TESTICULAR DYSGENESIS AND MENTAL-RETARDATION IN 2 SIBLINGS WITH MALE PSEUDOHERMAPHRODITISM

Authors
VARDAR MA ZEREN H DEMIR C EVRUKE C OZPOYRAZ N KADAYIFCI O ALTINTAS A ZORLUDEMIR S
Citation
Ma. Vardar et al., TESTICULAR DYSGENESIS AND MENTAL-RETARDATION IN 2 SIBLINGS WITH MALE PSEUDOHERMAPHRODITISM, Adolescent and pediatric gynecology, 8(3), 1995, pp. 132-134
Citations number
NO
Categorie Soggetti
Obsetric & Gynecology",Pediatrics
Journal title
Adolescent and pediatric gynecology → ACNP
ISSN journal
09328610
Volume
8
Issue
3
Year of publication
1995
Pages
132 - 134
Database
ISI
SICI code
0932-8610(1995)8:3<132:TDAMI2>2.0.ZU;2-7
Abstract
Two mentally disabled phenotypic females, 46, XY dysgonadal sisters fr om a consanguineous marriage are described. Low plasma testosterone le vels and an inadequate response of plasma-testosterone to human chorio nic gonadotrophin (hCG) stimulation were noted in both cases. Dysgenet ic testes and remnants of the mullerian and wolffian ducts were identi fied at exploratory laparotomy. Mild mental retardation was also prese nt in both cases, These data may suggest the specific role of genetic factors in the etiology of testicular dysgenesis.