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Familial Hypercholesterolemia and Early Coronary Death-Reply

Roger R. Williams, MD
JAMA. 1986;256(3):348. doi:10.1001/jama.1986.03380030050016.
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In Reply.—  Dr Ravenholt's respect for smoking as a modifiable risk factor for early coronary disease is shared by the authors of this article. In the "Comment" section of the JAMA article, we suggested a multiplicative interaction of cigarette smoking with family history. We noted in our report that two brothers with familial hypercholesterolemia had very different outcomes according to their smoking status. One who smoked died at age 32 years, and a nonsmoker died at age 45 years. Furthermore, we cited our prior published analysis suggesting magnified effects of cigarette smoking in high-risk families.1Unfortunately, Dr Ravenholt's conclusion that our four pioneer Utah males with the gene for familial hypercholesterolemia survived to ages 62, 68, 72, and 81 years primarily because they were nonsmokers is not supported by our data. Most of the 20 other males with familial hypercholesterolemia in recent generations were also nonsmokers and yet still


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