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Vol. 46. Issue 9.
Pages 397-402 (January 2003)
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Vol. 46. Issue 9.
Pages 397-402 (January 2003)
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Hiperplasia adrenal congénita con pérdida de sal: historia obstétrica y ginecológica
Salt-losing congenital adrenal hyperplasia. Obstetric and gynecological history
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A. Padillaa,
Corresponding author
ngonzalezg@sego.es

Correspondencia: Servicio de Obstetricia y Ginecología, 3.a planta. Hospital Universitario de Canarias. Ofra, s/n. La Laguna. Tenerife. España
, I. Clementea, M.N. Suáreza, J.L. Pérezb, N.L. González-Gonzáleza
a Servicio de Obstetricia y Ginecología (Prof. J. Parache). Hospital Universitario de Canarias. Facultad de Medicina de la Universidad de la Laguna. Tenerife
b Servicio de Endocrinología del Hospital Universitario de Canarias. Facultad de Medicina de la Universidad de La Laguna. Tenerife. España
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Resumen

Se presenta el caso de una mujer afectada de un déficit de 21-hidroxilasa en su forma clásica que ha sido controlado desde el período neonatal inmediato hasta la vida adulta. Se describen las 2 intervenciones quirúrgicas que se practicaron para corregir las anomalías anatómicas genitourinarias secundarias al síndrome de masculinización, así como el curso del embarazo conseguido de forma espontánea. Tras dar a luz a una niña sana, la mujer tuvo problemas de identificación personal y de comportamiento sexual que la llevaron a realizar 2 intentos de suicidio tras romper su relación de pareja

Palabras clave:
Hiperplasia adrenal congénita con pérdida de sal
Síndrome adrenogenital congénito
Seudohermafroditismo femenino
Summary

Congenital adrenal hyperplasia is due to 21 hydroxylase deficiency and leads to hyperandrogenism, which causes the masculinization of these patients, and reduced cortisol and aldosterone production, which produces the salt-losing form of this disease. We present the case of a women with severe saltlosing congenital adrenal hyperplasia who had undergone surgery in childhood for virilization of the genitals. Spontaneous pregnancy was achieved and a healthy girl was born. During the puerperium she presented a severe crisis of sexual identity that led to an unsuccessful suicide attempt

Keywords:
Salt-losing congenital adrenal hyperplasia
Congenital adrenogenital syndrome
Female pseudohermaphroditism
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Copyright © 2003. Sociedad Española de Ginecología y Obstetricia
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