Hypertension as a result of stenosis of a renal artery was diagnosed in three patients, aged 20, 17, and 9 respectively, among 14 siblings from one family. The oldest patient was treated by vasoplasty; a hypoplastic kidney had previously been removed in an unsuccessful attempt to control hypertension. Nephrectomy was performed in the other two patients. The pathological picture of the main renal artery that could be examined differed from that usually seen in the fibromuscular dysplasia group. The absence of microscopic signs of atrophy in the small kidneys behind the stenosis argues for a developmental defect. More than three years after operation blood pressure and renal function in all three patients have remained normal.
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