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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/29465806/principles-of-haemophilia-care-the-asia-pacific-perspective
#1
REVIEW
S Dunkley, J C M Lam, M J John, R S M Wong, H Tran, R Yang, S C Nair, M Shima, A Street, A Srivastava
Optimal haemophilia care is best established and implemented through a well-coordinated plan guided by clearly defined principles and priorities. A document which enunciates those details is therefore important. A successful example of this approach is the definition of principles of haemophilia care (PHC) outlined by the European Association for Haemophilia and Associated Disorders (EAHAD) and also the World Federation of Hemophilia. A similar document applicable to the Asia-Pacific region must take into account not only the highly varied healthcare systems but also the tremendous socio-economic and cultural diversities which impact provision of such care...
February 21, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29461004/effects-of-pre-analytical-heat-treatment-in-factor-viii-fviii-inhibitor-assays-on-fviii-antibody-levels
#2
B Boylan, C H Miller
INTRODUCTION: The use of pre-analytical heat treatment (PHT) with the Nijmegen-Bethesda assay (NBA) for inhibitors to factor VIII (FVIII) can remove/destroy infused or endogenous FVIII from patient plasma samples, allowing testing of recently infused patients with haemophilia. Two PHT methods have been described as follows: heating to 56°C for 30 minutes and heating to 58°C for 90 minutes. Data examining the effects of PHT on anti-FVIII IgG4 , the antibodies known to correlate most closely with the presence of FVIII inhibitors, are limited...
February 20, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29446525/laboratory-testing-for-factor-viii-and-ix-inhibitors-in-haemophilia-a-review
#3
REVIEW
C H Miller
Inhibitors are antibodies directed against haemophilia treatment products which interfere with their function. Factor VIII (FVIII) inhibitors in haemophilia A and factor IX (FIX) inhibitors in haemophilia B are significant clinically when they require a change in a patient's treatment regimen. Their persistence may increase morbidity and mortality. Multiple laboratory tests are now available for detecting and understanding inhibitors in haemophilia. Inhibitors are traditionally measured by their interference in clotting or chromogenic factor assays...
February 15, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29446520/lenalidomide-as-a-novel-therapy-for-gastrointestinal-angiodysplasia-in-von-willebrand-disease
#4
N V Khatri, B Patel, D R Kohli, S S Solomon, K Bull-Henry, C M Kessler
INTRODUCTION: Lenalidomide is a thalidomide analog with anti-angiogenic properties. Previous case reports suggest its efficacy in preventing gastrointestinal bleeding (GIB) secondary to angiodysplasia (AD) in hereditary haemorrhagic telangiectasia and potentially in reversing AD. We present the first case series to explore lenalidomide as a treatment for AD-related GIB in patients with von Willebrand disease (VWD). METHODS: A retrospective chart review was conducted to include patients with VWD, who were evaluated from 2010 to 2013 and who had received lenalidomide to treat recurrent GIB secondary to AD...
February 15, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436084/clinical-trials-and-registries-in-haemophilia-opponents-or-collaborators-comparison-of-pup-data-derived-from-different-data-sources
#5
C Keipert, C J Jonker, H M van den Berg, A Hilger
INTRODUCTION: The "Guideline on the clinical investigation of recombinant and human plasma-derived factor VIII products" (ClinGL) provides the requirements for the performing of clinical trials (CTs) for marketing authorization in Europe. The number of eligible previously untreated patients (PUPs) for CTs might be difficult to meet because of the concurrent development of FVIII concentrates, and additional data sources must be explored. AIM: The extent to which CTs and the PedNet registry met relevant parameters, identified in the ClinGL, as well as inhibitor incidences were investigated in patients from both sources...
February 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436077/recombinant-factor-viii-fc-fusion-protein-for-immune-tolerance-induction-in-patients-with-severe-haemophilia-a-with%C3%A2-inhibitors-a-retrospective-analysis
#6
M Carcao, A Shapiro, J M Staber, N Hwang, C Druzgal, K Lieuw, M Belletrutti, C D Thornburg, S P Ahuja, J Morales-Arias, J Dumont, G Miyasato, E Tsao, N Jain, S W Pipe
INTRODUCTION: Immune tolerance induction (ITI) is the gold standard for eradication of factor VIII inhibitors in severe haemophilia A; however, it usually requires treatment for extended periods with associated high burden on patients and healthcare resources. AIM: Review outcomes of ITI with recombinant factor VIII Fc fusion protein (rFVIIIFc) in patients with severe haemophilia A and high-titre inhibitors. METHODS: Multicentre retrospective chart review of severe haemophilia A patients treated with rFVIIIFc for ITI...
February 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436075/ligneous-cervicitis-and-endometritis-a-gynaecological-presentation-of-congenital-plasminogen-deficiency
#7
REVIEW
M Baithun, T Freeman-Wang, P Chowdary, R A Kadir
BACKGROUND: Congenital plasminogen deficiency is a rare autosomal recessive condition. Plasminogen deficiency is thought to result in an inability of fibrin breakdown and therefore accumulation of fibrin and formation of ligneous changes. Ligneous lesions can form on a number of mucosal membranes including the cervix and endometrium. METHODS: We report the case of a 25-year-old woman with type 1 plasminogen deficiency with ligneous cervicitis and endometritis and her treatment and clinical course over the last few years...
February 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436074/hepatitis-c-infection-in-a-greek-population-with-inherited-bleeding-disorders
#8
LETTER
O Giouleme, P Paschos, A Katsoula, K Panteliadou, S Vakalopoulou, V Garipidou
No abstract text is available yet for this article.
February 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29436079/pocket-handheld-ultrasound-for-evaluation-of-the-bleeding-haemophilic-joint-a-novel-and-reliable-way-to-recognize-joint-effusions
#9
LETTER
J Y Zhou, K C Rappazzo, L Volland, R F W Barnes, M Brackman, B Steiner, R Kruse-Jarres, D V Quon, C Bailey, E Y Chang, A von Drygalski
No abstract text is available yet for this article.
February 12, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418048/a-brief-survey-of-clinicians-perceptions-of-parent-preferences-for-involvement-in-obstetrical-and-perinatal-management-decisions-in-haemophilia
#10
LETTER
K J Lucier, R V Movilla, M K Parvizian, R Siddiqui, E H Gabriele, M Bourque, J MacIsaac, P C Moorehead, A K Chan, N M Heddle, S J Lane
No abstract text is available yet for this article.
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418046/management-of-brothers-with-haemophilia-a-and-familial-cerebral-cavernous-malformations
#11
LETTER
Kevin J Zhang, M Chitlur
No abstract text is available yet for this article.
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418045/laboratory-monitoring-issues-in-recombinant-porcine-fviii-replacement-in-acquired-haemophilia-a
#12
LETTER
V R Vanguru, G Kershaw, M Konda, V M Chen
No abstract text is available yet for this article.
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418043/advantage-of-recombinant-von-willebrand-factor-for-peri-operative-management-in-paediatric-acquired-von-willebrand-syndrome
#13
LETTER
A C Weyand, R Jesudas, S W Pipe
No abstract text is available yet for this article.
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418042/the-importance-of-abo-blood-group-in-pharmacokinetic-studies-in-haemophilia-a
#14
LETTER
M Franchini, C Mengoli, G Marano, S Pupella, P M Mannucci, G M Liumbruno
No abstract text is available yet for this article.
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29418040/male-gender-school-attendance-and-sports-participation-are-positively-associated-with-health-related-quality-of-life-in-children-and-adolescents-with-congenital-bleeding-disorders
#15
P F Limperg, M M H Joosten, K Fijnvandraat, M Peters, M A Grootenhuis, L Haverman
BACKGROUND: This study assesses health-related quality of life (HRQOL), and variables associated with HRQOL, in children and adolescents with haemophilia and congenital bleeding disorders (CBD) in the Netherlands. METHODS: Patients <18 years with CBD under treatment at the Hemophilia Comprehensive Care Center of the Academic Medical Center were included. Participants completed generic HRQOL questionnaires (TAPQOL 0-5 years; PedsQL 6-18 years). Differences and effect sizes in HRQOL compared to healthy peers, and between hemophilia severity groups, were tested using Mann Whitney U-tests...
February 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29405496/clinical-use-of-recombinant-factor-viii-fc-and-recombinant-factor-ix-fc-in-patients-with-haemophilia-a-and-b
#16
C Wang, G Young
INTRODUCTION: Although clinical trials have demonstrated extended half-life (EHL) VIII and IX fusion proteins to be safe and efficacious in patients with haemophilia A and B, studies on real-world clinical application have not been performed. AIM: To retrospectively examine the real-world experience of rFVIII Fc and rFIX Fc in patients. METHODS: A retrospective review of existing medical records of patients with haemophilia A or haemophilia B who had been prescribed rFVIII Fc or rFIX Fc was conducted from the Children's Hospital Los Angeles Haemostasis and Thrombosis Centre database...
February 5, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29405493/a-novel-missense-mutation-p-phe360cys-in-fix-gene-results-in-haemophilia-b-in-a-female-patient-with-skewed-x-inactivation
#17
LETTER
C Yang, Z Yu, W Zhang, L Cao, W Ouyang, F Hu, P Zhang, X Bai, C Ruan
No abstract text is available yet for this article.
February 5, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29388750/high-proportion-of-patients-with-bleeding-of-unknown-cause-in-persons-with-a-mild-to-moderate-bleeding-tendency-results-from-the-vienna-bleeding-biobank-vibb
#18
J Gebhart, S Hofer, S Panzer, P Quehenberger, R Sunder-Plassmann, G Hoermann, E Eigenbauer, H Haslacher, S Kepa, P A Kyrle, S Eichinger, P Knöbl, L Eischer, C Mannhalter, C Ay, I Pabinger
INTRODUCTION: Data on clinical characteristics and the prevalence of underlying coagulopathies in patients with mild-to-moderate bleeding disorders (MBDs) are scarce. AIM: We established the Vienna Bleeding Biobank (VIBB) to characterize and thoroughly investigate Austrian patients with MBDs. RESULTS: Four hundred eighteen patients (female = 345, 82.5%) were included. A platelet function defect (PFD) was diagnosed in 26 (6.2%) and a possible PFD in 30 (7...
February 1, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29388742/a-prospective-surveillance-study-of-inhibitor-development-in-haemophilia-a-patients-following-a-population-switch-to-a-third-generation-b-domain-deleted-recombinant-factor-viii
#19
E Dubé, A Bonnefoy, C Merlen, J-F Castilloux, S Cloutier, C Demers, C A Sabapathy, J St-Louis, C Vezina, M Warner, G-É Rivard
INTRODUCTION: Following a provincial tender, most subjects with haemophilia A in Quebec switched their treatment to a third-generation recombinant B-domain-deleted factor VIII (FVIII). AIM: Our objective was to evaluate the incidence of inhibitor development and FVIII recovery in patients following the switch of factor replacement therapy. METHODS: One hundred and thirty-five subjects were enrolled and tested for FVIII activity and inhibitors every 6 months during 1 year...
February 1, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29388741/successful-delivery-in-an-patient-with-afibrinogenemia-after-three-abortions-a-case-report-and-review-of-the-literature
#20
LETTER
M Karimi, M Bordbar, M Aali, A Bazrafshan, H Tavoosi, J Gerdabi
No abstract text is available yet for this article.
February 1, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
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