Analysis of 45 episodes of arterial occlusive disease in Japanese patients with congenital protein C deficiency.

Abstract

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.

• Authors: T Sakata, K Kario, Y Katayama, T Matsuyama, H Kato, T Miyata
• Journal: Thrombosis research (Thromb Res) Vol. 94 Issue 2 Pg. 69-78 (Apr 15 1999) ISSN: 0049-3848 [Print] United States
• PMID: 10230891 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adult
• Age of Onset
• Aged
• Aged, 80 and over
• Arterial Occlusive Diseases (diagnosis, epidemiology, genetics)
• Cerebral Infarction (diagnosis, epidemiology, genetics)
• Chi-Square Distribution
• Disease-Free Survival
• Female
• Heterozygote
• Humans
• Immunoenzyme Techniques
• Japan (epidemiology)
• Male
• Middle Aged
• Myocardial Infarction (diagnosis, epidemiology, genetics)
• Protein C Deficiency (genetics)
• Risk Factors
• Statistics, Nonparametric