5-alpha-reductase deficiency epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Epidemiology and Demographics

The number of people with this condition varies geographically, depending on how much of a given population is interrelated. In 1974, Jullianne Imperato-McGinley has estimated an incidence of 1:90 males in the Dominican Republic. This can be seen in that certain regions have evolved terminology for the condition:

Local names

The term Guevedoche or Guevedoces is Spanish slang for the condition. It originated in the Dominican Republic where more than three dozen cases have occurred in the small village of Salinas, all descended from a single individual named Altagracia Carrasco. It stands for the vulgar expression huevo a los doce, which translates literally as "balls at twelve". It is also known locally as 'Machihembras' ('first women, then man').

A similar cluster of cases among the Simbari of the Eastern Highlands of Papua New Guinea has the local name 'Kwolu-aatmwol' ('female thing transforming into male thing').