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PubMed Journal Database | Haemophilia : the official journal of the World Federation of Hemophilia RSS

10:48 EDT 25th March 2019 | BioPortfolio

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Showing PubMed Articles 1–25 of 226 from Haemophilia : the official journal of the World Federation of Hemophilia

Publishing in Haemophilia.

Congenital Factor X deficiency in women: A systematic review of the literature.

Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. A total number of 49 relevant articles were identified, including 332...

Mortality of patients with haemophilia in Brazil: First report.

Brazil has the fourth largest world population of patients with haemophilia. However, mortality rates in this population are unknown.

Cross-sectional comparative study of pharmacokinetics and efficacy between sucrose-formulated recombinant factor VIII (Kogenate ) and BAY 81-8973 (Kovaltry ) in patients with severe or moderate haemophilia A in prophylaxis.

Sustained high trough factor IX activity levels with continued use of rIX-FP in adult and paediatric patients with haemophilia B.

Spinal dorsal rhizotomy plus concurrent left and right gastrocnemii releases in a 7-year-old child with haemophilia A and spastic cerebral palsy.

An exploratory comparison of single intra-articular injection of platelet-rich plasma vs hyaluronic acid in treatment of haemophilic arthropathy of the knee.

Intra-articular platelet-rich plasma (PRP) injection therapy has been extensively applied in clinical practice to treat musculoskeletal disorders such as osteoarthritis, but the treatment for haemophilic arthropathy is rarely reported.

Characterization of two large duplications of F9 associated with mild and severe haemophilia B, respectively.

Only two large duplications of F9 causing haemophilia B (HB) have been reported.

Generation of integration-free induced pluripotent stem cells from blood-derived cells isolated from patient with severe haemophilia A.

Why plasma-derived factor VIII?

Emicizumab prophylaxis to facilitate anticoagulant therapy for management of intra-atrial thrombosis in severe haemophilia with an inhibitor.

Outpatient treatment for haemophilic arthropathy with radiosynovectomy: Radiation dose to family members.

One of the key features of good practice in medicine is the doctor-patient communication. Radiation protection standards for radiosynovectomy (RS) is limited. Yttrium-90 is a beta-emitting radioisotope used in RS to treat joint pain from haemophilic arthritis. ICRP 94 states that if a patient is treated with up to 200 MBq, there is no need for further precautions when it comes to public exposure, however, activities can go up to 370 MBq in RS for the knee. This study analysed 119 family members' safety (1...

Survey of non-physiotherapy department medical staff in China and their understanding of physiotherapy in comprehensive management of haemophilia.

Physiotherapy plays a very important role in comprehensive care of patients with haemophilia. Due to limited resources and a lack of understanding among medical personnel, physiotherapy has not become a standard component of comprehensive management of haemophilia in China.

Anterior osteophyte resection of the ankle joint to increase range of motion in haemophilic ankle arthropathy.

Haemophilic ankle arthropathy may cause joint bleeding, pain, stiffness, erosion and progressive motion limitations, including a decreased range of motion (ROM) secondary to anterior osteophyte impingement.

Whole blood ristocetin-induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease.

The haemorrhagic phenotype in patients with von Willebrand disease (VWD) is heterogeneous, and assays of von Willebrand factor ristocetin cofactor activity (VWF:RCo) do not always reflect clinical severity, especially in those individuals classed as type 1 VWD. Recent studies have shown that whole blood ristocetin-induced platelet agglutination (WB-RIPA) using an easy-to-use analyzer, Multiplate® platelet impedance technique, could be informative as a diagnostic test in VWD, although inconsistencies were e...

Scope of practice of haemophilia physiotherapists: A European survey.

European guidelines on the care of haemophilia recommend ready access to a range of services provided by a multidisciplinary team of specialists including physiotherapy. However, the scope of physiotherapy provided is unknown.

Prevalence and risk factors associated with hepatitis C among Brazilian male patients with haemophilia: A long-term follow-up.

People with haemophilia represent a population with a high prevalence of HCV infection due to the use of blood components and plasma-derived clotting factor concentrates before the introduction of viral-inactivating procedures (in the 1980s) and screening for HCV (in the 1990s). About 80% of HCV-infected patients have chronic HCV infection, and at least 20% develop end-stage liver disease. The aim of the study was to assess current anti-HCV positivity in a large cohort of Brazilian haemophilia patients and ...

Exploring regional variations in the cross-cultural, international implementation of the Patient Reported Outcomes Burdens and Experience (PROBE) study.

The Patient Reported Outcomes Burdens and Experience (PROBE) study has developed and validated the PROBE questionnaire for assessing patient-reported outcomes in people with haemophilia and participants without bleeding disorders.

Rapid and sustained immune tolerance to inhibitors induced by a plasma-derived, VWF-containing FVIII concentrate.

Evaluation of a standardized protocol for thrombin generation using the calibrated automated thrombogram: A Nordic study.

The thrombin generation assay-calibrated automated thrombogram (TGA-CAT) method is used to measure the overall coagulation capacity in plasma. However, the method is still considered to be a research tool, mainly because of its' lack of standardization.

Relapse pattern and long-term outcomes in subjects with acquired haemophilia A.

Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy.

Splicing analysis of 26 F8 nucleotide variations using a minigene assay.

Classically, the study of splicing impact of variation located near the splice site is performed by both in silico and mRNA analysis. However, RNA sample was rarely available.

Expecting the unexpected: Acquired haemophilia A in a patient with homozygous factor V deficiency.

Parent's report on oral health-related quality of life of children with haemophilia.

Among children with haemophilia and their caregivers; problems arising from the teeth and the surrounding tissues have an important role in the treatment of this disease and it affects the quality of life of children and their parents.

Sports participation and physical activity in adult Dutch and Swedish patients with severe haemophilia: A comparison between intermediate- and high-dose prophylaxis.

Differences in treatment and outcome have been reported for persons with haemophilia (PWH) on intermediate-dose (Dutch) and high-dose (Swedish) prophylaxis, but the potential influence of sports participation has not been considered.


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