Unilateral true hermaphrodite with 46,XX/46,XY dispermic chimerism.

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Unilateral true hermaphrodite with 46,XX/46,XY dispermic chimerism.

A 13 year old female presented with ambiguous external genitalia, right inguinal ovotestis, left ovary, apparently normal Mullerian system, and absent Wolffian system. Cultured lymphocytes showed a 46,XX/46,XY karyotype. Histopathology of the gonads confirmed true hermaphroditism. The presence of two genetically different erythrocyte populations was observed. The findings suggested that the pat...

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True Hermaphrodite: A Case Report

True hermaphrodite is one of the rarest variety of disorders of sexual differentiation (DSD) and represents only 5% cases of all. A 3-year-old child presented with left sided undescended testis and penoscrotal hypospadias. Chordee correction was performed 18 months back, elsewhere. At laparotomy Mullerian structures were present on left side. On right side testis was normally descended into the...

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A Case of True Hermaphrodite Presenting as Cyclical Hematuria

True hermaphrodite (also known as ovotesticular disorder of sexual development or ovotesticular-DSD), is one of the rare varieties of disorder of sexual development. It is characterized by histologically confirmed both ovarian and testicular tissue in one individual. Here we report the case of a16-year-old phenotypic male with 46, XX genotype(true hermaphrodite) presenting with cyclical hematur...

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Hermaphrodite with Mosaic

SIR,-In a recent review de Grouchy 1 compiles the following karyotypes described in true hermaphroditism: 46 XX, 46 XY, 46 XX/46 XY, 45 XO/46 XY, 46 XX/47 XXY, 46 XX/47 XXX, 46 XX/46 XY/45 XO, 46 XX/47 XXY/ 49 XXYYY, and 45 XO/46 X isoY/47 XY isoY. We have studied the chromosomes of a true hermaphrodite with a new mosaicism, 46 XX/46 XY/47 XXY, which might throw some light on the possible multi...

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Bilateral gonadoblastomas with a left sided dysgerminoma in a true hermaphrodite (disorder of sexual differentiation) with 46, XY karyotype.

Ovotesticular DSD is not an uncommon disorder. The presence of Y chromosome confers a high risk of neoplastic transformation in dysgenetic gonads. The neoplastic development in these patients is associated with the presence of Y chromosome and intra abdominal location of the abnormal gonad. We report histogenetic details of a rare occurrence of bilateral gonadoblastomas and left sided dysgermin...

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 1987

ISSN: 1468-6244

DOI: 10.1136/jmg.24.12.784