Adrenocortical adenoma associated with inadequately treated congenital adrenal hyperplasia.

Abstract	We report a 6 year-old boy with the simple virilizing form of 21-hydroxylase deficiency in whom an adrenal adenoma developed following 5 years of steroid treatment. Extremely high levels of basal serum 17alpha-hydroxyprogesterone as well as an exaggerated response of 17alpha-hydroxyprogesterone to adrenocorticotropic hormone confirmed congenital adrenal hyperplasia at 7 years of age. Initially elevated serum steroid levels were restrained by high dose hydrocortisone therapy, but he chronically tended to take inadequate doses of glucocorticoid. At 12 years of age an adenoma was found in the cortex of the hyperplastic right adrenal gland. The importance of early diagnosis and compliance with medication in the simple virilizing form of 21-hydroxylase deficiency is stressed.
Authors	Selim Kurtoğlu, M Emre Atabek, Mehmet Keskin, Tahir Ercan Patiroglu
Journal	Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) Vol. 16 Issue 9 Pg. 1311-4 (Dec 2003) ISSN: 0334-018X [Print] Germany
PMID	14714757 (Publication Type: Case Reports, Journal Article)
Chemical References	Glucocorticoids Hydroxyprogesterones Testosterone Androstenedione Dehydroepiandrosterone Sulfate 17-alpha-Hydroxyprogesterone Adrenocorticotropic Hormone Mixed Function Oxygenases Steroid 21-Hydroxylase Hydrocortisone
Topics	17-alpha-Hydroxyprogesterone (blood) Adrenal Hyperplasia, Congenital (complications, diagnosis, drug therapy) Adrenocortical Adenoma (complications, surgery, ultrastructure) Adrenocorticotropic Hormone (pharmacology) Androstenedione (blood) Child Dehydroepiandrosterone Sulfate (blood) Drug Administration Schedule Glucocorticoids (therapeutic use) Hair (growth & development) Humans Hydrocortisone (therapeutic use) Hydroxyprogesterones (blood, pharmacology) Male Mixed Function Oxygenases (blood, deficiency, genetics) Patient Compliance Steroid 21-Hydroxylase (blood, genetics) Testosterone (blood) Treatment Failure Virilism (diagnosis, rehabilitation)

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