Poor Response to Substitution Therapy with Cortisone Acetate in Patients with Congenital Adrenal Hyperplasia
| Autor: | Tsunekazu Yamano, Takuji Imamura, Hiroshi Inada, Ryoichi Nakajima |
|---|---|
| Rok vydání: | 2004 |
| Předmět: |
Drug
endocrine system medicine.medical_specialty medicine.drug_class 11β-hydroxysteroid dehydrogenase (HSD) type 1 Endocrinology Diabetes and Metabolism media_common.quotation_subject 21-hydroxylase deficiency Endocrinology hydrocortisone (HC) Internal medicine medicine Congenital adrenal hyperplasia In patient Substitution therapy cortisone acetate (CA) media_common Hydrocortisone business.industry medicine.disease Cortisone acetate Pediatrics Perinatology and Child Health Corticosteroid Original Article business congenital adrenal hyperplasia (CAH) medicine.drug |
| Zdroj: | Clinical Pediatric Endocrinology |
| ISSN: | 1347-7358 0918-5739 |
| DOI: | 10.1297/cpe.13.11 |
| Popis: | Although cortisone acetate is approved worldwide as corticosteroid substitution therapy in congenital adrenal hyperplasia (21-hydroxylase deficiency), its effectiveness is uncertain since its biologic activity depends on activation by 11β-hydroxysteroid dehydrogenase (11β-HSD). We sought to compare the effect of cortisone acetate with that of hydrocortisone. In 10 patients with congenital adrenal hyperplasia, cortisone acetate was replaced with hydrocortisone in substitution therapy. During this change, blood concentrations of 17-hydroxy-progesterone, adrenocorticotropin (ACTH), and requirements for each drug were monitored. Concentrations of 17-hydroxyprogesterone decreased (mean 10.1 vs. 48.6 ng/ml), as did those of ACTH. Cortisone acetate dose requirements averaged 33.9 mg/m(2), while hydrocortisone dose requirements averaged only 20.3 mg/m(2). In one of the patients resistant to cortisone acetate therapy, DNA sequences in the coding regions and promoter of the 11β-HSD gene were analyzed, detecting no genetic abnormalities. Cortisone acetate is inferior to hydrocortisone as substitution therapy in patients with congenital adrenal hyperplasia. |
| Databáze: | OpenAIRE |
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