gonadal dysgenesis


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Related to gonadal dysgenesis: gonadal agenesis, Swyer syndrome, XY gonadal dysgenesis

dysgenesis

 [dis-jen´ĕ-sis]
defective development; see also dysplasia and malformation.
gonadal dysgenesis
1. defective development of the gonads.
2. turner's syndrome and its variants.

go·nad·al dys·gen·e·sis

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

gonadal 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 dysgenesis

A 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.
References in periodicals archive ?
This a case of pure gonadal dysgenesis in a 46,XY phenotypically female patient, who presented with primary amenorrhoea and infertility.
A case report of successful pregnancy in a patient with pure 46, XY gonadal dysgenesis.
In the setting of gonadal dysgenesis, prophylactic gonadectomy specimens should be assessed for the presence of both gonadoblastoma and malignant germ cell-tumor elements.
Gonadoblastoma associated with pure gonadal dysgenesis in monozygotic twins.
Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism--a critical analysis.
Control males never contained testicular oocytes, although two control males contained two to three degenerating extragonadal oocytes (not within lobules), and a single control male showed gonadal dysgenesis (Figure 9).
In addition, in high-use York County, Nebraska, 28% of the males examined had gonadal dysgenesis (Figure 13), and testicular oocytes were found in a single male.
Both the proportion of males with gonadal dysgenesis and the proportion with testicular oocytes (hermaphrodites) were higher at the lower dose.
Wild populations that contained males with gonadal dysgenesis and testicular oocytes (hermaphrodites) were associated with localities with atrazine use and/or atrazine contamination.