Reifenstein's syndrome : case report of two siblings
Two cases of penoscrotal hypospadias and gynecomastias in a 14-year-old and 16-year-old, who were siblings are reported. These cases, with rather small testes and 46, XY karyotypes, were classified as familial incomplete male pseudohermaphroditism, type 1, so called Reifenstein's syndrome. Thei...
Publié dans: | Hinyokika kiyo. Acta urologica Japonica. - 1962. - 33(1987), 4 vom: 01. Apr., Seite 545-50 |
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Auteur principal: | |
Autres auteurs: | , , , , , |
Format: | Article |
Langue: | Japanese |
Publié: |
1987
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Accès à la collection: | Hinyokika kiyo. Acta urologica Japonica |
Sujets: | Case Reports English Abstract Journal Article Estrogen Antagonists |
Résumé: | Two cases of penoscrotal hypospadias and gynecomastias in a 14-year-old and 16-year-old, who were siblings are reported. These cases, with rather small testes and 46, XY karyotypes, were classified as familial incomplete male pseudohermaphroditism, type 1, so called Reifenstein's syndrome. Their serum testosterone and estrogen levels were high, but the luteinizing hormone (LH) and follicle-stimulating hormone levels were within normal limits. LH-releasing hormone tests revealed good response of LH. Urethrographies showed no prostatic utricles. They had urethroplasties at the ages of 5 and 6. Anti-estrogen therapy had some effect on the gynecomastias |
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Description: | Date Completed 03.09.1987 Date Revised 18.11.2010 published: Print Citation Status MEDLINE |
ISSN: | 0018-1994 |