2 . C A R D I O L O G Y
Children with HGPS are at high risk for heart attacks and strokes at any
age. Cardiovascular disease in Progeria is a gradual process. Blood pressure
and ECG are often in the normal range until a child is older. Careful, repeatmeasurements are recommended because the best way to detect a problem
is by asking if there has been a change over time.
The following testing should be considered annually, and more often ifhome physicians recommend:
•Cardiology visit with physical examination
•Measurement of fasting lipids and glucose
• Carotid duplex ultrasound*, if available
*Note, carotid duplex ultrasound and/or pulse wave velocity are available in some centers,
but are not yet routinely performed on pediatric patients. 2 . 1 T H E P R O G E R I A H A N D B O O K
Studies in adults have shown that the benefits of low dose aspirin therapyincrease with increasing cardiovascular risk. Recommendations here come
from experience in adults and in children with diseases which predisposethem to heart attacks and stroke.
Low dose aspirin should be considered for all children with HGPS at any
age, regardless of whether the child has exhibited overt cardiovascular abnormalities or abnormal lipid profiles. Low dose aspirin may help to prevent thrombotic events, including transient ischemic attacks (TIAs)stroke, and heart attacks, by inhibiting platelet aggregation. Aspirin dosageis determined by patient weight, and should be 2-3 mg/kg given once dailyor every other day. Platelets may become “stickier” (i.e., more likely to formclots) at times of stress with illness, fever, etc. While these recommendationsare guidelines, individuals may make adjustment in aspirin dosing basedon their clinical course.
Once a child begins to develop signs or symptoms of vascular decline, suchas hypertension, TIA, strokes, seizures, angina, dyspnea on exertion, ECGchanges, echocardiogram changes, or heart attacks, a higher level of intervention is warranted. Antihypertensive medication, anticoagulants,anti-seizure, and other medications usually administered to adults withsimilar medical issues have been given to children with HGPS. All medica-tion should be dosed according to weight, and carefully adjusted according toaccompanying toxicity (negative side effects) and efficacy (effectiveness).
Aspirin may rarely cause stomach discomfort. If excessive bleeding or bruis-ing is detected, stop aspirin therapy and consult your physician. Aspirintherapy will probably need to be discontinued 1 week prior to any surgery;consult your physician if any surgery is being planned.
If your child becomes ill with chickenpox, stop the aspirin therapy (seebelow).
There is a weak association between aspirin usage during Varicella (chicken-pox) infection and Fatty Liver With Encephalopathy (Reye’s Syndrome) inchildren under 15 years of age. The risk of Reye’s syndrome is extremelysmall compared to the potential benefits of low dose aspirin treatment,given the risk of cardiovascular events in HGPS.
T h e P r o g e r i a R e s e a r c h F o u n d a t i o n ;
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DM STAT-1 CONSULTING BRUCE RATNER, PhD 574 Flanders Drive North Woodmere, NY 11581 [email protected] 516.791.3544 fax 516.791.5075 1 800 DM STAT-1 www.dmstat1.com The first half of the following material is copyrighted material, belonging to Bruce Ratner, as found in his book Statis-tical Modeling and Analysis for Database Marketing: Effective Techniques for Mining Big Data , CRC Press, Boca