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Abstract
Fifty-four children referred for investigation of hypertension had renovascular disease.
In eight patients it was associated with neurofibromatosis, in three with idiopathic
hypercalcemia of infancy, and in five cases it followed an arteritic illness. Fibromuscular
dysplasia was the underlying abnormality in the majority of cases (46%). Twenty-six
patients (48%) were first seen with accelerated hypertension; 38 children (70%) had
bilateral renal arterial disease, and in 41 (76%), disease of the small intrarenal
vessels was found. Renal vein renin ratios indicated unilateral disease in 31 cases;
the results correlated with arteriography findings in 32 (62%) of 51 patients. Eleven
children also had the middle aortic syndrome, and 9 of 16 patients, investigated by
cerebral arteriography because of cranial bruits or focal neurologic signs, had cerebral
vascular abnormalities. Twenty patients were treated surgically--10 by reconstructive
procedures, 11 by nephrectomy or heminephrectomy, and 6 by transluminal angioplasty.
Of these, 9 (45%) are normotensive with no treatment, 10 have a decreased requirement
for antihypertensive drugs, and 1 had no improvement. Thirty-four patients were treated
medically because of the extent of their disease; two patients have died of hypertensive
complications. We conclude that renal vascular disease in children is often widespread,
may be associated with intracerebral vascular disease, frequently affects both kidneys,
including both intrarenal and extrarenal vessels, and is therefore not always amenable
to surgical intervention and cure.