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...

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Bibliographische Detailangaben
Veröffentlicht in:Hinyokika kiyo. Acta urologica Japonica. - 1962. - 33(1987), 4 vom: 01. Apr., Seite 545-50
1. Verfasser: Sugao, H (VerfasserIn)
Weitere Verfasser: Tsujimoto, Y, Takiuchi, H, Sakurai, T, Kobayashi, Y, Kato, G, Suzuki, T
Format: Aufsatz
Sprache:Japanese
Veröffentlicht: 1987
Zugriff auf das übergeordnete Werk:Hinyokika kiyo. Acta urologica Japonica
Schlagworte:Case Reports English Abstract Journal Article Estrogen Antagonists
Beschreibung
Zusammenfassung: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
Beschreibung:Date Completed 03.09.1987
Date Revised 18.11.2010
published: Print
Citation Status MEDLINE
ISSN:0018-1994