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[Defects of adrenal steroidogenesis in patients with hirsutism].
Med Clin (Barc). 1998 Feb 14; 110(5):171-6.MC

Abstract

BACKGROUND

To determine the frequency and the type of adrenal steroidogenic abnormalities in hirsute women.

SUBJECTS AND METHODS

ACTH test was performed during follicular phase in 127 hirsute and 40 normal (control) women. Before ACTH injection we measured in serum by RIA: 17-OH-pregnenolone (17-OH-P5), 17-OH-progesterone (17-OH-P4), androstenedione (AN), cortisol (CT), 11-deoxycortisol (DCT), dehydroepiandrosterone (DHEA) and its sulphate (DHEAS), total (TT) and free (FT) testosterone, oestradiol (E2), progesterone (PR), androstenediol glucuronide (AG), LH, FSH and prolactin. After 60 min of ACTH injection 17-OH-P5, 17-OH-P4, AN, DHEA, CT and DCT were measured. Net increment of stimulated steroids and the ratios 17-OH-P5/17-OH-P4, DHEA/AN, 17-OH-P4/CT, 17-OH-P5/CT and DCT/CT were calculated. Pelvic ultrasonographic exploration was done when irregular menses were reported.

RESULTS

Up to 31% of the patients presented enzymatic defects in adrenal steroidogenesis. Diagnostic criteria for enzyme defects were established. Late-onset 21-hydroxylase deficiency was diagnosed in 6 (4.5%) patients, HLA typing of these patients demonstrated that 4 out of 6 had B14-DR1. Sixteen women (12.6%) displayed a 17-OH-P4 response and the net increment 2 SD above the normal mean concentration, which are diagnostic criteria for late-onset 21-hydroxylase deficiency carriers. We diagnosed a 3 beta-hydroxysteroid dehydrogenase defect when 17-OH-P5 and DHEA responses, their net increment and the 17-OH-P5/17-OH-P4 and 17-OH-P5/CT ratios were 2 SD above the normal mean after ACTH: 14 women were diagnosed. 11 beta-hydroxylase deficiency diagnosis was made when DCT response, its net increment and the DCT/CT ratio after ACTH were 2 SD above the normal mean: 7 women were detected. Associated biosynthetic defects were described.

CONCLUSIONS

One third of our patients with hirsutism presented anomalous response to ACTH, consistent with enzymatic abnormalities in adrenal steroidogenesis.

Authors+Show Affiliations

Servicio de Endocrinologia, Hospital Universitari Germans Trias i Pujol, Badalona, Universidad Autónoma de Barcelona.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
English Abstract
Journal Article

Language

spa

PubMed ID

9547720

Citation

Salinas Vert, I, et al. "[Defects of Adrenal Steroidogenesis in Patients With Hirsutism]." Medicina Clinica, vol. 110, no. 5, 1998, pp. 171-6.
Salinas Vert I, Audí Parera L, Granada Ybern ML, et al. [Defects of adrenal steroidogenesis in patients with hirsutism]. Med Clin (Barc). 1998;110(5):171-6.
Salinas Vert, I., Audí Parera, L., Granada Ybern, M. L., Lucas Martín, A., Pizarro Lozano, E., Foz Sala, M., & Sanmartí Sala, A. (1998). [Defects of adrenal steroidogenesis in patients with hirsutism]. Medicina Clinica, 110(5), 171-6.
Salinas Vert I, et al. [Defects of Adrenal Steroidogenesis in Patients With Hirsutism]. Med Clin (Barc). 1998 Feb 14;110(5):171-6. PubMed PMID: 9547720.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Defects of adrenal steroidogenesis in patients with hirsutism]. AU - Salinas Vert,I, AU - Audí Parera,L, AU - Granada Ybern,M L, AU - Lucas Martín,A, AU - Pizarro Lozano,E, AU - Foz Sala,M, AU - Sanmartí Sala,A, PY - 1998/4/21/pubmed PY - 1998/4/21/medline PY - 1998/4/21/entrez SP - 171 EP - 6 JF - Medicina clinica JO - Med Clin (Barc) VL - 110 IS - 5 N2 - BACKGROUND: To determine the frequency and the type of adrenal steroidogenic abnormalities in hirsute women. SUBJECTS AND METHODS: ACTH test was performed during follicular phase in 127 hirsute and 40 normal (control) women. Before ACTH injection we measured in serum by RIA: 17-OH-pregnenolone (17-OH-P5), 17-OH-progesterone (17-OH-P4), androstenedione (AN), cortisol (CT), 11-deoxycortisol (DCT), dehydroepiandrosterone (DHEA) and its sulphate (DHEAS), total (TT) and free (FT) testosterone, oestradiol (E2), progesterone (PR), androstenediol glucuronide (AG), LH, FSH and prolactin. After 60 min of ACTH injection 17-OH-P5, 17-OH-P4, AN, DHEA, CT and DCT were measured. Net increment of stimulated steroids and the ratios 17-OH-P5/17-OH-P4, DHEA/AN, 17-OH-P4/CT, 17-OH-P5/CT and DCT/CT were calculated. Pelvic ultrasonographic exploration was done when irregular menses were reported. RESULTS: Up to 31% of the patients presented enzymatic defects in adrenal steroidogenesis. Diagnostic criteria for enzyme defects were established. Late-onset 21-hydroxylase deficiency was diagnosed in 6 (4.5%) patients, HLA typing of these patients demonstrated that 4 out of 6 had B14-DR1. Sixteen women (12.6%) displayed a 17-OH-P4 response and the net increment 2 SD above the normal mean concentration, which are diagnostic criteria for late-onset 21-hydroxylase deficiency carriers. We diagnosed a 3 beta-hydroxysteroid dehydrogenase defect when 17-OH-P5 and DHEA responses, their net increment and the 17-OH-P5/17-OH-P4 and 17-OH-P5/CT ratios were 2 SD above the normal mean after ACTH: 14 women were diagnosed. 11 beta-hydroxylase deficiency diagnosis was made when DCT response, its net increment and the DCT/CT ratio after ACTH were 2 SD above the normal mean: 7 women were detected. Associated biosynthetic defects were described. CONCLUSIONS: One third of our patients with hirsutism presented anomalous response to ACTH, consistent with enzymatic abnormalities in adrenal steroidogenesis. SN - 0025-7753 UR - https://www.unboundmedicine.com/medline/citation/9547720/[Defects_of_adrenal_steroidogenesis_in_patients_with_hirsutism]_ L2 - http://www.elsevier.es/en/linksolver/ft/ivp/0025-7753/110/171 DB - PRIME DP - Unbound Medicine ER -