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Year of Publication
2.  The European Haemophilia Network (EUHANET) 
Blood Transfusion  2014;12(Suppl 3):s515-s518.
doi:10.2450/2014.0008-14s
PMCID: PMC4044796  PMID: 24922291
haemophilia; EUHANET; reference networks; European; certification
3.  The European standards of Haemophilia Centres 
Blood Transfusion  2014;12(Suppl 3):s525-s530.
Introduction
The European haemophilia community of professionals and patients has agreed on the principles of haemophilia care to address comprehensive optimal delivery of care which is nowadays scattered throughout Europe. Many of the health facilities call themselves Haemophilia Centres despite their variation in size, expertise and services provided. Only a small number of countries have Haemophilia Centre accreditation systems in place.
Methods
In the framework of the European Haemophilia Network project, following an inclusive process of stakeholder involvement, the European Guidelines for the certification of haemophilia centres have been developed in order to set quality standards for European Haemophilia Centres and criteria for their certification.
Results
The Guidelines define the standards and criteria for the designation of two levels of care delivery: European Haemophilia Treatment Centres, providing local routine care, and European Haemophilia Comprehensive Care Centres, providing specialised and multi-disciplinary care and functioning as tertiary referral centres. Additionally, they define standards about general requirements, patient care, provision of an advisory service and establishment of network of clinical and specialised services.
Conclusions
The implementation of the European Guidelines for the certification of Haemophilia Centres will contribute to the reduction of health inequalities through the standardisation of quality of care in European Union Member States and could represent a model to be taken into consideration for other rare disease groups.
doi:10.2450/2014.0056-14s
PMCID: PMC4044800  PMID: 24922293
standards and criteria; certification system; haemophilia care; EUHANET
4.  The methodology for defining the European Standards for the certification of Haemophilia Centres in Europe 
Blood Transfusion  2014;12(Suppl 3):s519-s524.
Introduction
Work Package 4 Development of the standardisation criteria of the European Haemophilia Network project has the main objective of implementing a common and shared European strategy for a certification system for two levels of Haemophilia Centres: European Haemophilia Treatment Centres and European Haemophilia Comprehensive Care Centres in the Member States of the European Union.
Materials and methods
An inclusive and participatory process for developing shared standards and criteria for the management of patients with inherited bleeding disorders has been carried out. The process has been implemented through four different consultation events involving the entire European community of stakeholders that significantly contributed in the drafting of the European Guidelines for the certification of Haemophilia Centres.
Results
The Guidelines set the standards for the designation of centres that provide specialised and multidisciplinary care (Haemophilia Comprehensive Care Centres) as well as local routine care (Haemophilia Treatment Centres). Standards cover several issues such as: general requirements; patient care; advisory services; laboratory; networking of clinical and specialised services.
Conclusions
The drafting of the European Guidelines for the certification of Haemophilia Centres was performed adopting a rigorous methodological approach. In order to build the widest possible consensus to the quality standards, the main institutional and scientific stakeholders have been involved. The resulting document will significantly contribute in promoting standardisation in the quality of diagnosis and treatment in European Haemophilia Centres.
doi:10.2450/2014.0046-14s
PMCID: PMC4044802  PMID: 24922292
standards; criteria; certification system; haemophilia care; EUHANET
5.  A certification/accreditation model for Haemophilia Centres in Italy 
Blood Transfusion  2014;12(Suppl 3):s505-s509.
Background
The Italian Association of Haemophilia Centres has developed a voluntary programme of professional accreditation of Haemophilia Centres, run by its members. Participation in the programme, which aims to foster staff involvement in clinical governance, includes both medical personnel and nurses.
Materials and methods
Accreditation is awarded provided the candidate Haemophilia Centre is able to adhere to a pre-established set of quality standards and meet a number of clinical and organisational requirements, previously defined on the basis of evidence-based medicine. Self-evaluation is the first step in the programme, followed by a site visit by a team of peer professionals experienced in quality auditing.
Results
The programme has so far involved 21 Italian Haemophilia Centres. The comparison between self- and peer-evaluation revealed less discrepancies for disease-related than for organisational requirements, the latter being met to a lesser degree by most Haemophilia Centres.
Discussion
This programme of professional accreditation developed by the Italian Association of Haemophilia Centres has the potential to describe, monitor and improve clinical and organisational performances in the management of patients with haemophilia and allied inherited coagulation disorders. It should also be seen as a contribution to the implementation of the strategy for improving professional governance in Haemophilia Centres.
doi:10.2450/2014.0331-13s
PMCID: PMC4044808  PMID: 24922289
professional accreditation; quality of care; standards
6.  Prophylactic efficacy and pharmacokinetically guided dosing of a von Willebrand factor/factor VIII concentrate in adults and children with von Willebrand's disease undergoing elective surgery: a pooled and comparative analysis of data from USA and European Union clinical trials 
Blood Transfusion  2013;11(4):533-540.
Background
Surgical intervention may pose significant risk of life-threatening bleeding in patients with von Willebrand's disease; prophylactic treatment with von Willebrand factor/factor VIII concentrate is generally indicated for von Willebrand's disease characterized by moderate to severe qualitative and quantitative deficiencies of Willebrand factor to raise and maintain both Willebrand factor and FVIII at haemostatic levels for surgical prophylaxis.
Materials and methods
Since prospective clinical data in such situations were lacking, two recent, prospective, multicentre studies evaluated the prophylactic perioperative use of the on Willebrand factor/ factor VIII concentrates, Humate-P® and Haemate P. Despite some differences in the two studies, one conducted in the USA (n =35) and one in the European Union (n =27), the designs were similar enough to allow for a limited pooled analysis of data. In both studies, preoperative loading doses and subsequent maintenance doses were calculated using individual subject-derived incremental in vivo recovery values, although von Willebrand factor:ristocetin cofactor and FVIII:coagulation activity target levels differed between the protocols. Efficacy was rated daily by the investigator as excellent, good, moderate, or poor.
Results
Overall haemostatic efficacy (rating of excellent/good), assessed 24 hours after the last infusion (USA) or taken as the worst rating between surgery and day 14 (EU), was achieved in 95% of the pooled population of 62 adults and children. Efficacy did not appear to be affected by dosing variations. The rate of possibly related adverse events was low (8 subjects; 13%); one of these events was considered serious (pulmonary embolism).
Discussion
This pooled analysis of a relatively large number of patients for a rare disease confirms the feasibility of pharmacokinetically guided dosing of von Willebrand factor/factor VIII concentrate and highlights its efficacy and safety in the prevention of excessive perioperative bleeding.
doi:10.2450/2013.0254-12
PMCID: PMC3827397  PMID: 23736911
von Willebrand's disease; factor VIII; prophylaxis; elective surgery
7.  Recombinant factor VIIa as haemostatic therapy in advanced liver disease 
Blood Transfusion  2013;11(4):487-490.
doi:10.2450/2012.0066-12
PMCID: PMC3827390  PMID: 23114524
recombinant factor VIIa; tranexamic acid; liver cirrhosis; liver transplantation; hepatectomy
8.  Plasma-derived medicinal products: demand and clinical use 
Blood Transfusion  2013;11(Suppl 4):s2-s5.
doi:10.2450/2013.002s
PMCID: PMC3853983  PMID: 24333309
9.  Present and future challanges in the treatment of haemophilia: a clinician’s perspective 
Blood Transfusion  2013;11(Suppl 4):s77-s81.
doi:10.2450/2013.012s
PMCID: PMC3853988  PMID: 24333317
haemophilia; factor VIII; factor IX; inhibitors; recombinant technology
10.  The demand for factor VIII and for factor IX and the toll fractionation product surplus management 
Blood Transfusion  2013;11(Suppl 4):s64-s76.
doi:10.2450/2013.011s
PMCID: PMC3853991  PMID: 24333316
plasma-derived medicinal products; factor VIII; factor IX; demand; surplus management
11.  Liver disease, coagulopathies and transfusion therapy 
Blood Transfusion  2013;11(1):32-36.
doi:10.2450/2012.0151-12
PMCID: PMC3557474  PMID: 23058863
liver disease; coagulopathy; transfusion therapy
12.  Hypercoagulability in congenital haemolytic anaemias 
Blood Transfusion  2012;10(4):423-427.
doi:10.2450/2011.0031-11
PMCID: PMC3496237  PMID: 22044960
thalassaemia; sickle cell anaemia; thrombosis; therapy
13.  Prophylaxis for adults with haemophilia: towards a personalised approach? 
Blood Transfusion  2012;10(2):123-124.
doi:10.2450/2012.0182-11
PMCID: PMC3320771  PMID: 22337279
14.  Factor VIII inhibitor and source of replacement therapy 
Blood Transfusion  2012;10(1):112-113.
doi:10.2450/2011.0064-11
PMCID: PMC3259001  PMID: 22044948
16.  Evidence-based recommendations on the treatment of von Willebrand disease in Italy 
Blood Transfusion  2009;7(2):117-126.
Background
von Willebrand disease (VWD) is the most common hereditary bleeding disorder affecting both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes bleeding of mucous membranes and soft tissues. The aim of treatment is to correct the dual defect of haemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII).
Material and methods
This document contains evidence-based recommendations for the management of VWD compiled by AICE (the Italian Association of Haemophilia Centres). All the evidence supporting these recommendations are based on non-randomised comparative studies or case series, because randomised controlled clinical trials or meta-analyses are not available for this disease.
Results and conclusions
Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD with FVIII and VWF levels of 10 U/dL or more, while VWF/FVIII concentrates are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2 and 3 VWD). VWF concentrates devoid of FVIII, not yet licensed in Italy, may be considered for short-term prophylaxis in elective surgery or for long-term secondary prophylaxis.
doi:10.2450/2008.0052-08
PMCID: PMC2689066  PMID: 19503633
von Willebrand disease; desmopressin; von Willebrand factor
17.  Prophylaxis in congenital coagulation disorders: past, present and future 
Blood Transfusion  2008;6(Suppl 2):s1-s3.
doi:10.2450/2008.0029-08
PMCID: PMC2652216  PMID: 19105502
18.  Advantages and limits of ADAMTS13 testing in thrombotic thrombocytopenic purpura 
Blood Transfusion  2008;6(3):127-135.
doi:10.2450/2008.0056-07
PMCID: PMC2626866  PMID: 18705236
ADAMTS13; thrombotic thrombocytopenic purpura; TTP
19.  Autoimmune haemophilia 
Blood Transfusion  2008;6(1):6-7.
doi:10.2450/2008.0004-08
PMCID: PMC2626853  PMID: 18661917

Results 1-19 (19)