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[Male pseudohermaphroditism. Testicular feminization syndrome]. 1992

W A Liu
Second Affiliated Hospital, Fujian Medical College.

Male pseudohermaphroditism--testicular feminization syndrome is a rare genetic entity with considerably familial predisposition. Of the 9 cases, 7 except 2 children were confirmed surgically. Clinical manifestations of the disorder were primary amenorrhea, infertility and a mass in groin. We suggested that orchiectomy is necessary after puberty. The genetic pattern, clinical characteristics, diagnosis and treatment of the condition were briefly discussed with a review of the literature.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D009919 Orchiectomy The surgical removal of one or both testicles. Castration, Male,Orchidectomy,Castrations, Male,Male Castration,Male Castrations,Orchidectomies,Orchiectomies
D011627 Puberty A period in the human life in which the development of the hypothalamic-pituitary-gonadal system takes place and reaches full maturity. The onset of synchronized endocrine events in puberty lead to the capacity for reproduction (FERTILITY), development of secondary SEX CHARACTERISTICS, and other changes seen in ADOLESCENT DEVELOPMENT. Puberties
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012734 Disorders of Sex Development In gonochoristic organisms, congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical. Effects from exposure to abnormal levels of GONADAL HORMONES in the maternal environment, or disruption of the function of those hormones by ENDOCRINE DISRUPTORS are included. Disorders of Sexual Development,Sex Development Disorders,Sex Differentiation Disorders,Ambiguous Genitalia,Genital Ambiguity,Hermaphroditism,Intersex Conditions,Intersexuality,Pseudohermaphroditism,Sexual Development Disorders,Sexual Differentiation Disorders,Ambiguities, Genital,Ambiguity, Genital,Condition, Intersex,Conditions, Intersex,Differentiation Disorder, Sex,Differentiation Disorder, Sexual,Differentiation Disorders, Sex,Differentiation Disorders, Sexual,Disorder, Sex Differentiation,Disorder, Sexual Differentiation,Disorders, Sex Differentiation,Disorders, Sexual Differentiation,Genital Ambiguities,Genitalia, Ambiguous,Intersex Condition,Intersexualities,Sex Development Disorder,Sex Differentiation Disorder,Sexual Development Disorder,Sexual Differentiation Disorder
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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