[Complete testicular feminization syndrome. Report of a case]. 1993

F Ramirez, and S Salazar, and B Madurga, and R Ibañez, and J M Baez Perea, and J Flores Gines
Hospital Universitario Puerta del Mar, Cádiz, España.

A case of complete testicular feminization (Morris syndrome) in a male pseudohermaphrodite is described. The results of the hormone analyses and the histological features of the resected gonad are presented. The literature is reviewed with special reference to whether gonadectomy should be performed in the pre or post-pubertal period. We advocate early castration because of the high incidence of germ cell neoplasia and the possibility of virilization in case of incomplete androgenic resistance. Our approach is based on the satisfactory feminization achieved by exogenous estrogen therapy.

UI MeSH Term Description Entries
D008297 Male Males
D009919 Orchiectomy The surgical removal of one or both testicles. Castration, Male,Orchidectomy,Castrations, Male,Male Castration,Male Castrations,Orchidectomies,Orchiectomies
D003131 Combined Modality Therapy The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used. Multimodal Treatment,Therapy, Combined Modality,Combined Modality Therapies,Modality Therapies, Combined,Modality Therapy, Combined,Multimodal Treatments,Therapies, Combined Modality,Treatment, Multimodal,Treatments, Multimodal
D004967 Estrogens Compounds that interact with ESTROGEN RECEPTORS in target tissues to bring about the effects similar to those of ESTRADIOL. Estrogens stimulate the female reproductive organs, and the development of secondary female SEX CHARACTERISTICS. Estrogenic chemicals include natural, synthetic, steroidal, or non-steroidal compounds. Estrogen,Estrogen Effect,Estrogen Effects,Estrogen Receptor Agonists,Estrogenic Agents,Estrogenic Compounds,Estrogenic Effect,Estrogenic Effects,Agents, Estrogenic,Agonists, Estrogen Receptor,Compounds, Estrogenic,Effects, Estrogen,Effects, Estrogenic,Receptor Agonists, Estrogen
D006065 Gonadotropins, Pituitary Hormones secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR) that stimulate gonadal functions in both males and females. They include FOLLICLE STIMULATING HORMONE that stimulates germ cell maturation (OOGENESIS; SPERMATOGENESIS), and LUTEINIZING HORMONE that stimulates the production of sex steroids (ESTROGENS; PROGESTERONE; ANDROGENS). Pituitary Gonadotropins
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012739 Gonadal Steroid Hormones Steroid hormones produced by the GONADS. They stimulate reproductive organs, germ cell maturation, and the secondary sex characteristics in the males and the females. The major sex steroid hormones include ESTRADIOL; PROGESTERONE; and TESTOSTERONE. Gonadal Steroid Hormone,Sex Hormone,Sex Steroid Hormone,Sex Steroid Hormones,Sex Hormones,Hormone, Gonadal Steroid,Hormone, Sex,Hormone, Sex Steroid,Hormones, Gonadal Steroid,Hormones, Sex Steroid,Steroid Hormone, Gonadal,Steroid Hormone, Sex,Steroid Hormones, Gonadal,Steroid Hormones, Sex
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D013734 Androgen-Insensitivity Syndrome A disorder of sexual development transmitted as an X-linked recessive trait. These patients have a karyotype of 46,XY with end-organ resistance to androgen due to mutations in the androgen receptor (RECEPTORS, ANDROGEN) gene. Severity of the defect in receptor quantity or quality correlates with their phenotypes. In these genetic males, the phenotypic spectrum ranges from those with normal female external genitalia, through those with genital ambiguity as in Reifenstein Syndrome, to that of a normal male with INFERTILITY. Testicular Feminization,AR Deficiency,Androgen Insensitivity Syndrome,Androgen Insensitivity, Partial,Androgen Receptor Deficiency,Androgen Resistance Syndrome,Androgen-Insensitivity Syndrome, Complete,Androgen-Insensitivity Syndrome, Partial,DHTR Deficiency,Dihydrotestosterone Receptor Deficiency,Male Pseudohermaphroditism Due to Androgen Insensitivity,Reifenstein Syndrome,Reifenstein's Syndrome,Testicular Feminization Syndrome,AR Deficiencies,Androgen Insensitivities, Partial,Androgen Insensitivity Syndrome, Complete,Androgen Insensitivity Syndrome, Partial,Androgen Insensitivity Syndromes,Androgen Receptor Deficiencies,Androgen Resistance Syndromes,Androgen-Insensitivity Syndromes,Androgen-Insensitivity Syndromes, Complete,Androgen-Insensitivity Syndromes, Partial,Complete Androgen-Insensitivity Syndrome,Complete Androgen-Insensitivity Syndromes,DHTR Deficiencies,Deficiencies, AR,Deficiencies, Androgen Receptor,Deficiencies, DHTR,Deficiencies, Dihydrotestosterone Receptor,Deficiency, AR,Deficiency, Androgen Receptor,Deficiency, DHTR,Deficiency, Dihydrotestosterone Receptor,Dihydrotestosterone Receptor Deficiencies,Feminization Syndrome, Testicular,Feminization Syndromes, Testicular,Feminization, Testicular,Feminizations, Testicular,Insensitivities, Partial Androgen,Insensitivity Syndrome, Androgen,Insensitivity Syndromes, Androgen,Insensitivity, Partial Androgen,Partial Androgen Insensitivities,Partial Androgen Insensitivity,Partial Androgen-Insensitivity Syndrome,Partial Androgen-Insensitivity Syndromes,Receptor Deficiencies, Androgen,Receptor Deficiencies, Dihydrotestosterone,Receptor Deficiency, Androgen,Receptor Deficiency, Dihydrotestosterone,Reifensteins Syndrome,Resistance Syndrome, Androgen,Resistance Syndromes, Androgen,Testicular Feminization Syndromes,Testicular Feminizations

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