parenteral nutrition program, and liver-intestinal transplantation experience. Timing for referral of patients in specialized centers remains a crucial issue. The main causes of IF are briefly reviewed emphasizing the medico-surgical strategy for prevention and care-provision, adapted to each type and stage of IF.
Goulet
Olivier
O
National Reference Center for Rare Digestive Diseases, Hospital Necker Enfants Malades, Universite Paris 5 Rene Descartes, Paris, France. olivier.goulet@nck.aphp.fr
Fusaro
Fabio
F
Lacaille
Florence
F
Sauvat
Frédérique
F
D015415:Biomarkers; D002956:Citrulline / Q000097:blood; D004065:Digestive System Abnormalities / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D006801:Humans; D007410:Intestinal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D007422:Intestines / Q000637:transplantation; D016031:Liver Transplantation; D010288:Parenteral Nutrition; D012778:Short Bowel Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy
D015415:Biomarkers; D002956:Citrulline