[Male pseudohermaphroditism]. 1997

K Fukutani
Department of Urology, Asoka Hospital.

Male pseudohermaphroditism is a condition of sex differentiation disorders in which the gonads are tests, but the genital ducts and/or external genitalia are incompletely masculinized. This syndrome is caused by a failure of the sequential process in embryonal development of the testis. In the presence of functioning testis the Müllerian ducts regress, while the mesonephric ducts and urogenital sinus differentiate into the internal and external male genitalia. Male pseudohermaphroditism is classified to subtypes according to etiological factors: (1) testicular unresponsiveness to hCG and LH; (2) defect in testosterone biosynthesis; (3) end-organ resistance to androgen; (4) defects in the intracellular metabolism of testosterone; (5) aberrations in testicular organogenesis; (6) defects in anti-Müllerian hormone.

UI MeSH Term Description Entries
D007985 Leydig Cells Steroid-producing cells in the interstitial tissue of the TESTIS. They are under the regulation of PITUITARY HORMONES; LUTEINIZING HORMONE; or interstitial cell-stimulating hormone. TESTOSTERONE is the major androgen (ANDROGENS) produced. Interstitial Cells, Testicular,Leydig Cell,Testicular Interstitial Cell,Testicular Interstitial Cells,Cell, Leydig,Cell, Testicular Interstitial,Cells, Leydig,Cells, Testicular Interstitial,Interstitial Cell, Testicular
D008297 Male Males
D006059 Gonadal Dysgenesis A number of syndromes with defective gonadal developments such as streak GONADS and dysgenetic testes or ovaries. The spectrum of gonadal and sexual abnormalities is reflected in their varied sex chromosome (SEX CHROMOSOMES) constitution as shown by the karyotypes of 45,X monosomy (TURNER SYNDROME); 46,XX (GONADAL DYSGENESIS, 46XX); 46,XY (GONADAL DYSGENESIS, 46,XY); and sex chromosome MOSAICISM; (GONADAL DYSGENESIS, MIXED). Their phenotypes range from female, through ambiguous, to male. This concept includes gonadal agenesis. Gonadal Agenesis,Dysgenesis, Gonadal
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
D013196 Dihydrotestosterone A potent androgenic metabolite of TESTOSTERONE. It is produced by the action of the enzyme 3-OXO-5-ALPHA-STEROID 4-DEHYDROGENASE. 5 alpha-Dihydrotestosterone,Androstanolone,Stanolone,17 beta-Hydroxy-5 beta-Androstan-3-One,17beta-Hydroxy-5alpha-Androstan-3-One,5 beta-Dihydrotestosterone,5-alpha Dihydrotestosterone,5-alpha-DHT,Anaprotin,Andractim,Dihydroepitestosterone,Gelovit,17 beta Hydroxy 5 beta Androstan 3 One,17beta Hydroxy 5alpha Androstan 3 One,5 alpha DHT,5 alpha Dihydrotestosterone,5 beta Dihydrotestosterone,Dihydrotestosterone, 5-alpha,beta-Hydroxy-5 beta-Androstan-3-One, 17
D013739 Testosterone A potent androgenic steroid and major product secreted by the LEYDIG CELLS of the TESTIS. Its production is stimulated by LUTEINIZING HORMONE from the PITUITARY GLAND. In turn, testosterone exerts feedback control of the pituitary LH and FSH secretion. Depending on the tissues, testosterone can be further converted to DIHYDROTESTOSTERONE or ESTRADIOL. 17-beta-Hydroxy-4-Androsten-3-one,17-beta-Hydroxy-8 alpha-4-Androsten-3-one,8-Isotestosterone,AndroGel,Androderm,Andropatch,Androtop,Histerone,Sterotate,Sustanon,Testim,Testoderm,Testolin,Testopel,Testosterone Sulfate,17 beta Hydroxy 4 Androsten 3 one,17 beta Hydroxy 8 alpha 4 Androsten 3 one,8 Isotestosterone

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