Growth patterns and outcomes in congenital adrenal hyperplasia; effect of chronic treatment regimens.

AIM

To assess outcome (final height and sexual maturation), growth patterns and blood pressure in 16 children with congenital adrenal hyperplasia treated at one institution over a 30 year period.

METHODS

Growth patterns and maturation were determined by retrospective review (median follow up period 14 years). Dose adjustment of corticosteroid replacement treatment, sufficient to maintain normal levels of adrenal precursor secretion, was determined using assays of urinary pregnanetriol excretion (up to 1975) or early morning measurements of plasma 17 hydroxy progesterone and plasma renin activity at intervals of 4-6 months.

RESULTS

In 7 of 15 patients the growth pattern during infancy was retarded--13 not exceeding the population mean. Catchup growth as steroid dose fell with age was not usually observed. In boys, height potential was further compromised by a significant reduction in growth velocity during puberty. None of nine patients evaluated at final height had attained the target height. During the first year of life, plasma renin activity was suppressed below the reference range in six of nine infants. Despite this, and lower than normal levels of plasma renin activity in childhood, most children were normotensive.

CONCLUSIONS

Avoiding salt depletion in infancy and excessive androgen secretion during childhood do not ensure normal growth patterns or normal final height. Impaired final height in congenital adrenal hyperplasia is more likely to be due to over treatment, particularly in infancy when lower doses of corticosteroids may improve height prognosis.