Abstract
BACKGROUND: Chronic intestinal failure (CIF) in childhood is caused by congenital malformations and inflammatory diseases of the gut. Its reported prevalence is 13.7 per million population. Long-term home parenteral nutrition has dramatically improved the life expectancy and quality of life of children with CIF. The affected children are now treated with parenteral nutrition at home as soon as their medical state and family circumstances allow. METHODS: The authors present data from a patient registry and review publications retrieved by a selective literature search. RESULTS AND CONCLUSION: Children with CIF can now be expected to survive beyond adolescence, at the very least, and enjoy good quality of life. This goal can only be achieved if nutritional therapy is carried out safely and the affected children's development is closely monitored by an interdisciplinary team that consists of primary care physicians/family doctors, neonatologists, pediatric gastroenterologists, and pediatric surgeons. Moreover, the prevention, early detection, and appropriate treatment of complications such as infection, liver disease, renal dysfunction, and disturbances of bone metabolism is of vital importance. The patients' families must be supported by specially qualified ambulatory nurses and social workers. Treatment with parenteral, enteral, and oral nutrition and surgery enables most infants with CIF to meet all their nutritional needs orally by the time they start going to school. For children who suffer from intractable complications, intestinal transplantation provides a real and increasing chance of survival.