Therapy
The best direct therapy for a severe protein C deficiency, which can also become apparent for the first time in adulthood, is the administration of concentrated protein C via an infusion directly into the circulation. This directly remedies the deficiency and helps to restore microcirculation in the capillaries. This is the only way to prevent a fatal outcome, especially in newborns with congenital protein C deficiency.
The administration of heparin, another anticoagulant, is a quick action to maintain anticoagulation during the period of protein C deficiency. At least as important is the treatment of a potentially reversible primary cause. This includes quickly bringing sepsis under control or, if wrongly medicated, discontinuing the substances that inhibit the formation of protein C. Liver diseases must also be tackled, since this is where protein C is formed.
Summary
Protein C deficiency is a rare congenital or acquired disease associated with an increased tendency to form blood clots. This tendency to thrombosis can lead to microthromboses and necrosis of the skin, blockage of important arteries with liver damage, stomach ulcers and stroke and, in untreated cases, to multiple organ failure and death of the patient. Protein C is a protein produced in the liver, which normally blocks the coagulation cascade with protein S, thus preventing unnecessary blood clotting and blood clot formation.
However, if a deficiency of Protein C or Protein S occurs for genetic or other reasons (see: Protein S Magel), uninhibited clot formation occurs, which in turn is associated with thromboses and embolisms. Typical causes of an acquired protein C deficiency include cirrhosis of the liver with liver failure or sepsis in the context of untreated meningitis; the so-called meningococcal sepsis. This leads to an enormous consumption and thus to a deficiency of anticoagulant enzymes such as protein C and S. Especially in pregnant women, a protein C deficiency can be life-threatening.
Protein-C deficiency and pregnancy
Studies have shown that pregnant women have a five-fold higher risk of thrombosis compared to non-pregnant women. This is due to the slower blood flow in the vessels of pregnant women on the one hand, and the altered concentrations of blood clotting enzymes on the other. Blood-clotting inhibiting enzymes such as Protein C and Protein S decrease, while blood-clotting promoting enzymes increase.
Women who also have a genetic or acquired tendency to thrombosis experience thromboses and embolisms all the more frequently. Pregnancy complications such as pre-eclampsia and HELLP syndrome can also be the result of a protein C deficiency. These are two typical and dreaded diseases during pregnancy, the cause of which is, among other things, a blood clotting disorder (e.g.B.
a protein C and S deficiency). In severe cases these diseases can be accompanied by embolisms, childhood circulatory disorders and miscarriages, liver failure and death. To prevent these complications, women at risk are recommended to wear compression stockings during and after pregnancy. Women who have already experienced thrombosis or who have been diagnosed with a blood clotting disorder are recommended additional therapy with heparin injections.
