Steroid responsive hypertension secondary to 11-beta hydroxylase deficiency--a case report
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Date
2008-07
Authors
Isiavwe, A.R
Ekpebegh, C.O
Fasanmade, O.A
Ohwovoriole, A.E
Journal Title
Journal ISSN
Volume Title
Publisher
West African College of Physicians
Abstract
BACKGROUND:
Congenital adrenal hyperplasia (CAH), due to deficiency in 11â hydroxylase enzyme is rare a cause of virilization associated with glucocorticoid responsive hypertension.
OBJECTIVE:
To present a rare cause of CAH and secondary hypertension responsive to glucocorticoid therapy.
METHODS:
Clinical and laboratory evaluation of a young woman with a" phallus" and hypertension. Investigations carried out included serum biochemistry, bone age determination, sex chromatin evaluation and serum levels of androgens and 17-OH progesterone, as well as pelvic ultrasonography.
RESULTS:
There was a history of cliteromegaly, first noticed at age 14 years, but menstrual periods were said to be regular. Physical findings included male type hairline and musculature with a blood pressure of 160/110mmHg. Breast development was Tanner Grade 3 while the public hair was male in pattern. The clitoris was 5cm long. Serum electrolytes were normal but levels of testosterone, dehydroepiandosterone sulphate and 17-OH progesterone were elevated. Features were compatible with a diagnosis of congenital adrenal hyperplasia (CAH) due to Il-beta-hydroxylase defficiency. Hypertension responded to glucocorticoid therapy, recurred on default and was corrected again on re-starting hydrocortisone.
CONCLUSION:
Congenital adrenal hyperplasia due to 11â-hydroxylase deficiency though rare, should be considered in the differential diagnosis of hypertension with virilization in young females.
Description
Staff publications
Keywords
Congenital adrenal hyperplasia , Secondary hypertension , Steroid responsive , Research Subject Categories::MEDICINE
Citation
Isiavwe AR, Ekpebegh CO, Fasanmade OA, Ohwovoriole AE. Steroid responsive hypertension secondary to 11-beta hydroxylase deficiency--a case report. West African Journal of Medicine, 2008;7(3);182-185.