Guevedoces

Guevedoces

Guevedoces is a term used in the Dominican Republic to describe individuals who are born with a condition known as 5-alpha-reductase deficiency. This condition is a form of intersex variation where individuals are genetically male (46,XY) but are born with ambiguous genitalia or female-appearing genitalia. The term "guevedoces" translates to "penis at twelve," reflecting the common occurrence of these individuals developing male secondary sexual characteristics at puberty.

Genetics and Pathophysiology

Guevedoces is caused by a deficiency in the enzyme 5-alpha-reductase type 2, which is responsible for converting testosterone into dihydrotestosterone (DHT). DHT is a potent androgen that plays a crucial role in the masculinization of the external genitalia during fetal development. In individuals with 5-alpha-reductase deficiency, the lack of DHT results in underdeveloped male genitalia at birth.

During puberty, the surge in testosterone levels can lead to the development of male secondary sexual characteristics, such as increased muscle mass, deepening of the voice, and growth of facial and body hair. The external genitalia may also masculinize, leading to the development of a penis and scrotum.

Clinical Presentation

At birth, individuals with guevedoces may be assigned female due to the appearance of their external genitalia. However, as they reach puberty, the changes in their bodies often lead to a re-evaluation of their gender identity. Some individuals may choose to live as males, while others may continue to identify as female or adopt a non-binary identity.

Cultural and Social Aspects

In the Dominican Republic, the condition is relatively well-known, particularly in certain rural communities where it is more prevalent. The cultural understanding of guevedoces allows for a degree of acceptance and flexibility in gender roles that may not be present in other societies.

Diagnosis and Management

Diagnosis of 5-alpha-reductase deficiency can be confirmed through genetic testing and hormone analysis. Management of the condition is individualized and may include hormone therapy or surgical interventions, depending on the individual's gender identity and personal preferences.

Also see

• Intersex
• Androgen Insensitivity Syndrome
• Gender Identity
• Hormone Replacement Therapy