[Polycystic ovary syndrome as expression of 3-beta-hydroxysteroid dehydrogenase deficiency].
Late-onset adrenal enzymatic deficiencies are a common cause of hirsutism. They can produce a PCO syndrome-like clinical course, underscoring the multi-causality of this condition. We report a 18 year old woman with secondary amenorrhea and hirsutism, whose serum levels of testosterone and DHEA-S were 175 ng/dl and 7.3 micrograms/ml and the LH/FSH ratio was 5.9. Dexamethasone decreased testosterone and DHEA-S levels, while the ACTH stimulation test increased the 17 hydroxypregnenolone serum concentration and the 17 hydroxypregnenolone/17 hydroxyprogesterone ratio to values considered diagnostic of 3 beta hydroxysteroid dehydrogenase deficiency.
Sapunar
J
J
Departamento de Endocrinología, Metabolismo y Nutrición, Hospital Clínico, Universidad Católica de Chile.
Triantafilo
Y
Y
Arteaga
E
E
Rojas
A
A
Campino
C
C
López
J M
JM
Rodríguez
J A
JA
D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency*; D000293:Adolescent; D000311:Adrenal Glands / Q000378:metabolism; D000324:Adrenocorticotropic Hormone; D005260:Female; D006628:Hirsutism / Q000378:metabolism; D006801:Humans; D011085:Polycystic Ovary Syndrome / Q000175:diagnosis / Q000201:enzymology*
D000324:Adrenocorticotropic Hormone; D015096:3-Hydroxysteroid Dehydrogenases
Síndrome de ovario poliquístico como expresión de déficit de la enzima 3 beta hidroxiesteroide deshidrogenasa.