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

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.

Effects of growth hormone and/or sex steroid administration on whole-body protein turnover in healthy aged women and men.

Aging is associated with reduced activities of the growth hormone (GH), insulin-like growth factor I (IGF-I), and sex steroid axes, and with decreased lean body mass and protein synthesis. Using a randomized, double-blinded, placebo-controlled design, we studied the effects of 6 months of administration of GH alone, sex hormone alone (hormone replacement therapy in women, testosterone enanthate [T] in men), or GH plus sex hormone on protein turnover in healthy men (n=60) and women (n=43), aged 65 to 88 years (mean, 71+/-4.4 years). Growth hormone administration significantly increased IGF-I levels in both sexes, more markedly in men. Sex steroid administration increased the levels of estrogen and testosterone in women and men, respectively (P=.05). Protein turnover was measured before and after the 26-week treatment period by means of a primed, constant l-[1-(13)C]leucine infusion. In men, GH plus T administration increased leucine flux from 80.2+/-2.8 to 93.6+/-4.2 micromol.h-1.kg-1 (P=.02). Leucine oxidation did not change significantly after hormone treatment in either sex. Growth hormone treatment led to nonsignificant upward trends in nonoxidative leucine disposal in men (9.1+/-5.2 mol.h-1.kg-1) and women (7.6+/-7.1 mol.h-1.kg-1). Among all groups combined, changes in nonoxidative leucine disposal were directly related to those of serum IGF-I level (r=0.248, P<.02). Whole-body protein turnover increased in GH plus T-treated men (0.6+/-0.2 g protein.kg-1.d-1; P<.01). These data suggest that low-dose GH administration increases protein synthesis in healthy aged women and men, and that the coadministration of testosterone plus GH enhances this effect in elderly men.