ABSTRACT
Neurofibromatosis type 1 (NF1) is associated with vascular lesions, such as renal artery stenosis, and secondary hypertension. The real prevalence is largely unknown, particularly in children. We observed 27 patients with NF1, mean age 12.8 years (range 4.2-24 years), for 2-10 years to assess the association of NF1 with vascular abnormalities and secondary hypertension. Patients were studied with angiography, 24-h blood pressure monitoring, a captopril test, and Doppler ultrasonography of aorta and renal arteries. The prevalence of hypertension was 18.5%; 61.5% of patients studied with angiography had vascular lesions, half of whom were apparently normotensive. However, they had abnormal 24-h blood pressure monitoring, which was a first sign of poor blood pressure control. Those patients with severe hypertension (11.1%) were successfully treated with percutaneous transluminal angioplasty (PTA); stenosis recurred in 2 of 3 patients after a 2-year follow-up period, and was responsive to drugs. We conclude that hypertension is a frequent complication of NF1 in pediatric patients, it is usually secondary to typical vascular lesions, and requires careful follow-up. Ambulatory blood pressure monitoring (24-h) is a sensitive method for detecting initial alterations of the blood pressure pattern. PTA may be an effective treatment in this condition.
Subject(s)
Hypertension/complications , Neurofibromatosis 1/complications , Renal Artery Obstruction/complications , Adolescent , Adult , Aorta, Abdominal , Aortic Coarctation/physiopathology , Blood Pressure , Blood Pressure Monitoring, Ambulatory , Child , Child, Preschool , Female , Humans , Hypertension/epidemiology , Hypertension/physiopathology , Incidence , Male , Neurofibromatosis 1/physiopathology , Renal Artery Obstruction/epidemiology , Renal Artery Obstruction/physiopathologyABSTRACT
We used the captopril test (CT) in 32 children, 8 with renovascular hypertension (RVH), 17 with renal hypertension (RH) and 7 with normal blood pressure, in order to study the renin-angiotensin system activation (RASA). All children affected by RVH presented a positive CT: a post-captopril plasma renin activity (PRA) of 12 ng ml-1 h-1 or more, an absolute PRA increase of 10 ng ml-1 h-1 or more and a 150% increase or more, or 400% or more if the baseline PRA was less than 3 ng ml-1 h-1. The CT may be useful for demonstrating the RASA in RVH.
Subject(s)
Angiotensin-Converting Enzyme Inhibitors , Captopril , Hypertension, Renal/diagnosis , Adolescent , Blood Pressure , Child , Child, Preschool , Humans , Hypertension, Renal/physiopathology , Infant , Renin/blood , Renin-Angiotensin SystemABSTRACT
A 9-year-old boy with hypertension was found to have neurofibromatosis associated with stenosis of the right renal artery. Percutaneous transluminal angioplasty (PTA) was performed. Immediately post angioplasty angiography showed that the stenosis persisted, but over the next few days his blood pressure rapidly decreased and remained well controlled even when treatment was discontinued. The captopril stimulation test, performed after PTA, confirmed the return of plasma renin activity to normal values. A digital subtraction aortogram, performed 2.5 years after PTA, was unchanged. His blood pressure remained persistently normal, without anti-hypertensive agents. Based on these results, PTA is suggested as the first step in correcting renal artery stenosis due to neurofibromatosis. A complete anatomical resolution of the stenosis is probably not required since slight improvements in the renal artery lumen may be accompanied by important functional improvement.
