Except for sterility this change results in few or no

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Unformatted text preview: birth an unambiguous assignment of male or female is not possible. Androgyny is the union of two sexes in one individual. Androgynous is the adjective form. 175 176 Glossary androstenedione A steroid hormone from which the body derives both male and female sex hormones. clitorectomy A surgical procedure in which the clitoris is removed. clitoroplasty Surgical alteration of the clitoris, often to reduce a large clitoris to a more standard size. cloacal exstrophy A birth defect that results in malformation of the pelvic region and associated internal organs. The cloaca is a common passageway for feces, urine, and reproduction found in some adult animals such as birds. But during human development, the cloaca forms, then usually disappears later as the individual parts separate to form a rectum, a bladder, and genitalia. In exstrophy of the cloaca, the cloaca never fully separates into the individual parts, so the lower region of the abdomen doesn’t fuse, and what would normally become the bladder, etc., is open to the outside. Cloacal exstrophy affects boys and girls but is most severe in boys, because boys with cloacal exstrophy have cryptorchidism (failure of the testes to descend into the scrotum) and very severe epispadias (a malformation of the penis in which the urethral opening is above the phallus). congenital adrenal hyperplasia This disorder is also known as adrenogenital syndrome or 21-hydroxylase deficiency. Congenital adrenal hyperplasia covers a range of inher- Glossary 177 ited disorders of the adrenal gland. It can affect either boys or girls. People with congenital adrenal hyperplasia partially or completely lack an enzyme that aids in production of cortisol and aldosterone. Under these conditions the adrenal glands produce much higher levels of androgens, which causes male characteristics to appear early (or inappropriately). dihydrotestosterone A steroid hormone derived from testosterone using the enzyme 5-alpha reductase. Along with testosterone, this hormone is essential for normal masculinization of the external genitalia of male fetuses. disorders of sex development A catchall phrase to describe any alterations in the normal pattern of development of internal and external genitalia in animals, mostly humans. estrogen A steroid hormone that plays a role during primary (present at birth) and secondary (arises at puberty) sex development in females. Fallopian tube Either of a pair of ducts that deliver ova from the ovaries to the uterus in the female reproductive system of humans and most other mammals. Also the site of fertilization. gender identity A person’s own sense of being male or female. Gender identity may be unrelated to the person’s external genitalia or karyotype. 178 Glossary gender reassignment In children, this refers to surgical and hormonal procedures usually undertaken to give a 46,XY male child the sexual characteristics of a female. Usually only attempted when, for one reason or another (such as cloacal exstrophy), traditional development as a male is not an option. genitoplasty Surgical alteration of the external genitalia. Usually done to make a person more closely resemble an average male or female. gonadal dysgenesis—pure and mixed, complete and partial Abnormal development of one or more gonads. Complete failure of gonadal development is known as pure or complete gonadal dysgenesis. Partial development of the gonads is known as partial or mixed gonadal dysgenesis. gonads Organs inside plants and animals that produce gametes—either ovaries or testes producing ova or sperm, respectively. hermaphrodite; true hermaphrodite; pseudohermaphrodite An animal or plant with reproductive tissues of both sexes. In humans, this is a person with one testis and one ovary or one or more ovotestes. Traditionally, the term “true hermaphrodite” has been used to refer to people with gonadal tissue of both sexes—either one testis and one ovary, or one or two ovotestes. “Pseudohermaphrodite” has been used to refer to people whose external genitalia are not consistent with their chromosomal makeup. Most pediatric endocrinologists consider this term archaic and potentially offensive when use to refer to a human being. Glossary 179 hypospadias Most commonly described in males, this disorder of development results in the urethral opening forming somewhere between the glans and the base of the penis instead at the tip. In females, it may result in the urethra opening into the vagina. intersex Human beings who cannot be easily classified as either male or female. karyotype The complement of chromosomes found in an organism. For humans, the usual karyotype is 46,XY or 46,XX—human beings usually have a total of 46 chromosomes including either one X and one Y sex chromosome (male) or two X sex chromosomes (female). Klinefelter syndrome A genetic variation in males that usually results in a karyotype of 47,XXY. Except for sterility, this change results in few or no physical or menta...
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This document was uploaded on 02/04/2014.

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