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Vol 82, No 6 (2011)
ARTICLES
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Frasier syndrome in 17-year-old girl – case report

Ewa Matuszczak, Wojciech Debek, Iwona Chomczyk
Ginekol Pol 2011;82(6).

open access

Vol 82, No 6 (2011)
ARTICLES

Abstract

Abstract The authors present a case of Frasier syndrome in a 17-year-old girl with nephrotic syndrome and male pseudohermaphroditism. Due to the existing risk of developing tumors in dysgenetic gonads, the patient was admitted to the clinic for prophylactic gonadectomy. The operation was then postponed as a result of rapid progression to end-stage renal failure, and the patient was placed on hemodialysis. During subsequent laparoscopy, both ovaries and dysgenetic gonads were resected. Histopathological examination revealed the presence of both seminal ducts and epididymis. Early prophylactic resection of dysgenetic gonads, such as was undertaken in this patient, is indicated in children with Frasier syndrome to prevent the development of germ cell tumors.

Abstract

Abstract The authors present a case of Frasier syndrome in a 17-year-old girl with nephrotic syndrome and male pseudohermaphroditism. Due to the existing risk of developing tumors in dysgenetic gonads, the patient was admitted to the clinic for prophylactic gonadectomy. The operation was then postponed as a result of rapid progression to end-stage renal failure, and the patient was placed on hemodialysis. During subsequent laparoscopy, both ovaries and dysgenetic gonads were resected. Histopathological examination revealed the presence of both seminal ducts and epididymis. Early prophylactic resection of dysgenetic gonads, such as was undertaken in this patient, is indicated in children with Frasier syndrome to prevent the development of germ cell tumors.
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Keywords

Frasier syndrome, Pseudohermaphroditism, ambiguous genitalia, renal failure, nephrotic syndrome

About this article
Title

Frasier syndrome in 17-year-old girl – case report

Journal

Ginekologia Polska

Issue

Vol 82, No 6 (2011)

Bibliographic record

Ginekol Pol 2011;82(6).

Keywords

Frasier syndrome
Pseudohermaphroditism
ambiguous genitalia
renal failure
nephrotic syndrome

Authors

Ewa Matuszczak
Wojciech Debek
Iwona Chomczyk

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