Protein S Deficiency Differential Diagnoses
- Author: John E Godwin, MD, MS; Chief Editor: Emmanuel C Besa, MD more...
Castoldi E, Hackeng TM. Regulation of coagulation by protein S. Curr Opin Hematol. Sep 2008;15(5):529-36. [Medline].
Soare AM, Popa C. Deficiencies of proteins C, S and antithrombin and activated protein C resistance--their involvement in the occurrence of Arterial thromboses. J Med Life. Oct-Dec 2010;3(4):412-5. [Medline]. [Full Text].
Castoldi E, Maurissen LF, Tormene D, Spiezia L, Gavasso S, Radu C, et al. Similar hypercoagulable state and thrombosis risk in type I and type III protein S-deficient individuals from families with mixed type I/III protein S deficiency. Haematologica. Sep 2010;95(9):1563-71. [Medline]. [Full Text].
Marlar RA, Gausman JN. Protein S abnormalities: a diagnostic nightmare. Am J Hematol. May 2011;86(5):418-21. [Medline].
Heeb MJ, Prashun D, Griffin JH, Bouma BN. Plasma protein S contains zinc essential for efficient activated protein C-independent anticoagulant activity and binding to factor Xa, but not for efficient binding to tissue factor pathway inhibitor. FASEB J. Jul 2009;23(7):2244-53. [Medline].
Lijfering WM, Brouwer JL, Veeger NJ, Bank I, Coppens M, Middeldorp S, et al. Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives. Blood. May 21 2009;113(21):5314-22. [Medline].
Brouwer JL, Lijfering WM, Ten Kate MK, Kluin-Nelemans HC, Veeger NJ, van der Meer J. High long-term absolute risk of recurrent venous thromboembolism in patients with hereditary deficiencies of protein S, protein C or antithrombin. Thromb Haemost. Jan 2009;101(1):93-9. [Medline].
Edlich RF, Cross CL, Dahlstrom JJ, Long WB 3rd. Modern concepts of the diagnosis and treatment of purpura fulminans. J Environ Pathol Toxicol Oncol. 2008;27(3):191-6. [Medline].
Alvi AR, Khan S, Niazi SK, Ghulam M, Bibi S. Acute mesenteric venous thrombosis: improved outcome with early diagnosis and prompt anticoagulation therapy. Int J Surg. Jun 2009;7(3):210-3. [Medline].
Tripodi A, Asti D, Chantarangkul V, Biguzzi E, Mannucci PM. Interference of factor V Leiden on protein S activity: evaluation of a new prothrombin time-based assay. Blood Coagul Fibrinolysis. Sep 2007;18(6):543-6. [Medline].
Adachi T. Protein S and congenital protein S deficiency: the most frequent congenital thrombophilia in Japanese. Curr Drug Targets. Aug 2005;6(5):585-92. [Medline].
Baker ME, French FS, Joseph DR. Vitamin K-dependent protein S is similar to rat androgen-binding protein. Biochem J. Apr 1 1987;243(1):293-6. [Medline].
Brill-Edwards P, Ginsberg JS, Johnston M, Hirsh J. Establishing a therapeutic range for heparin therapy. Ann Intern Med. Jul 15 1993;119(2):104-9. [Medline].
Dahlback B, Villoutreix BO. The anticoagulant protein C pathway. FEBS Lett. Jun 13 2005;579(15):3310-6. [Medline].
Dahlbäck B, Stenflo J. High molecular weight complex in human plasma between vitamin K-dependent protein S and complement component C4b-binding protein. Proc Natl Acad Sci U S A. Apr 1981;78(4):2512-6. [Medline].
Di Scipio RG, Hermodson MA, Yates SG, Davie EW. A comparison of human prothrombin, factor IX (Christmas factor), factor X (Stuart factor), and protein S. Biochemistry. Feb 22 1977;16(4):698-706. [Medline].
Francis RB Jr. Protein S deficiency in sickle cell anemia. J Lab Clin Med. May 1988;111(5):571-6. [Medline].
Giri TK, Villoutreix BO, Wallqvist A, Dahlbäck B, de Frutos PG. Topological studies of the amino terminal modules of vitamin K-dependent protein S using monoclonal antibody epitope mapping and molecular modeling. Thromb Haemost. Nov 1998;80(5):798-804. [Medline].
Gomez E, Poort SR, Bertina RM, Reitsma PH. Identification of eight point mutations in protein S deficiency type I--analysis of 15 pedigrees. Thromb Haemost. May 1995;73(5):750-5. [Medline].
Hackeng TM, Rosing J. Protein S as cofactor for TFPI. Arterioscler Thromb Vasc Biol. Aug 6 2009;epub ahead of print. [Medline].
Hackeng TM, van 't Veer C, Meijers JC, Bouma BN. Human protein S inhibits prothrombinase complex activity on endothelial cells and platelets via direct interactions with factors Va and Xa. J Biol Chem. Aug 19 1994;269(33):21051-8. [Medline].
Heeb MJ, Rosing J, Bakker HM, Fernandez JA, Tans G, Griffin JH. Protein S binds to and inhibits factor Xa. Proc Natl Acad Sci U S A. Mar 29 1994;91(7):2728-32. [Medline].
Hillarp A, Dahlback B. Novel subunit in C4b-binding protein required for protein S binding. J Biol Chem. Sep 5 1988;263(25):12759-64. [Medline].
Ploos van Amstel HK, Huisman MV, Reitsma PH, Wouter ten Cate J, Bertina RM. Partial protein S gene deletion in a family with hereditary thrombophilia. Blood. Feb 1989;73(2):479-83. [Medline].
Ploos van Amstel HK, Reitsma PH, van der Logt CP, Bertina RM. Intron-exon organization of the active human protein S gene PS alpha and its pseudogene PS beta: duplication and silencing during primate evolution. Biochemistry. Aug 28 1990;29(34):7853-61. [Medline].
Ridker PM, Hennekens CH, Lindpaintner K, Stampfer MJ, Eisenberg PR, Miletich JP. Mutation in the gene coding for coagulation factor V and the risk of myocardial infarction, stroke, and venous thrombosis in apparently healthy men. N Engl J Med. Apr 6 1995;332(14):912-7. [Medline].
Schmidel DK, Nelson RM, Broxson EH Jr, Comp PC, Marlar RA, Long GL. A 5.3-kb deletion including exon XIII of the protein S alpha gene occurs in two protein S-deficient families. Blood. Feb 1 1991;77(3):551-9. [Medline].
Schmidel DK, Tatro AV, Phelps LG, Tomczak JA, Long GL. Organization of the human protein S genes. Biochemistry. Aug 28 1990;29(34):7845-52. [Medline].
Siddique RM, Siddique MI, Connors AF Jr, Rimm AA. Thirty-day case-fatality rates for pulmonary embolism in the elderly. Arch Intern Med. Nov 11 1996;156(20):2343-7. [Medline].
Simmonds RE, Zöller B, Ireland H, Thompson E, de Frutos PG, Dahlbäck B, et al. Genetic and phenotypic analysis of a large (122-member) protein S-deficient kindred provides an explanation for the familial coexistence of type I and type III plasma phenotypes. Blood. Jun 15 1997;89(12):4364-70. [Medline].
Tait RC, Walker ID, Reitsma PH, Islam SI, McCall F, Poort SR, et al. Prevalence of protein C deficiency in the healthy population. Thromb Haemost. Jan 1995;73(1):87-93. [Medline].
Walker MC, Garner PR, Keely EJ, Rock GA, Reis MD. Changes in activated protein C resistance during normal pregnancy. Am J Obstet Gynecol. Jul 1997;177(1):162-9. [Medline].
Watkins PC, Eddy R, Fukushima Y, Byers MG, Cohen EH, Dackowski WR, et al. The gene for protein S maps near the centromere of human chromosome 3. Blood. Jan 1988;71(1):238-41. [Medline].
Zhang D, Hao J, Yang N. Protein C and D-dimer are related to portal vein thrombosis in patients with liver cirrhosis. J Gastroenterol Hepatol. Aug 3 2009;epub ahead of print. [Medline].

