Congenital Adrenal Hyperplasia as a Cause of Secondary Hypertension in Adults: Three Cases

Congenital adrenal hyperplasia is a group of hereditary disorders originating from enzymatic defects in steroidogenesis, resulting in impaired cortisol synthesis in the adrenal cortex. The rare forms of congenital adrenal hyperplasia characterized by hypertension and hypokalemia include 11- β and 17...

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Bibliographic Details
Published in:Turkish journal of endocrinology and metabolism 2019, Vol.23 (2), p.135-140
Main Authors: ANAFOROĞLU, İnan, ALGÜN, Ekrem, ERSOY, Kerem
Format: Article
Language:English
Subjects:
Adrenal glands
Congenital diseases
Hyperplasia
Hypertension
Physical growth
Puberty
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Summary:Congenital adrenal hyperplasia is a group of hereditary disorders originating from enzymatic defects in steroidogenesis, resulting in impaired cortisol synthesis in the adrenal cortex. The rare forms of congenital adrenal hyperplasia characterized by hypertension and hypokalemia include 11- β and 17-α hydroxylase deficiencies. We described three cases of congenital adrenal hyperplasia, two with 11-β hydroxylase and one with 17-α hydroxylase deficiency, each presenting with hypertension and hypokalemia in adulthood. In addition, 11-β hydroxylase deficiency cases showed precocious puberty with testicular adrenal rest tumor and adrenal myelolipoma, whereas the patient with 17-α hydroxylase deficiency showed sexual infantilism. Congenital adrenal hyperplasia is a rare cause of secondary hypertension in adults. It is particularly considered in patients with sexual maturation disorders, such as precocious puberty, delayed puberty, or sexual infantilism, and in those with hypertension and hypokalemia.
ISSN:1301-2193
1308-9846
DOI:10.25179/tjem.2019-65085