HaemophiliaThis is an RSS file. You can use it to subscribe to this data in your favourite RSS reader, such as GoogleReader, or to display this data on your own website or blog. Subscribe to this data using MyMedWorm.Subscribe to this data using GoogleReader.Subscribe to this data using Bloglines.Subscribe to this data using MyYahoo.

This page shows you the latest items in this publication.

Pages: 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15

747 records returned

VERITAS-Pro: a new measure of adherence to prophylactic regimens in haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Prophylactic treatment is recommended for severe haemophilia. Non-adherence to a prophylactic regimen can limit treatment effectiveness and compromise outcomes. The aim of this study is to validate a new prophylactic treatment adherence scale entitled Validated Hemophilia Regimen Treatment Adherence Scale [ndash] Prophylaxis (VERITAS-Pro), a self-/parent-report questionnaire consisting of 24 questions on six (four-item) subscales (Time, Dose, Plan, Remember, Skip, Communicate) that takes approximately 10 min to complete and is currently available in English only. Participants were recruited to complete the VERITAS...
Source: Haemophilia - November 19, 2009 Category: Hematology Authors: N. DUNCAN, W. KRONENBERGER, C. ROBERSON, A. SHAPIRO Source Type: journals

Cubital tunnel syndrome in patients with haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Elbow is the second most common joint involved in patients with haemophilia; however, there is little data about the involvement of ulnar nerve at elbow in patients with haemophilic arthropathy. The purpose of this study was to address this problem in the elbow and evaluate the results of anterior subcutaneous transposition of the ulnar nerve in a small group of patients with haemophilia who had been managed in two institutions. Information on six patients who were diagnosed with tardy ulnar nerve palsy in two institutions was retrospectively collected. All patients suffered form severe haemophilia A. Anterior sub...
Source: Haemophilia - November 18, 2009 Category: Hematology Authors: S. M. JAVAD MORTAZAVI, R. S. GILBERT, M. S. GILBERT Source Type: journals

The optimal mode of delivery for the haemophilia carrier expecting an affected infant is vaginal deliveryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. The optimal mode of delivery of a haemophilia carrier expecting a child is still a matter of uncertainty and debate. The aim of this commentary/review is to suggest that normal vaginal delivery should be the recommended mode of delivery for the majority of carriers, based on review of studies on obstetric aspects of haemophilia. About 2.0[ndash]4.0% of all haemophilia boys born in countries with a good standard of health care will suffer from ICH during the neonatal period. This is an average figure including all modes of delivery and regardless of whether the carrier status of the mother or the haemophilia status...
Source: Haemophilia - November 18, 2009 Category: Hematology Authors: R. LJUNG Source Type: journals

Major orthopaedic surgeries for haemophilia with inhibitors using rFVIIaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This report is the largest clinical report on major orthopaedic surgeries at a single institute. We have concluded that the combination of bolus and continuous infusion of rFVIIa is safe and effective, and more convenient to administer than simple bolus infusion therapy to achieve haemostasis at peri-operative periods. In addition, our data also concurs with the data of several previous reports which showed that orthopaedic surgery for haemophilia patients with inhibitors by means of rFVIIa is safe and effective. (Source: Haemophilia)
Source: Haemophilia - November 18, 2009 Category: Hematology Authors: H. TAKEDANI, H. KAWAHARA, M. KAJIWARA Source Type: journals

Haemorrhagic manifestations and prevalence of factor V deficiency in north-eastern IranEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - November 12, 2009 Category: Hematology Authors: H. MANSOURITORGHABEH, L. MANAVIFAR, A. MOBALEGH, A. SHIRDEL Source Type: journals

A review of long-term prophylaxis in the rare inherited coagulation factor deficienciesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. The rare inherited coagulation factor deficiencies (deficiencies of factors I, II, V, VII, XI, XIII, combined FV + FVII deficiency, combined deficiency of the vitamin K dependent factors and von Willebrand disease type 3) have an aggregate prevalence of approximately 1:100 000. They may cause recurrent life or function threatening haemorrhage. In this article we review the available literature on long-term prophylaxis and, where possible, make recommendations on this important area. (Source: Haemophilia)
Source: Haemophilia - November 11, 2009 Category: Hematology Authors: T. TODD, D. J PERRY Source Type: journals

Malignancy in patients with haemophilia: a review of the literatureEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Haemophilia A and B are rare X-linked conditions. Elevated rates of HIV and hepatitis C related malignancies in these patients are well reported, however rates of other types of cancers are not. Therefore, a retrospective literature review of cancer in patients with haemophilia was conducted. A Medline search of articles from January 1966 to July 2009 utilizing the keywords haemophilia, leukaemia, malignancy, mortality, neoplasm and cancer was performed. The articles were reviewed and additional relevant publications were located from the references. Data on age, type and severity of haemophilia, HIV status, type ...
Source: Haemophilia - November 11, 2009 Category: Hematology Authors: A. L. DUNN Source Type: journals

Purification and characterization of a new recombinant factor VIII (N8)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, the present data show that N8 is a pure and well-characterized FVIII product with biochemical properties that equal other FVIII products. (Source: Haemophilia)
Source: Haemophilia - November 11, 2009 Category: Hematology Authors: L. THIM, B. VANDAHL, J. KARLSSON, N. K. KLAUSEN, J. PEDERSEN, T. N. KROGH, M. KJALKE, J. M. PETERSEN, L. B. JOHNSEN, G. BOLT, P. L. NØRBY, T. D. STEENSTRUP Source Type: journals

Successful management of fresh-frozen plasma transfusion therapy based upon clinical symptoms for total knee arthroplasty in a patient with severe factor V deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - November 11, 2009 Category: Hematology Authors: C. RIDEAU, E. GAERTNER, M. BLAY, C. TROJANI, P. TOULON Source Type: journals

Development and preliminary testing of a Paediatric Version of the Haemophilia Activities List (pedhal)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Worldwide, children with haemophilia suffer from limitations in performing activities of daily living. To measure such limitations in adults a disease-specific instrument, the Haemophilia Activities List (HAL), was created in 2004. The aim of this study was to adapt the HAL for children with haemophilia and to assess its psychometric properties. The structure and the main content were derived from the HAL. Additionally, items of the Childhood Health Assessment Questionnaire and the Activity Scale for Kids were considered for inclusion. This version was evaluated by health professionals (n = 6), patients (n = 4), a...
Source: Haemophilia - November 11, 2009 Category: Hematology Authors: W. G. GROEN, J. van der NET, P. J. M. HELDERS, K. FISCHER Source Type: journals

Upper gastrointestinal bleeding in children with haemophilia: a clinical significance of Helicobacter pylori infectionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. For patients with haemophilia, gastrointestinal (GI) bleeding is a life-threatening complication and can be caused by the Helicobacter pylori infection. Among children with haemophilia who had visited with GI bleeding, the prevalence of H. pylori infection and the recurrence rate after H. pylori eradication was investigated. Seven children with haemophilia A with hematemesis (age: 5.3[ndash]17.0 years) were evaluated for the causes of GI bleeding and the detection of H. pylori. Gastroendoscopy was done to find the bleeding focus and for further evaluation including rapid urease test and mucosal biopsy. Four patien...
Source: Haemophilia - November 5, 2009 Category: Hematology Authors: B. H. CHOE, J. Y. KIM, J. H. LEE, J. M. KIM, M. A. CHU, S. M. CHO, K. S. LEE Source Type: journals

Thrombin generation in vitro in the presence of by-passing agents in siblings with severe haemophilia AEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we evaluated and compared thrombin generation in vitro using plasma samples in the presence of by-passing agents (FEIBA® and NovoSeven®) in 21 unrelated brother pairs with and without inhibitors enrolled in the Malmö International Brother Study (MIBS). Mean maximum thrombin formation in patients with a current inhibitor titer was 182.0 ± 52.8 mmol mL[minus]1 (FEIBA®) and 130.7 ± 54.9 mmol mL[minus]1 (rFVIIa), respectively, and somewhat higher in those without inhibitors, 222.7 ±85.5 mmol mL[minus]1 (FEIBA®) and 142.8 ±53.6mmol mL[minus]1 (rFVIIa) (P = 0.16 and 0.29). The variance regarding the maxim...
Source: Haemophilia - October 31, 2009 Category: Hematology Authors: J. KLINTMAN, E. BERNTORP, J. ASTERMARK Source Type: journals

Postmarketing surveillance study of KOGENATE® Bayer with Bio-Set® in patients with haemophilia A: evaluation of patients' satisfaction after switch to the new reconstitution systemEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. KOGENATE® Bayer (rFVIII-FS) with Bio-Set® is designed to prevent patient contact with exposed needles during recombinant factor VIII reconstitution. This postmarketing surveillance study evaluated patient satisfaction before and after switching to the new Bio-Set reconstitution method. Male children and adults with haemophilia A were enrolled from nine European countries. A preference questionnaire was administered to patients after Bio-Set training and at the end of the observation period ([ge]20 exposure days or 3 months). Physician assessments of patient compliance and satisfaction were conducted at the end o...
Source: Haemophilia - October 30, 2009 Category: Hematology Authors: N. VIDOVIC, R. MUSSO, R. KLAMROTH, M. M. ENRIQUEZ, K. ACHILLES Source Type: journals

A ferric chloride induced arterial injury model used as haemostatic effect modelEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, we have demonstrated that under optimized conditions the FeCl3 induced arterial injury model provides a therapeutic window that makes it an useful effect model for evaluation of the haemostatic potential of procoagulant drugs. (Source: Haemophilia)
Source: Haemophilia - October 30, 2009 Category: Hematology Authors: F. MØLLER, M. TRANHOLM Source Type: journals

Mosaicism and haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Mosaicism may affect the haemophilia phenotype. Well-known instances include chromosomal mosaicism due to aneuploidy and pseudo-mosaicism due to varying patterns of X-chromosome inactivation. Chromosomal mosaicism in a chimera is a potential source of phenotypic variation. Gene mosaicism is commonplace. Its pattern and effect depend on the stage of development at which a mutation occurs. Proven or possible genetic mosaicism is an important consideration when predicting the likelihood of transmission of haemophilia to a future generation. (Source: Haemophilia)
Source: Haemophilia - October 30, 2009 Category: Hematology Authors: C. K. KASPER, C. H. BUZIN Source Type: journals

How do we treat: upper gastrointestinal bleeding in adults with haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: P. A. KOUIDES, P. F. FOGARTY Source Type: journals

Early long-term FEIBA prophylaxis in haemophilia A patients with inhibitor after failing immune tolerance inductionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study was performed to assess the effectiveness and safety of early prophylaxis with FEIBA in preventing bleeding and joint damage after failed ITI. Seven paediatric patients proceeded immediately after failed ITI to long-term FEIBA prophylaxis at 60[ndash]100 IU kg[minus]1 dosages and various dosing intervals depending upon bleeding tendency. Bleeding episodes and joint status were assessed. Thrombin generation assays (TGA) were also used to gauge treatment response. FEIBA prophylaxis was commenced at a median age of 6.0 years (range, 1.5[ndash]11.8 years) and continued for a median duration of 6.9 years (range, 0.8[...
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: C. ESCURIOLA ETTINGSHAUSEN, W. KREUZ Source Type: journals

Long-term prophylaxis in patients with factor XIII deficiency complicated by intracranial haemorrhage in IranEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: P. ESHGHI, S. B. MAHJOUR, M. NADERI, J. DEHBOZORGIAN, M. KARIMI Source Type: journals

Rectus muscle sheath haematoma in a patient with congenital FX deficiency and in another with congenital FVII deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: A. GIROLAMI, E. ALLEMAND, F. TEZZA, D. PELLATI, R. SCANDELLARI Source Type: journals

Physical activity levels and participation in sport in Irish people with haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Physical activity and sport are associated with a range of health and social benefits. The aim of this study was to assess the level of sports participation and physical activity of Irish people with haemophilia (PWH). A questionnaire was administered to Irish PWH attending the National Centre for Hereditary Coagulation Disorders over a 3-month period. This included the International Physical Activity Questionnaire (IPAQ) and the Haemophilia Activities List (HAL). Comparisons with EU average data from European Physical Activity Surveillance System for physical activity scores (IPAQ) were made using independent t-t...
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: E. SHERLOCK, J. S. O'DONNELL, B. WHITE, C. BLAKE Source Type: journals

New early prophylaxis regimen that avoids immunological danger signals can reduce FVIII inhibitor developmentEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. The most problematic complication of haemophilia A treatment is the development of inhibitors to FVIII. The highest risk of developing inhibitors is during the first 20 exposure days (EDs). If the patient can be brought through this high risk period without inhibitor development, the subsequent risk is low. Therefore, as a pilot project, we developed a prophylaxis regimen for the first 20[ndash]50 EDs specifically designed to induce tolerance to the administered FVIII and to minimize inhibitor development by avoiding immunological danger signals. Twenty-six consecutive previously untreated patients (PUPs) with sev...
Source: Haemophilia - October 29, 2009 Category: Hematology Authors: K. KURNIK, C. BIDLINGMAIER, W. ENGL, H. CHEHADEH, B. REIPERT, G. AUERSWALD Source Type: journals

A study of reported factor VIII use around the worldEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of this paper was to study the reported factor VIII (FVIII) use on a country-by-country basis. Data on the reported FVIII use for 104 countries were obtained from the Marketing Research Bureau, Inc. and the World Federation of Hemophilia. The results show that FVIII use varies considerably among countries, even among the wealthiest of countries. The use of FVIII concentrate increases as economic capacity increases; in addition, consumption of FVIII has been increasing at a greater rate in high income countries. Given these trends, there probably will be a global increase in FVIII concentrates usage. Such info...
Source: Haemophilia - October 21, 2009 Category: Hematology Authors: J. S. STONEBRAKER, M. BROOKER, R. E. AMAND, A. FARRUGIA, A. SRIVASTAVA Source Type: journals

Cost of care of haemophilia with inhibitorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. In Western countries, the treatment of patients with inhibitors is presently the most challenging and serious issue in haemophilia management, direct costs of clotting factor concentrates accounting for >98% of the highest economic burden absorbed for the healthcare of patients in this setting. Being designed to address questions of resource allocation and effectiveness, decision models are the golden standard to reliably assess the overall economic implications of haemophilia with inhibitors in terms of mortality, bleeding-related morbidity, and severity of arthropathy. However, presently, most data analyses stem...
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: M. N. D. DI MINNO, G. DI MINNO, M. DI CAPUA, A. M. CERBONE, A. COPPOLA Source Type: journals

How we treat: patients with haemophilia undergoing screening colonoscopyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: P. F. FOGARTY, P. KOUIDES Source Type: journals

Development and validation of a new questionnaire for the assessment of subjective physical performance in adult patients with haemophilia – the HEP-Test-QEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Specific research studies for the investigation of physical performance in haemophilic patients are rare. However, these instruments become increasingly more important to evaluate therapeutic treatments. Within the frame of the Haemophilia & Exercise Project (HEP), a new questionnaire, namely HEP-Test-Q, has been developed for the assessment of subjective physical performance in haemophilic adults. In this article, the development and validation of the HEP-Test-Q is described. The development consisted of different phases including item collection, pilot testing and field testing. The preliminary version was pilot...
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: S. von MACKENSEN, D. CZEPA, M. HERBSLEB, T. HILBERG Source Type: journals

To circumcise or not to circumcise? Circumcision in patients with bleeding disordersEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Circumcision is one of the most common procedures performed in male neonates, but few published reports have described circumcision in patients with bleeding disorders. The aim of this study was to analyse outcomes of circumcision among children evaluated at our institution to determine the extent of complications and to provide guidelines for circumcision management. We searched our patient database for records of children who were followed up at the Mayo Clinic Comprehensive Hemophilia Center from 2000 through 2007 and who had been circumcised. We retrospectively reviewed the medical records to document complica...
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: V. RODRIGUEZ, R. TITAPIWATANAKUN, C. MOIR, K. A. SCHMIDT, R. K. PRUTHI Source Type: journals

Effect of glycoPEGylation on factor VIIa binding and internalizationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Recombinant coagulation factor VIIa (rFVIIa), which is widely used for treatment of bleeding episodes in haemophilia patients with inhibitors, is cleared from the circulation relatively fast with a plasma half-life of 2[ndash]4 h. PEGylation is an established and clinically proven strategy for prolonging the circulatory life-time of bio-therapeutic proteins. The aim of this study was to investigate the effect of glycoPEGylation of rFVIIa on rFVIIa binding to its cellular receptors and its subsequent internalization. rFVIIa and glycoPEGylated rFVIIa were labeled with 125I and the radio-iodinated proteins were used ...
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: P. SEN, S. GHOSH, M. EZBAN, U. R. PENDURTHI, L. VIJAYA MOHAN RAO Source Type: journals

A study of variations in the reported haemophilia A prevalence around the worldEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objectives of this paper were to study the reported haemophilia A prevalence (per 100 000 males) on a country-by-country basis and address the following: Does the reported prevalence of haemophilia A vary by national economies? We collected prevalence data for 106 countries from the World Federation of Hemophilia (WFH) annual global surveys and the literature. We found that the reported haemophilia A prevalence varied considerably among countries, even among the wealthiest of countries. The prevalence (per 100 000 males) for high income countries was 12.8 ± 6.0 (mean ± SD) whereas it was 6.6 ± 4.8 for the rest of th...
Source: Haemophilia - October 20, 2009 Category: Hematology Authors: J. S. STONEBRAKER, P. H. B. BOLTON-MAGGS, J. MICHAEL SOUCIE, I. WALKER, M. BROOKER Source Type: journals

Study of mutations in Jordanian patients with haemophilia A: identification of five novel mutationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Haemophilia A (HA) is an X-linked recessive bleeding disorder caused by mutations in the factor VIII gene (F8), which encodes factor VIII (FVIII) protein, a plasma glycoprotein, that plays an important role in the blood coagulation cascade. In the present study, our aim was to identify F8 gene mutations in HA patients from Jordan. One hundred and seventy-five HA patients from 42 unrelated families were included in this study. Among these patients, 117 (67%) had severe HA, 13 (7%) had moderate HA and 45 (26%) had mild HA. Severe patients were first tested for intron-22 inversion using long range polymerase chain re...
Source: Haemophilia - October 7, 2009 Category: Hematology Authors: A. AWIDI, M. RAMAHI, D. AlHATTAB, R. MEFLEH, M. DWEIRI, N. BSOUL, A. MAGABLAH, E. ARAFAT, M. BARQAWI, M. BISHTAWI, E. HADDADEEN, M. FALAH, B. TARAWNEH, S. SWAIDAN, S. FAUORI Source Type: journals

Commentary on Knight et al.: A systematic review of the cost-effectiveness of rFVIIa and APCC in the treatment of minor/moderate bleeding episodes for haemophilia patients with inhibitorsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 6, 2009 Category: Hematology Authors: J. W. HAY, Z.-Y. ZHOU Source Type: journals

Efficacy and safety of highly purified, doubly virus-inactivated VWF/FVIII concentrates in inherited von Willebrand's disease: results of an Italian cohort study on 120 patients characterized by bleeding severity scoreEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. The efficacy of highly purified VWF/FVIII concentrates with standardized ristocetin cofactor content (VWF:RCo) has been already proven in patients with von Willebrand's disease (VWD). Aim of this retrospective study is to confirm efficacy and safety of two highly purified, doubly virus-inactivated VWF/FVIII concentrates in a large cohort of patients with VWD who were characterized at enrolment by bleeding severity score. Study drugs Alphanate or Fanhdi were given to 120 cases (51 males, 69 females, median age 50 years, range 6[ndash]83 years). Patients had VWD3 (10), VWD2A (19), VWD2B (25), VWD2M (10) and DDAVP-un...
Source: Haemophilia - October 5, 2009 Category: Hematology Authors: A. B. FEDERICI, G. BARILLARI, E. ZANON, M. G. MAZZUCCONI, R. MUSSO, R. TARGHETTA, P. M. MANNUCCI Source Type: journals

Sorafenib as a feasible therapeutic option in haemophiliacs with hepatocellular carcinomaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 4, 2009 Category: Hematology Authors: M. LAPECORELLA, M. NAPOLITANO, M. TUDINI, G. BRUERA, A. LUCCHESI, A. V. GIORDANO, G. MARIANI, E. RICEVUTO Source Type: journals

Influence of aquatic training on the motor performance of patients with haemophilic arthropathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Thirteen patients with haemophilia A took part in this study voluntarily. They underwent an aquatic training programme over a 9-week period (27 sessions; three sessions per week; 1 h per session). Their motor performance was assessed by the following cardio-respiratory and mechanical variables before and after the training programme: oxygen uptake (VO2, mL min[minus]1), relative oxygen uptake (rel VO2, mL min[minus]1·kg[minus]1), carbon dioxide (CO2, mL min[minus]1), respiratory quotient (R), heart rate (bpm) and the distance covered in 12 min (the Cooper test, m). Nine patients successfully completed the interve...
Source: Haemophilia - October 4, 2009 Category: Hematology Authors: L. VALLEJO, A. PARDO, M. GOMIS, J. E. GALLACH, S. PÉREZ, F. QUEROL Source Type: journals

Effectiveness of a balance training home exercise programme for adults with haemophilia: a pilot studyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Adults with haemophilia and other bleeding disorders often develop lower limb musculoskeletal problems associated with bleeds into joints and muscles, which may affect balance performance and increase likelihood of falling. The aim of this study was to evaluate the effectiveness of an individualized balance and strength home exercise programme on improving balance and related outcomes for adults with haemophilia and other bleeding disorders. Twenty male adults with haemophilia and other bleeding disorders (mean age 39.4 years, 95% CI = 33.7[ndash]45.1) were recruited to participate. They underwent a comprehensive ...
Source: Haemophilia - October 4, 2009 Category: Hematology Authors: K. HILL, M. FEARN, S. WILLIAMS, L. MUDGE, C. WALSH, P. McCARTHY, M. WALSH, A. STREET Source Type: journals

Prosthetic heart valves and rare hypersensitivity to vitamin K antagonists resulting from factor IX mutation: how to manage anticoagulation?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - October 4, 2009 Category: Hematology Authors: A. HOLBRO, G. A. MARBET, T. H. TRAN, J. OLDENBURG, O. FRIESEWINKEL, D. A. TSAKIRIS Source Type: journals

Mycophenolate in the remission induction of a patient with acquired haemophilia AEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - September 24, 2009 Category: Hematology Authors: Y. S. LEE, H. J. NG Source Type: journals

Haemophilia care in India: innovations and integrations by various chapters of Haemophilia Federation of India (HFI)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, several of these approaches are discussed with the understanding that 80% of worlds' haemophilia population needs similar help, and the national haemophilia organizations (NMO) of various developing countries will find some of the approaches useful and adaptable to their own circumstances. (Source: Haemophilia)
Source: Haemophilia - September 23, 2009 Category: Hematology Authors: K. GHOSH, S. SHETTY, D. SAHU Source Type: journals

FEIBA prophylaxis in haemophilia patients: a clinical update and treatment recommendationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. In patients with severe haemophilia and inhibitors, regular factor VIII inhibitor bypassing activity (FEIBA) prophylaxis has been shown to reduce the frequency of bleeding by up to 85% and to improve patient quality of life. FEIBA is well tolerated; the incidence of thrombotic events and of allergic reactions is extremely low. The concept of prophylaxis in haemophilia patients with inhibitors is relatively new and some clinicians may be unsure of how to use FEIBA in this context. These treatment recommendations, based on published evidence plus the collective experience of a group of haematologists (with practical...
Source: Haemophilia - September 22, 2009 Category: Hematology Authors: D. PERRY, E. BERNTORP, C. TAIT, G. DOLAN, P. A. HOLME, M. LAFFAN, R. LASSILA, A. MUMFORD, J. PASI, J. WILDE, A. WILL, T. T. YEE Source Type: journals

In vitro and in vivo stability of diluted recombinant factor VIII for continuous infusion use in haemophilia AEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Factor VIII (FVIII) replacement by continuous infusion (CI) is used postoperatively or after significant bleeding. For young paediatric patients, CI may require FVIII dilution. Variable stabilities of diluted full-length recombinant FVIII Kogenate® FS (KG-FS) have been reported under different storage conditions. We investigated the recovery and stability of diluted KG-FS in vitro and in vivo. Kogenate® FS was diluted to 50[ndash]120 U mL[minus]1 and its recovery and stability in glass vials or polypropylene syringes was determined. Furthermore, stability of KG-FS diluted to 80 U mL[minus]1'administered' via sin...
Source: Haemophilia - September 16, 2009 Category: Hematology Authors: S. REVEL-VILK, V. S. BLANCHETTE, M. SCHMUGGE, D. S. CLARK, D. LILLICRAP, M. L. RAND Source Type: journals

Pregnancy in a patient with severe factor X deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - September 16, 2009 Category: Hematology Authors: A. MAMOPOULOS, S. VAKALOPOULOU, E. LEFKOU, A. FILELI, V. GARIPIDOU, G. MAVROMATIDIS, K. DINAS, V. KARAGIANNIS Source Type: journals

Current challenges of pharmacovigilance in bleeding disorders: converting the burden to benefitEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary Safety surveillance studies have proven essential in research and development of new biological therapies for bleeding disorders as well as other diseases. Although product safety regarding HIV, hepatitis, and other blood-borne infections is currently excellent, potential new infectious agents require continued vigilant monitoring. Inhibitor development is the most common serious side effect of haemophilia replacement therapy. Several aetiological factors associated with inhibitors have been identified, but their true impact is still largely unknown. Moreover, whether plasma-derived and recombinant factor products ...
Source: Haemophilia - September 15, 2009 Category: Hematology Authors: R. LASSILA, E. ARMSTRONG Source Type: journals

Development, implementation and evaluation of a new self-help programme for mothers of haemophilic children in Korea: a pilot studyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Developing an effective support group programme is necessary to help the mothers of haemophilic children to encourage their children to live healthily and independently through early management, as well as to reduce the mothers' depression and stress. Although the need is high, there is no self-help group programme for mothers in Korea yet.The purpose of this study was to develop, implement and evaluate a new self-help group programme for mothers of children with haemophilia.Pre-experimental design was used to evaluate the effect of a pilot group. Participants were 12 mothers of haemophilic children below 15 years...
Source: Haemophilia - September 15, 2009 Category: Hematology Authors: H. S. KANG, W. O. KIM, K. J. CHO, Y. JEONG Source Type: journals

Determinants of factor VIII plasma levels in carriers of haemophilia A and in control womenEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Factor VIII (FVIII) levels show a considerable variability in female carriers of haemophilia A. Presently, the reasons for this are poorly understood. The aim of the study was to elucidate the influence of genetic and non-genetic parameters on FVIII plasma levels in carriers (n = 42). Results were compared with age-matched healthy women without carriership of haemophilia A (n = 42). Each carrier was tested for the family-specific mutation, ABO blood group, FVIII level, von Willebrand factor (VWF) antigen and activity and C-reactive protein (CRP). FVIII levels were lower in carriers compared to non-carriers [74% (5...
Source: Haemophilia - September 15, 2009 Category: Hematology Authors: C. AY, K. THOM, F. ABU-HAMDEH, B. HORVATH, P. QUEHENBERGER, C. MALE, C. MANNHALTER, I. PABINGER Source Type: journals

VERITAS-PRN: a new measure of adherence to episodic treatment regimens in haemophiliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Episodic treatment of bleeding disorders is defined as utilization of clotting factor concentrates in response to acute bleeding episodes to achieve haemostasis. Non-adherence to prescribed episodic regimens can limit treatment effectiveness and result in target joint formation, pain and disability. Evaluation of and interventions to promote adherence may improve health outcomes. The purpose of this study was to validate a new adherence scale developed for individuals with bleeding disorders treated on episodic infusion regimens, entitled VERITAS-PRN [Validated Hemophilia Regimen Treatment Adherence Scale [ndash] ...
Source: Haemophilia - September 14, 2009 Category: Hematology Authors: N. A. DUNCAN, W. G. KRONENBERGER, C. P. ROBERSON, A. D. SHAPIRO Source Type: journals

The national registry of haemophilia A and B in Spain: results from a census of patientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Haemophilia)
Source: Haemophilia - September 14, 2009 Category: Hematology Authors: J. A. AZNAR, L. ABAD-FRANCH, V. R. CORTINA, P. MARCO Source Type: journals

Assessment of the progression of haemophilic arthropathy in childrenEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Arthropathy is considered as an irreversible and progressive complication in patients with haemophilia, even in children on prophylaxis. To estimate the progression of haemophilic arthropathy, 85 joints of 24 boys with severe (n = 18) and moderate (n = 6) haemophilia (A: 22, B: 2) were investigated with clinical examination, X-rays and magnetic resonance imaging (MRI) at two time periods (time 0 and 1). Patients' age at time 0 was 10.5 ± 3.6 years and time elapsed to time 1 was 3.8 ± 1.4 years. At time 0: all investigated joints had more than three bleeds. Sixteen boys were on secondary prophylaxis for 5.4 ± 2....
Source: Haemophilia - September 9, 2009 Category: Hematology Authors: H. PERGANTOU, H. PLATOKOUKI, G. MATSINOS, O. PAPAKONSTANTINOU, A. PAPADOPOULOS, P. XAFAKI, D. PETRATOS, S. ARONIS Source Type: journals

Haemopoietic stem cell transplantation induces tolerance to donor antigens but not to foreign FVIII peptidesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. A 22-year-old male with severe haemophilia A and high responding factor VIII (FVIII) inhibitor underwent sibling haematopoietic stem cell transplantation in an attempt to eradicate the inhibitor. A reduced intensity conditioning regimen was followed by bone marrow infusion and continuous FVIII administration during immune reconstitution. Although substantial levels of FVIII:C (>100 IU dL[minus]1) were maintained initially, at day +23 inhibitor titres rose, indicating boosting of recipient memory repertoire, despite complete donor chimerism. On day +46, he developed Klebsiella pneumoniae septicaemia and died. This ...
Source: Haemophilia - September 3, 2009 Category: Hematology Authors: J. UPRICHARD, F. DAZZI, J. F. APPERLEY, M. A. LAFFAN Source Type: journals

Profiling of factor VIII mutations in Korean haemophilia AEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. Haemophilia A (HA) is an X chromosome-linked inherited bleeding disorder caused by heterogeneous mutations of coagulation factor VIII (FVIII). Although more than 900 mutations of FVIII gene are reported in the HAMSTeRS database, the mutation data regarding the FVIII gene in the Korean population is currently insufficient. The aim of this study was to profile the mutations of FVIII in Korean HA, 38 unrelated Korean HA male patients were examined. Peripheral blood samples were obtained from the patients. Long distance-PCR was performed for the identification of inversions in intron 22 and intron 1. Then gross exon d...
Source: Haemophilia - August 30, 2009 Category: Hematology Authors: S. H. HWANG, M. J. KIM, J. A. LIM, H. C. KIM, H. S. KIM Source Type: journals

Thrombin generation testing in haemophilia comprehensive care centresEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study will review unmet laboratory needs in haemophilia and the potential applications of TGT in the management of haemophiliacs. Furthermore, technical and standardization issues of the method will be discussed. (Source: Haemophilia)
Source: Haemophilia - August 27, 2009 Category: Hematology Authors: Y. DARGAUD, C. NEGRIER Source Type: journals

Uncomplicated neurosurgical resection of a malignant glioneuronal tumour under haemostatic cover of rFVIIa in a severe haemophilia patient with a high-titre inhibitor: a case report and literature review of rFVIIa use in major surgeriesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary. The development of inhibitors following factor VIII replacement therapy is a serious complication in severe inherited haemophilia. Whereas significant experience, notably in orthopaedic surgery, is now obtained with the use of bypassing agents in haemophilia with high-titre inhibitor, new surgical challenges might occur due to patients' increasing life expectancy. A 56-year-old severe haemophilia A patient with a high-titre inhibitor was diagnosed for probable right temporoparietal malignant glioneuronal tumour on cerebral magnetic resonance imaging (MRI) (4 cm x 3 cm cerebromeningeal tumour with perilesional oede...
Source: Haemophilia - August 25, 2009 Category: Hematology Authors: A. AOUBA, E. DEZAMIS, A. SERMET, C. ROTHSCHILD, O. HERMINE, D. LASNE, M-F. TORCHET Source Type: journals