Skip to main content Accessibility help
×
Home

Stein-Leventhal Syndrome in Sisters — The Possible Role of Genetic Factors in the “Polycystic Ovary Syndrome”

  • A. Borghi (a1), M. Maiello (a1) and G. Giusti (a1)

Summary

Two pairs of sisters are reported who showed clinical, morphologic, and hormone abnormalities, such as to be classified in the picture of the so-called Stein-Leventhal syndrome.

These cases, as other few similar observations, may suggest the possibility that, at least in some instances, the Stein-Leventhal syndrome can be governed by factors of a genetic order.

After a short review of the literature on this subject, it is pointed out that enough evidence supports the existence of predisposing genetic factors to the manifestation of the syndrome, although a true heredity of the disease cannot at the moment be entirely supported. Also some fraternal cases could find an explanation in lesional actions repeated during subsequent pregnancies.

    • Send article to Kindle

      To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

      Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

      Find out more about the Kindle Personal Document Service.

      Stein-Leventhal Syndrome in Sisters — The Possible Role of Genetic Factors in the “Polycystic Ovary Syndrome”
      Available formats
      ×

      Send article to Dropbox

      To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

      Stein-Leventhal Syndrome in Sisters — The Possible Role of Genetic Factors in the “Polycystic Ovary Syndrome”
      Available formats
      ×

      Send article to Google Drive

      To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

      Stein-Leventhal Syndrome in Sisters — The Possible Role of Genetic Factors in the “Polycystic Ovary Syndrome”
      Available formats
      ×

Copyright

Corresponding author

Via Andrea del Castagno 34, 50132 Firenze, Italy

References

Hide All
Axelrod, L.R., Goldzieher, J.W. 1961. The polycystic ovary. III - Steroid biosynthesis in normal and polycystic ovarian tissue. J. Clin. Endocrinol. Metab., 22: 431.
Belaisch, J., Sebaoun, J., Gilbert-Dreyfus, , Zara, M. 1958. Sexe chromosomique et états intersexuels. Sem. Hop. Paris, 34: 1313.
Bigozzi, U., Giusti, G., Borghi, A. 1957. Iperfunzione surrenale androgena in gemelle (Considerazioni endocrinologiche e genetiche). Atti del Simposio Internazionale sulla Genetica della Tubercolosi e dei Tumori, Torino. Edizioni Istituto Mendel, Roma.
Bishun, N.P., Morton, W.R.M. 1964. Chromosome mosaicism in Stein-Leventhal syndrome. Br. Med. J., 5418: 1200.
Bonilla, F. 1962. El virilismo en ginecologia. Atti del Simposio Internazionale sui Virilismi in Ginecologia, Firenze. Ed. Salpietra, Firenze.
Botella-Llusià, J. 1967. Le syndrome de Stein-Leventhal. Gynecol. Prat., 18: 21.
Bret, A.J., Duperrat, B., Bourey, J. 1955. À propos du syndrome de Stein-Leventhal. Presse Med., 62: 1561.
Broster, L.R., Patterson, J., Camber, B. 1953. Adrenal pseudohermaphroditism. Br. Med. J., 4849: 1288.
Byrd, J.R., Mahesh, V.B., Greenblatt, R.B. 1964. Chromosomal studies in Stein-Leventhal syndrome. J. Clin. Endocrinol. Metab., 24: 939.
Cahill, G.F., Melicow, M.M. 1950. Tumors of the adrenal gland. J. Urol., 64: 1.
Case Records of the Massachusetts General Hospital 1957. Case 43481. N. Engl. J. Med., 257: 1086.
Case Records of the Massachusetts General Hospital 1962. Case 85. N. Engl. J. Med., 267: 1311.
Cerviño, J.M., Ravera, J.J., Grosso, O.F., Pou de Santiago, A., Moratò-Manaro, J., Moussio-Fournier, J.C. 1956. Syndrome de Stein-Leventhal. À propos de deux sœurs atteintes de ce syndrome. Ann. Endocrinol. Paris, 17: 355.
Cooper, H.E., Spellacy, W.N., Prem, K.A., Cohen, W.D. 1968. Hereditary factors in the Stein-Leventhal syndrome. Am. J. Obstet. Gynecol., 100: 371.
De Grouchy, J., Lamy, M., Yaneva, H., Salomon, Y., Netter, A. 1961. Further abnormalities on the X chromosome in primary amenorrhoea or in severe oligomenorrhoea. Lancet, 2: 777.
De Lozzio, C.B., Bur, G., Sonnerschein, C., Valencia, J.I. 1963. Estudio citogenetico en casos de patologia gonadal femenina. Prensa Med. Argent., 50: 1322.
Evans, T.N., Riley, G.M. 1958. Polycystic ovariandisease (Stein-Leventhal syndrome): ethiology and rationale for surgical treatment. Obstet. Gynecol., 12: 168.
Fréderich, L., Lennès, G., Rougé-Collard, E., Mouchette, R., Hausman-Hagemeijer, A. 1967. Mise en évidence par analyse statistique d'une petite translocation chez un sujet atteint du syndrome de Stein-Leventhal. J. Genet. Hum., 16: 25.
Galbis, M., Monmeneu, S., Forteza, G., Esteve, F., Baguena, R. 1964. Citogenetica de la poliquistosis ovàrica. Rev. Esp. Obstet. Ginecol., 23: 270.
Giusti, G., Borghi, A., Zurli, A., Cavina, C., Toccafondi, R. 1964. Studi sull'irsutismo « semplice ». VII - I risultati dell'indagine peneumopelvigrafica in 127 casi: correlazione fra il quadro radiologico ovarico e i dati clinici ed ormonali. Rass. Neurol. Veg., 18: 1.
Giusti, G., Borghi, A., Zurli, A., Maiello, M. 1966. A proposito dei casi di disgenesia gonadica a fenotipo femminile con segni di funzione gonadica spontanea. Considerazioni cliniche ed etiopatogenetiche. Rass. Neurol. Veg., 20: 63.
Goldzieher, J.W., Green, A. 1962. The polycystic ovary. I - Clinical and histologic features. J. Clin. Endocrinol. Metab., 22: 325.
Gonzales-Gutierrez, J.T. 1969. Nuestra experiencia acerca de los factores etiologicos en la ovariosis poliquistica. Ginecol. Obstet. Mex., 25: 323.
Hargrave, D.C. 1965. Chromosome mosaicism in Stein-Leventhal syndrome. Br. Med. J., 1: 997.
Jacobs, P.A., Harnden, D.G., Buckton, K.E., Court-Brown, W.M., King, M.J., McBride, J.A., MacGregor, T.N., Maclean, N. 1961. Cytogenetic studies in primary amenorrhoea. Lancet, 1: 1183.
Jeffcoate, T.N.A. 1964. The androgenic ovary, with special reference to the Stein-Leventhal syndrome. Am. J. Obstet. Gynecol., 88: 143.
Kase, N., Kowal, J., Perloff, W., Soffer, L. J. 1963. In vitro production of androgens by a virilizing adrenal adenoma and associated polycystic ovaries. Acta Endocrinol. (Kbh.), 44: 15.
Lanthier, A., Sandor, T. 1960. “In vitro” production of androgenic steroids by human normal and “Stein-Leventhal type” ovarian slices. Metabolism, 9: 853.
Leon, N., Beçac, W., Beçac, M.L., Dorfman, R.I. 1963. On the ethiology of the Stein-Leventhal syndrome. Acta Genet. (Basel), 13: 252.
Lloyd, C.W., Lobotsky, J., Segre, E.J., Kobayashi, T., Taymor, M.L., Batt, R.E. 1966. Plasma testosterone and urinary 17-ketosteroids in women with hirsutism and polycystic ovaries. J. Clin. Endocrinol. Metab., 26: 314.
Lopez, J.M., Migeon, C.J., Jones, G.E.S. 1967. Hirsutism and evaluation of the dexametasone suppression and chronic gonadotropin stimulation test. Am. J. Obstet. Gynecol., 98: 749.
Mahesh, V., Greenblatt, R.B. 1962. Isolation of dehydroepiandrosterone and 17a-hydroxy-⊿5pregnenolone from the polycystic ovaries of the Stein-Leventhal syndrome. J. Clin. Endocrinol. Metab., 22: 441.
Mauvais-Jarvis, P., Baulieu, E.E. 1962. Urinary 17-ketosteroid conjugates in hirsutism. J. Clin. Invest., 41: 1690.
Milcu, S.M., Maximilian, C. 1962. Aspecte genetice in heterotricoža. Stud. Cercet. Endocrinol., 13: 391.
Milcu, S.M., Maximilian, C., Ionescu, B. 1968. Endocrinopatile Genetice. Ed. Acad. Republ. Social. Romania, Bucuresti.
Moncada-Lorenzo, E. 1970. Familial study of hirsutism. J. Clin. Endocrinol. Metab., 31: 556.
Moore, J. 1962. The Stein-Leventhal syndrome. Atti del Simposio Internazionale sui Virilismi in Ginecologia, Firenze. Ed. Salpietra, Firenze.
Muller, P., Weill, G. 1961. La chromatine sexuelle dans le syndrome de Stein-Leventhal et syndromes apparentés. Presse Med., 69: 2563.
Netter, A., Mauvais-Jarvis, P. 1961. Recherches sur la maladie de Stein-Leventhal. Sem. Hop. Paris, 37: 52.
Netter, A., Bloch-Michel, H., Salomon, Y., Thévenet, F., De Grouchy, J., Lamy, M. 1961 a. Etude du caryotype dans la maladie de Stein-Leventhal. Ann. Endocrinol. (Paris), 22: 841.
Netter, A., Bloch-Michel, H., Salomon, Y. 1961 b. Etude du caryotype d'une malade atteinte de syndrome de Stein-Leventhal. Presse Med., 69: 2563.
Netter, A., Thoret, Y. 1967. La maladie de Stein-Leventhal. Gynecol. Prat., 18: 71.
Nicolosi, G. 1960. La prova del progesterone in alcune consanguinee di donne con sindrome di Stein-Leventhal. Progr. Med., 16: 753.
Philipp, E., Stange, H.H. 1954. Das polyzystische Ovarium (Stein-Leventhal Syndrom). Dtsch. Med. Wochenschr., 79: 1519.
Rabinovich, L. 1955. Ovario poliquistico con hirsutismo-hipertenciòn esencial. Semana Med., 16: 103.
Rashad, M.N., Morton, W.R.M., Sloan, W.E., Bishun, N.P., Robinson, J.S. 1964. Polycystic sclerotic ovaries. Lancet, 1: 771.
Serment, H., Ruf, H., Codaccioni, J.L. 1961. Relations entre les aplasies ovariennes et le syndrome de Stein-Leventhal. Bull. Fed. Soc. Gynecol. Obstet. Lang. Fr., 13: 613.
Stein, I.F. 1967. La durée de la fertilité après la résection cunéiforme de l'ovaire au cours du syndrome de Stein-Leventhal. Gynecol. Prat., 18: 9.
Stenchever, M.A., MacIntyre, M.N., Jarvis, J.A., Hempel, J.M. 1968. Cytogenetic evaluation of 41 patients with Stein-Leventhal syndrome. Obstet. Gynecol., 32: 794.
Suzor, R., Falloux, P. 1961. Le syndrome de Stein-Leventhal est-il héréditaire? Bull. Fed. Soc. Gynecol. Obstet. Lang. Fr., 13: 12.
Trace, R.J., Keaty, E.C., McCall, M.L. 1960. An investigation of ovarian tissue and urinary 17-ketosteroids in patients with bilateral polycystic ovaries. Am. J. Obstet. Gynecol., 79: 310.
Touraine, A. 1955. L'Hérédité en Médecine. Masson, Paris.
Vague, J., Picard, D., Vithy, G., Galiner, L., Favier, G., Miller, G. 1956. Etude cytologique de la chromatine sexuelle dans diverses endocrinopathies. Ann. Endocrinol. (Paris), 17: 51.
Valsecchi, A., Beolchini, P.E., Gualandri, V. 1965. Considerazioni su di un caso di sindrome di Stein-Leventhal. Minerva Ginecol., 17: 1046.
Van Campenhout, J., Simmer, H., Dignam, W., Moore, J. 1964. Polycystic sclerotic ovaries. Lancet, 2: 1380.
Van Ek, K.H. 1964. Twee zusters met het syndroom van Stein-Leventhal. Ned. Tijdschr. Geneeskd., 109: 1307.
Wais, S. 1960. Bilateral polycystic ovaries with negative chromatin pattern. Acta Endocrinol. (Suppl.) (Kbh.), 35: 615.
Williams, G. H., Rose, L.I., Jaccer, P.L., Lauler, D.P. 1969. A Turner's syndrome variant with polycystic ovaries and idiopathic myocardial hypertrophy. Ann. Intern. Med., 70: 572.

Stein-Leventhal Syndrome in Sisters — The Possible Role of Genetic Factors in the “Polycystic Ovary Syndrome”

  • A. Borghi (a1), M. Maiello (a1) and G. Giusti (a1)

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed