[Growth and adult height in patients with congenital adrenal hyperplasia]. / Rust a dospelá výska u pacientu s kongenitální adrenální hyperplazií.

BACKGROUND: The growth of children with congenital adrenal hyperplasia (CAH) can be impaired by overproduction of endogenous androgens which after the initial growth acceleration lead to early ossification of the growth plates and a low adult height, and by excessive glucocorticoid treatment as the latter antagonize the effect of growth hormone and cause growth retardation. In order to identify the life period decisive for the adult height of patients with CAH the authors analyzed retrospectively their growth data. METHODS AND RESULTS: In 35 children with 21-hydroxylase deficiency (24 girls, age 4.8 - 22.3 years and 11 boys, age 6.3 - 22.3 years) the height and bone maturation was evaluated at the age of 3, 7, 10 and 12 years and the adult height. In all girls and in 5/11 boys treatment of CAH was started at the age of 0.0 - 1.5 years (median 0.1) (early diagnosis), in 6/11 boys at the age of 4.5 - 7.0 years (median 5.2) (late diagnosis). In children with an early diagnosis growth retardation by the age of 3 years occurred (SDS of the girls' height' -1.4 +/- 0.2, n = 24, boys -0.7 +/- 0.4, n = 5). The height deficit in girls at the age of 3 predicted significantly the loss of adult height, as compared with the expected height in the family (r = 0.68, p = 0.02). By the age of 7 years the height deficit declined insignificantly (SDS of girls' height -1.0 +/- 0.2, n = 21), but bone maturation proceeded rapidly (3 years: SDS 0.1 +/- 0.4, n = 7, 7 years: SDS 1.4 +/- 0.5, n = 11). The adult height of girls (n = 12) is 147.0 - 162.5 cm (mean 156.7), i.e. -1.6 +/- 0.2 SDS, in early diagnosed boys (n = 4) 159 - 176.6 cm (mean 167.6), i.e. -1.7 +/- 0.5 SDS, in boys with a late diagnosis (n =4) 165.0 - 172.0 (mean 169.5), i.e. -1.5 +/- 0.3 SDS. CONCLUSIONS: In treated children with CAH growth retardation occurs already from the age of 3 years, apparently due to excessive glucocorticoid doses. This retardation predicts a loss of adult height. Between the age of 3 and 7 years the growth prognosis deteriorates further due to accelerated bone maturation resulting from inadequate suppression of androgens. Optimal treatment during these periods in life can improve the prerequisites for attaining the predicted adult height.