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RESEARCH PRODUCT

The endocrine pattern of late onset adrenal hyperplasia (21-hydroxylase deficiency)

Enrico CarminaM. MaggioreFrancesca RosatoAlberto JanniA. M. Gagliano

subject

AdultHirsutismendocrine systemmedicine.medical_specialtyAdolescentmedicine.drug_classEndocrinology Diabetes and MetabolismLate onsetEndocrinologyAdrenocorticotropic HormoneInternal medicineHydroxyprogesteronesmedicineHumansEndocrine systemGonadal Steroid HormoneshirsutismAdrenal Hyperplasia Congenitalbiologybusiness.industry17-alpha-HydroxyprogesteroneVirilization21-HydroxylaseLuteinizing Hormonemedicine.diseasePolycystic ovaryProlactinProlactinEndocrinologySteroid Hydroxylasesbiology.proteinFemaleSteroid 21-HydroxylaseFollicle Stimulating Hormonemedicine.symptomGonadotropinbusinesshormones hormone substitutes and hormone antagonists

description

We describe 5 adult women with severe hirsutism due to late onset 21-hydroxylase deficiency. Diagnosis was performed on the finding of high serum 17-hydroxyprogesterone (17OHP) levels with a marked hyperresponse to an ACTH test. The endocrine study showed in most patients a gonadotropin behavior similar to that observed in classical polycystic ovary (PCO) syndrome. Prolactin levels were slightly increased in basal conditions and presented an exaggerated response to TRH stimulation.

https://doi.org/10.1007/bf03348395