Analysis of 45 episodes of arterial occlusive disease in Japanese patients with congenital protein C deficiency.
Hereditary protein C deficiency is associated with a predisposition to venous thrombosis. It is not clear whether the deficiency is involved in arterial occlusion. In the present study, we screened for protein C amidolytic activity in patients admitted to the National Cardiovascular Center Hospital, and we identified among them 43 probands and 51 relatives with heterozygous protein C deficiency. Among them, 34 patients with heterozygous protein C deficiency had manifested 45 episodes of arterial occlusive disease. Venous thrombotic diseases were less common. In the examination of whether protein C deficiency hastens arterial occlusion, we found a significant difference (p =0.02) in the age at onset of acute myocardial infarction between the patients with protein C deficiency (n=10; 49.4+/-14.8 years) and a group of patients with normal protein C levels (n=42; 60.5+/-10.6 years). Acute myocardial infarction occurred before 40 years of age in a significantly greater proportion of the patients with protein C deficiency (3:10, 30%) as compared with the controls (2:42, 5%) (chi2=5.9, p=0.015). At the onset of atherothrombotic cerebral infarction the patients with protein C deficiency were significantly (p=0.022) younger (n= 11; 57.4+/-12.8 years) than those with normal protein C levels (n=48; 64.6+/-10.1 years). Venous thrombosis was the most frequent clinical manifestation (21 of 31 episodes) in the patients with antithrombin III deficiency (n=26; 68% of the total), who were admitted to our hospital. Thus, our study suggests that congenital protein C deficiency contributes to earlier onset of arterial occlusive diseases, especially acute myocardial infarction, in Japanese subjects.
Sakata
T
T
Laboratory of Clinical Chemistry, National Cardiovascular Center, Suita, Japan.
Kario
K
K
Katayama
Y
Y
Matsuyama
T
T
Kato
H
H
Miyata
T
T
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D002544:Cerebral Infarction / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D016009:Chi-Square Distribution; D018572:Disease-Free Survival; D005260:Female; D006579:Heterozygote; D006801:Humans; D007124:Immunoenzyme Techniques; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009203:Myocardial Infarction / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D020151:Protein C Deficiency / Q000235:genetics*; D012307:Risk Factors; D018709:Statistics, Nonparametric