A CASE OF RENOVASCULAR HYPERTENSION ASSOCIATED WITH NEUROFIBROMATOSIS

We report a case of renovascular hypertension associated with neurofib romatosis complicated by moderate proteinuria. A 16-year-old female wa s admitted to Kensei General Hospital with a complaint of headache and a blood pressure of 230/120 mm Hg. She was referred to us for further evaluation of the hypertension. On examination, cafe-au-lait spots we re seen over her extremities and flank, and a bruit was heard in the r ight upper abdomen. The urinary protein excretion was 2.1 g/day. The p lasma renin activity (PRA) and plasma aldosterone concentration were h igh, but the levels of catecholamines were normal. The renogram was as ymmetric and on venous sampling, the PRA in the right renal vein was 5 8.3 ng/ml/h and that in the left was 22.1 ng/ml/h. CT scan detected an approximately 10-mm mass in the proximal right renal artery. Arteriog raphy disclosed severe stenosis in the right renal artery and the supe rior mesenteric artery. Therefore, we concluded that her hypertension resulted from stenosis of the right renal artery due to neurofibromato sis. Accordingly, she underwent an operation to reconstruct that arter y. After the operation, her blood pressure and PRA normalized without administration of any anti-hypertensive drug and urinary protein disap peared.