Defects in the prostaglandin-system--heredity, prevalence and vascular risk analysis

2180 healthy adults, 620 patients (90% with cardiovascular disease, 10% others), 143 newborns and 351 relatives of persons in whom any abnormality of the prostaglandin (PG)-system had been discovered were involved in the Viennese Initiative for Prostaglandin (VIP)-Screening from 1984 through May 1989. The defects discovered were either inborn or acquired ones and were either familial or non-familial. The highest number of defects (54.8% of the total) identified were associated with PGI2. They indicated a lack of the PGI2-synthesis stimulating plasma factor (PF)-activity (n = 17) and PGI2-stabilizing capacity in plasma (defect 'Vienna-Döbling', n = 4, 12.9% of total), absence of high-affinity PGI2-binding sites on platelet membranes and lack of response of platelets to PGI2 (defect 'Vienna-Hietzing', n = 2, 6.5% of total). Furthermore, a lack of cyclooxygenase (n = 2, 6.5% of total), 12-lipoxygenase (defect 'Vienna-Penzing', n = 3, 9.7% of total) and thromboxane synthetase (n = 3, 9.7% of total) activities were diagnosed. In newborn screening, the PF-absence amounted to 2.8% (4/143), the lack of PGI2-stabilizing capacity to 0.7% (1/143). In adults, the prevalence is difficult to assess. Nevertheless, our findings indicate a frequency of 0.93% (26/2800) of defects in the PG-system among the adult Viennese population. The lack of PF-activity seems to be by far the most frequent one (0.46%, 13/2800). Other defects are much less common. It is not clear whether the incidence in healthy subjects or in patients with cardiovascular disease is different from each other. Further, it remains to be assessed whether there is a causative relation to vascular events or just an association. More intensive screening activities should validate the diagnostic, pathogenetic and therapeutic impact of the various defects in the PG-system.