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

Published online by Cambridge University Press:  01 August 2014

A. Borghi ,
M. Maiello  and
G. Giusti
A. Borghi*
Affiliation:
Institute of General Medical Clinic and Medical Therapy, University of Florence, Italy
M. Maiello
Affiliation:
Institute of General Medical Clinic and Medical Therapy, University of Florence, Italy
G. Giusti
Affiliation:
Institute of General Medical Clinic and Medical Therapy, University of Florence, Italy
*
Via Andrea del Castagno 34, 50132 Firenze, Italy

Summary

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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.

Type
Research Article
Information
Acta geneticae medicae et gemellologiae: twin research , Volume 21 , Issue 1-2 , April 1972 , pp. 79 - 93
Copyright
Copyright © The International Society for Twin Studies 1972

References

REFERENCES

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.CrossRefGoogle Scholar
Belaisch, J., Sebaoun, J., Gilbert-Dreyfus, , Zara, M. 1958. Sexe chromosomique et états intersexuels. Sem. Hop. Paris, 34: 1313.Google Scholar
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.Google Scholar
Bishun, N.P., Morton, W.R.M. 1964. Chromosome mosaicism in Stein-Leventhal syndrome. Br. Med. J., 5418: 1200.Google Scholar
Bonilla, F. 1962. El virilismo en ginecologia. Atti del Simposio Internazionale sui Virilismi in Ginecologia, Firenze. Ed. Salpietra, Firenze.Google Scholar
Botella-Llusià, J. 1967. Le syndrome de Stein-Leventhal. Gynecol. Prat., 18: 21.Google Scholar
Bret, A.J., Duperrat, B., Bourey, J. 1955. À propos du syndrome de Stein-Leventhal. Presse Med., 62: 1561.Google Scholar
Broster, L.R., Patterson, J., Camber, B. 1953. Adrenal pseudohermaphroditism. Br. Med. J., 4849: 1288.CrossRefGoogle Scholar
Byrd, J.R., Mahesh, V.B., Greenblatt, R.B. 1964. Chromosomal studies in Stein-Leventhal syndrome. J. Clin. Endocrinol. Metab., 24: 939.Google Scholar
Cahill, G.F., Melicow, M.M. 1950. Tumors of the adrenal gland. J. Urol., 64: 1.Google Scholar
Case Records of the Massachusetts General Hospital 1957. Case 43481. N. Engl. J. Med., 257: 1086.Google Scholar
Case Records of the Massachusetts General Hospital 1962. Case 85. N. Engl. J. Med., 267: 1311.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
Evans, T.N., Riley, G.M. 1958. Polycystic ovariandisease (Stein-Leventhal syndrome): ethiology and rationale for surgical treatment. Obstet. Gynecol., 12: 168.Google Scholar
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.Google Scholar
Galbis, M., Monmeneu, S., Forteza, G., Esteve, F., Baguena, R. 1964. Citogenetica de la poliquistosis ovàrica. Rev. Esp. Obstet. Ginecol., 23: 270.Google Scholar
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.Google Scholar
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.Google Scholar
Goldzieher, J.W., Green, A. 1962. The polycystic ovary. I - Clinical and histologic features. J. Clin. Endocrinol. Metab., 22: 325.Google Scholar
Gonzales-Gutierrez, J.T. 1969. Nuestra experiencia acerca de los factores etiologicos en la ovariosis poliquistica. Ginecol. Obstet. Mex., 25: 323.Google Scholar
Hargrave, D.C. 1965. Chromosome mosaicism in Stein-Leventhal syndrome. Br. Med. J., 1: 997.CrossRefGoogle ScholarPubMed
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.Google Scholar
Jeffcoate, T.N.A. 1964. The androgenic ovary, with special reference to the Stein-Leventhal syndrome. Am. J. Obstet. Gynecol., 88: 143.Google Scholar
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.Google Scholar
Lanthier, A., Sandor, T. 1960. “In vitro” production of androgenic steroids by human normal and “Stein-Leventhal type” ovarian slices. Metabolism, 9: 853.Google ScholarPubMed
Leon, N., Beçac, W., Beçac, M.L., Dorfman, R.I. 1963. On the ethiology of the Stein-Leventhal syndrome. Acta Genet. (Basel), 13: 252.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
Mauvais-Jarvis, P., Baulieu, E.E. 1962. Urinary 17-ketosteroid conjugates in hirsutism. J. Clin. Invest., 41: 1690.Google Scholar
Milcu, S.M., Maximilian, C. 1962. Aspecte genetice in heterotricoža. Stud. Cercet. Endocrinol., 13: 391.Google Scholar
Milcu, S.M., Maximilian, C., Ionescu, B. 1968. Endocrinopatile Genetice. Ed. Acad. Republ. Social. Romania, Bucuresti.Google Scholar
Moncada-Lorenzo, E. 1970. Familial study of hirsutism. J. Clin. Endocrinol. Metab., 31: 556.Google Scholar
Moore, J. 1962. The Stein-Leventhal syndrome. Atti del Simposio Internazionale sui Virilismi in Ginecologia, Firenze. Ed. Salpietra, Firenze.Google Scholar
Muller, P., Weill, G. 1961. La chromatine sexuelle dans le syndrome de Stein-Leventhal et syndromes apparentés. Presse Med., 69: 2563.Google Scholar
Netter, A., Mauvais-Jarvis, P. 1961. Recherches sur la maladie de Stein-Leventhal. Sem. Hop. Paris, 37: 52.Google Scholar
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.Google Scholar
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.Google Scholar
Netter, A., Thoret, Y. 1967. La maladie de Stein-Leventhal. Gynecol. Prat., 18: 71.Google Scholar
Nicolosi, G. 1960. La prova del progesterone in alcune consanguinee di donne con sindrome di Stein-Leventhal. Progr. Med., 16: 753.Google Scholar
Philipp, E., Stange, H.H. 1954. Das polyzystische Ovarium (Stein-Leventhal Syndrom). Dtsch. Med. Wochenschr., 79: 1519.Google Scholar
Rabinovich, L. 1955. Ovario poliquistico con hirsutismo-hipertenciòn esencial. Semana Med., 16: 103.Google Scholar
Rashad, M.N., Morton, W.R.M., Sloan, W.E., Bishun, N.P., Robinson, J.S. 1964. Polycystic sclerotic ovaries. Lancet, 1: 771.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google ScholarPubMed
Suzor, R., Falloux, P. 1961. Le syndrome de Stein-Leventhal est-il héréditaire? Bull. Fed. Soc. Gynecol. Obstet. Lang. Fr., 13: 12.Google Scholar
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.Google Scholar
Touraine, A. 1955. L'Hérédité en Médecine. Masson, Paris.Google Scholar
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.Google Scholar
Valsecchi, A., Beolchini, P.E., Gualandri, V. 1965. Considerazioni su di un caso di sindrome di Stein-Leventhal. Minerva Ginecol., 17: 1046.Google Scholar
Van Campenhout, J., Simmer, H., Dignam, W., Moore, J. 1964. Polycystic sclerotic ovaries. Lancet, 2: 1380.Google Scholar
Van Ek, K.H. 1964. Twee zusters met het syndroom van Stein-Leventhal. Ned. Tijdschr. Geneeskd., 109: 1307.Google Scholar
Wais, S. 1960. Bilateral polycystic ovaries with negative chromatin pattern. Acta Endocrinol. (Suppl.) (Kbh.), 35: 615.Google Scholar
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.CrossRefGoogle Scholar