ISSN: 1301-2193 E-ISSN: 1308-9846
  • Turkish Journal of
    Endocrinology and Metabolism

Congenital Adrenal Hyperplasia as a Cause of Secondary Hypertension in Adults: Three Cases
Sekonder Hipertansiyon Sebebi Olarak Konjenital Adrenal Hiperplazi: Üç Vaka
Department of Endocrinology, Bahcesehir University Faculty of Medicine, Department of Endocrinology and Metabolism, Istanbul, Turkey *Giresun Ada Hastanesi, Giresun, Turkey **Ordu Devlet Hastanesi, Ordu, Turkey
Doi: 10.25179/tjem.2019-65085 - Makale Dili: EN
Turk J Endocrinol Metab 2019;23:135-140
ABSTRACT
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.
ÖZET
Konjenital adrenal hiperplaziler; adrenal kortekste bozulmuş kortizol senteziyle sonuçlanan enzimatik defektlere sebep olan herediter bir grup hastalıktır. Konjenital adrenal hiperplazilerin hipertansiyon ve hipokalemiye sebep olan nadir formları 11-β hidroksilaz ve 17-α hidroksilaz eksikliği ile gider. Bu çalışmada, hipertansiyon ve hipokalemi ile gelen iki erişkin 11-β hidroksilaz ve bir erişkin 17-α hidroksilaz hastasının sunulması amaçlanmıştır. Prekoks puberte öyküsü olan 11-β hidroksilaz hastalarında, aynı zamanda testiküler adrenal rest tümör ve adrenal miyelolipoma mevcut iken, 17-α hidroksilaz hastamızda da seksüel infantilizim mevcut idi. Konjenital adrenal hiperplaziler erişkinlerde sekonder hipertansiyonun nadir sebeplerindendir. Prekoks puberte, gecikmiş puberte veya seksüel infantilizim gibi seksüel olgunlaşma bozuklukları ve hipertansiyonla birlikte hipokalemi olması durumlarında konjetinal adrenal hiperplaziler akla gelmelidir.
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