This page is being served from the Urological Sciences Research Foundation web repository, and was originally posted between 1996-2008. In January 2009 USRF’s founder, Dr. Leonard S. Marks and his staff joined UCLA’s Department of Urology where they are continuing their research. Click for more information.
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In an isolated village of the southwestern Dominican Republic, 2% of the live births were in the 1970's, guevedoces (actually male pseudohermaphrodites). These children appeared to be girls at birth, but at puberty these 'girls' sprout muscles, testes, and a penis. For the rest of their lives they are men in nearly all respects (see photograph 6 below). Their underlying pathology was found to be a deficiency of the enzyme, 5-alpha Reductase.
The story of the guevedoces was originally presented at a Federation meeting in Atlantic City, NJ in 1973 and published in Science in 1974 (Imperato-McGinley J, Guerrero L, Gautier T, Peterson RE. Steroid 5alpha-reductase deficiency in man: an inherited form of male pseudohermaphroditism. Science 1974 Dec 27; 186 (4170): 1213-5). Specific inhibition of 5-alpha Reductase is the mechanism of action of the prostate drug, finasteride (Proscar), which in adults, shrinks the prostate without affecting the male phenotype. |