Normalization of height and excess body fat in children with salt-wasting 21-hydroxylase deficiency

To evaluate growth and body composition of patients with the salt wasting form of classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and to compare them with healthy children. Twenty-one prepubertal patients (eight boys and 13 girls) between 2.1 and 10.2 years and 67 prepubert...

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Bibliographic Details
Published in:Jornal de pediatria 2011-05, Vol.87 (3), p.263-268
Main Authors: Mendes-Dos-Santos, Carolina T, Lemos-Marini, Sofia H V, Baptista, Maria Tereza M, Guerra-Junior, Gil, De-Mello, Maricilda P, Paulino, Maria Fernanda V M, Morcillo, André M
Format: Article
Language:eng ; por
Subjects:
Adipose Tissue - physiopathology
Adrenal Hyperplasia, Congenital - drug therapy
Adrenal Hyperplasia, Congenital - physiopathology
Anti-Inflammatory Agents - administration & dosage
Body Composition - physiology
Body Height - physiology
Body Mass Index
Bone Development - physiology
Child
Child, Preschool
Epidemiologic Methods
Female
Growth - physiology
Humans
Hydrocortisone - administration & dosage
Infant
Male
Skinfold Thickness
Time Factors
Treatment Outcome
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Summary:To evaluate growth and body composition of patients with the salt wasting form of classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and to compare them with healthy children. Twenty-one prepubertal patients (eight boys and 13 girls) between 2.1 and 10.2 years and 67 prepubertal healthy controls (36 boys and 31 girls) between 1.2 and 11.7 years were included. Weight, height, upper-arm circumference, skinfolds, body composition determined by bioimpedance, and bone age were measured. The following data were obtained from the medical records: parents' height, serum levels of 17-hydroxyprogesterone and Δ4-androstenedione, prescribed hydrocortisone doses, weight and length at birth, in the beginning of the treatment, and at 2 years. Patients had lower weight and length z scores at the first appointment compared with the same data at birth, showing recovery after the beginning of the treatment without advanced bone age. Mean height z score was higher in controls (0.28 ± 0.86) than in patients (-0.61 ± 0.99, p < 0.001); this difference disappeared when the patients' height was adjusted to their bone age (0.33 ± 1.68, p = 0.912). Patients had higher body mass index (p < 0.001), fat mass (p < 0.001), and fat mass index (p < 0.001) than controls. There was no difference in the skinfolds between the two groups (p = 0.157). Patients had growth recovery with mean height similar to the general population; however, they had higher body fat, which seems to be visceral, since there was no difference between the skinfolds of both groups.
ISSN:0021-7557
1678-4782
DOI:10.2223/JPED.2095