A case of primary amenorrhea due to 17alpha- hydroxylase deficiency / 대한내과학회지
Korean Journal of Medicine
;
: 543-547, 2002.
Artículo
en Coreano
| WPRIM
| ID: wpr-209361
ABSTRACT
The enzyme, 17 -hydroxylase, is necessary for both cortisol and estrogen synthesis. Deficiency of the hormone results in increased adrenocorticotrophic hormone (ACTH), follicle-stimulating hormone (FSH). Synthesis of progesterone, 11-deoxycorticosterone (DOC), corticosterone and aldosterone don't require the enzyme. The lack of estrogen results in primary amenorrhea and absent sexual maturation. The replacement of dexamethasone and estrogens has lowered the blood pressure and produced feminization. A 19-year-old female had 46,XX genotype and presented amonorrhea, absence of sexual characteristics, hypertension and hypokalemia. Endocrinologic studies demonstrated increased plasma progesterone, ACTH levels and low production of 17 -hydroxyprogesterone and testosterone. We report a rare case of 17 -hydroxylase deficency with a brief history and review of the literature.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Plasma
/
Progesterona
/
Maduración Sexual
/
Testosterona
/
Presión Sanguínea
/
Corticosterona
/
Dexametasona
/
Hidrocortisona
/
Hiperplasia Suprarrenal Congénita
/
Hormona Adrenocorticotrópica
Límite:
Femenino
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Medicine
Año:
2002
Tipo del documento:
Artículo
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