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defective gonadal development, varying types and degrees of which have been identified, including gonadal aplasia or agenesis, rudimentary gonads, congenitally defective gonads, and true hermaphroditism; the character of the external genitalia, genital ducts, and secondary sexual development are only sometimes uniquely related to a given type of gonadal dysgenesis XO GONADAL DYSGENESIS51 consists of monosomy X with a gonadal streak rather than a true ovary, notably seen in Turner syndrome; XX GONADAL DYSGENESIS51 is an autosomal recessive disorder with a female karyotype, streak gonads, and primary amenorrhea, but with no body features of Turner syndrome; XY GONADAL DYSGENESIS51 is an X-linked disorder associated with a male karyotype and a female habitus, streaked gonads, and absence of secondary sexual characteristics.
Synonym(s): ovarian dysgenesis
a general designation for a variety of conditions involving anomalies in the development of the gonads, such as Turner's syndrome, hermaphroditism, and gonadal aplasia.
gonadal dysgenesisA condition characterized by underdeveloped or imperfectly formed gonads; the prototypic GD is Turner syndrome–45, X0, which occurs in 1:2-7000 ♀ births Clinical Short stature, webbed neck, cubitus valgus, micrognathia with high arched palate, epicanthal folds, lymphedema of hands and feet, aortic coarctation, renal malformation, osteoporosis, DM, widely spaced nipples, sexual infantilism. See Intersex syndromes.
go·nad·al dys·gen·e·sis(gō-nad'ăl dis-jen'ĕ-sis)
Defective gonadal development; types include gonadal aplasia or agenesis, rudimentary gonads, congenitally defective gonads, and true hermaphroditism.