Male pseudohermaphroditism secondary to panhypopituitarism.
نویسندگان
چکیده
An infant with a 46XY karyotype was born with ambiguous genitalia, including microphallus and perineal hypospadias. A female gender was assigned due to extreme failure of development of the external genitalia. Subsequent investigations demonstrated panhypopituitarism, and it is believed that severe gonadotrophin deficiency was responsible for the intersex state. This case illustrates the need to evaluate the hypothalamic-pituitary axis in selected cases of intersex, and also questions the prevailing assumption that testosterone secretion during embryogenesis is largely pituitary gonadotrophin independent, under the control of human chorionic gonadotrophin.
منابع مشابه
Suprasellar tuberculoma presenting as panhypopituitarism.
Suprasellar tuberculomas are exceptionally rare. We present the case of a middle aged male who presented with generalised apathy and meningism of two weeks duration. Neuroimaging was suggestive of tuberculomas in suprasellar cistern, while endocrinological investigation showed panhypopituitarism manifesting as pituitary hypothyroidism, hypocortisolism, and hyperprolactinemia. Cerebrospinal flui...
متن کاملMale Pseudohermaphroditism Presented with Sudden Cardiac Arrest
Torsades de pointes is a life-threatening arrhythmia associated with a number of causes, but is very rare among endocrinologic disorders. We report a case of male pseudohermaphroditism with hyperaldosteronism due to a 17α-hydroxylase deficiency presented with sudden cardiac arrest.
متن کاملGonadal tumor in Frasier syndrome: a review and classification.
Frasier syndrome is a rare inherited disease characterized by steroid-resistant nephrotic syndrome, gonadal tumor, and male pseudohermaphroditism (female external genitalia with sex chromosomes XY), which is based on a splice site mutation of Wilms tumor-suppressor gene 1 (WT1). Several unusual Frasier syndrome cases have been reported in which male pseudohermaphroditism was absent. We reviewed...
متن کاملClinical, genetic, and pathological features of male pseudohermaphroditism in dog
Male pseudohermaphroditism is a sex differentiation disorder in which the gonads are testes and the genital ducts are incompletely masculinized. An 8 years old dog with normal male karyotype was referred for examination of external genitalia abnormalities. Adjacent to the vulva subcutaneous undescended testes were observed. The histology of the gonads revealed a Leydig and Sertoli cell neoplasi...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Archives of disease in childhood
دوره 75 2 شماره
صفحات -
تاریخ انتشار 1996