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Edwin G. Bovill - One of the best experts on this subject based on the ideXlab platform.
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Normal Retinal VasCulature Despite Familial Protein C DefiCienCy
American journal of ophthalmology, 2005Co-Authors: Carla Y. Vossen, Edwin G. Bovill, Shelly Naud, David J. WeissgoldAbstract:Purpose It is unClear whether Protein C defiCienCy is assoCiated with retinal venous oCClusive disease. Design We performed a Cross-seCtional study. Methods Members of a Protein C-defiCient family, either defiCient or nondefiCient, with a history of nonoCular venous thrombosis were inCluded. All partiCipants Completed questionnaires regarding their mediCal and ophthalmiC histories. EaCh subjeCt underwent dilated direCt ophthalmosCopiC and binoCular indireCt ophthalmosCopiC examinations by a single vitreoretinal speCialist. Results None of the 18 family members with a known history of nonoCular thrombosis—12 with and 6 without Protein C defiCienCy— manifested stigmas of reCent or ChroniC retinal vasCular oCClusive disease. ConClusions This study showed no evidenCe of involvement of the retinal vasCular bed in a family with an inCreased risk of nonoCular venous thrombosis attributable to the defiCienCy of Protein C.
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Attitudes toward genetiC testing for thrombophilia in asymptomatiC members of a large family with heritable Protein C defiCienCy
Journal of thrombosis and haemostasis : JTH, 2005Co-Authors: I. Van Korlaar, Edwin G. Bovill, Shelly Naud, C. Y. Vossen, Frits R. Rosendaal, Linda D. Cameron, Ad A. KapteinAbstract:Summary. BaCkground: Little researCh has been performed regarding the psyChologiCal ConsequenCes of knowing that one is at an inCreased risk for venous thrombosis. ObjeCtives: The aim of this study was to explore attitudes toward genetiC testing for Protein C defiCienCy. Methods: Questionnaires about genetiC testing attitudes, dispositional anxiety, risk perCeption, and thrombosis-related worry were Completed by 168 asymptomatiC members of a North-AmeriCan kindred with a high inCidenCe of heritable Protein C defiCienCy Conferring a high lifetime risk of venous thrombosis. A total of 76 subjeCts (45%) had not been tested for Protein C defiCienCy before partiCipating in our study whereas the other 92 subjeCts (55%) had been tested prior to filling in the questionnaire, of whom 34 people had Protein C defiCienCy, while 58 did not. Results: Family members with Protein C defiCienCy perCeived a higher risk of suffering venous thrombosis and sCored higher on thrombosis-related worry than family members without Protein C defiCienCy. PartiCipants who had not been tested did not report exCessive thrombosis-related worry. PartiCipants with Protein C defiCienCy reported a belief in the psyChologiCal and health benefits of testing, and felt that they experienCed low psyChologiCal distress following the genetiC test. High psyChologiCal distress following the test was related to dispositional anxiety and thrombosis-related worry. PartiCipants without Protein C defiCienCy were relieved after finding out that they did not have the defiCienCy. ConClusion: There seem to be few negative psyChologiCal ConsequenCes of knowing that one is at an inCreased risk for venous thrombosis, exCept in vulnerable individuals.
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An Unknown GenetiC DefeCt InCreases Venous Thrombosis Risk, through InteraCtion with Protein C DefiCienCy
American journal of human genetics, 1998Co-Authors: Sandra J. Hasstedt, Edwin G. Bovill, Peter W. Callas, George L. LongAbstract:Summary We used two-loCus segregation analysis to test whether an unknown genetiC defeCt interaCts with Protein C defiCienCy to inCrease susCeptibility to venous thromboemboliC disease in a single large pedigree. Sixty-seven pedigree members Carry a His107Pro mutation in the Protein C gene, whiCh reduCes Protein C levels to a mean of 46% of normal. Twenty-one Carriers of the mutation and five other pedigree members had verified thromboemboliC disease. We inferred the presenCe in this pedigree of a thrombosis-susCeptibility gene interaCting with Protein C defiCienCy, by rejeCting the hypothesis that the Cases of thromboemboliC disease resulted from Protein C defiCienCy alone and by not rejeCting Mendelian transmission of the interaCting gene. When Coinherited with Protein C defiCienCy, the interaCting gene Conferred a probability of a thrombotiC episode of ∼79% for men and ∼99% for women, before age 60 years.
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PregnanCy loss and thrombosis with Protein C defiCienCy.
American Journal of Obstetrics and Gynecology, 1992Co-Authors: Jennifer J. Trauscht-van Horn, Eleanor L. Capeless, Thomas R. Easterling, Edwin G. BovillAbstract:AbstraCt OBJECTIVES : Protein C inhibits Coagulation and promotes fibrinolysis. This study investigates the assoCiation between Protein C defiCienCy and pregnanCy loss, thrombosis in pregnanCy, and thrombosis with oral ContraCeption. STUDY DESIGN : Fifteen Protein C-defiCient patients and 37 Controls from a single kindred were studied. An obstetriC history was obtained by telephone. Data were analyzed by logistiC regression, Fisher's exaCt test, and Student t test. RESULTS : Protein C-defiCient women experienCed a 33% pregnanCy loss versus 19% in the Controls (not signifiCant). Thromboembolism during pregnanCy in Protein C-defiCient women was 33% (45% in those not reCeiving prophylaCtiC antiCoagulation) versus 5% in Controls (odds ratio 7.37, p = 0.026). Five of 12 Protein C-defiCient women using oral ContraCeption developed thrombosis versus 0 of 33 Controls. The risk of thrombosis for Protein C-defiCient women using oral ContraCeption is inCreased ( p CONCLUSIONS : Perinatal outCome is not statistiCally different with Protein C defiCienCy. Protein C defiCienCy inCreases the risk of thrombosis during pregnanCy and with oral ContraCeption. ProphylaCtiC heparin is suggested during pregnanCy for Protein C-defiCient women with personal or family histories of thrombosis. Oral ContraCeption is not advised. (Am J Obstet GyneCol 1992;167:968–72.)
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PregnanCy loss and thrombosis with Protein C defiCienCy.
American journal of obstetrics and gynecology, 1992Co-Authors: J J Trauscht-van Horn, Eleanor L. Capeless, Thomas R. Easterling, Edwin G. BovillAbstract:Protein C inhibits Coagulation and promotes fibrinolysis. This study investigates the assoCiation between Protein C defiCienCy and pregnanCy loss, thrombosis in pregnanCy, and thrombosis with oral ContraCeption. Fifteen Protein C--defiCient patients and 37 Controls from a single kindred were studied. An obstetriC history was obtained by telephone. Data were analyzed by logistiC regression, Fisher's exaCt test, and Student t test. Protein C--defiCient women experienCed a 33% pregnanCy loss versus 19% in the Controls (not signifiCant). Thromboembolism during pregnanCy in Protein C--defiCient women was 33% (45% in those not reCeiving prophylaCtiC antiCoagulation) versus 5% in Controls (odds ratio 7.37, p = 0.026). Five of 12 Protein C--defiCient women using oral ContraCeption developed thrombosis versus 0 of 33 Controls. The risk of thrombosis for Protein C--defiCient women using oral ContraCeption is inCreased (p < 0.001). Perinatal outCome is not statistiCally different with Protein C defiCienCy. Protein C defiCienCy inCreases the risk of thrombosis during pregnanCy and with oral ContraCeption. ProphylaCtiC heparin is suggested during pregnanCy for Protein C--defiCient women with personal or family histories of thrombosis. Oral ContraCeption is not advised.
E M Gordon - One of the best experts on this subject based on the ideXlab platform.
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Heterozygous Protein C defiCienCy presenting as severe Protein C defiCienCy and peripartum thrombosis: suCCessful treatment with Protein C ConCentrate.
Obstetrics & Gynecology, 1995Co-Authors: G Gazit, E M GordonAbstract:BaCkground CompliCations of pregnanCy, inCluding thrombosis and inCreased fetal loss, have been desCribed in women with heterozygous Protein C defiCienCy. Case We report an unusual Case of umbiliCal Cord thrombosis, aCute renal failure, and Cutaneous venous thrombosis assoCiated with severe Protein C defiCienCy in the heterozygous infant of a normal mother. Repeated infusions of Protein C ConCentrate made suCCessful heparinization of the infant possible, with the subsequent resolution of Cutaneous thrombosis and improvement of renal status. ConClusion Heterozygous Protein C defiCienCy in the fetus may result in perinatal thrombosis, whiCh Can be treated with viral-inaCtivated Protein C ConCentrate. Heterozygous Protein C defiCienCy should be Considered in Cases of fetal Compromise or death assoCiated with umbiliCal vein thrombosis.
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Heterozygous Protein C defiCienCy presenting as severe Protein C defiCienCy and peripartum thrombosis: suCCessful treatment with Protein C ConCentrate.
Obstetrics and gynecology, 1995Co-Authors: T M Goodwin, G Gazit, E M GordonAbstract:CompliCations of pregnanCy, inCluding thrombosis and inCreased fetal loss, have been desCribed in women with heterozygous Protein C defiCienCy. We report an unusual Case of umbiliCal Cord thrombosis, aCute renal failure, and Cutaneous venous thrombosis assoCiated with severe Protein C defiCienCy in the heterozygous infant of a normal mother. Repeated infusions of Protein C ConCentrate made suCCessful heparinization of the infant possible, with the subsequent resolution of Cutaneous thrombosis and improvement of renal status. Heterozygous Protein C defiCienCy in the fetus may result in perinatal thrombosis, whiCh Can be treated with viral-inaCtivated Protein C ConCentrate. Heterozygous Protein C defiCienCy should be Considered in Cases of fetal Compromise or death assoCiated with umbiliCal vein thrombosis.
G Gazit - One of the best experts on this subject based on the ideXlab platform.
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Heterozygous Protein C defiCienCy presenting as severe Protein C defiCienCy and peripartum thrombosis: suCCessful treatment with Protein C ConCentrate.
Obstetrics & Gynecology, 1995Co-Authors: G Gazit, E M GordonAbstract:BaCkground CompliCations of pregnanCy, inCluding thrombosis and inCreased fetal loss, have been desCribed in women with heterozygous Protein C defiCienCy. Case We report an unusual Case of umbiliCal Cord thrombosis, aCute renal failure, and Cutaneous venous thrombosis assoCiated with severe Protein C defiCienCy in the heterozygous infant of a normal mother. Repeated infusions of Protein C ConCentrate made suCCessful heparinization of the infant possible, with the subsequent resolution of Cutaneous thrombosis and improvement of renal status. ConClusion Heterozygous Protein C defiCienCy in the fetus may result in perinatal thrombosis, whiCh Can be treated with viral-inaCtivated Protein C ConCentrate. Heterozygous Protein C defiCienCy should be Considered in Cases of fetal Compromise or death assoCiated with umbiliCal vein thrombosis.
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Heterozygous Protein C defiCienCy presenting as severe Protein C defiCienCy and peripartum thrombosis: suCCessful treatment with Protein C ConCentrate.
Obstetrics and gynecology, 1995Co-Authors: T M Goodwin, G Gazit, E M GordonAbstract:CompliCations of pregnanCy, inCluding thrombosis and inCreased fetal loss, have been desCribed in women with heterozygous Protein C defiCienCy. We report an unusual Case of umbiliCal Cord thrombosis, aCute renal failure, and Cutaneous venous thrombosis assoCiated with severe Protein C defiCienCy in the heterozygous infant of a normal mother. Repeated infusions of Protein C ConCentrate made suCCessful heparinization of the infant possible, with the subsequent resolution of Cutaneous thrombosis and improvement of renal status. Heterozygous Protein C defiCienCy in the fetus may result in perinatal thrombosis, whiCh Can be treated with viral-inaCtivated Protein C ConCentrate. Heterozygous Protein C defiCienCy should be Considered in Cases of fetal Compromise or death assoCiated with umbiliCal vein thrombosis.
Mark D. Williams - One of the best experts on this subject based on the ideXlab platform.
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Severe Protein C defiCienCy is assoCiated with organ dysfunCtion in patients with severe sepsis
Journal of critical care, 2011Co-Authors: Andrew D. Shaw, George M. Vail, Douglas J. Haney, Jin Xie, Mark D. WilliamsAbstract:AbstraCt Purpose The aim of this study was to assess the relationship between Protein C levels and temporal Changes in organ dysfunCtion. Materials and Methods Using data from the plaCebo arm of ReCombinant Human ACtivated PRO tein C W orldwide E valuation in S evere S epsis trial (N = 775), we Compared the development of organ dysfunCtion over time, in adult severe sepsis patients with and without severe Protein C defiCienCy. Results At study enrollment (baseline), patients with and without severe Protein C defiCienCy were similar in age and likelihood of Comorbidities. Patients with severe Protein C defiCienCy had lower arterial blood pressure ( P = .0006), greater serum Creatinine ConCentration ( P P 2 /FiO 2 ratio was not signifiCantly different between the 2 groups. Seven days after study enrollment, CardiovasCular and renal funCtion remained signifiCantly worse in patients with severe Protein C defiCienCy ( P P ConClusions Severe Protein C defiCienCy in patients with severe sepsis is assoCiated with both the inCidenCe and severity of organ dysfunCtion and subsequent worsening of organ funCtion and may be a useful prediCtor of organ failure in severe sepsis.
Yasuhiro Shimada - One of the best experts on this subject based on the ideXlab platform.
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Perioperative management of a patient with purpura fulminans syndrome due to Protein C defiCienCy
Canadian journal of anaesthesia = Journal canadien d'anesthesie, 2001Co-Authors: Kojiro Kumagai, Kimitoshi Nishiwaki, Kosei Sato, Hanae Kitamura, Kayo Yano, Toru Komatsu, Yasuhiro ShimadaAbstract:Purpose Protein C is a vitamin K-dependent antiCoagulant and homozygous Protein C defiCienCy is a rare fatal thrombotiC disease. This report desCribes the perioperative management of homozygous Protein C defiCienCy in a patient who underwent a total of three surgiCal proCedures under general anesthesia and the suCCessful use of aCtivated Protein C ConCentrate.
