1973 Volume 15 Issue 2 Pages 81-89
Fibromuscular dysplasia accompanied with contralateral hypoplastic kidney is relatively rare. The report described here is dealt with the hypertensive woman of 21 years-old. Physically she was found with high blood pressure of 216/134 mmHg, bruit heard on the left upper quadrant of the abdomen, hypertensive retinopathy KW-2 and harelip. Laboratory examination showed proteinuria and slightly impaired GFR. Serum electrolytes were within normal limits. Plasma renin activity in peripheral venous blood was elevated as well as plasma angiotensin-III level in arterial blood, as determined radioimmunologically. Aldosterone secretion rate was not augumented. High renin level was also detected in renal venous blood ; the value of 8.40 ng of angiotension generation/ml/hr in the left was not differed significantly from that of 7.76 ng/ml/hr in the right. On the aortogram, the left renal artery was narrowed with the feature of “string-of-beads” suggesting the fibromuscular dysplasia of the artery and the arteriovenous fibromuscular dysplasia of the artery and the arteriovenous fistula was concomitantly demonstrated in the left kidney. The right kidney was contracted and its main artery was seeming small, which possibly showed the hypoplastic kidney. The descending aorta was also suggested hypoplastic. These findings of the harelip and abnormalities in the aorta and kidneys may be of the genetic origin, Fibromuscular dysplasia and renal hypoplasia are equally responsible to release of renin in excess. It is wellknown that high renin activity in peripheral blood is causatively related to hypertension in renovascular disease. However, the patient was not treated surgically because the both kidneys were involved. Conservative therapy with antihypertensive drugs caused a fall in arterial pressure and the patient is now kept in good control.
