Steroid responsive hypertension secondary to 11-beta hydroxylase deficiency--a case report.
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.


CONCLUSIONS
Congenital adrenal hyperplasia due to 11â-hydroxylase deficiency though rare, should be considered in the differential diagnosis of hypertension with virilization in young females.
Isiavwe
A R
AR
Department of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria. PMB 12003 Lagos, Nigeria.
Ekpebegh
C O
CO
Fasanmade
O A
OA
Ohwovoriole
A E
AE
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D000328:Adult; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D006973:Hypertension / Q000209:etiology*; D013252:Steroid 11-beta-Hydroxylase / Q000378:metabolism*; D014770:Virilism / Q000175:diagnosis / Q000601:surgery*
D013252:Steroid 11-beta-Hydroxylase; D006854:Hydrocortisone