Causes and Management of Intestinal Failure in Children.pdf

CO I D I p s s m a s p L l c u b C t e t a e r t q t c e t i I l c q t c p s p f f n GASTROENTEROLOGY 2006;130:S16–S28auses and Management of Intestinal Failure in Children LIVIER GOULET and FRANK RUEMMELE ntegrated Program of Intestinal Failure, Home Parenteral Nutrition and Intestinal Transplantation, National Reference Center for Rare igestive Disease, H?pital Necker-Enfants Malades, Université Reni Descartes, Paris, France a I I t s d m a l p m m m v s S g c r n i p s d s S p e I s H C g d i d p P Sntestinal failure is a condition requiring the use of arenteral nutrition as long as it persists. Causes of evere protracted intestinal failure include short bowel yndrome, congenital diseases of enterocyte develop- ent, and severe motility disorders (total or subtotal ganglionosis or chronic intestinal pseudo-obstruction yndrome). Intestinal failure may be irreversible in some atients, thus requiring permanent parenteral nutrition. iver disease may develop with subsequent end-stage iver cirrhosis in patients with intestinal failure as a onsequence of both underlying digestive disease and nadapted parenteral nutrition. Death will occur if com- ined liver-intestine transplantation is not performed. atheter-related sepsis and/or extensive vascular hrombosis may impede the continuation of a safe and fficient parenteral nutrition and may also require intes- inal transplantation in some selected cases. Thus man- gement of patients with intestinal failure requires an arly recognition of the condition and the analysis of its isk of irreversibility. Timing of referral for intestinal ransplantation remains a crucial issue. As a conse- uence, management should include therapies adapted o each stage of intestinal failure based on a multidis- iplinary approach in centers involving pediatric gastro- nterology, parenteral nutrition expertise, home paren- eral nutrition program, pediatric surgery, and liver ntestinal transplantation program. ntestinal failure (IF) is a condition of