[A case of congenital protein C deficiency with pulmonary thromboembolism].
A 47-year-old woman, with a history of recurrent venous thrombosis in her lower limbs (1988 and June, 1992), was admitted because of pulmonary thromboembolism in the right middle lung lobe. She was given anti-coagulant therapy with warfarin. The consolidation in the right middle lobe disappeared within two months. Hematological examinations concerning the coagulation and fibrinolytic system showed a significant decrease in both the serum concentration and the activity of protein C, a vitamin K-dependent hepatic protein which acts as an anticoagulant by shutting off fibrin formation and stimulating fibrinolysis. Since a sister of the patient also has a history of venous thrombosis, several members of her family were tested for protein C deficiency. The familial study revealed that her sister and mother had both a decreased concentration and depressed activity of protein C, indicating that this is a case of congenital protein C deficiency. Warfarin therapy has been continued to reduce the prothrombin time to 70% of the normal control level, resulting in no further episodes of thrombosis.
Takeda
K
K
Department of Internal Medicine III, Osaka University School of Medicine, Japan.
Kumagai
H
H
Hayashi
S
S
Tanio
Y
Y
Kawase
I
I
Kishimoto
T
T
Fujiwara
M
M
D005260:Female; D006801:Humans; D008875:Middle Aged; D011486:Protein C / Q000235:genetics; D020151:Protein C Deficiency; D011517:Prothrombin Time; D011655:Pulmonary Embolism / Q000188:drug therapy / Q000209:etiology*; D012008:Recurrence; D013924:Thrombophlebitis / Q000188:drug therapy / Q000209:etiology*; D014859:Warfarin / Q000008:administration & dosage / Q000627:therapeutic use
D011486:Protein C; D014859:Warfarin