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. 1966 Jan 1;94(1):1–7.

Polycystic Ovaries Associated with Congenital Adrenal Hyperplasia

O J Lucis, R Hobkirk, C H Hollenberg, S A MacDonald, P Blahey
PMCID: PMC1935165  PMID: 5901591

Abstract

Polycystic ovaries were found in a 16-year-old female with congenital absence of vagina, male-like external genitalia, and congenital adrenal hyperplasia. Masculinization was sufficiently severe to cause the patient to be reared as a male. Biochemical studies of ovarian tissue revealed hyperactivity and an imbalance of enzyme systems concerned with steroid-hormone biosynthesis, which led to production of large amounts of androgens. The pathway towards estrogens was preserved but less efficient than normal. Urinary steroid metabolites before and after hysterectomy and bilateral salpingo-oophorectomy revealed an absence of Porter-Silber chromogens and tetrahydrocortisone. Excretion of aldosterone was normal and that of corticosterone slightly higher than normal. The patterns of urinary 17-ketosteroids, pregnanediol, pregnanetriol and pregnanetriolone were similar to those commonly seen in congenital adrenal hyperplasia with steroid 21-hydroxylase deficiency. Urinary estrogens after panhysterectomy were low, being in the post-menopausal range. The pathogenesis of polycystic ovaries and their possible contribution to masculinization are discussed.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. AXELROD L. R., GOLDZIEHER J. W., ROSS S. D. CONCURRENT 3-BETA-HYDROXYSTEROID DEHYDROGENASE DEFICIENCY IN ADRENAL AND SCLEROCYSTIC OVARY. Acta Endocrinol (Copenh) 1965 Mar;48:392–412. doi: 10.1530/acta.0.0480392. [DOI] [PubMed] [Google Scholar]
  2. BARRACLOUGH C. A., LEATHEM J. H. Infertility induced in mice by a single injection of testosterone propionate. Proc Soc Exp Biol Med. 1954 Apr;85(4):673–674. doi: 10.3181/00379727-85-20989. [DOI] [PubMed] [Google Scholar]
  3. BONGIOVANNI A. M., ROOT A. W. The adrenogenital syndrome. N Engl J Med. 1963 Jun 6;268:1283–contd. doi: 10.1056/NEJM196306062682308. [DOI] [PubMed] [Google Scholar]
  4. BROWN J. B., BULBROOK R. D., GREENWOOD F. C. An additional purification step for a method for estimating oestriol, oestrone and oestradiol-17 beta in human urine. J Endocrinol. 1957 Nov;16(1):49–56. doi: 10.1677/joe.0.0160049. [DOI] [PubMed] [Google Scholar]
  5. EVANS T. N., RILEY G. M. Polycystic ovarian disease (Stein-Leventhal syndrome); etiology and rationale for surgical treatment. Obstet Gynecol. 1958 Aug;12(2):168–179. [PubMed] [Google Scholar]
  6. GREENBLATT R. B. Clinical aspects of sexual abnormalities in man. Recent Prog Horm Res. 1958;14:335–404. [PubMed] [Google Scholar]
  7. HAAS R. L., RILEY G. M. Polycystic ovaries and amenorrhea; laboratory and clinical observations. Obstet Gynecol. 1955 May;5(5):657–668. [PubMed] [Google Scholar]
  8. ITTRICH G. [Studies on the extraction of red Kober dyes by organic solvents for the determination of urinary estrogens]. Acta Endocrinol (Copenh) 1960 Sep;35:34–48. [PubMed] [Google Scholar]
  9. JEFFCOATE T. N. THE ANDROGENIC OVARY, WITH SPECIAL REFERENCE TO THE STEIN-LEVENTHAL SYNDROME. Am J Obstet Gynecol. 1964 Jan 15;88:143–156. doi: 10.1016/0002-9378(64)90248-0. [DOI] [PubMed] [Google Scholar]
  10. KEETTEL W. C., BRADBURY J. T., STODDARD F. J. Observations on the polycystic ovary syndrome. Am J Obstet Gynecol. 1957 May;73(5):954-62; discussion, 962-5. [PubMed] [Google Scholar]
  11. LEVENTHAL M. L. Functional and morphologic studies of the ovaries and suprarenal glwnds in the Stein-Leventhal syndrome. Am J Obstet Gynecol. 1962 Jul 15;84:154–164. doi: 10.1016/0002-9378(62)90418-0. [DOI] [PubMed] [Google Scholar]
  12. LEVENTHAL M. L. The Stein-Leventhal syndrome. Am J Obstet Gynecol. 1958 Oct;76(4):825–838. doi: 10.1016/0002-9378(58)90019-x. [DOI] [PubMed] [Google Scholar]
  13. PERLOFF W. H., JACOBSOHN G. EFFECT OF HUMAN CHORIONIC GONADOTROPIN (HCG) ON URINARY 17-KETOSTEROIDS IN A PATIENT WITH STEIN-LEVENTHAL SYNDROME BEFORE AND AFTER OOPHORECTOMY. J Clin Endocrinol Metab. 1963 Nov;23:1177–1179. doi: 10.1210/jcem-23-11-1177. [DOI] [PubMed] [Google Scholar]
  14. SOMMERS S. C., WADMAN P. J. Pathogenesis of polycystic ovaries. Am J Obstet Gynecol. 1956 Jul;72(1):160–169. doi: 10.1016/s0002-9378(16)37526-3. [DOI] [PubMed] [Google Scholar]

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