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J Pediatr Gastroenterol Nutr. 2007 Mar;44(3):347-53.

Long-term outcome of children receiving home parenteral nutrition: a 20-year single-center experience in 302 patients.

Author information

1
Department of Pediatric Gastroenterology and Nutrition and HPN Center, Necker-Enfants Malades Hospital, Paris, France. virginie.colomb@nck.ap-hop-paris.fr

Abstract

BACKGROUND:

More information is needed regarding the prognosis of children receiving home parenteral nutrition (HPN). This article describes 20-year outcome data in children receiving HPN and provides separate profiles for the major pediatric diagnostic subgroups.

PATIENTS AND METHODS:

This retrospective study included children who started receiving HPN between January 1, 1980, and December 31, 1999, in a single pediatric HPN center.

RESULTS:

A total of 302 children were recruited, 230 (76%) with primary digestive disorders and 72 (24%) with nonprimary digestive disorders. Median age at HPN onset was 1.5 years. Median duration of HPN was 1.3 years. By January 1, 2000, 54% had weaned from HPN, 26% were still receiving HPN, 16% had died, and 4% had undergone intestinal transplantation. The survival probabilities at 2, 5, 10, and 15 years were 97%, 89%, 81%, and 72%, respectively. The likelihood and cause of death depended on the underlying diagnosis. Nine percent of children with primary digestive disorders died, 24% from their primary disease and 48% from liver disease or sepsis. Children with intractable diarrhea of infancy had the highest mortality rate (25%) and the highest incidence of liver disease (48%; P = 0.0002). Thirty-eight percent of children with primary nondigestive diseases died, 94% from their primary disease and 6% from liver disease or sepsis.

CONCLUSIONS:

Outcome and survival of children receiving HPN are mainly determined by their underlying diagnosis. Nearly all children with primary digestive disease survive if referred early to an expert center.

PMID:
17325556
DOI:
10.1097/MPG.0b013e31802c6971
[Indexed for MEDLINE]

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