Protocol Number: 96-CH-0033 Title: An Open, Randomized, Long-Term Clinical Trial of Flutamide, Testolactone, and Reduced Hydrocortisone Dose vs. Conventional Treatment of Children with Congenital adrenal hyperplasia Number: 96-CH-0033 Summary: This study was developed to determine if a combination of four drugs (fllutamide, testolactone, reduced hydrocortisone dose, and fludrocortisone) can normalize growth in children with congenital adrenal hyperplasia. The study will take 60 children, boys and girls and divide them into 2 groups based on the medications given. Group one will receive the new four- drug combination. Group two will receive the standard treatment for congenital adrenal hyperplasia (hydrocortisone and fludrocortisone). The boys in group one will take the medication until the age of 14 at which time they will stop taking the four drug combination and begin receiving the standard treatment for congenital adrenal hyperplasia. Girls in group one will take the four drug combination until the age of 13, at which time they will stop and begin receiving the standard treatment for congenital adrenal hyperplasia plus futamide. Futamide will be given to the girls until six months after their first menstrual period. All of the children will be followed until they reach their final adult height. The effectiveness of the treatment will be determined by measuring the patient's adult height, body mass index, and bone density. Sponsoring Institute: National Institute of Child Health and Human Development (NICHD) Recruitment Detail Type: Active Accrual Of New Subjects Gender: Male & Female Referral Letter Required: No Population Exclusion(s): None Eligibility Criteria: Boys with bone ages 2 to 13 years and girls with bone ages 2 to 11 years with classic 21-hydroxylase deficiency. Subjects must either not yet have undergone pubertal activation of the hypothalamic-pituitary-gonadal axis, or, if pubertal activation has occurred, must be receiving an LHRH agonist to suppress secondary central precocious puberty. Subjects must not have concurrent illnesses requiring glucocorticoid treatment (such as severe asthma), or requiring drugs that markedly alter hydrocortisone metabolism (such as anticonvulsants), and unable to be brought into reasonable control with conventional treatment (an unusual occurrence). Special Instructions: Currently Not Provided Disease Category: Endocrine, Nutritional, Metabolic, & Immunity Keywords: 21-Hydroxylase Deficiency 11-Hydroxylase Deficiency Antiandrogen Aromatase Inhibitor Growth Disorder Hydrocortisone Recruitment Keywords: Congenital adrenal hyperplasia (CAH) Investigational Drug(s): Flutamide and Testolactone Deslorelin Investigational Device(s): None Contacts: Patient Recruitment and Public Liaison Office, CC. Building 61 10 Cloister Court Bethesda, Maryland 20892-4754 Long Distance Calls: 1-800-411-1222 Fax: (301) 480-9793 Electronic Mail:prpl@mail.cc.nih.gov Citations: Cutler. 1990. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency, N Engl J Med, Vol. 323, p. 1806 Laue. 1996. A preliminary study of flutamide, testolactone, and reduced hydrocortisone dose in the treatment of congenital adrenal hyperplasia, J Clin Endocrinol Metab, Vol. 81, p. 3535 Merke. 1997. New approaches to the treatment of congenital adrenal hyperplasia, JAMA, Vol. 277, p. 1073 If you have: Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC. Questions about specific studies, or the database in general, please contact the Protocol Coordination Service Center, CC. Technical questions regarding the Clinical Center web site, please contact the Information Systems Department, CC.