[The clinical characteristics, complications and therapeutic problems of protein C deficiency].
Examination was performed of 20 patients from 15 families with heterozygous deficiency of protein C (27-62% against the mean standard). Recurrent thromboses were seen in 17 of them. Thromboses of the lower veins, pulmonary artery thromboembolism, thromboses of the surface veins, those of cerebral vessels, myocardial infarction, of renal and mesenterial vessels were registered in 15, 5, 4, 3, 3, 1 and 1 patients, respectively. There was a tendency to recurrent thrombosis during pregnancy with high risk of spontaneous or induced abortions. The highest effect was produced with low-molecular heparin combined with transfusions of recently frozen plasma and introduction of disaggregating agents. Anabolic hormones were used to prevent thrombosis. The data are provided on a 4-fold increase in the prevalence of protein C deficiency in polythrombotic patients living in the West Siberia vs. deficiency of antithrombin III.
Barkagan
Z S
ZS
Belykh
S I
SI
Karpenko
A A
AA
D000293:Adolescent; D000328:Adult; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011486:Protein C / Q000032:analysis; D020151:Protein C Deficiency; D012008:Recurrence; D013927:Thrombosis / Q000175:diagnosis / Q000209:etiology / Q000235:genetics / Q000628:therapy
D011486:Protein C
Klinicheskaia kharakteristika, oslozhneniia i problemy terapii defitsita proteina C.