Keywords
Hermafroditismo Verdadero
estados intersexuales
citogenética
estados intersexuales
citogenética
How to Cite
Jácome Roca, A., & Medina Murillo, J. E. (2018). Aspectos Citogenéticos, Patológicos y Endocrinos de Cuatro Pacientes con Posible Hermafroditismo Verdadero. Revista Colombiana De Endocrinología, Diabetes &Amp; Metabolismo, -8(2), 5–20. https://doi.org/10.53853/encr.-8.2.216
Abstract
Fragmento
Los estados intersexuales manifestados primordialmente por ambigüedad de los genitales externos se observan cada día con mayor frecuencia en los centros universitarios colombianos a donde son referidos los pacientes para estudio. Los servicios pediátricos los reciben e intervienen en el diagnóstico y en el tratamiento tanto los endocrinólogos como los urólogos, ginecólogos, citogenetistas y eventualmente los psiquiatras. Entre estas anomalías descolla el Hermafroditismo Verdadero como fenómeno interesante y como reto terapéutico.
Para su diagnóstico se requiere la presencia tanto de tejido ovárico como testicular, y deben diferenciarse de los más conocidos seudohermafroditismos, usualmente debidos a síndromes adrenogenitales.
* Trabajo presentado en la VIII reunión anual de la Sociedad Colombiana de Endocrinología, Noviembre de 1971, Manizales, Colombia.
References
1. BAIN. A. D., and SCOTT, J. S.: Mixed gonadal dysgenesis with XX/XY mosaicim, The evidence for the occurrence of fertilisation by two Spermatozoa in man, Lancet
1:1035, 1965.
2. BREWER. J. I., JONES. H. O., and CULVER, H.: True hermaphroditism, JAMA 148: 431-435, 1952.
3. BUNGE. R. G., and BRADBURY, J. T.: Oocytes in Seminiferous Tubules: H. A. case report of bilateral ovotestes, J. Clin. Endocrinol. 19: 1661-1666, 1959,
4. CLAYTON, G. W. SMITH, J. D., and ROSEMBERG, J. S.: Familial true hermaphroditism in pre and post-puberal genetic females. Hormonal and morphologic Studies, J. Clin. Endocrino. 18: 1349, 1968.
5. FEDERMAN, D. C.: Disorders of sexual development, N. Eng. J. Med. 277: 35-360, 1967.
6. FORD, C. E.: Mosaics and chimaeras, Brit. Med. Bull. 25:104, 1969.
7. GARTLER, S. M., WAXMAN, S. H. and GIBLETT, E.: and XX/XY human hermaphrodite resulting fron double fertilization, Proc. Nat. Acad. Scienc. 48: 332, 1962.
8. GRUMBACH, M. M., VAN WYK, J. J., and WILKINS, L.: Chromosonal Sex in gonadal dysgenesis (ovarian agenesis): relationship to male pseudohermaphroditism and theories of human sex differentiation, J. Clin. Endocrinol. 15: 1161, 1955.
9. GRUMBACH, M. M., and BARR, M. L.: Cytologic tests of chromosomal sex in relation to sexual anomalies in man, Rec. Progr., Hormone Res., 14: 335, 1958.
10. GUINET, P., et PUTELAT, R.: Hermaphrodisme Authentique, Rev. Lyon Med. 9: 67-97, 1960.
11. HAMPSON, J. G., MONEY, J., and HAMPSON, J. L.: Hermaphrodism: recomendations concerning case menagement, J. Clin. Endocrinol. 16: 547-556, 1956.
12. HARDEN, D. G., and AMSTRONG. C. N.: The chromosomes of a true hermaphrodite, Brit. M. J. 2: 12-87-1288, 1959.
13. HUGHES, W. H., ERIKSON, C. C., FLEISCHMAN, W., and ETTELDORF, J. N.; True hermaphroditism: report of a case, J. Pediat, 52: 662-669, 1958.
14. JACOME R., A, y GARCIA C., F.: Deficiencia congénita virilizante de la-hidroxilasa: estudio de 3 casos y revisión de los síndromes adrenogenitales (para ser publicado).
15. M. JONES. H., and SCOTT, W. W.: Hermaphroditism, Genital anomalies and related endocrine disorders, Baltimore 1958, Williams and Wilkins Co.,
16. JOSSO, N. DE GROUCHY. J. ANVERT. J. NEZELOF, C, JAYLE, M. F., MONLLEC, J., FREZAL, J, DE CASANBON. A., and LAMY, M.: True hermaphroditism with XX/XY mosaicism, probably due to double fertilization of the ovum, J. Clin. Endocrino. 25:114, 1965.
17. JOST. A. In JONES. H. M., and SCOTT. W. W.: Hermaphroditism, Genital anomalies and related endocrine disorders, Baltimore, 1958, Williams and Wilkins Co.
18. KOONTZ, W. W., and YOUNG, R. B.: True hermaphroditism, a report of three cases, J. Urol. 101: 102, 1969.
19. MEDINA M., J.: Comunicación personal.
20. MILLER. O. J.: The sex chromosome anomalies, Am. J. Obst. Gynecol. 90: 1078, 1964
21. MILNER, W. A. GARLICK, W. B., FINK, A. J., and STEIN, J. J.: The Hermaphrodite Siblings, J. Urol. 79: 1003-1009, 1958.
22. MOORE, J., FRANKLIN, R. R., Wills, S. H., and CLAYTON, G. W. Diagnosis and management of individuals with hermaphroditic external genitalia, Am. J. Obst, Gyne. 83: 1175-83, 1962.
23. MOORE, K. L.: The sex chromatin, Philadelphia, W. B. Saunders. Co., 1966.
24. OVERZIER, C.: Intersexuality, Editado por New York Academic Fress, 1963.
25. OVERZIER, C.: Ein XX/XY – Hermaphrodit mit einem “intratubularen Ei" und einem Gonadoblastom (gonocytom 3), Klin wchschr 42: 1052, 1964. 26 Rosemberg. H. S., Clayton, G. W.
26. ROSEMBERG. H. S. CLAYTON. G. W. and Hso. T. C.: Familial true hermaphroditism, J. Clin. Endocrinol. 23: 203, 1963.
27. SCHWIEBINGER, G. W., and HODGES, C. V.: A true hermaphrodite, J. Urol. 73 424-29, 1955.
28. SEGAL, S. J., and NELSON, W. O.: Developmental aspects of human hermaphrodism: The significance of sex chromatine patterns, J. Clin. Endocrinol. 17: 676-92.1957.
29. TURNER, C. D.: Special mechanisms in anomalies of sex differentiation, Am. J. Obst. Gynec, and Hsu, T. C.: Familial true hermaphroditism, J. Clin. Endocrinol. 23: 203, 1963. 90: 1208, 1964.
30. MEED, J. C., SEGALOFF, A., MIENER, W. B., and DOUGLAS, J. W.: True hermaphroditism, endocrine studies in a case of ovotestis, J. Clin. Endocrinol. 7: 741-748, 1947.
31. WILKINS, IL, GRUMBACH, M. M., VAN WYK, J. J., SHEPARD, T. H., and PAPADA TOS, C.: Hermaphroditism: clasification, diagnosis, selection of sex and treatment Pediatrics 16: 287-302, 1955.
32. FERGUESON-SMITH M. A., "Karyotype-phenotype Correlations in Gonadal Dysgenesis and Their Bearing on the Pathogenesis of J. Med. Genet. 2:142, 1965.
33. ARMENDARIS S. Citogenética Humana. Interamericana 1. edición 1968, pág. 136. 145.
34. HAMETON, J. L. "Sex Chromosomes and Their Anormalities in Man and Mammals" in A. Lima de Faria. Handbook of Molecular Cytology. North-Holand, Amsterdao pag. 751. London. 1969.
35. SINGH, R. P. and CARR, D. H. "Tha anatomy and histology of XO human embryos- and fetuses". Anat. Record 155: 369, 1966.
36. PARK I. J., JONES H. W., Bias W. B. "True Hermaphroditism with 46, XX/46, XY. Chromosome Complement' Report of a case Obst. & Gynec. 36: 377, 1970.
37. ZUELZER. W. W. BEATTIE. K. M., and REISMAN L. E. "Generalized imbalanced mosaicism attributable to dispermy a problable fertilization of a polar body" At of Hum. Genet. 16:38, 1964.
1:1035, 1965.
2. BREWER. J. I., JONES. H. O., and CULVER, H.: True hermaphroditism, JAMA 148: 431-435, 1952.
3. BUNGE. R. G., and BRADBURY, J. T.: Oocytes in Seminiferous Tubules: H. A. case report of bilateral ovotestes, J. Clin. Endocrinol. 19: 1661-1666, 1959,
4. CLAYTON, G. W. SMITH, J. D., and ROSEMBERG, J. S.: Familial true hermaphroditism in pre and post-puberal genetic females. Hormonal and morphologic Studies, J. Clin. Endocrino. 18: 1349, 1968.
5. FEDERMAN, D. C.: Disorders of sexual development, N. Eng. J. Med. 277: 35-360, 1967.
6. FORD, C. E.: Mosaics and chimaeras, Brit. Med. Bull. 25:104, 1969.
7. GARTLER, S. M., WAXMAN, S. H. and GIBLETT, E.: and XX/XY human hermaphrodite resulting fron double fertilization, Proc. Nat. Acad. Scienc. 48: 332, 1962.
8. GRUMBACH, M. M., VAN WYK, J. J., and WILKINS, L.: Chromosonal Sex in gonadal dysgenesis (ovarian agenesis): relationship to male pseudohermaphroditism and theories of human sex differentiation, J. Clin. Endocrinol. 15: 1161, 1955.
9. GRUMBACH, M. M., and BARR, M. L.: Cytologic tests of chromosomal sex in relation to sexual anomalies in man, Rec. Progr., Hormone Res., 14: 335, 1958.
10. GUINET, P., et PUTELAT, R.: Hermaphrodisme Authentique, Rev. Lyon Med. 9: 67-97, 1960.
11. HAMPSON, J. G., MONEY, J., and HAMPSON, J. L.: Hermaphrodism: recomendations concerning case menagement, J. Clin. Endocrinol. 16: 547-556, 1956.
12. HARDEN, D. G., and AMSTRONG. C. N.: The chromosomes of a true hermaphrodite, Brit. M. J. 2: 12-87-1288, 1959.
13. HUGHES, W. H., ERIKSON, C. C., FLEISCHMAN, W., and ETTELDORF, J. N.; True hermaphroditism: report of a case, J. Pediat, 52: 662-669, 1958.
14. JACOME R., A, y GARCIA C., F.: Deficiencia congénita virilizante de la-hidroxilasa: estudio de 3 casos y revisión de los síndromes adrenogenitales (para ser publicado).
15. M. JONES. H., and SCOTT, W. W.: Hermaphroditism, Genital anomalies and related endocrine disorders, Baltimore 1958, Williams and Wilkins Co.,
16. JOSSO, N. DE GROUCHY. J. ANVERT. J. NEZELOF, C, JAYLE, M. F., MONLLEC, J., FREZAL, J, DE CASANBON. A., and LAMY, M.: True hermaphroditism with XX/XY mosaicism, probably due to double fertilization of the ovum, J. Clin. Endocrino. 25:114, 1965.
17. JOST. A. In JONES. H. M., and SCOTT. W. W.: Hermaphroditism, Genital anomalies and related endocrine disorders, Baltimore, 1958, Williams and Wilkins Co.
18. KOONTZ, W. W., and YOUNG, R. B.: True hermaphroditism, a report of three cases, J. Urol. 101: 102, 1969.
19. MEDINA M., J.: Comunicación personal.
20. MILLER. O. J.: The sex chromosome anomalies, Am. J. Obst. Gynecol. 90: 1078, 1964
21. MILNER, W. A. GARLICK, W. B., FINK, A. J., and STEIN, J. J.: The Hermaphrodite Siblings, J. Urol. 79: 1003-1009, 1958.
22. MOORE, J., FRANKLIN, R. R., Wills, S. H., and CLAYTON, G. W. Diagnosis and management of individuals with hermaphroditic external genitalia, Am. J. Obst, Gyne. 83: 1175-83, 1962.
23. MOORE, K. L.: The sex chromatin, Philadelphia, W. B. Saunders. Co., 1966.
24. OVERZIER, C.: Intersexuality, Editado por New York Academic Fress, 1963.
25. OVERZIER, C.: Ein XX/XY – Hermaphrodit mit einem “intratubularen Ei" und einem Gonadoblastom (gonocytom 3), Klin wchschr 42: 1052, 1964. 26 Rosemberg. H. S., Clayton, G. W.
26. ROSEMBERG. H. S. CLAYTON. G. W. and Hso. T. C.: Familial true hermaphroditism, J. Clin. Endocrinol. 23: 203, 1963.
27. SCHWIEBINGER, G. W., and HODGES, C. V.: A true hermaphrodite, J. Urol. 73 424-29, 1955.
28. SEGAL, S. J., and NELSON, W. O.: Developmental aspects of human hermaphrodism: The significance of sex chromatine patterns, J. Clin. Endocrinol. 17: 676-92.1957.
29. TURNER, C. D.: Special mechanisms in anomalies of sex differentiation, Am. J. Obst. Gynec, and Hsu, T. C.: Familial true hermaphroditism, J. Clin. Endocrinol. 23: 203, 1963. 90: 1208, 1964.
30. MEED, J. C., SEGALOFF, A., MIENER, W. B., and DOUGLAS, J. W.: True hermaphroditism, endocrine studies in a case of ovotestis, J. Clin. Endocrinol. 7: 741-748, 1947.
31. WILKINS, IL, GRUMBACH, M. M., VAN WYK, J. J., SHEPARD, T. H., and PAPADA TOS, C.: Hermaphroditism: clasification, diagnosis, selection of sex and treatment Pediatrics 16: 287-302, 1955.
32. FERGUESON-SMITH M. A., "Karyotype-phenotype Correlations in Gonadal Dysgenesis and Their Bearing on the Pathogenesis of J. Med. Genet. 2:142, 1965.
33. ARMENDARIS S. Citogenética Humana. Interamericana 1. edición 1968, pág. 136. 145.
34. HAMETON, J. L. "Sex Chromosomes and Their Anormalities in Man and Mammals" in A. Lima de Faria. Handbook of Molecular Cytology. North-Holand, Amsterdao pag. 751. London. 1969.
35. SINGH, R. P. and CARR, D. H. "Tha anatomy and histology of XO human embryos- and fetuses". Anat. Record 155: 369, 1966.
36. PARK I. J., JONES H. W., Bias W. B. "True Hermaphroditism with 46, XX/46, XY. Chromosome Complement' Report of a case Obst. & Gynec. 36: 377, 1970.
37. ZUELZER. W. W. BEATTIE. K. M., and REISMAN L. E. "Generalized imbalanced mosaicism attributable to dispermy a problable fertilization of a polar body" At of Hum. Genet. 16:38, 1964.
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