|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Treatment of haemophilia A/B patients comprises factor VIII (FVIII) or factor IX (FIX) concentrate replacement therapy, respectively.
|
31606899 |
2020 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Larger international studies comparing the clinical presentation and treatment modalities of mild clotting FVIII and FIX deficiencies in both haemophilia males and females should be encouraged.
|
31815335 |
2020 |
|
Hemophilia A
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
Patients with haemophilia A (HA) or B (HB) experience spontaneous limb- or life-threatening bleedings which are prevented by regular prophylactic intravenous infusions of the deficient coagulation factor (FVIII or FIX).
|
31676141 |
2020 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
The prevalences of HA and HB fall within the ranges reported in more developed countries; the consumption of FVIII and FIX was in line with that of other European countries (France, United Kingdom) and Canada.
|
30865582 |
2020 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
It is recommended that each haemophilia centre should ensure that appropriate laboratory assays are available for FVIII and FIX products in local clinical use.
|
31846168 |
2020 |
|
Hemophilia A
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
Severe hemophilia is classically characterized by a factor VIII (FVIII) or factor IX (FIX) coagulant activity below 1% of normal (spontaneous and severe bleeds).
|
31517708 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Hemophilia A and B, diseases caused by the lack of factor VIII (FVIII) and factor IX (FIX) respectively, lead to insufficient thrombin production, and therefore to bleeding.
|
31383642 |
2019 |
|
Hemophilia A
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
It has been proved that the level of factor IX (FIX) is lesser with haemophilia patient and the attempt here is focused to quantify FIX level by interdigitated electrode (IDE) sensor.
|
31743722 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Hemophilia A (HA) and hemophilia B (HB) are X-linked bleeding disorders due to inheritable deficiencies in either coagulation factor VIII (FVIII) or factor IX (FIX), respectively.
|
30705923 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Haemophilia is a serious inherited bleeding disorder resulting from a deficiency of coagulation factor VIII (haemophilia A) or coagulation factor IX (haemophilia B).
|
31069799 |
2019 |
|
Hemophilia A
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
After a second control with a normal FIX level and a second genetic confirmation of hemophilia, no FIX concentrates was administered to perform the infiltration, which occurred without hemorrhagic complication.
|
31272859 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Hemophilia is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) in hemophilia A or factor IX (FIX) in hemophilia B.
|
31427262 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
AHCDC: Association of Hemophilia Clinic Directors of Canada; AICE: Italian Association of Hemophilia Centres; ATHN: American Thrombosis and Hemostasis Network; EAHAD: European Association for Haemophilia and Allied Disorders; EHC: European Hemophilia Consortium; FIX: Coagulation Factor IX; FVIII: Coagulation Factor VIII; HAL: Haemophilia Activity List; HJHS: Haemophilia Joint Health Score; HTC: Hemophilia Treatment Centre; HTCCNC: Hemophilia Treatment Centre Collaborative Network of China; MASAC: Medical and Scientific Advisory Council; MDT: Multidisciplinary team; NHD: National Haemophilia Database; NHF: National Hemophilia Foundation; PK: Pharmacokinetics; POCUS: Point of care ultrasound; PWH: People with haemophilia; SHIELD: Supporting Hemophilia through International Education, Learning and Development; WFH: World Federation of Hemophilia.
|
30073913 |
2019 |
|
Hemophilia A
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
Emicizumab, a bispecific humanized monoclonal antibody, bridges activated factor IX (FIX) and FX to restore the function of missing activated FVIII in hemophilia A. Emicizumab prophylaxis in children with hemophilia A and FVIII inhibitors was investigated in a phase 3 trial (HAVEN 2).
|
31697801 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Patients with haemophilia who have developed inhibitors against factor VIII (FVIII) or factor IX present a significant concern to those surgeons who operate on them.
|
30507046 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
The median (range) clotting FVIII was 0.85 IU/mL (0.24-1.90 IU/mL) and FIX 0.60 IU/mL (0.42-1.76 IU/mL) in hemophilia A and B carriers, respectively.
|
30709356 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Two of these proteins, produced in the liver, factor VIII and factor IX, are deficient or present a functional defect in people with haemophilia.
|
30999657 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Further elucidation of the shared mechanisms underlying abnormal bone homeostasis in the absence of FVIII or FIX is needed to guide evidence-based approaches to the screening and treatment of the prevalent bone defects in hemophilia A and B.
|
31594977 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
The standard therapy for patients with haemophilia is prophylactic treatment with replacement factor VIII (FVIII) or factor IX (FIX).
|
30411401 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Here, we explore this topic using CPDs from FVIII and FIX and data concerning carriers' hemophilia severity.
|
31267011 |
2019 |
|
Hemophilia A
|
0.500 |
CausalMutation
|
disease |
CLINVAR |
Diagnostic high-throughput sequencing of 2396 patients with bleeding, thrombotic, and platelet disorders.
|
31064749 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Nor is there sound evidence as to how subcutaneous non-FVIII/FIX replacement approaches (concizumab; emicizumab; fitusiran) or single intravenous injections of adeno-associated viral vectors (when employing gene therapy) will revolutionize adherence in haemophilia.
|
30146094 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
CLINVAR |
Diagnostic high-throughput sequencing of 2396 patients with bleeding, thrombotic, and platelet disorders.
|
31064749 |
2019 |
|
Hemophilia A
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Hemophilia mice that express a mutant allele of human coagulation factor IX (FIX) containing nonsense mutation R338X were treated with <i>eRF1</i>- or <i>eRF3a</i>-ASO.
|
31070517 |
2019 |
|
Hemophilia A
|
0.500 |
Biomarker
|
disease |
BEFREE |
Clinical trials in hemophilia using adeno-associated virus (AAV) vectors to transfer functional factor IX (FIX) have reported increases in FIX activity to functional levels, reduced bleed frequency, and a lessening or abrogation of the need for costly FIX replacement.
|
29624465 |
2018 |