Postpartum intracranial venous thrombosis associated with dysfunctional protein C and deficiency of protein S

Article Abstract:

Thrombosis (blood clot formation) in the venous system within the brain is a complication that may occur in women shortly after childbirth. The symptoms include severe headache, nausea, vomiting, partial paralysis, and seizures. If the clot blocks the flow of blood through the venous sinuses (dilated channels through which blood flows), intracranial pressure can increase, leading to serious illness. This condition is generally attributed to the increased tendency of blood to coagulate that is present in pregnancy, which is known as the hypercoagulability of pregnancy. The case of an 18-year-old woman who developed headache, lethargy, and seizures 10 days after giving birth is described. She underwent treatment with anticonvulsants and hospitalization in the intensive care unit to allow reduction of the increased intracranial pressure. This was successful, and she was discharged several days later with no neurologic abnormalities. The pathophysiology of venous blockage is described. The hypercoagulability of pregnancy is the result of several factors in the blood, including protein C and its cofactor, protein S. The patient in the report had abnormally low protein C activity, although the protein C antigenic concentration was normal. The concentration of free protein S was also low. Even in the absence of a history of clotting disorders (as was the case for this patient), evaluation of protein C and protein S levels may be useful for diagnosis and treatment of patients who develop blood clots in the postpartum period. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Measurement, Blood coagulation factors, Pregnancy, Complications of, Pregnancy complications, Protein C deficiency, Puerperal disorders, Cerebral embolism and thrombosis, Cerebral embolism, Cerebral thrombosis, Protein S deficiency

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Brief report: complete deficiency of plasminogen-activator inhibitor type 1 due to a frame-shift mutation

Article Abstract:

A case study of a nine-year-old girl with abnormal bleeding confirms the role of plasminogen-activator inhibitor type 1 (PAI-1) in the formation of blood clots. No previous studies have established the function of PAI-1 in the body nor discovered a link between the absence of PAI-1 and the incidence of disease. The patient had experienced unusually severe bleeding following surgery and accidents but was otherwise healthy. DNA analysis and a study of the girl's family history revealed a genetic mutation resulting in the patient's complete lack of PAI-1. PAI-1 is thought to be important to many body functions but the absence of any other disorders in the patient suggests its role may be limited to blood clot regulation. However, the girl is too young to assess the potential role of PAI-1 in ovulation or tumor metastasis.

Genetic aspects, Fibrinolysis

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Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia

Article Abstract:

Effective ways to prevent arthropathy in boys with severe hemophilia A are examined. It is shown that prophylaxis with recombinant factor VIII can prevent joint damage and decrease the frequency of joint and other hemorrhages in young boys with severe hemophilia A.

Care and treatment, Prevention, Hemophilia, Joint diseases, Clinical report

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