'True agonadism': A misnomer?
| Autor: | George A. Limbeck, Maria I. New, Kenneth W. Dumars, Gary A. Parks, W. Leslie G. Quinlivan |
|---|---|
| Rok vydání: | 1974 |
| Předmět: |
Male
endocrine system medicine.medical_specialty Exploratory laparotomy medicine.medical_treatment Disorders of Sex Development Radioimmunoassay Uterus Misnomer Labial fusion Chorionic Gonadotropin Diagnosis Differential Internal medicine Methods medicine Humans Endocrine system Testosterone Pregnanetriol Mullerian Ducts Hydroxysteroids Fetus urogenital system business.industry Androstenedione Leydig Cells Estrogens Dehydroepiandrosterone Luteinizing Hormone medicine.disease 17-Ketosteroids Thyroxine Endocrinology medicine.anatomical_structure Child Preschool Karyotyping Pediatrics Perinatology and Child Health Male pseudohermaphroditism Female Follicle Stimulating Hormone Corticosterone business |
| Zdroj: | The Journal of Pediatrics. 84:375-380 |
| ISSN: | 0022-3476 |
| DOI: | 10.1016/s0022-3476(74)80720-1 |
| Popis: | A 3 1/2-year-old "girl" whose condition had been diagnosed as "true agonadism" underwent endocrine evaluation, which suggested the presence of Leydig cells. The child was considered to be female, although born with mild phallic enlargement and posterior labial fusion. At one year of age, evaluation revealed a, 46,XY karyotype; at exploratory laparotomy, no gonads, uterus, Fallopian tubes, or Wolffian remnants were observed. Base-line gonadotropins (FSH, 0.72 ng.per milliliter; LH, 1.9 ng. per milliliter), were not elevated and a significant rise in plasma testosterone (63 to 169 ng. per 100 ml.) followed stimulation with HCG; both features suggested the presence of testosterone-secreting Leydig cells. The obsence of Mullerian anlagen suggests that at some time during fetal development Mullerian-inhibiting factor was secreted by testes. "True agonadism" is probably a misnomer and actually represents that form of male pseudohermaphroditism with a scant amount of functioning testicular tissue present. |
| Databáze: | OpenAIRE |
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