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Haemophilia

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https://www.readbyqxmd.com/read/28326647/exploring-some-intersections-between-pharmacokinetics-factor-viii-measurement-and-human-morphometrics-impact-of-recent-advances-in-haemophilia-study-design-on-our-understanding-of-optimal-haemophilia-treatment
#1
A Iorio, A N Edginton
No abstract text is available yet for this article.
March 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28322357/assessment-of-dental-caries-experience-and-periodontal-health-status-among-children-with-haemophilia-in-new-delhi-india-a-case-control-study
#2
Mridula Goswami, Babita Jangra
PURPOSE: To assess the caries experience and periodontal health status among children with haemophilia in New Delhi. MATERIALS AND METHODS: This case control study was conducted in New Delhi over a 14-month period. Data were collected from 110 children, of which 55 children had haemophilia and 55 were healthy. Caries was assessed in terms of decayed, missing and filled teeth, according to WHO criteria, while periodontal health status was assessed regarding plaque present, and recorded by utilising the Silness and Löe plaque index...
March 20, 2017: Oral Health & Preventive Dentistry
https://www.readbyqxmd.com/read/28321962/psychosocial-care-for-children-with-haemophilia-and-their-parents-in-the-netherlands
#3
REVIEW
P F Limperg, L Haverman, M Beijlevelt, M van der Pot, G Zaal, W A de Boer, K Fijnvandraat, M Peters, M A Grootenhuis
INTRODUCTION: Children growing up with haemophilia are at greater risk for psychosocial problems than their healthy peers. Providing psychosocial care to children with haemophilia and their families is indispensable, since psychosocial factors can have a significant impact on health and health-related quality of life (HRQOL). AIMS: Our aim was to give a description of psychosocial care provided by the multidisciplinary team of the Hemophilia Comprehensive Care Centre (HCCC) at the Emma Children's Hospital in Amsterdam, the Netherlands...
March 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28324767/the-health-assessment-questionnaire-disability-index-haq-di-as-a-valid-alternative-for-measuring-the-functional-capacity-of-people-with-haemophilia
#4
Hortensia De La Corte-Rodriguez, E Carlos Rodriguez-Merchan
BACKGROUND: There are several scales for evaluating the functional capacity of people with haemophilia (PWH). OBJECTIVE: To demonstrate the value and simplicity of the "Health Assessment Questionnaire Disability Index" (HAQ-DI) for making a functional assessment of persons with haemophilia (PWH) in those contexts in which the specific physical assessment scales cannot be used (the "World Federation of Haemophilia Physical Examination Score" (WFH-PES) and the "Haemophilia Joint Health Score 2...
March 14, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28306626/pregnancy-and-delivery-in-a-woman-with-severe-haemophilia-a
#5
Jelena N Bodrozic, Predrag S Miljic, Danijela R Lekovic, Milos A Petronijevic, Darko A Antic, Mirjana M Mitrovic, Svetlana M Petronijevic-Vrzic, Irena S Djunic
Severe form of haemophilia in women is an extremely rare condition. Owing to the rarity of the disease there are no precise recommendations concerning the optimal management of pregnancy and delivery in these patients. We are reporting the clinical course and management of a 30-year-old woman with a severe form of haemophilia A (factor VIII <1 IU/dl) during her first pregnancy and delivery. Antepartum, she was treated on demand by FVIII concentrate and she delivered at 37 weeks of gestation by cesarean section...
March 16, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28306191/biomechanical-markers-and-theoretical-concepts-related-to-haemophilic-ankle-and-subtalar-joint-arthropathy-introducing-the-term-haemophilic-tarsal-pan-arthropathy
#6
REVIEW
S Lobet, A McCarthy, C Hermans, K Peerlinck, G A Matricali, F Staes, K Deschamps
BACKGROUND: Although regular factor replacement can reduce the incidence of joint bleeds and slow down the development of haemophilic arthropathy, the ankle joint remains particularly vulnerable even in children with haemophilia on primary or secondary prophylaxis and is now the primary joint affected. The heterogeneity in the pathoaetiology of haemophilic ankle arthropathy means that the functional consequences of early stage of ankle arthropathy are difficult to define as early morphological and structural changes can be observed in clinically asymptomatic ankles...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306186/sippet-methodology-analysis-and-generalizability
#7
REVIEW
F Peyvandi, P M Mannucci, R Palla, F R Rosendaal
The development of anti-FVIII neutralizing alloantibodies (inhibitors), occurring in about one-third of previously untreated patients (PUPs) with severe haemophilia A, depends on various genetic and environmental risk factors. Several previous studies have reported on the immunogenicity of FVIII concentrates, and due to differences in study design, study period, inhibitor testing frequency and follow-up duration the results were inconclusive. The first randomized trial on this unresolved question (SIPPET) included 251 previously untreated or minimally treated patients with severe haemophilia A treated with either a single plasma-derived FVIII (pdFVIII) containing VWF or a recombinant FVIII (rFVIII)...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28302350/primary-prophylaxis-in-haemophilia-care-guideline-update-2016
#8
Kathelijn Fischer, Rolf Ljung
This paper reviews the current status on recommendations or guidelines for primary prophylaxis based on recent published papers from organizations or group of experts as well as some original key papers. A rather uniform view exists that prophylaxis should be initiated at an early age before or after no more than a single joint bleed and, if possible, preferably be continued for life. The dose and dose frequency of prophylaxis is dependent on the goal of treatment, bleeding phenotype, compliance, venous access and economic resources in the health care system and should be tailored individually based on clinical outcome and pharmacokinetics...
February 17, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28300362/hepatitis-c-in-haemophilia-time-for-treatment-for-all
#9
EDITORIAL
M Makris, B A Konkle
No abstract text is available yet for this article.
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28299792/life-experience-of-the-adult-and-ageing-patient-with-haemophilia-practical-aspects-for-psychological-support
#10
REVIEW
A Torres-Ortuño, R Cid-Sabatel, J Barbero, M García-Dasí
This article discusses, from a psychological perspective, the life experience of the adult and ageing person with haemophilia, including psychological issues, aspects of his personal and social integration, decision-making, communication and other factors that may affect treatment adherence and quality of life. The aim was to provide haematologists and healthcare staff with knowledge and resources to improve communication and support for adult persons with haemophilia, and raise awareness on psychosocial issues related to quality of life, sexuality and aspects associated with ageing with haemophilia...
March 15, 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/28294101/screening-of-intron-1-inversion-of-factor-viii-gene-in-130-patients-with-severe-haemophilia-a-from-pakistani-cohort
#11
Azhar Sattar, Shabbir Hussain, Muhammad Ikram Ullah, Saqib Mahmood, Shahida Mohsin
No abstract text is available yet for this article.
March 15, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28289747/physiotherapy-treatments-in-musculoskeletal-pathologies-associated-with-haemophilia-a-literature-review
#12
Jesús Guodemar-Pérez, Montserrat Ruiz-López, Elena Rodríguez-López, Pablo García-Fernández, Juan Pablo Hervás-Pérez
The aim of this study is to offer physiotherapists a synthesis of the main therapeutic tools available for the treatment of musculoskeletal pathologies in patients with haemophilia, according to the scientific literature. Although bleeds are recognised as no longer being a cause of death for people with haemophilia, the accompanying musculoskeletal injuries now represent the main problem associated with this disorder. There is a lack of clear guidelines to date regarding the physiotherapy treatment of these disorders...
March 14, 2017: Hämostaseologie
https://www.readbyqxmd.com/read/28252515/factor-viii-mutation-spectrum-in-haemophilia-a-patients-in-the-population-of-henan-china
#13
Nan Bai, Xiaofan Zhu, Zhenhua Zhao, Ying Bai, Qinghua Wu, Ning Liu, Duo Chen, Xiangdong Kong
Defects in the coagulation factor VIII gene cause haemophilia A, which is the most common X-linked recessive bleeding disorder. In total, 45 affected families were investigated to elucidate the factor VIII gene mutation spectrum. The families were subjected to clinical, biochemical, and molecular analyses. Inverse-shifting PCR was first applied to severe haemophilia A patients to identify inversions in introns 22 and 1. Then, next-generation sequencing was performed to detect mutations in inversion-negative patients with severe haemophilia A and moderate-mild haemophilia A patients...
March 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28220685/incidence-of-low-titre-factor-viii-inhibitors-in-patients-with-haemophilia-a-meta-analysis-of-observational-studies
#14
A Messori, F Peyvandi, D Mengato, P M Mannucci
INTRODUCTION: A few studies have been focused on low-titre inhibitors in patients with haemophilia A. Although several putative factors have been implicated in the development of these inhibitors, solid data are still lacking. AIM: The aim of this study was to perform a proportion meta-analysis on the incidence of low-titre inhibitors in haemophilia A. METHODS: We surveyed the PubMed database to identify studies on de novo development of low-titre inhibitors in haemophilia A patients...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220631/treatment-of-bleeding-episodes-with-recombinant-factor-viii-fc-fusion-protein-in-a-long-study-subjects-with-severe-haemophilia-a
#15
A D Shapiro, J N Mahlangu, D Perry, J Pasi, D V Quon, P Chowdary, E Tsao, S Li, A Innes, G F Pierce, G A Allen
INTRODUCTION: The Phase 3 A-LONG study demonstrated the safety and efficacy of rFVIIIFc for the control and prevention of bleeding episodes in severe haemophilia A. AIM: To describe the treatment of bleeding episodes with rFVIIIFc in the A-LONG study. METHODS: A-LONG subjects (<1 IU dL(-1) endogenous FVIII) were treated with individualized prophylaxis (Arm 1), weekly prophylaxis (Arm 2) or episodic treatment (Arm 3). Information recorded for each bleeding episode included type, location and dose to treat the episode...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220573/a-comparison-of-two-types-of-ankle-supports-in-men-with-haemophilia-and-unilateral-ankle-pain-from-arthropathy
#16
D Oleson, L Fox, T Nguyen, P Sochacki, M McCarthy, E Adams, M Recht
INTRODUCTION: Adults with haemophilia frequently have a painful and disordered gait due to ankle arthropathy. AIM: The aim of this study was to determine if pain and gait parameters were affected by the use of different types of ankle bracing. METHODS: We investigated the gait patterns of 17 men with severe haemophilia without bracing, using a fracture boot (FB) and a carbon fibre floor reaction ankle foot orthosis (CF-AFO). Pain relief was determined in each condition...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28220555/population-pharmacokinetic-characterization-of-bay-81-8973-a-full-length-recombinant-factor-viii-lessons-learned-importance-of-including-samples-with-factor-viii-levels-below-the-quantitation-limit
#17
D Garmann, S McLeay, A Shah, P Vis, M Maas Enriquez, B A Ploeger
INTRODUCTION: The pharmacokinetics (PK), safety and efficacy of BAY 81-8973, a full-length, unmodified, recombinant human factor VIII (FVIII), were evaluated in the LEOPOLD trials. AIM: The aim of this study was to develop a population PK model based on pooled data from the LEOPOLD trials and to investigate the importance of including samples with FVIII levels below the limit of quantitation (BLQ) to estimate half-life. METHODS: The analysis included 1535 PK observations (measured by the chromogenic assay) from 183 male patients with haemophilia A aged 1-61 years from the 3 LEOPOLD trials...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205396/can-the-diagnostic-reliability-of-the-thrombin-generation-test-as-a-global-haemostasis-assay-be-improved-the-impact-of-calcium-chloride-concentration
#18
L A Parunov, S S Surov, Y Liang, T K Lee, M V Ovanesov
BACKGROUND: Thrombin generation test (TGT) is a global haemostasis assay with a potential to predict bleeding tendencies and treatment effects in patients with haemophilia. Despite 15 years of clinical research, the diagnostic value of TGT remains controversial, possibly due to suboptimal sensitivity to coagulation deficiencies, robustness and reproducibility. OBJECTIVE: The goal of this study was to explore the effect of calcium chloride (CaCl2 ) concentration on the TGT's response to intrinsic coagulation factors (F) VIII, IX and XIa...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205367/multiplex-ligation-dependent-probe-amplification-as-first-mutation-screening-for-large-deletions-and-duplications-in-haemophilia
#19
D Belvini, R Salviato, P Radossi, G Tagariello
INTRODUCTION: Molecular characterization has shown a wide mutational spectrum underlying haemophilia A (HA) and haemophilia B (HB). Different molecular assays have allowed laboratories to perform genetic testing for F8 and F9 mutations. AIM: Recently, multiplex ligation-dependent probe amplification (MLPA), a simple technique for relative quantitation of targeted genomic regions, has been introduced in HA and HB for detection of large deletions and duplications...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28205285/natural-history-and-clinical-characteristics-of-inhibitors-in-previously-treated-haemophilia-a-patients-a-case-series
#20
A Iorio, A M Barbara, M Makris, K Fischer, G Castaman, C Catarino, E Gilman, K Kavakli, T Lambert, R Lassila, T Lissitchkov, E Mauser-Bunschoten, M E Mingot-Castellano, N Ozdemir, I Pabinger, R Parra, J Pasi, K Peerlinck, A Rauch, V Roussel-Robert, M Serban, A Tagliaferri, J Windyga, E Zanon
BACKGROUND: Development of inhibitors is the most serious complication in haemophilia A treatment. The assessment of risk for inhibitor formation in new or modified factor concentrates is traditionally performed in previously treated patients (PTPs). However, evidence on risk factors for and natural history of inhibitors has been generated mostly in previously untreated patients (PUPs). The purpose of this study was to examine cases of de novo inhibitors in PTPs reported in the scientific literature and to the EUropean HAemophilia Safety Surveillance (EUHASS) programme, and explore determinants and course of inhibitor development...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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