Nonclassic Congenital Adrenal Hyperplasia: What Do Endocrinologists Need to Know?
Endocrinol Metab Clin North Am
; 50(1): 151-165, 2021 03.
Article
em En
| MEDLINE
| ID: mdl-33518183
ABSTRACT
Congenital adrenal hyperplasia encompasses a group of autosomal recessive defects in cortisol biosynthesis, and 21-hydroxylase deficiency accounts for 95% of such cases. Non-classic 21-hydroxylase deficiency is due to partial enzymatic defects, which present with normal cortisol synthesis, but excessive production of adrenal androgens, including 11-oxygenated androgens. Non-classic 21-hydroxylase deficiency is relatively common, and its phenotype resembles closely that of polycystic ovary syndrome. This review focuses primarily on non-classic 21-hydroxylase deficiency, its clinical features, diagnosis, and management.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome do Ovário Policístico
/
Hiperplasia Suprarrenal Congênita
Tipo de estudo:
Diagnostic_studies
Limite:
Female
/
Humans
Idioma:
En
Revista:
Endocrinol Metab Clin North Am
Assunto da revista:
ENDOCRINOLOGIA
/
METABOLISMO
Ano de publicação:
2021
Tipo de documento:
Article