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A Rare Case of True Hermaphrodite with Features of Klinefelter Syndrome

Volume 51 Number 4 October-December, 2013 1 ** Resident, Department of Internal Medicine, Southern Philippines Medical Center, Davao City ** Consultant, Section of Endocrinology, Department of Internal Medicine, Southern Philippines Medical Center Synopsis: A 16-year-old male presented with cyclical hypogastric pain, eunochoid features, male external gen i ta l ia w i th hypospadia , male and female reproductive structures on imaging tests and a Klinefelter syndrome of karyotype 46XX/47XXY. Only two cases of true hermaphroditism with features of Klinefelter syndrome and a karyotype of 46XX/47XXY have been published. Our patient is the second true hermaphrodite with mosaic Klinefelter syndrome 46XX/47XXY karyotype to be reported.