Steroid responsive hypertension secondary to 11-beta hydroxylase deficiency--a case report.
| Autor: | Isiavwe AR; Department of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria. PMB 12003 Lagos, Nigeria., Ekpebegh CO, Fasanmade OA, Ohwovoriole AE |
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| Jazyk: | angličtina |
| Zdroj: | West African journal of medicine [West Afr J Med] 2008 Jul; Vol. 27 (3), pp. 182-5. |
| Abstrakt: | 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. |
| Databáze: | MEDLINE |
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