Congenital adrenal hyperplasia complicated by central precocious puberty: Linear growth during infancy and treatment with gonadotropin-releasing hormone analog

• Published in: Metabolism, clinical and experimental Vol. 46; no. 5; pp. 513 - 517
• Main Authors: Soliman, Ashraf T., AlLamki, Mohammed, AlSalmi, Issa, Asfour, Maurice
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.05.1997; Elsevier
• Subjects: Adrenal Hyperplasia, Congenital - blood; Adrenal Hyperplasia, Congenital - complications; Adrenal Hyperplasia, Congenital - physiopathology; Biological and medical sciences; Child; Child Development; Female; Follicle Stimulating Hormone - blood; Gonadotropin-Releasing Hormone - analogs & derivatives; Hormones. Endocrine system; Humans; Leuprolide - therapeutic use; Luteinizing Hormone - blood; Male; Medical sciences; Pharmacology. Drug treatments; Puberty - drug effects; Puberty, Precocious - drug therapy; Puberty, Precocious - etiology; Endocrinopathy; Human; Puberty; Sexual development; Adrenal cortex diseases; Treatment efficiency; Gonadotropin RH; Hyperadrenocorticism; Corporal biometry; Enzymopathy; Precocious puberty; Congenital adrenal hyperplasia syndrome; Chemotherapy; Treatment; Analog; Adrenal gland diseases; Complication; Somatic growth; Child
• ISSN: 0026-0495; 1532-8600
• DOI: 10.1016/S0026-0495(97)90186-4

Some children with congenital adrenal hyperplasia (CAH) develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We have seen six such children who had the diagnosis of CAH with late initiation of corticosteroid treatment and/or poor compliance who developed central precocious puberty (CPP). These patients were treated with standard-dose hydrocortisone and fludrocortisone. Administration of depot leuprorelin (3.75 mg subcutaneously every 28 days) for 2 years or longer was effective in arresting the manifestations of puberty, decelerating the pretreatment growth velocity ([GV] 10.8 ± 1.5 v 3.65 ± 0.95 cm/yr), increasing the predicted adult height ([PAHT] 147.5 ± 7.8 v 153.4 ± 8.3 cm), and decreasing the bone age to statural age ratio (1.26 ± 0.13 v 1.16 ± 0.09). Analysis of auxanological data during the first 2 years of life showed that linear growth was significantly accelerated and bone age was advanced in patients who developed CPP compared with 11 age-matched patients. It appears that proper glucocorticoid replacement to achieve adequate control of hyperandrogenemia during early life might prevent development of CPP in these patients. Gonadotropin-releasing hormone agonist (GnRHa) therapy can improve the final adult height, bringing it closer to that expected from the genetic potential.