Should CAH in Females Be Classified as DSD?

  title={Should CAH in Females Be Classified as DSD?},
  author={Ricardo Gonz{\'a}lez and Barbara M Ludwikowski},
  journal={Frontiers in Pediatrics},
Great controversies and misunderstandings have developed around the relatively recently coined term disorders of sex development (DSD). In this article, we question the wisdom of including XX individuals with congenital adrenal hyperplasia (CAH) in the DSD category and develop arguments against it based on the published literature on the subject. It is clear that females with CAH assigned the female gender before 24 months of age and properly managed retain the female gender identity regardless… 
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Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries and were largely facilitated by the confrontational activities of a number of support groups.
Disorders or Differences of Sex Development? Views of Affected Individuals on DSD Terminology
The data do not support the view that, in general, the term Disorders of Sex Development is insensitive to concerns of affected persons and that it should therefore be abandoned, and it is recommended that clinicians evaluate each patient’s preferences.
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Surgical, Patient, and Parental Considerations in the Management of Children with Differences of Sex Development
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Long-term outcomes of the surgical treatment of girls with a classical form of congenital adrenal hyperplasia
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Restoring normal anatomy in female patients with atypical genitalia.
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There remains a need for continued monitoring of patients as they go through puberty with the possibility of additional surgery for vaginal stenosis, and Anatomically based surgery and refinement in surgical techniques with acceptance of moderate degrees of clitoral hypertrophy as normal should improve long-term outcomes.


Gender Dysphoria in 46,XX Persons with Adrenogenital Syndrome Raised as Females: An Addendum
This review found out how many cases of XX individuals with CAH who actually decided to change their gender role after puberty have been reported, since this information is important for clinicians to advise parents in the neonatal period.
Long-term management of patients with disorders of sex development (DSD).
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The ultimate challenge of cloacal exstrophy.
Sexual Orientation in Women with Classical or Non-classical Congenital Adrenal Hyperplasia as a Function of Degree of Prenatal Androgen Excess
The findings support a sexual-differentiation perspective involving prenatal androgens on the development of sexual orientation and are linked to the first study to relate sexual orientation to the specific molecular genotypes of CAH.
Congenital adrenal hyperplasia patient perception of ‘disorders of sex development’ nomenclature
The results indicate that the majority of parents and patients with CAH are dissatisfied with the term DSD, and reconsideration of the current nomenclature and ongoing dialogue between the medical community and patients will eventually lead to removal of stigmatization, better management protocols, and improved outcomes.
Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia
It is concluded that the assignment to the female gender as a general policy for 46, XX patients with CAH appears justified, even in severely masculinized 46,XX newborns withCAH (Prader stage IV or V).
Misrepresentation of Evidence Favoring Early Normalizing Surgery for Atypical Sex Anatomies: Response to Baratz and Feder (2015)
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Psychosexual development of children and adolescents with hypospadias.
Boys with corrected hypospadias may show a psychosexual development that is similar to healthy children, and Puberty could be a critical time for the patients, however, during which they might require regular urological follow-ups and may benefit from age-appropriate information about their penile condition.