GROWTH-PATTERNS AND OUTCOMES IN CONGENITAL ADRENAL-HYPERPLASIA - EFFECT OF CHRONIC TREATMENT REGIMENS

Aim. To assess outcome (final height and sexual maturation), growth pa tterns and blood pressure in 16 children with congenital adrenal hyper plasia treated at one institution over a 30 year period. Methods. Grow th patterns and maturation were determined by retrospective review (me dian follow up period 14 years). Dose adjustment of corticosteroid rep lacement treatment, sufficient to maintain normal levels of adrenal pr ecursor secretion, was determined using assays of urinary pregnanetrio l excretion (up to 1975) or early morning measurements of plasma 17 hy droxy progesterone and plasma renin activity at intervals of 4-6 month s. Results. In 7 of 15 patients the growth pattern during infancy was retarded - 13 not exceeding the population mean. Catchup growth as ste roid dose fell with age was not usually observed. In boys, height pote ntial was further compromised by a significant reduction in growth vel ocity 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 a ctivity in childhood, most children were normotensive. Conclusions. Av oiding salt depletion in infancy and excessive androgen secretion duri ng childhood do not ensure normal growth patterns or normal final heig ht. Impaired final height in congenital adrenal hyperplasia is more li kely to be due to over treatment, particularly in infancy when lower d oses of corticosteroids may improve height prognosis.