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Holstein K.,Universitatsklinikum Hamburg Eppendorf | Klamroth R.,Klinik fuer Innere Medizin und Haemophiliezentrum | Richards M.,Paediatric Haematology | Perez-Garrido R.,Haematology | Gringeri A.,University of Milan
Haemophilia | Year: 2012

There are no evidence-based guidelines on pain management in people with haemophilia (PWH), who may suffer acute, disabling pain from haemarthroses and chronic arthropathic pain. To review evidence and to investigate current clinical practice in pain assessment and management in PWH the European Haemophilia Therapy Standardisation Board undertook a literature review and a survey in 22 Haemophilia Treatment Centres (HTC), using a questionnaire and seven clinical scenarios. Consensus was sought on pain assessment and management in PWH. Few clinical studies on pain management in PWH were identified. The HTCs care for 1678 children (47% severe haemophilia, 84% on prophylaxis, 17% with arthropathy and 8% with chronic pain) and 5103 adults (44% severe haemophilia, 40% on prophylaxis, 67% with arthropathy and 35% with chronic pain). Analgesics are prescribed by HTCs in 80% of cases (median; range 0-100%) and in 10% (median; range 0-80%) are bought over the counter. Pain and analgesic use are assessed when reported by patients and at check-ups. Only eight centres use a specific pain scale and/or have specific pain guidelines. Two HTCs arrange regular consultations with pain specialists. For acute pain, the preferred first-line drug is paracetamol for children, and paracetamol or non-steroidal anti-inflammatory drugs (NSAIDs) for adults. Children with chronic pain are treated with paracetamol or NSAIDs, whereas adults usually receive Cox-2 inhibitors. Second-line therapy is heterogeneous. There is little published evidence to guide pain assessment and management in PWH, and clinical practice varies considerably across Europe. General and specific recommendations are needed. © 2012 Blackwell Publishing Ltd.

Srivastava A.,Christian Medical College | Brewer A.K.,Royal Infirmary | Mauser-Bunschoten E.P.,University Utrecht | Key N.S.,University of North Carolina at Chapel Hill | And 7 more authors.
Haemophilia | Year: 2013

Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies. © 2012 Blackwell Publishing Ltd.

Gratwohl A.,University of Basel | Baldomero H.,University of Basel | Schwendener A.,University of St. Gallen | Gratwohl M.,University of St. Gallen | And 3 more authors.
Bone Marrow Transplantation | Year: 2011

Six hundred and fifteen centers from 45 countries reported a total 30 293 HSCT to this 2008 EBMT survey with 26 810 first transplants (40% allogeneic, 60% autologous). This corresponds to an increase of 7% for the allogeneic and 3% for the autologous HSCT. Main indications were leukemias (32%; 89% allogeneic); lymphomas (56%; 89% autologous); solid tumors (6%; 96% autologous); and non-malignant disorders (6%; 88% allogeneic). There were more unrelated than HLA-identical sibling donors (49 vs 46%). The proportion of peripheral blood transplants remained stable with 99% for autologous and 70% for allogeneic HSCT. One fifth of the teams with >80 HSCT performed more than half of all HSCT. This trend towards teams with higher numbers of HSCT was stronger for allogeneic (Gini coefficient 57%) than for autologous HSCT (Gini coefficient 38%). Transplant rates (number of transplants per 10 million inhabitants) increased in a close to linear way with increasing team density (number of transplant teams per 10 million inhabitants) and without saturation (R2=0.54); this connection was even stronger for allogeneic HSCT (R2=0.67). These data illustrate status and trends for HSCT in Europe. They provide a rational basis for planning and patient counseling. © 2011 Macmillan Publishers Limited All rights reserved.

Neufeld E.J.,Harvard University | Galanello R.,University of Cagliari | Viprakasit V.,Mahidol University | Aydinok Y.,Ege University | And 10 more authors.
Blood | Year: 2012

This was a 24-week, multicenter phase- 2 study designed to assess safety, tolerability, and pharmacodynamics of FBS0701, a novel oral chelator, in adults with transfusional iron overload. Fifty-one patients, stratified by transfusional iron intake, were randomized to FBS0701 at either 14.5 or 29 mg/kg/d (16 and 32 mg/kg/d salt form). FBS0701 was generally well tolerated at both doses. Forty-nine patients (96%) completed the study. There were no drug-related serious adverse events. No adverse events (AEs) showed dose-dependency in frequency or severity. Treatment-related nausea, vomiting, abdominal pain, and diarrhea were each noted in < 5% of patients. Mean serum creatinine did not change significantly from Baseline or between dose groups. Transaminases wer increased in 8 (16%), three of whom acquired HCV on-study from a single blood bank while five had an abnormal baselineALT. The 24 week mean change in liver iron concentration (ΔLIC) at 14.5 mg/kg/d was +3.1 mg/g (dw); 29% achieved a decrease in LIC. Mean ΔLIC at 29 mg/kg/d was -0.3 mg/g (dw); 44% achieved a decrease in LIC (P < .03 for ΔLIC between doses). The safety and tolerability profile at therapeutic doses compare favorably to other oral chelators. This trialwasregistered atwww.clinicaltrials. gov as NCT01186419. © 2012 by The American Society of Hematology.

Davies J.,Haematology | Chant R.,Basingstoke and North Hampshire Foundation Trust | Simpson S.,Basingstoke and North Hampshire Foundation Trust | Powell R.,Peninsula Medical School
Transfusion Medicine | Year: 2011

Background/Aims: Prophylactic anti-D given during pregnancy can be detected in current indirect antiglobulin tests (IAT). Using this to measure the persistence of prophylactic anti-D, this study set out to determine whether there is an association between anti-D detectable at delivery and the RhD status of the foetus and/or the duration of the pregnancy post the standard 28 week dose of routine antenatal anti-D prophylaxis (RAADP). The study also investigated the detection rates of anti-D at delivery when given in a two dose regime or a one dose regime. Method: All IAT screening was undertaken using fully automated Diamed gel technology. The results from 407 women were included in the two dose regime study, and 157 in the one dose regime study. Results: 160/407 (39%) women receiving one dose of prophylactic anti-D had no detectable anti-D at delivery. 123/157 (78%) women on the one dose regime had no detectable anti-D at delivery. No association was found between detectable anti-D at delivery and the RhD status of the infant in either study arm. A strong association was found between detectable anti-D Ig at delivery and the duration of the pregnancy post the 28 week dose in each study arm. Conclusion: Our data show that neither the two dose nor the one dose regime appear to provide adequate cover at delivery for a large percentage of pregnant women. This appears to be associated with the duration of the pregnancy past the 28 week dose but not associated with the RhD status of the foetus. © 2011 The Authors. Transfusion Medicine © 2011 British Blood Transfusion Society.

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