Importance of early diagnosis and gonadectomy in 46, XY females
Autor: | W.A.W. Walters, L.J. Cussen, R.A. MacMahon |
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Rok vydání: | 1980 |
Předmět: |
Adult
endocrine system medicine.medical_specialty Time Factors Adolescent Secondary sex characteristic Gonadal dysgenesis Y chromosome Gonadal Dysgenesis XY gonadal dysgenesis Internal medicine Medicine Humans Castration Gonadal Dysgenesis 46 XY urogenital system business.industry Karyotype General Medicine medicine.disease Sertoli cell Abdominal mass Endocrinology medicine.anatomical_structure Child Preschool Pediatrics Perinatology and Child Health Male pseudohermaphroditism Surgery Female medicine.symptom business |
Zdroj: | Journal of pediatric surgery. 15(5) |
ISSN: | 0022-3468 |
Popis: | Gonadal neoplasms developed in three 46, XY females aged 3 yr and 10 mo, 16 yr, and 19 yr. One patient died from metastatic neoplasia. Early diagnosis of 46, XY gonadal dysgenesis is essential, and should be considered in female infants and children with any features of Turner's syndrome, ambiguous genitalia, phallic hypertrophy, excessive height, large hands and feet, failure of development of secondary sexual characteristics, primary amenorrhea, abdominal mass, or unexplained hypertension. Any female with gonadal dysgenesis and a Y chromosome in her karyotype should have prophylactic gonadectomy as soon as possible. The absence of Sertoli cells in these patients, causing lack of androgen binding protein with deficient local concentration of androgens and consequent failure of maturation of spermatogonia, may lead to unregulated proliferation of germ cells, and hence explain the frequency of gonadal neoplasia in the 46, XY female. |
Databáze: | OpenAIRE |
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