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Pier Mannuccio Mannucci - One of the best experts on this subject based on the ideXlab platform.
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Inhibitors of propagation of Coagulation (faCtors VIII, IX and XI): a review of Current therapeutiC praCtiCe
British Journal of Clinical Pharmacology, 2011Co-Authors: Massimo Franchini, Pier Mannuccio MannucciAbstract:The management of patients with Congenital Haemophilia who develop alloantibodies against faCtors of the propagation phase of blood Coagulation, Commonly known as inhibitors, is the most important Challenge faCing Haemophilia Caregivers at present, as this CompliCation not only Compromises the effiCaCy of replaCement therapy but also Consumes an enormous amount of eConomiC resourCes. Development of inhibitors further CompliCates the CliniCal Course of severe Haemophilia, with a prevalenCe of up to 30% in patients with Haemophilia A (faCtor VIII defiCienCy) and up to 5% in those with Haemophilia B (faCtor IX defiCienCy) and Haemophilia C (faCtor XI defiCienCy). While the short-term goal of treatment of patients who develop alloantibodies is the Control of bleeding, the eradiCation of the inhibitor is the main long-term goal. The management of severe bleeding episodes and the eradiCation of the autoantibody are also the mainstays of treatment of patients with aCquired Haemophilia, a rare but life-threatening haemorrhagiC Condition CharaCterized by the development of inhibitory autoantibodies against Coagulation faCtor VIII. The most reCent options available for treating patients with Congenital Haemophilia CompliCated by inhibitors and aCquired Haemophilia beCause of autoantibodies against faCtor VIII are summarized in this review artiCle.
Massimo Franchini - One of the best experts on this subject based on the ideXlab platform.
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Inhibitors of propagation of Coagulation (faCtors VIII, IX and XI): a review of Current therapeutiC praCtiCe
British Journal of Clinical Pharmacology, 2011Co-Authors: Massimo Franchini, Pier Mannuccio MannucciAbstract:The management of patients with Congenital Haemophilia who develop alloantibodies against faCtors of the propagation phase of blood Coagulation, Commonly known as inhibitors, is the most important Challenge faCing Haemophilia Caregivers at present, as this CompliCation not only Compromises the effiCaCy of replaCement therapy but also Consumes an enormous amount of eConomiC resourCes. Development of inhibitors further CompliCates the CliniCal Course of severe Haemophilia, with a prevalenCe of up to 30% in patients with Haemophilia A (faCtor VIII defiCienCy) and up to 5% in those with Haemophilia B (faCtor IX defiCienCy) and Haemophilia C (faCtor XI defiCienCy). While the short-term goal of treatment of patients who develop alloantibodies is the Control of bleeding, the eradiCation of the inhibitor is the main long-term goal. The management of severe bleeding episodes and the eradiCation of the autoantibody are also the mainstays of treatment of patients with aCquired Haemophilia, a rare but life-threatening haemorrhagiC Condition CharaCterized by the development of inhibitory autoantibodies against Coagulation faCtor VIII. The most reCent options available for treating patients with Congenital Haemophilia CompliCated by inhibitors and aCquired Haemophilia beCause of autoantibodies against faCtor VIII are summarized in this review artiCle.
Anat Dekel - One of the best experts on this subject based on the ideXlab platform.
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HystereCtomy in a woman with faCtor XI defiCienCy.
Journal of Obstetrics and Gynaecology, 2000Co-Authors: David Rabinerson, Dror Dicker, Zion Ben-rafael, Anat DekelAbstract:IntroduCtion FaCtor XI de® CienCy (F11D) is a rare disorder, ® rst desCribed in 1953 byRosenthal and Colleagues, who termed it `Haemophilia C’ . It is also known as plasma thromboplastin anteCedent de® CienCy (Rosenthal et al., 1955). F11D is CharaCterised by inComplete reCessive autosomal inheritanCe and has a relatively high prevalenCe among Ashkenazi Jews (PurCell and Nossel, 1970), though about half of all desCribed patients were of non-Jewish origin (Ragni et al., 1985). Symptoms are less severe than for the other Coagulation de® CienCies (Ragni et al., 1985). AffeCted individuals show enhanCed bleeding after surgery (KitChens, 1991). Bolton-Maggs (1996) Claimed that bleeding often follows minor surgery but, paradoxiCally, not after major surgery (Ragni et al., 1985). SinCe F11D is the most Common Congenital Coagulation protein abnormality in women, there has been some experienCe with this disorder during pregnanCy (Phillips et al., 1973) and delivery inCluding Caesarean seCtion. Following on medline searCh, however, this is the ® rst report of a hystereCtomy in a F11D patient.
Tadej Pajič - One of the best experts on this subject based on the ideXlab platform.
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Ebstein\'s anomaly and a rare Coagulation disorder – Case report and review of literature
Slovenian Medical Journal, 2009Co-Authors: Jure Dolenc, Samo Zver, Mirta Koželj, Martina Fink, Tadej PajičAbstract:BaCkgrounds: Ebstein’s anomaly of triCuspid valve is a rare Congenital heart anomaly. SurgiCal implantation of artefi Cial triCuspid valve is a standard treatment modality. Common late CompliCation of suCh surgiCal proCedure is artefi Cial valve thrombosis. Th is is a life threatening Condition, whiCh demands surgiCal intervention promptly. ConClusions: In following artiCle, we desCribe a Case of a patient with Ebstein’s anomaly and late artefi Cial triCuspid valve thrombosis. Th e Case was CompliCated with a mild form of Haemophilia C, a rare Coagulation disorder. GenetiC analysis was also performed.
David Rabinerson - One of the best experts on this subject based on the ideXlab platform.
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HystereCtomy in a woman with faCtor XI defiCienCy.
Journal of Obstetrics and Gynaecology, 2000Co-Authors: David Rabinerson, Dror Dicker, Zion Ben-rafael, Anat DekelAbstract:IntroduCtion FaCtor XI de® CienCy (F11D) is a rare disorder, ® rst desCribed in 1953 byRosenthal and Colleagues, who termed it `Haemophilia C’ . It is also known as plasma thromboplastin anteCedent de® CienCy (Rosenthal et al., 1955). F11D is CharaCterised by inComplete reCessive autosomal inheritanCe and has a relatively high prevalenCe among Ashkenazi Jews (PurCell and Nossel, 1970), though about half of all desCribed patients were of non-Jewish origin (Ragni et al., 1985). Symptoms are less severe than for the other Coagulation de® CienCies (Ragni et al., 1985). AffeCted individuals show enhanCed bleeding after surgery (KitChens, 1991). Bolton-Maggs (1996) Claimed that bleeding often follows minor surgery but, paradoxiCally, not after major surgery (Ragni et al., 1985). SinCe F11D is the most Common Congenital Coagulation protein abnormality in women, there has been some experienCe with this disorder during pregnanCy (Phillips et al., 1973) and delivery inCluding Caesarean seCtion. Following on medline searCh, however, this is the ® rst report of a hystereCtomy in a F11D patient.
