Male pseudohermaphroditism secondary to panhypopituitarism

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...

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Bibliographic Details
Published in:Archives of disease in childhood 1996-08, Vol.75 (2), p.153-155
Main Authors: Burgner, D P, Kinmond, S, Wallace, A M, Young, D G, Forest, M G, Donaldson, M D
Format: Article
Language:English
Subjects:
Biological and medical sciences
Birth control
Disorders of Sex Development - etiology
Embryonic growth stage
Genitalia - abnormalities
Gonadotropins, Pituitary - deficiency
Gynecology. Andrology. Obstetrics
Hormonal contraception
Humans
Hypopituitarism - complications
Infant, Newborn
Karyotypes
Karyotyping
Male
Medical sciences
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Summary: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.
ISSN:0003-9888
1468-2044
DOI:10.1136/adc.75.2.153