Japanese Journal of Thrombosis and Hemostasis
Online ISSN : 1880-8808
Print ISSN : 0915-7441
ISSN-L : 0915-7441
Hereditary Protein S Deficiency Associated with Dysplasminogenemia. A Case of Deep vein Thrombosis with Various Clinical Symptoms
Takashi FUJINAGAShigenori YUTANIHiroshi TAMURAKoji SUZUKI
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JOURNAL FREE ACCESS

1990 Volume 1 Issue 4 Pages 330-336

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Abstract

Protein S is a vitamin K-dependent plasma protein which acts as a cofactor for an anticoagulant, activated protein C.
We observed a patient with recurrent deep vein thrombosis complicated by fever, central nervus system involvement and acute abdomen. This case, a 17-year-old Japanese male, was hospitalized on 13-year-old at the first time. He complained fever, chest pain, and convulsion of unknown origin. Laboratory studies showed an elevated erythrocyte sedimentation rate and an elevated C-reactive protein. He had a meningismus and a severe abdominal pain (acute abdomen), but no inflammatory change. A phleborreogram showed evidence of left femoral deep venous thrombosis. He had recurrent thrombosis and familial thrombosis. The family analysis revealed that the reduced levels of protein S activity, total and free forms of protein S antigen and plasminogen activity in his father and himself. This case suggests that patients with a protein S deficiency associated with dysplasminogenemia may have various clinical features.

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© The Japanese Society on Thrombosis and Hemostasis
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