Growth, nutritional status, body composition, and energy expenditure in prepubertal children with Alagille syndrome

• Published in: The Journal of pediatrics Vol. 134; no. 2; pp. 172 - 177
• Main Authors: Wasserman, Dror, Zemel, Babette S., Mulberg, Andrew E., John, Helen A., Emerick, Karan M., Barden, Elizabeth M., Piccoli, David A., Stallings, Virginia A.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.02.1999; Elsevier; Mosby-Year Book, Inc
• Subjects: Alagille Syndrome - physiopathology; Biological and medical sciences; Body Composition; Child; Child, Preschool; Energy Metabolism; Female; Gastroenterology. Liver. Pancreas. Abdomen; Growth; Humans; Liver Failure; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Malformations; Medical sciences; Nutritional Status; United States; North America; America; REE; FM; Fat; MAMA; DXA; FFM; AGS; CFR; MAC; WHO; Human; Portal circulation disease; Pathophysiology; Cardiovascular disease; Arteriohepatic dysplasia; Body composition; Vascular disease; Energetic cost; Prepuberty; Digestive diseases; Somatic growth; Child; Nutritional status
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/S0022-3476(99)70411-7

Objectives: To describe the patterns of growth, nutritional status, body composition, and resting energy expenditure (REE) in prepubertal children with Alagille syndrome (AGS) 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 skinfolds and by dual-energy x-ray absorptiometry, and REE by indirect calorimetry. The children with AGS were compared with a healthy, age-matched reference group of 37 prepubertal children. Results: Compared with healthy children, children with AGS had significantly reduced ( P < .05) growth (weight, weight z score, height, height z score), nutritional status (midarm circumference, triceps skinfold, and midarm muscle 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 body fat ( P < .02). Conclusions: Growth and body composition abnormalities are common in prepubertal children with AGS. (J Pediatr 1999;134:172-7)