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Severe form of 3βHSD2D is associated with varying manifestations of incomplete masculinization including severe hypospadia, micropenis, bifid scrotum, and undescended testis [16, 20, 29]. In case of SW phenotype, 3βHSD2D is usually diagnosed within the first few weeks of life. In case of non-SW phenotype, patients may be diagnosed at any time before puberty [37]. However, the diagnosis has rarely been further delayed and patients can present with gender role related concerns during adulthood [38]. Overall, the patients tend to be diagnosed at a younger age in 46,XY children due to a higher rate of genital ambiguity compared to females [34, 39]. Also, there seems to be an underrepresentation of 46,XX patients, which might be explained by lack of diagnosis in milder form of 3βHSD2D in females. Also, females with severe form may die undiagnosed in a neonatal adrenal crisis more often than males [15]. 50 Rodadas dinamarca 2 divisao este Sem Depósito. Pubertal status.
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