TY - JOUR
T1 - Natural history of intestinal failure, investigated through a national network-based approach
AU - Guarino, Alfredo
AU - De Marco, Giulio
AU - Castro, M.
AU - Gambarara, M.
AU - Diamanti, A.
AU - Barabino, A.
AU - Gandullia, P.
AU - Roggero, P.
AU - Bruzzese, E.
AU - Berni Canani, R.
AU - Tramontano, A.
AU - Sordino, D.
AU - Settimi, A.
AU - Martelossi, S.
AU - Faraguna, D.
AU - Indrio, F.
AU - Campanozzi, A.
AU - Giovanni, S.
AU - Amarri, S.
AU - Lionetti, P.
AU - Bazzigaluppi, E.
AU - Bosi, E.
AU - Collins, R. S.
AU - Piana, G.
AU - Goulet, O.
AU - Marino, C.
AU - Morroni, M.
PY - 2003/8
Y1 - 2003/8
N2 - Objectives: Intestinal failure (IF) is a condition whose treatment requires advanced knowledge and techniques and a multidisciplinary approach. Intestinal failure is the endpoint of many intestinal diseases and may result in full recovery, in life-long parenteral nutrition, or in the death of the child. The aim of this study was to evaluate the natural history of IF in children using a national network of resources. Methods: Italian centers of pediatric gastroenterology merged in a national network, developing a collaborative management approach for children with IF. A consensus definition of IF was achieved. A database was set up to investigate the cause, epidemiologic factors, and natural history of IF. Results: One hundred nine children were enrolled in 5 years. The cause of IF was: short bowel syndrome (n = 48), disorders of motility (n = 16), structural enterocyte defects (n = 14), multiple food intolerance (n = 10), autoimmune enteropathy (n = 7), and others or unknown (n = 14). The eventual outcome was closely related to the primary cause, ranging from full and permanent intestinal sufficiency in children with multiple food intolerance to high death rate or total dependance upon parenteral nutrition in those with structural enterocyte defects. Intermediate outcomes were observed for the other causes. Four children received intestinal transplantation. Conclusions: The network approach for IF provides an effective model to optimize resources and to investigate prospectively the natural history of IF. Based on the work on this series, a European network for IF could be an effective model for fulfilling the diagnostic and management needs, including intestinal transplantation.
AB - Objectives: Intestinal failure (IF) is a condition whose treatment requires advanced knowledge and techniques and a multidisciplinary approach. Intestinal failure is the endpoint of many intestinal diseases and may result in full recovery, in life-long parenteral nutrition, or in the death of the child. The aim of this study was to evaluate the natural history of IF in children using a national network of resources. Methods: Italian centers of pediatric gastroenterology merged in a national network, developing a collaborative management approach for children with IF. A consensus definition of IF was achieved. A database was set up to investigate the cause, epidemiologic factors, and natural history of IF. Results: One hundred nine children were enrolled in 5 years. The cause of IF was: short bowel syndrome (n = 48), disorders of motility (n = 16), structural enterocyte defects (n = 14), multiple food intolerance (n = 10), autoimmune enteropathy (n = 7), and others or unknown (n = 14). The eventual outcome was closely related to the primary cause, ranging from full and permanent intestinal sufficiency in children with multiple food intolerance to high death rate or total dependance upon parenteral nutrition in those with structural enterocyte defects. Intermediate outcomes were observed for the other causes. Four children received intestinal transplantation. Conclusions: The network approach for IF provides an effective model to optimize resources and to investigate prospectively the natural history of IF. Based on the work on this series, a European network for IF could be an effective model for fulfilling the diagnostic and management needs, including intestinal transplantation.
KW - Intestinal failure
KW - Intestinal transplantation
KW - Parenteral nutrition
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U2 - 10.1097/00005176-200308000-00010
DO - 10.1097/00005176-200308000-00010
M3 - Article
C2 - 12883298
AN - SCOPUS:1542497371
VL - 37
SP - 136
EP - 141
JO - Journal of Pediatric Gastroenterology and Nutrition
JF - Journal of Pediatric Gastroenterology and Nutrition
SN - 0277-2116
IS - 2
ER -