Classification, epidemiology and aetiology.
Intestinal failure and its most important cause, short-bowel syndrome (SBS), are rare clinical entities leading to a vast complex of symptoms and complications with significant morbidity and mortality. Both conditions occur as the result of a massive reduction in enteral nutrient absorptive capacity. Disease manifestation is based on aetiological and anatomical characteristics such as remaining intestinal length and the presence of a functionally intact colon. Congenital and perinatal conditions, for example, intestinal atresia, necrotizing enterocolitis (NEC) and intestinal volvulus are the most important causes in children. The aetiology in adults is based on diseases inducing loss of intestinal function or loss of intestinal surface area after extensive surgical resections. The most frequent causes are mesenteric infarction, radiation enteritis and Crohn's disease. Knowledge of the epidemiology of intestinal failure and SBS is limited, being mainly based on the extrapolated figures of home parenteral nutrition centres and single-centre studies. At present, the incidence of SBS is estimated to be 2-5 per million.
Koffeman
Geert I
GI
Paediatric Surgical Centre Amsterdam, Academic Medical Center, Amsterdam, P.O. Box 22700, 1105 AZ, Amsterdam, The Netherlands. g.i.koffeman@amc.uva.nl
van Gemert
Wim G
WG
George
Elvira K
EK
Veenendaal
Roeland A
RA
D000328:Adult; D002648:Child; D003424:Crohn Disease / Q000150:complications; D004751:Enteritis / Q000150:complications / Q000209:etiology; D020345:Enterocolitis, Necrotizing / Q000150:complications; D020139:Gastroschisis / Q000150:complications; D006554:Hernia, Umbilical / Q000150:complications; D006627:Hirschsprung Disease / Q000150:complications; D006801:Humans; D007238:Infarction / Q000150:complications; D007408:Intestinal Absorption; D007409:Intestinal Atresia / Q000150:complications; D045822:Intestinal Volvulus / Q000150:complications; D012778:Short Bowel Syndrome / Q000145:classification / Q000453:epidemiology / Q000209:etiology; D013152:Splanchnic Circulation