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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.