D. Wasserman et al., Growth, nutritional status, body composition, and energy expenditure in prepubertal children with Alagille syndrome, J PEDIAT, 134(2), 1999, pp. 172-177
Objectives: To describe the patterns of growth, nutritional status, body co
mposition, and resting energy expenditure (REE) in prepubertal children wit
h Alagille syndrome (ACS) before the onset of end-stage liver disease.
Study design: Thirteen prepubertal subjects with AGS (8 male; mean age, 6.8
+/- 2.8 years) were evaluated for growth parameters, body composition by s
kinfolds and by dual-energy x-ray absorptiometry, and REE by indirect calor
imetry. The children with AGS were compared with a healthy, age-matched ref
erence group of 37 prepubertal children.
Results: Compared with healthy children, children with AGS had significantl
y reduced (P < .05) growth (weight, weight z score, height, height z score)
, nutritional status (midarm circumference, triceps skinfold, and mid-arm m
uscle area), and body composition (fat mass and fat-free mass). Subscapular
thickness, percent body fat, and REE were not different. The AGS subgroup
(n = 4) with REE greater than 110% predicted value had a reduced percent bo
dy fat (P < .02).
Conclusions: Growth and body composition abnormalities are common in prepub
ertal children with AGS.