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Table 1 DSD examples and sex assignment considerations.

From: Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team

Condition Sex assignment Evidence Grade
CAH Usually female Berenbaum and Bailey [92] Strong recommendation with very low quality Evidence for female sex assignment.
CAIS Female Wisniewski et al. [93] Strong recommendation with very low quality evidence for female sex assignment.
PAIS Depends on the phenotype Köhler et al. [94] Consensus statement to base sex assignment on the phenotype.
Gonadal dysgenesis Usually female Sarafoglou and Ostrer [95] Consensus statement for female sex assignment in most instances.
Hypospadias Usually male Boisen et al. [96] Weak recommendation with low quality evidence for male sex assignment.
Hypopituitarism/ Hypogonadotropic hypogonadism Male Grumbach [14] Consensus statement for male sex assignment, and to evaluate babies within the first 6 months and treat with testosterone.
Ovotesticular syndrome Depends on the continent Hadjiathanasiou et al. [97] Strong recommendation with very low quality evidence. assign gender on an individual basis.
5-Reductase deficiency Depends on the continent Imperato-McGinley et al. [21] Strong recommendation with very low quality evidence. assign gender on an individual basis with appropriate consideration of the patient's degree of masculinization.