Gonadotropin secretion in congenital adrenal hyperplasia before and during glucocorticoid therapy. Differences between the newborn infant and late onset forms
Medina, M.; Martín, O.; Flores, M.; Zárate, A.
Revista de Investigacion Clinica; Organo del Hospital de Enfermedades de la Nutricion 38(4): 401-406
1986
ISSN/ISBN: 0034-8376 PMID: 3105017 Document Number: 274004
Basal gonadotropin levels and pituitary secretory response to LHRH were studied in three children and three late-onset postpubertal patients with non-salt-losing congenital adrenal hyperplasia (CAH) before and during cortisone replacement therapy. Before initiation of hydrocortisone treatment, the three children with the classic form of CAH showed the typical prepubertal pattern of low circulating levels of LH and FSH, as well as a lack of response to LHRH administration. Once these children were on hydrocortisone therapy, the administration of LHRH induced a significant incrase in LH serum levels with minor change in FSH values. The patients with late-onset congenital adrenal hyperplasia had a normal pubertal development before the enzyme deficiency was manifested. These patients exhibited basal normal gonadotropin levels and the administration of LHRH produced a postpubertal pattern of response before and during glucocorticoid therapy. These results demonstrate the existence of two different patterns of gonadotropin secretion in CAH. In the untreated children the pattern of response to LHRH corresponded to their sexual development stage as well as to their advanced bone age, but there was no correspondence to their chronological age. During treatment the response to LHRH administration changed to a pubertal pattern. In the patients with late-onset CAH, the postpubertal pattern of response of LHRH was found before and during glucocorticoid therapy. We conclude that the type and amount of adrenal steroids as well as the age of onset of the enzyme deficiency are involved in the regulation of gonadotropin secretion.