Reproductive system

• XX ovotesticular DSD
  7 October 2010

  XX OT-DSD; XX true hermaphrodism; XX ovotesticular disorder of sex development: XX true hermaphroditism

• 46,XX male
  7 October 2010

  XX maleness, XX males

• Wolffian duct remnant
  10 September 2010

  Wolffian duct remnants, mesonephric duct remants

• 17,20-desmolase deficiency
  22 July 2010

  Synopsis
  ambiguous genitalia
  in utero virilization of female fetus
  fetal and maternal virilization associated with pregnancy
  normal karyotype / normal female genotype
  extremely high levels of pregnenolone, pregnenolone sulfate, progesterone d in association with low plasma levels of delta 4-androstenedione, testosterone, dehydroepiandrosterone and its sulfate
  cortisol 17OH-progesterone and ACTH levels and plasma renin activity normal.
  all the progestagens rise further after (...)

• neonatal bilateral ovarian sex cord stromal tumors
  22 July 2010

  Synopsis
  macrosomy
  clitoromegaly
  labial hypertrophy
  ovarian cysts
  bilateral ovarian enlargement fibrothecoma juvenile granulosa cell tumor (JGCT)
  References
  Neonatal bilateral ovarian sex cord stromal tumors. Capito C, Flechtner I, Thibaud E, Emond S, Kalfa N, Jaubert F, Sarnacki S. Pediatr Blood Cancer. 2009 Mar;52(3):401-3. PMID: #19058207#

• maternal ingestion of progestins or androgens
  20 May 2010

  Maternal ingestion of synthetic progestins was implicated as a cause of female pseudohermaphroditism in the late 1950s when such treatment was employed for threatened or habitual abortion.
  Subsequently, progestins have also been implicated in the development of hypospadias in male offspring.
  Most cases of female pseudohermaphroditism in this category developed after maternal ingestion of Norlutin (17α-ethinyl-19-nortestosterone), less often with ethisterone (17α-ethinyltestosterone), and (...)

• placental aromatase deficiency
  19 May 2010

  placental aromatase defect

• gonadotropin-independent precocious puberty
  21 April 2010

  A group of children exists in whom premature sexual maturation occurs in the absence of pubertal levels of gonadotropins; that is, they have gonadotropin-independent precocious puberty.
  Children with gonadotropin-independent precocity demonstrate an absence of gonadotropin pulsations, variable responses to luteinizing hormone-releasing hormone, lack of suppression of puberty in response to LHRHa, and cyclic steroidogenesis.
  Tissue from testicular biopsies performed in boys with (...)

• NR5A1-associated DSD
  20 April 2010

  SF1-associated intersexuality; SF-1-associated intersexuality, NR5A1-associated intersexuality; NR5A1-associated disorder of sexual development

• NROB1 duplication-associated DSD
  20 April 2010

  DAX1-associated intersexuality; DAX1 duplication-associated DSD

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