21-hydroxylase deficiency secondary prevention

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Congenital adrenal hyperplasia main page

Overview

Classification

21-hydroxylase deficiency
11β-hydroxylase deficiency
17 alpha-hydroxylase deficiency
3 beta-hydroxysteroid dehydrogenase deficiency
Cytochrome P450-oxidoreductase (POR) deficiency (ORD)
Lipoid congenital adrenal hyperplasia

Differential Diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Secondary Prevention

Preventing hyperandrogenism and optimizing fertility

As growth ends, management in girls with CAH changes focus to optimizing reproductive function. Both excessive testosterone from the adrenals and excessive glucocorticoid treatment can disrupt ovulation, resulting in irregularity of menses or amenorrhea, as well as infertility. Continued monitoring of hormone balance and careful readjustment of glucocorticoid dose can usually restore fertility, but as a group, women with CAH have a lower fertility rate than a comparable population.

CAH has little effect on male fertility unless an adult stops taking his glucocorticoid medication entirely for an extended period of time, in which case excessive adrenal testosterone may reduce testicular production as well as spermatogenesis.

References


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