Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications

References

• Alhenc-GelasMEmmerichJGandrilleS1995Protein C infusion in a patient with inherited protein C deficiency caused by two missense mutations: Arg 178 to Gln and Arg-1 to HisBlood Coagul Fibrinolysis635417795152
   PubMed Web of Science ®Google Scholar
• AllaartCFPoortSRRosendaalFR1993Increased risk of venous thrombosis in carriers of hereditary protein C deficiency defectLancet34113488093743
   PubMed Web of Science ®Google Scholar
• AubergerK1992Evaluation of a new protein-C concentrate and comparison of protein-C assays in a child with congenital protein-C deficiencyAnn Hematol64146511571410
   PubMed Web of Science ®Google Scholar
• BaligaVThwaitesRTillyerML1995Homozygous protein C deficiency-management with protein C concentrateEur J Pediatr15453487556318
   PubMed Web of Science ®Google Scholar
• BarnesCNewallFHigginsS2002Perinatal management of patients at high risk of homozygous protein C deficiencyThromb Haemost88370112195720
   PubMed Web of Science ®Google Scholar
• BeckmanRJSchmidtRJSanterreRF1985The structure and evolution of a 461 amino acid human protein C precursor and its messenger RNA, based upon the DNA sequence of cloned human liver cDNA’sNucl Acids Res1352332991859
   PubMed Web of Science ®Google Scholar
• BertinaRMBroekmansAWvan der LindenIK1982Protein C deficiency in a Dutch family with thrombotic diseaseThromb Haemost48156897135
   PubMed Web of Science ®Google Scholar
• BertinaRMKoelemanBPCKosterT1994Mutation in blood coagulation factor V associated with resistance to activated protein CNature3696478164741
   PubMed Web of Science ®Google Scholar
• BovillEGBauerKADickermanJD1989The clinical-spectrum of heterozygous protein C deficiency in a large new England KindredBlood7371272521802
   PubMed Web of Science ®Google Scholar
• BransonHEKatzKMarbleR1983Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infantLancet2116586139528
   PubMed Web of Science ®Google Scholar
• BroekmansAWBertinaRMLoeligerEA1983Protein C and the development of skin necrosis during anticoagulant therapyThromb Haemost49244
   Web of Science ®Google Scholar
• BrueckmannMHornSLangS2005Recombinant human activated protein C upregulates cyclooxygenase-2 expression in endothelial cells via binding to endothelial cell protein C receptor and activation of protease-activated receptor-1Thromb Haemost937435015841323
   PubMed Web of Science ®Google Scholar
• ClarkeRCJohnstonJRMayneEE2000Meningococcal septicaemia: treatment with protein C concentrateIntensive Care Med26471310872142
   PubMed Web of Science ®Google Scholar
• ConardJBauerKAGruberA1993Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiencyBlood8241159648353282
   PubMed Web of Science ®Google Scholar
• DahlbäckBCarlssonMSvenssonPJ1993Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: Prediction of a cofactor to activated protein CProc Natl Acad Sci USA9010048430067
   PubMed Web of Science ®Google Scholar
• DahlbäckBStenfloJ1994The protein C anticoagulant system. In The Molecular Basis of Blood DiseasesPhiladelphiaW.B. Saunders Company599
   Google Scholar
• De FouwNJde JongYFHaverkateF1988Activated protein C increases fibrin clot lysis by neutralization of plasminogen activator inhibitor – no evidence for a cofactor role of protein SThromb Haemost60328332975409
   PubMed Web of Science ®Google Scholar
• De KleijnEDde GrootRHackCE2003Activation of protein C following infusion of protein C concentrate in children with severe meningococcal sepsis and purpura fulminans: a randomized, double-blinded, placebo-controlled, dose-finding studyCrit Care Med3118394712794428
   PubMed Web of Science ®Google Scholar
• De StefanoVMastrangeloSSchwarzHP1993Replacement therapy with a purified protein C concentrate during initiation of oral anticoagulation in severe protein C congenital deficiencyThromb Haemost7024798236128
   PubMed Web of Science ®Google Scholar
• DreyfusMMagnyJFBrideyF1991Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrateN Engl J Med325156581944440
   PubMed Web of Science ®Google Scholar
• DreyfusMMastersonMDavidM1995Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiencySemin Thromb Hemost21371818747700
   PubMed Web of Science ®Google Scholar
• DreyfusMLadouziAChambostH2007Treatment of inherited protein C deficiency by replacement therapy with the French purified plasma-derived protein C concentrate (PROTEXEL)Vox Sang932334017845261
   PubMed Web of Science ®Google Scholar
• EsmonCT1987The regulation of natural anticoagulant pathwaysScience2351348523029867
   PubMed Web of Science ®Google Scholar
• EsmonCT1989The roles of protein C and thrombomodulin in the regulation of blood coagulationJ Biol Chem264474362538457
   PubMed Web of Science ®Google Scholar
• EsmonCT2001Role of coagulation inhibitors in inflammationThromb Haemost8651611487041
   PubMed Web of Science ®Google Scholar
• EsmonCT2003The protein C pathwayChest1243 Suppl26S32S12970121
   PubMed Web of Science ®Google Scholar
• EsmonCT2006Inflammation and the activated protein C anticoagulant pathwaySemin Thromb Hemost32Suppl 1496016673266
   PubMed Web of Science ®Google Scholar
• EsmonCTXuJGuJM1999Endothelial Protein C ReceptorThromb Haemost82251810605711
   PubMed Web of Science ®Google Scholar
• EttingshausenCEVeldmanABeegT1999Replacement therapy with protein C concentrate in infants and adolescents with meningococcal sepsis and purpura fulminansSemin Thromb Hemost255374110632475
   PubMed Web of Science ®Google Scholar
• FavierRDeschampsABelhocineR1998Simultaneous administration of antithrombin III and protein C concentrates for the treatment of a devastating coagulopathy in a childHematol Cell Ther4067709615249
   PubMedGoogle Scholar
• FeistritzerCRiewaldM2005Endothelial barrier protection by activated protein C through PAR1-dependent sphingosine 1–phosphate receptor-1 crossactivationBlood10531788415626732
   PubMed Web of Science ®Google Scholar
• FloodEPRedishMHBociekSJ1943Thrombophlebitis migrans disseminata : report of a case in which gangrene of a breast occuredN J State J Med4311214
   Google Scholar
• FouassierMMoreauDThiolliereF2005Evolution of thrombin formation and fibrinolysis markers, including thrombin-activatable fibrinolysis inhibitor, during severe meningococcemiaPathophysiol Haemost Thromb34284716772741
   PubMedGoogle Scholar
• FourrierFChopinCGoudemandJ1992Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficienciesChest101816231531791
   PubMed Web of Science ®Google Scholar
• FourrierFLeclercFAidanK2003Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic studyIntensive Care Med291081712761614
   PubMed Web of Science ®Google Scholar
• GattiLCarnelliVRusconiR2003Heparin-induced thrombocytopenia and warfarin-induced skin necrosis in a child with severe protein C deficiency: successful treatment with dermatan sulfate and protein C concentrateJ Thromb Haemost1387812871519
   PubMed Web of Science ®Google Scholar
• GersonWTDickermanJDBovillEG1993Severe acquired protein C deficiency in purpura fulminans associated with disseminated intra-vascular coagulation: treatment with protein C concentratePediatrics91418228424021
   PubMed Web of Science ®Google Scholar
• GoodwinTMGazitGGordonEM1995Heterozygous protein C deficiency presenting as severe protein C deficiency and peripartum thrombosis: successful treatment with protein C concentrateObstet Gynecol8666247675405
   PubMed Web of Science ®Google Scholar
• GreengardJSFisherCLVilloutreixB1994Structural basis for Type I and Type II deficiencies of antithrombotic plasma protein C: Patterns revealed by three-dirnensional molecular modelling of mutations of the protease domainProteins183678208728
   PubMed Web of Science ®Google Scholar
• GriffinJHEvattBZimmermanTS1981Deficiency of protein C in congenital thrombotic diseaseJ Clin Invest68137036895379
   PubMed Web of Science ®Google Scholar
• HattenbachLOBeegTKreuzW1999Ophthalmic manifestation of congenital protein C deficiencyJ AAPOS31889010428594
   PubMed Web of Science ®Google Scholar
• JoyceDEGrinnellBW2002Recombinant human activated protein C attenuates the inflammatory response in endothelium and monocytes by modulating nuclear factor-kappaBCrit Care Med305 SupplS2889312004250
   PubMed Web of Science ®Google Scholar
• KellyJGO’MalleyK1979Clinical pharmakokinetics of oral anticoagulantsClin Pharmacokinet4111369763
   PubMed Web of Science ®Google Scholar
• KreuzWVeldmanAEscuriola-EttingshausenC1998Protein-C concentrate for meningococcal purpura fulminansLancet35198679734967
   PubMed Web of Science ®Google Scholar
• LaneDAMannucciPMBauerKA1997Inherited thrombophilia: part 2Thromb Haemost76824348971998
   PubMed Web of Science ®Google Scholar
• LeclercFCremerRLeteurtreS2000Protein C concentrate and recombinant tissue plasminogen activator in meningococcal septic shockCrit Care Med281694710834762
   PubMed Web of Science ®Google Scholar
• LewandowskiKZawilskaK1994Protein C concentrate in the treatment of warfarin-induced skin necrosis in the protein C deficiencyThromb Haemost713958029804
   PubMed Web of Science ®Google Scholar
• MaciasWLNelsonDR2004Severe protein C deficiency predicts early death in severe sepsisCrit Care Med32SupplS223815118522
   PubMed Web of Science ®Google Scholar
• MestersRMHelterbrandJUtterbackBG2000Prognostic value of protein C concentrations in neutropenic patients at high risk of severe septic complicationsCrit Care Med2822091610921542
   PubMed Web of Science ®Google Scholar
• Manco-JohnsonMNussR1992Protein C concentrate prevents peripartum thrombosisAm J Hematol4069701566751
   PubMed Web of Science ®Google Scholar
• Manco-JohnsonMJKnapp-ClevengerR2004Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiencyJ Pediatr Hematol Oncol2625714707707
   PubMed Web of Science ®Google Scholar
• MarlarRAMontgomeryRRBroekmansAW1989Diagnosis and treatment of homozygous protein C deficiency. Report of the Working Party on Homozygous Protein C Deficiency of the Subcommittee on Protein C and Protein S, International Committee on Thrombosis and HaemostasisJ Pediatr114528342647943
   PubMed Web of Science ®Google Scholar
• MathiasMKhairKBurgessC2004Subcutaneous administration of protein C concentratePediatr Hematol Oncol21551615552819
   PubMed Web of Science ®Google Scholar
• MinfordAMParapiaLAStainforthCLeeD1996Treatment of homozygous protein C deficiency with subcutaneous protein C concentrateBr J Haematol9321568611462
   PubMed Web of Science ®Google Scholar
• MosnierLOZlokovicBVGriffinJH2007The cytoprotective protein C pathwayBlood10931617217110453
   PubMed Web of Science ®Google Scholar
• MullerFMEhrenthalWHafnerG1996Purpura fulminans in severe congenital protein C deficiency: monitoring of treatment with protein C concentrateEur J Pediatr1552058750805
   PubMed Web of Science ®Google Scholar
• MustafaSMannhalterCRintelenC1998Clinical features of thrombophilia in families with gene defects in protein C or protein S combined with factor V LeidenBlood Coagul Fibrinolysis98599607123
   PubMed Web of Science ®Google Scholar
• NakayamaTMatsushitaTHidanoH2000A case of purpura fulminans is caused by homozygous delta8857 mutation (protein C-nagoya) and successfully treated with activated protein C concentrateBr J Haematol1107273010997987
   PubMed Web of Science ®Google Scholar
• OkajimaK2001Regulation of inflammatory responses by natural anticoagulantsImmunol Rev1842587411918684
   PubMed Web of Science ®Google Scholar
• PabingerIAllaartCFHermansJ1992Hereditary protein C-deficiency. Laboratory values in transmitters and guidelines for the diagnostic procedure. Report on a study of the SSC Subcommittee on Protein C and Protein SThromb Haemost6847041448781
   PubMed Web of Science ®Google Scholar
• PabingerIKyrlePAHeistingerM1994The risk of thrombosis in asymptomatic patients with hereditary protein C or protein S deficiency. A prospective cohort studyThromb Haemost7144158052960
   PubMed Web of Science ®Google Scholar
• PabingerISchneiderB1996Thrombotic risk in hereditary antithrombin III, protein C, or protein S deficiency. A cooperative, retrospective study. Gesellschaft fuer Thrombose- und Haemostaseforschung (GTH) Study Group on Natural InhibitorsArterioscler Thromb Vasc Biol1674288640401
   PubMed Web of Science ®Google Scholar
• RadosevichMZhou1F-LHuartJ-J2003Chromatographic purification and properties of a therapeutic human protein C concentrateJ Chromatography790199207
   Web of Science ®Google Scholar
• ReitsmaPHBenuvdiFDoigRG1995Protein C deficiency- a database of mutations, 1995 update. On behalf of the Subcommittee on Plasma Coagulation Inhibitors of the Scientifie and Standardization Committee of the ISTHThromb Haemost73876897482420
   PubMed Web of Science ®Google Scholar
• ReitsmaPH1997Protein C deficiency: from gene defects to diseaseThromb Haemost78334509198175
   PubMed Web of Science ®Google Scholar
• RichardsEMMakrisMPrestonFE1997The successful use of protein C concentrate during pregnancy in a patient with type 1 protein C deficiency, previous thrombosis and recurrent fetal lossBr J Haematol9866019332323
   PubMed Web of Science ®Google Scholar
• RiewaldMPetrovanRJDonnerA2002Activation of endothelial cell protease activated receptor 1 by the protein C pathwayScience2961880212052963
   PubMed Web of Science ®Google Scholar
• RintalaESeppalaOPKotilainenP1998Protein C in the treatment of coagulopathy in meningococcal diseaseCrit Care Med2696589590329
   PubMed Web of Science ®Google Scholar
• RintalaEKauppilaMSeppalaOP2000Protein C substitution in sepsis-associated purpura fulminansCrit Care Med282373810921567
   PubMed Web of Science ®Google Scholar
• RivardGEDavidMFarrellC1995Treatment of purpura fulminans in meningococcemia with protein C concentrateJ Pediatr126646527699550
   PubMed Web of Science ®Google Scholar
• SalonvaaraMKuismanenKMononenT2004Diagnosis and treatment of a newborn with homozygous protein C deficiencyActa Paediatr93137914989454
   PubMed Web of Science ®Google Scholar
• SansonBJSimioniPTormeneD1999The incidence of venous thromboembolism in asymptomatic carriers of a deficiency of anti-thrombin, protein C, or protein S: a prospective cohort studyBlood943702610572082
   PubMed Web of Science ®Google Scholar
• Sanz-RodriguezCGil-FernandezJJZapaterP1999Long-term management of homozygous protein C deficiency: replacement therapy with subcutaneous purified protein C concentrateThromb Haemost818879010404762
   PubMed Web of Science ®Google Scholar
• SchrammWSpannaglMBauerKA1993Treatment of coumarin-induced skin necrosis with a monoclonal antibody purified protein C concentrateArch Dermatol12975368507079
   PubMedGoogle Scholar
• SekiyamaKItohHSagawaN2003Successful management of a pregnant woman with heterozygous protein C deficiency using activated protein C concentrateJ Obstet Gynaecol Res29412514641692
   PubMed Web of Science ®Google Scholar
• SharonCTirindelliMCMannucciPM1986Homozygous protein C deficiency with moderately severe clinical symptomsThromb Res4148383754354
   PubMed Web of Science ®Google Scholar
• ShimamuraKOkaKNakazawaM1983Distribution patterns of microthrombi in disseminated intravascular coagulationArch Path Lab Med10754376688518
   PubMed Web of Science ®Google Scholar
• SmithOPWhiteBVaughanD1997Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminansLancet350159039393338
   PubMed Web of Science ®Google Scholar
• StenfloJ1976A new vitamin K-dependent protein. Purification from bovine plasma and preliminary characterizationJ Biol Chem251355631245477
   PubMed Web of Science ®Google Scholar
• SugimotoMMaruhashiYKobayashiA1997Activated protein C concentrate: a new tool for the treatment of acute thromboembolism in patients with congenital protein C deficiencyThromb Haemost77122349241766
   PubMed Web of Science ®Google Scholar
• Tardy-PoncetBRayetIDamonG2001Protein C concentrates in a neonate with a cerebral venous thrombosis due to heterozygous type 1 protein C deficiencyThromb Haemost851118911434695
   PubMed Web of Science ®Google Scholar
• ToupanceONguyenPBrandtB1994Prevention of vascular thrombosis by human purified protein C concentrate in a patient with familial PC deficiency undergoing renal transplantationTranspl Int714458179804
   PubMed Web of Science ®Google Scholar
• VaccarellaGPelellaR2003Replacement treatment with protein C in an 18-year-old man with meningococcal sepsis and purpura fulminansMinerva Anestesiol696935
   Google Scholar
• Van de WouwerMCollenDConwayEM2004Thrombomodulin-protein C-EPCR system: integrated to regulate coagulation and inflammationArterioscler Thromb Vasc Biol2413748315178554
   PubMed Web of Science ®Google Scholar
• VukovichTAubergerKWeilJ1988Replacement therapy for a homozygous protein C deficiency-state using a concentrate of human protein C and SBr J Haematol70435402975502
   PubMed Web of Science ®Google Scholar
• WhiteBSchmidtMMurphyC2000aActivated protein C inhibits lipopolysaccharide-induced nuclear translocation of nuclear factor kappaB (NF-kappaB) and tumour necrosis factor alpha (TNF-alpha) production in the THP-1 monocytic cell lineBr J Haemato1101304
   PubMed Web of Science ®Google Scholar
• WhiteBLivingstoneWMurphyC2000bAn open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemiaBlood9637192411090052
   PubMed Web of Science ®Google Scholar
• YanSBHelterbrandJDHartmanDL2001Low levels of protein C are associated with poor outcome in severe sepsisChest1209152211555529
   PubMed Web of Science ®Google Scholar
• YeXFukudomeKTsuneyoshiN1999The endothelial cell protein C receptor (EPCR) functions as a primary receptor for protein C activation on endothelial cells in arteries, veins, and capillariesBiochem Biophys Res Commun259671710364477
   PubMed Web of Science ®Google Scholar