Adrenocortical adenoma associated with inadequately treated congenital adrenal hyperplasia


Kurtoglu S. , Atabek M. E. , Keskin M. , Patıroğlu T.

JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, cilt.16, ss.1311-1314, 2003 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 16 Konu: 9
  • Basım Tarihi: 2003
  • Doi Numarası: 10.1515/jpem.2003.16.9.1311
  • Dergi Adı: JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
  • Sayfa Sayıları: ss.1311-1314

Özet

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 virilizilng form of 21-hydroxylase deficiency is stressed.