keyword
MENU ▼
Read by QxMD icon Read
search

Haemophilia

keyword
https://www.readbyqxmd.com/read/28520202/haemophilia-treatment-for-all-and-the-role-of-tolerance-difference-and-education
#1
EDITORIAL
Alain Weill
No abstract text is available yet for this article.
May 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28515528/perioperative-management-of-a-patient-with-haemophilia-a-for-major-abdominal-surgery
#2
Mamta Sethi, Pavan Gurha
No abstract text is available yet for this article.
April 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28502190/two-rare-risk-factors-for-post-partum-haemorrhage-a-case-report-of-a-carrier-of-severe-haemophilia-a-with-a-uterine-arteriovenous-malformation
#3
Gemma L Malin, Gillian Swallow, Jane Rutherford
No abstract text is available yet for this article.
May 14, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28499509/ten-year-study-of-postoperative-complications-following-dental-extractions-in-patients-with-inherited-bleeding-disorders
#4
J-T Hsieh, K Klein, M Batstone
Dental extractions challenge the body's haemostatic mechanism. Postoperative bleeding from dental extraction can be prolonged, or even life threatening in patients with inherited bleeding disorders. Pre- and postoperative clotting factor replacements or systemic desmopressin (ddAVP) have been advocated at our institution to prevent bleeding complications in these patients. This study aimed to assess the postoperative bleeding rate in patients with inherited bleeding disorders that underwent dental extractions at our institution between 2003 and 2012...
May 9, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28492697/cd32-inhibition-and-high-dose-of-rhfviii-suppress-murine-fviii-specific-recall-response-by-distinct-mechanisms-in-vitro
#5
Nadine Vollack, Julia Friese, Sabine Bergmann, Andreas Tiede, Sonja Werwitzke
Development of neutralising antibodies (inhibitors) against factor VIII (FVIII) is a frequent and severe complication of replacement therapy in haemophilia A. Previous data from haemophilia A mouse model demonstrates that both CD32 inhibition and high doses of rhFVIII prevent the differentiation of FVIII-specific memory B cells (MBCs) into antibody secreting cells (ASCs). Here, cellular targets responsible for the suppression of ASC formation by means of CD32 inhibition and high dose of rhFVIII were analysed...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28492696/complex-recombination-with-deletion-in-the-f8-and-duplication-in-the-tmlhe-mediated-by-int22h-copies-during-early-embryogenesis
#6
Changming Chen, Xiaoling Xie, Xi Wu, Yeling Lu, Xuefeng Wang, Wenman Wu, Yiqun Hu, Qiulan Ding
Haemophilia A (HA) is a common X-linked recessive bleeding disorder and almost one half of patients with severe HA are caused by intron 22 inversion (Inv22) in the F8. Inv22 is considered to be almost exclusively of meiotic origin in germ cells during spermatogenesis and only one mosaic Inv22 female carrier with the mutation possibly occurring during mitosis of the embryo has been reported so far. Previously we have identified a novel complex recombination mediated by int22h copies in a sporadic severe HA pedigree and herein we have localised the sequences flanking the breakpoint region using genome walking technique, AccuCopy technique, gene chip and real-time PCR...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28492695/joint-assessment-in-von-willebrand-disease-validation-of-the-haemophilia-joint-health-score-and-haemophilia-activities-list
#7
Karin P M van Galen, Merel A Timmer, Piet de Kleijn, Kathelijn Fischer, Wouter Foppen, Roger E G Schutgens, Jeroen Eikenboom, Karina Meijer, Marjon H Cnossen, Karin Fijnvandraat, Johanna G van der Bom, Britta A P Laros-van Gorkom, Frank W G Leebeek, Eveline P Mauser-Bunschoten
Assessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28488976/haemophilia-a-the-consequences-of-de-novo-mutations-two-case-reports
#8
Federica Zarrilli, Antonio Coppola, Michele Schiavulli, Ernesto Cimino, Ausilia Elce, Giuseppe Rescigno, Giuseppe Castaldo, Felice Amato
No abstract text is available yet for this article.
April 5, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28475272/recombinant-factor-viia-addition-to-haemophilic-blood-perfused-over-collagen-tissue-factor-can-sufficiently-bypass-the-factor-ixa-viiia-defect-to-rescue-fibrin-generation
#9
R Li, K A Panckeri, P F Fogarty, A Cuker, S L Diamond
INTRODUCTION: Factor VIII (FVIII) or factor IX (FIX)-deficient haemophilic patients display deficits in platelet and fibrin deposition under flow detectable in microfluidics. Compared to fibrin generation, decreased platelet deposition in haemophilic blood flow is more easily rescued with recombinant factor VIIa (rFVIIa), whereas rFVIIa requires FXIIa participation to generate fibrin when tissue factor (TF) is absent. AIMS: Perfusion of haemophilic whole blood (WB) over collagen/TF surfaces was used to determine whether rFVIIa/TF was sufficient to bypass poor FIXa/FVIIIa function in blood from patients with haemophilia A and B...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28475226/molecular-cytogenetic-characterization-of-five-f8-complex-rearrangements-utility-for-haemophilia-a-genetic-counselling
#10
Y Jourdy, N Chatron, M Fretigny, M L Carage, H Chambost, S Claeyssens-Donadel, V Roussel-Robert, C Negrier, D Sanlaville, C Vinciguerra
BACKGROUND: Genomic inversions are usually balanced, but unusual patterns have been described in haemophilia A (HA) patients for intron 22 (Inv22) and intron 1 (Inv1) inversions leading to the hypothesis of more complex rearrangements involving deletions or duplications. AIM: To characterize five abnormal patterns either in Southern blot and long-range PCR for Inv22 or in PCR for Inv1. MATERIALS AND METHODS: All patients were studied using cytogenetic microarray analysis (CMA)...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470911/platelet-rich-plasma-for-chronic-synovitis-treatment-in-patients-with-haemophilia
#11
H Caviglia, M E Landro, C Daffunchio, G Galatro, A L Douglas Price, P Salgado, D Neme
INTRODUCTION: Haemophilic synovitis is caused by chronic accumulation of blood in the joint. Conservative treatment is insufficient to solve this pathology. Platelet-rich plasma (PRP) has a high concentration of growth factors (GFs) that play a key role in regulation and stimulation of healing processes. The aim of this study was to describe the effect of PRP injection in chronic synovitis of the joints in patients with haemophilia (PWH). PATIENTS AND METHODS: Nineteen patients with 28 joints were treated at our centre in Buenos Aires, Argentina between December 2014 and December 2015...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470862/first-report-on-the-safety-and-efficacy-of-an-extended-half-life-glycopegylated-recombinant-fviii-for-major-surgery-in-severe-haemophilia-a
#12
K Hampton, P Chowdary, S Dunkley, S Ehrenforth, L Jacobsen, A Neff, E Santagostino, J Sathar, H Takedani, C M Takemoto, C Négrier
BACKGROUND: N8-GP (turoctocog alfa pegol) is an extended half-life glycoPEGylated recombinant factor VIII (FVIII) product developed for the prevention and treatment of bleeds in haemophilia A patients. AIM: This is a planned interim analysis of pathfinder™3, an international, open-label, Phase 3 trial evaluating the efficacy and safety (including immunogenicity) of N8-GP administered before, during and after major surgery in severe haemophilia A patients aged ≥12 years...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28452151/pk-guided-personalized-prophylaxis-with-nuwiq-%C3%A2-human-cl-rhfviii-in-adults-with-severe-haemophilia-a
#13
T Lissitchkov, L Rusen, P Georgiev, J Windyga, R Klamroth, L Gercheva, L Nemes, A Tiede, J Bichler, S Knaub, L Belyanskaya, O Walter, K J Pasi
INTRODUCTION: Nuwiq(®) (human-cl rhFVIII) is a 4(th) generation recombinant human FVIII, without chemical modification or protein fusion, produced in a human cell-line. AIMS/METHODS: This study (NuPreviq) was a prospective, open-label, multicentre, phase IIIb study of the efficacy and safety of personalized prophylaxis with Nuwiq(®) in 66 previously treated adults with severe haemophilia A. NuPreviq had three phases: (i) a 72-h pharmacokinetic (PK) phase; (ii) a 1-3 month standard prophylaxis phase; and (iii) a 6-month personalized prophylaxis phase...
April 27, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28440032/discrepancies-between-the-one-stage-clotting-assay-and-the-chromogenic-assay-in-haemophilia-b
#14
K Kihlberg, K Strandberg, S Rosén, R Ljung, J Astermark
INTRODUCTION: Assay discrepancy in factor VIII activity between the one-stage and the chromogenic assays has been described in approximately one third of patients with non-severe haemophilia A. Whether assay discrepancy may also occur in patients with haemophilia B remains unknown. AIM: This study compared the results from the one-stage and the chromogenic assays in patients with haemophilia B. METHODS: Plasma samples from patients with haemophilia B attending the haemophilia centre in Malmö, Sweden, were collected after a wash-out period of more than 7 days and analysed with both assays...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28440005/comparing-the-burden-of-illness-of-haemophilia-between-resource-constrained-and-unconstrained-countries-the-s%C3%A3-o-paulo-toronto-hemophilia-study
#15
J D A Carneiro, V Blanchette, M C Ozelo, S V Antunes, P R Villaca, N L Young, D Castro, L R Brandão, M Carcao, A Abad, B M Feldman
INTRODUCTION: Although the regular replacement of clotting factor concentrates (prophylaxis) has been well established as the standard of care for severe haemophilia, the high cost of factor concentrates has limited access to prophylaxis in countries with under-developed or developing economies. AIMS: We studied the health gap that could be addressed by providing unlimited access to clotting factor concentrates with implementation of long-term prophylaxis initiated from an early age in life...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28440004/safety-and-effectiveness-of-room-temperature-stable-recombinant-factor-viia-in-patients-with-haemophilia-a-or-b-and-inhibitors-results-of-a-multinational-prospective-observational-study
#16
K Kavakli, F Demartis, M Karimi, P Eshghi, D Neme, H Chambost, L Sommer, M Zak, G Benson
INTRODUCTION: A room temperature stable formulation of recombinant activated factor VII (NovoSeven(®) ), allowing convenient storage and therefore improved treatment access, has been developed. Bioequivalence to the previous NovoSeven(®) was demonstrated in healthy humans, leading to European approval (2008). Although no confirmed cases of neutralising antibodies to rFVIIa in patients with haemophilia A or B have been observed with the original formulation, changes in formulation or storage condition may alter immunogenicity...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28439941/serological-biomarkers-detect-active-joint-destruction-and-inflammation-in-patients-with-haemophilic-arthropathy
#17
B Hua, E H N Olsen, S Sun, C N Gudme, L Wang, B Vandahl, K Roepstorff, M Kjelgaard-Hansen, B B Sørensen, Y Zhao, M A Karsdal, T Manon-Jensen
INTRODUCTION: Progressive arthropathy caused by recurrent joint bleeds is a severe complication in haemophilia. AIM: We investigated whether biomarkers of cartilage and bone degradation, and inflammation were altered in haemophilia patients and whether these biomarkers could identify haemophilia patients with arthropathy. METHODS: Serum from 35 haemophilia patients with varying degrees of arthropathy and 43 age- and gender-matched control subjects were analysed...
April 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28438758/diabetes-mellitus-and-acquired-haemophilia-new-association
#18
Carlos Tavares Bello, Yuliya Vasylenko, José Esteves, Carlos Augusto Vasconcelos
Diabetes mellitus encompasses a group of highly prevalent carbohydrate metabolic disorders with an increasing incidence. Some subtypes are thought to be associated with other immune-mediated diseases. Acquired haemophilia on the other hand is a quite rare autoimmune disease that is thought to be secondary to the emergence of inhibiting anticoagulation factor VIII antibodies (inhibitors) in patients with previously normal haemostatic function. More recently, numerous different diseases have been associated with acquired haemophilia namely immune-mediated diseases, drugs and solid and haematologic neoplasms...
April 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28429867/health-related-quality-of-life-questionnaires-in-individuals-with-haemophilia-a-systematic-review-of-their-measurement-properties
#19
REVIEW
P F Limperg, C B Terwee, N L Young, V E Price, S C Gouw, M Peters, M A Grootenhuis, V Blanchette, L Haverman
BACKGROUND: The evaluation of health related quality of life (HRQOL) is essential for a full assessment of the influence of an illness on patients' lives. The aim of this paper is to critically appraise and compare the measurement properties of HRQOL questionnaires studied in haemophilia. METHODS: Bibliographic databases (Embase, Medline, Cinahl and PsycInfo) were searched for articles evaluating measurement properties of HRQOL questionnaires in haemophilia. Articles were excluded that did not report HRQOL measurement properties, or when <50% of the study population had haemophilia...
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28429865/differential-effects-of-bleeds-on-the-development-of-arthropathy-basic-and-applied-issues
#20
REVIEW
L F D van Vulpen, S C Mastbergen, F P J G Lafeber, R E G Schutgens
Haemophilia is characterized by a spontaneous bleeding tendency, affecting mainly the synovial joints. Recurrent joint bleeds induce a cascade of inflammatory as well as degenerative processes injuring synovium, cartilage and bone. These processes affect each other and may occur in parallel and/or sequentially. Clinically, the effects of joint bleeds are heterogeneous. A marked variability in joint damage is observed in patients with a similar bleeding history. Also late stage effects differ with some patients developing chronic synovitis, and others suffering from osteochondral degeneration called haemophilic arthropathy...
April 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
keyword
keyword
4856
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"