Abstract
Vitamin-K=dependent protein C (PC) functions as a potent anticoagulant. Thrombo-embolism has been reported in kindreds with congenitally low PC levels suggesting that PC has an important role in hemostasis and that even a moderate reduction in this plasma protein may be associated with increased risk of thrombosis. Since thrombotic phenomena have important robs in the vaso-occlusive manifestations in SCD, PC was determined in 32 SCD during steady state and in controls (C).
The SCD had significantly lower PC levels as compared to controls. Six SCD were also studied during vaso-occlusive crisis (VOC). During VOC there was marked decrease in PC as compared to levels during steady state (49.5 ± 6.4 and 66.1 ± 10.0, respectively. P < 0.01). Decreased levels of PC during VOC increased to initial levels or higher with clinical improvement. It is postulated that the decreased levels of PC in SCD are probably secondary to increased consumption as well as decreased production because of altered liver functions. This data suggests that decreased levels of PC may increase the risk of thrombosis in these patients.
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Karayalcin, G., Rodriguez, R., Festa, R. et al. 920 PLASMA PROTEIN C LEVELS IN PATIENTS WITH SICKLE CELL DISEASE (SCD). Pediatr Res 19, 264 (1985). https://doi.org/10.1203/00006450-198504000-00950
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DOI: https://doi.org/10.1203/00006450-198504000-00950