Literature DB >> 1533892

[The clinical importance of protein C and S deficiency for surgical patients].

H Rabl1, H Fruhwirth.   

Abstract

Protein C and S are important factors in blood coagulation reported in many papers about people who suffered from thromboembolic diseases related to inherited or acquired deficiencies. Homozygous protein C/S deficiency is lethal in most cases without therapy. Heterozygous deficiency is moderate and complications occur between the 20.-50. year of age. Acquired protein C/S deficiency is a strong parameter for liver function. The typical clinical manifestations of protein C/S deficiencies are superficial and deep leg vein thrombosis, thrombosis of the mesenterial, cerebral, renal and axillary veins, portal vein thrombosis and pulmonary embolism. Most of the affected people live disease free over a longer period and develop thromboembolic complications during and after trauma, surgical interventions, pregnancy and puerperium. We report our experience with a 60 years old male who had developed a severe bilateral iliofemoral vein thrombosis with signs of pulmonary embolism after total hip replacement. An extended functional protein C deficiency (type II) was investigated by coagulation tests (Protein C Reagent, coagulometric from Behring Institute). A second female patient developed a descending iliofemoral vein thrombosis during pregnancy. Venous thrombectomy with arteriovenous fistula was performed, but reocclusion occurred after delivery. Redo-surgery was undertaken and a second reocclusion took place 10 days later. Further lysis therapy was not able to reopen the venous system. Whereas immunological and functional protein C levels showed normal ranges, the functional protein S level was markedly reduced (IL-Instrumentation Laboratory Protein S-Test).

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Year:  1992        PMID: 1533892     DOI: 10.1007/bf00184339

Source DB:  PubMed          Journal:  Langenbecks Arch Chir        ISSN: 0023-8236


  79 in total

1.  Inactivation of factor VIII by activated protein C and protein S.

Authors:  F J Walker; S I Chavin; P J Fay
Journal:  Arch Biochem Biophys       Date:  1987-01       Impact factor: 4.013

2.  Activated protein C stimulates the fibrinolytic activity of cultured endothelial cells and decreases antiactivator activity.

Authors:  Y Sakata; S Curriden; D Lawrence; J H Griffin; D J Loskutoff
Journal:  Proc Natl Acad Sci U S A       Date:  1985-02       Impact factor: 11.205

3.  Inhibition of platelet-dependent thrombus formation by human activated protein C in a primate model.

Authors:  A Gruber; J H Griffin; L A Harker; S R Hanson
Journal:  Blood       Date:  1989-02-15       Impact factor: 22.113

4.  Deficiencies of protein C, an inhibitor of blood coagulation.

Authors:  P M Mannucci; S Vigano
Journal:  Lancet       Date:  1982-08-28       Impact factor: 79.321

5.  Deficiency of protein C inhibitor in combined factor V/VIII deficiency disease.

Authors:  R A Marlar; J H Griffin
Journal:  J Clin Invest       Date:  1980-11       Impact factor: 14.808

6.  Liver dysfunction rather than intravascular coagulation as the main cause of low protein C and antithrombin III in acute leukemia.

Authors:  F Rodeghiero; P M Mannucci; S Viganò; T Barbui; L Gugliotta; M Cortellaro; E Dini
Journal:  Blood       Date:  1984-04       Impact factor: 22.113

7.  Radioimmunoassays for protein C and factor X. Plasma antigen levels in abnormal hemostatic states.

Authors:  D J Epstein; P W Bergum; S P Bajaj; S I Rapaport
Journal:  Am J Clin Pathol       Date:  1984-11       Impact factor: 2.493

8.  Homozygous protein C deficiency combined with heterozygous dysplasminogenemia found in a 21-year-old thrombophilic male.

Authors:  S Manabe; M Matsuda
Journal:  Thromb Res       Date:  1985-08-01       Impact factor: 3.944

9.  Inherited hypercoagulable states in children.

Authors:  J A Whitlock; R L Janco; J A Phillips
Journal:  Am J Pediatr Hematol Oncol       Date:  1989

10.  Isolation and sequence of the cDNA for human protein S, a regulator of blood coagulation.

Authors:  A Lundwall; W Dackowski; E Cohen; M Shaffer; A Mahr; B Dahlbäck; J Stenflo; R Wydro
Journal:  Proc Natl Acad Sci U S A       Date:  1986-09       Impact factor: 11.205

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