Güevedoce
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In the
Dominican Republic The Dominican Republic ( ; es, República Dominicana, ) is a country located on the island of Hispaniola in the Greater Antilles archipelago of the Caribbean region. It occupies the eastern five-eighths of the island, which it shares wit ...
, güevedoces (from es, güevedoce, from
Dominican Spanish Dominican Spanish () is Spanish language, Spanish as spoken in the Dominican Republic; and also among the Dominican diaspora, most of whom live in the United States, chiefly in New York City, New Jersey, Connecticut, Rhode Island, Massachusetts ...
es, güevos a los doce, label=none "testicles at twelve") are children with a specific
intersex Intersex people are individuals born with any of several sex characteristics including chromosome patterns, gonads, or genitals that, according to the Office of the United Nations High Commissioner for Human Rights, "do not fit typical bina ...
variation. Güevedoces are classified as girls when they are born but, around the age of 12, they start developing male genitalia. This is due to a deficiency in the production of 5α-reductase, an
enzyme Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...
involved in the
metabolism Metabolism (, from el, μεταβολή ''metabolē'', "change") is the set of life-sustaining chemical reactions in organisms. The three main functions of metabolism are: the conversion of the energy in food to energy available to run cell ...
of
testosterone Testosterone is the primary sex hormone and anabolic steroid in males. In humans, testosterone plays a key role in the development of Male reproductive system, male reproductive tissues such as testes and prostate, as well as promoting secondar ...
to
dihydrotestosterone Dihydrotestosterone (DHT, 5α-dihydrotestosterone, 5α-DHT, androstanolone or stanolone) is an endogenous androgen sex steroid and hormone. The enzyme 5α-reductase catalyzes the formation of DHT from testosterone in certain tissues includi ...
. The phenomenon also reportedly occurs in
Turkey Turkey ( tr, Türkiye ), officially the Republic of Türkiye ( tr, Türkiye Cumhuriyeti, links=no ), is a list of transcontinental countries, transcontinental country located mainly on the Anatolia, Anatolian Peninsula in Western Asia, with ...
and in
Papua New Guinea Papua New Guinea (abbreviated PNG; , ; tpi, Papua Niugini; ho, Papua Niu Gini), officially the Independent State of Papua New Guinea ( tpi, Independen Stet bilong Papua Niugini; ho, Independen Stet bilong Papua Niu Gini), is a country i ...
, where it is called ''kwolu-aatmwol'' (literally 'a female thing changing into a male thing') by the
Sambia people The Simbari people (also known as the Simbari Anga, called Sambia by Herdt) are a tribe of mountain-dwelling, hunting and horticultural people who inhabit the fringes of the Eastern Highlands Province of Papua New Guinea, and are extensively descr ...
.


Early research

The first scientific investigations of güevedoces occurred in the 1970s, when Julianne Imperato-McGinley, an endocrinologist from
Cornell University Cornell University is a private statutory land-grant research university based in Ithaca, New York. It is a member of the Ivy League. Founded in 1865 by Ezra Cornell and Andrew Dickson White, Cornell was founded with the intention to teach an ...
, traveled to the village of Las Salinas in the Dominican Republic to investigate reports of apparently
female Female (Venus symbol, symbol: ♀) is the sex of an organism that produces the large non-motile ovum, ova (egg cells), the type of gamete (sex cell) that fuses with the Sperm, male gamete during sexual reproduction. A female has larger gamet ...
children becoming
male Male (symbol: ♂) is the sex of an organism that produces the gamete (sex cell) known as sperm, which fuses with the larger female gamete, or ovum, in the process of fertilization. A male organism cannot reproduce sexually without access to ...
children at the onset of
puberty Puberty is the process of physical changes through which a child's body matures into an adult body capable of sexual reproduction. It is initiated by hormonal signals from the brain to the gonads: the ovaries in a girl, the testes in a boy. ...
. The cause was determined to be
5α-Reductase deficiency 5α-Reductases, also known as 3-oxo-5α-steroid 4-dehydrogenases, are enzymes involved in steroid metabolism. They participate in three metabolic pathways: bile acid biosynthesis, androgen and estrogen metabolism. There are three isozymes of ...
, and the results were published in the journal ''
Science Science is a systematic endeavor that builds and organizes knowledge in the form of testable explanations and predictions about the universe. Science may be as old as the human species, and some of the earliest archeological evidence for ...
'' in 1974. The frequency of the deficiency was found to be unusually high in Las Salinas, with occurrence ratio of 1 güevedoce to every 90 unaffected males. The transformation of a
phenotypically In genetics, the phenotype () is the set of observable characteristics or traits of an organism. The term covers the organism's morphology or physical form and structure, its developmental processes, its biochemical and physiological proper ...
female child into a phenotypically male adult at puberty, which is reported to be celebrated in Dominican culture, is the result of a
genotypic The genotype of an organism is its complete set of genetic material. Genotype can also be used to refer to the alleles or variants an individual carries in a particular gene or genetic location. The number of alleles an individual can have in a ...
male (with XY chromosomes) born with a deficiency in the
enzyme Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...
5α-reductase 5α-Reductases, also known as 3-oxo-5α-steroid 4-dehydrogenases, are enzymes involved in steroid metabolism. They participate in three metabolic pathways: bile acid biosynthesis, androgen and estrogen metabolism. There are three isozymes of ...
. 5α-Reductase is responsible for the reduction of testosterone to dihydrotestosterone. Dihydrotestosterone (DHT) is the
hormone A hormone (from the Greek participle , "setting in motion") is a class of signaling molecules in multicellular organisms that are sent to distant organs by complex biological processes to regulate physiology and behavior. Hormones are required ...
responsible for the majority of
embryonic development An embryo is an initial stage of development of a multicellular organism. In organisms that reproduce sexually, embryonic development is the part of the life cycle that begins just after fertilization of the female egg cell by the male sperm ...
of primary male sexual characteristics such as genital size. However, the
oxidized Redox (reduction–oxidation, , ) is a type of chemical reaction in which the oxidation states of substrate change. Oxidation is the loss of electrons or an increase in the oxidation state, while reduction is the gain of electrons or a d ...
form of the hormone, testosterone, promotes secondary sex characteristics. Thus, an XY child without functioning 5α-reductase cannot convert testosterone into the form that develops
primary sex characteristics A sex organ (or reproductive organ) is any part of an animal or plant that is involved in sexual reproduction. The reproductive organs together constitute the reproductive system. In animals, the testis in the male, and the ovary in the female, ...
and will have the appearance of
female genitalia A sex organ (or reproductive organ) is any part of an animal or plant that is involved in sexual reproduction. The reproductive organs together constitute the reproductive system. In animals, the testis in the male, and the ovary in the female, a ...
. At puberty, when large amounts of testosterone are produced, secondary sex characteristics (e.g., deepening voice, hair pattern changes, muscle
anabolism Anabolism () is the set of metabolic pathways that construct molecules from smaller units. These reactions require energy, known also as an endergonic process. Anabolism is the building-up aspect of metabolism, whereas catabolism is the breaking-do ...
, etc.) develop, thus producing a phenotypic male.


Status in society

In countries like the
United States The United States of America (U.S.A. or USA), commonly known as the United States (U.S. or US) or America, is a country primarily located in North America. It consists of 50 states, a federal district, five major unincorporated territorie ...
, intersex children are often operated on immediately after birth to make their genitals appear either typically male or female. In the Dominican Republic guevedoces are regarded as a third gender, experiencing ambivalent gender socialisation. In adulthood, güevedoces most commonly
self-identify Personal identity is the unique numerical identity of a person over time. Discussions regarding personal identity typically aim to determine the necessary and sufficient conditions under which a person at one time and a person at another time can ...
as men, but are not necessarily completely treated as such by society.
Anne Fausto-Sterling Anne Fausto-Sterling ( Sterling; born July 30, 1944) is an American sexologist who has written extensively on the biology of gender, sexual identity, gender identity, gender roles, and intersexuality. She is the Nancy Duke Lewis Professor Emer ...
states that güevedoces (as well as people in Papua New Guinea with 5α-reductase deficiency) "are recognised as a
third gender Third gender is a concept in which individuals are categorized, either by themselves or by society, as neither man nor woman. It is also a social category present in societies that recognize three or more genders. The term ''third'' is usually ...
" by their cultures, while the cultures "nevertheless recognize only two gender roles".


See also

* Gender system *
5α-Reductase deficiency 5α-Reductases, also known as 3-oxo-5α-steroid 4-dehydrogenases, are enzymes involved in steroid metabolism. They participate in three metabolic pathways: bile acid biosynthesis, androgen and estrogen metabolism. There are three isozymes of ...


References


External links

{{Male congenital malformations of genital organs, indeterminate sex and pseudohermaphroditism Intersex in society Gender systems