Document Detail

Phenotypical expression in XX males correlates with testicular response to exogenous choriogonadotropin in early infancy: does a variable degree of testicular failure determine the degree of genital ambiguity?
MedLine Citation:
PMID:  8827344     Owner:  NLM     Status:  MEDLINE    
The 46,XX male syndrome is characterized by the presence of testicular development in subjects who lack a Y chromosome. The majority of patients have male external genitalia without ambiguity; however, 10-15% show diverse degrees of hypospadias. Testicular function is normal at birth but deteriorates thereafter. However, it has not been clarified why some cases exhibit genital ambiguity. This study examined 10 affected patients, including 4 prepubertal (< 1 year old) with hypospadias (1 glandular, 1 penile, and 2 penoscrotal). In all subjects, testicular function was evaluated by performing a stimulation with choriogonadotropin. In the postpubertal individuals, basal and poststimuli testosterone were below the reference values. Prepubertal patients had age-appropriate basal test-osterone concentrations. All responded to the choriogonadotropin challenge; however, the most significant response was observed in the patient with the glandular hypospadias, the second highest response was presented by the patient with the penile hypospadias, while both patients with the penoscrotal hypospadias had the poorest responses. These results suggest that the degree of genital ambiguity is correlated with the impairment in testosterone response to choriogonadotropin in early infancy, indicating a defect in testosterone production in XX males with genital ambiguity.
J P Méndez; A Ulloa-Aguirre; S Kofman-Alfaro; P Canto; E Reyes; L Díaz-Cueto; G Pérez-Palacios
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of andrology     Volume:  37     ISSN:  0148-5016     ISO Abbreviation:  Arch. Androl.     Publication Date:    1996 Jul-Aug
Date Detail:
Created Date:  1996-12-03     Completed Date:  1996-12-03     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7806755     Medline TA:  Arch Androl     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  19-26     Citation Subset:  IM    
Unidad de Investigación Médica en Biología del Desarrollo, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, IMSS, México DF, México.
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MeSH Terms
Child, Preschool
Chorionic Gonadotropin / pharmacology*
Chromosome Aberrations*
Chromosome Disorders*
Estradiol / metabolism
Follicle Stimulating Hormone / metabolism
Luteinizing Hormone / metabolism
Sex Chromosomes*
Testis / drug effects*,  metabolism
Testosterone / metabolism
Reg. No./Substance:
0/Chorionic Gonadotropin; 50-28-2/Estradiol; 58-22-0/Testosterone; 9002-67-9/Luteinizing Hormone; 9002-68-0/Follicle Stimulating Hormone

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