Resumen

MEJIA, Yanire  y  GRUPO DE ENDOCRINOLOGIA MERIDA (ENDO-MER) et al. Manejo de la hiperplasia suprarrenal congénita. Rev. Venez. Endocrinol. Metab. [online]. 2014, vol.12, n.1, pp.41-51. ISSN 1690-3110.

Congenital adrenal hyperplasia (CAH) is a group of disorders caused by defects in the adrenal steroidogenic pathways. In its most common form, 21-α-hydroxylase deficiency, patients develop varying degrees of glucocorticoid and mineralocorticoid deficiency as well as androgen excess. It is generally classified as classical and non-classical forms. The goals of treatment are to replace the cortisol and aldosterone deficiency, avoid hyperandrogenism and achieve the best final height. Although these goals seem pretty straightforward, in practice, they are very difficult to achieve. Glucocorticoid and mineralocorticoid therapy is guided by monitoring clinical parameters as well as adrenal hormone and electrolytes concentrations. Practitioners must strike a fine balance; on height, overtreatment with glucocorticoids can lead to poor growth, and undertreatment, to androgen excess and premature epiphyseal maturation. The CAH diagnosis and treatment of the Endocrinology Service, Autonomous Institute University Hospital of The Andes, Mérida, Venezuela.

Palabras clave : Congenital adrenal hyperplasia; disorder of sexual differentiation; virilization; short stature.

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