To test the hypothesis that Paget's disease of bone is associated with a greater incidence of calcific aortic valve disease, a computer-generated list was obtained of all autopsy subjects from The Johns Hopkins Hospital in whom Paget's disease was diagnosed (n = 92). The severity of Paget's disease and cardiac valvular lesions was graded on a scale of 0 to 3, with 3 as the most severe. Two control cases were obtained for each case of Paget's disease. Each was the case either immediately before or after the Paget's case, and was matched for age, race, sex, and extent of autopsy. The incidences of moderate (10.9 percent) and severe (5.4 percent) calcific aortic valve disease were both fourfold greater than in the control group (chi-square analysis, p <0.01 and p <0.05, respectively). Additionally, the frequency of advancing grades of calcific aortic valve disease was greater in more advanced stages of Paget's disease. In fact, there was a dose-response effect of Paget's disease upon calcific aortic valve disease (trend analysis for proportion, p < 0.01). These data therefore support the hypothesis that Paget's disease is associated with calcific aortic valve disease in a dose-response manner.
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