Case Reports in Pediatrics

Case Report

Arterial Stiffness in a Toddler with Neurofibromatosis Type 1 and Refractory Hypertension

Table 1

Clinical, laboratory, and imaging data during follow-up visits.
 Age (yrs)Height (cm)Office BP (mmHg)/BP pcAmbulatory BP (mean day/night, mmHg)Office central BP1 (mmHg)Cf-PWV (m/s)2MRA aortic diameter (cm)CTA aortic diameter (cm)DTPA scan (right) % (left) %Renin active/rest (ng/ml/min) aldosterone active/rest (pg/dl)3Creatinine (mg/dl)/eGFR (ml/min/1.73 m2)4Urine protein (mg/m2/24h)Medication5
18 months2.595101/60
<90th		107/61         
106/61
108/61
1 month498125/85
>99th		122/80116/8650.30.2–0.3 8.75/30.940.58/9341.2 
123/81
119/77
Before↑
After↓		PTA
1 month499131/80
>99th		   0.35–0.5 25.8/74.2 0.79/68 Valsartan felodipine
4 months4100118/73
95–99th		127/76111/775.6 0.34–0.3817.7/82.3 0.72/7661.5Amlodipine
128/77Furosemide
125/73Atenolol
8 months5103133/91
>99th		112/65127/935   4.08/1.680.73/7749.4Amlodipine
115/67Furosemide
106/5610.8/8.52Atenolol
Clonidine
12 months5104135/81
>99th		128/76127/864.60.35 27/735.84/1.060.73/7848Amlodipine
122/7712.3/12.4Furosemide atenolol
115/74Clonidine
1Central BP was measured by an oscillometric device (SpygmoCor, AtCor Medical). 2Reference values available for children older than 7 yrs.: 5.26 m/sec 95th percentile for 7-year-old girls, 5.06 m/sec 95th percentile for 7-year-old girls. 3eGFR was based on Schwartz formula. 4Renin and aldosterone at rest was measured after night’s rest in the supine position and then active after 1 hour walking in the upright position.5 Antihypertensive drugs doses: valsartan:1.3 mg/kg/day qd; felodipine: 2.5 mg daily qd; amlodipine: 0.5 mg/kg/day qd; furosemide: 1 mg/day bid; atenolol:1 mg/kg/day bid; clonidine: 0.1 mg/day bid. BP: blood pressure; cf-PWV: carotid-femoral pulse wave velocity; MRA: magnetic resonance angiography; CTA: computed tomography angiography; DTPA: 99mTc-diethylenetriaminepentaacetic acid.