Haemophilia care in Europe: the ESCHQoL study.
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Haemophilia care in Europe: the ESCHQoL study. / Schramm, W; Gringeri, A; Ljung, R; Berger, K; Crispin, A; Bullinger, Monika; Giangrande, P L F; Mackensen von, Sylvia; Mantovani, L G; Nemes, L; Serban, M; Group, ESCHQOL Study.
in: HAEMOPHILIA, Jahrgang 18, Nr. 5, 5, 2012, S. 729-737.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Haemophilia care in Europe: the ESCHQoL study.
AU - Schramm, W
AU - Gringeri, A
AU - Ljung, R
AU - Berger, K
AU - Crispin, A
AU - Bullinger, Monika
AU - Giangrande, P L F
AU - Mackensen von, Sylvia
AU - Mantovani, L G
AU - Nemes, L
AU - Serban, M
AU - Group, ESCHQOL Study
PY - 2012
Y1 - 2012
N2 - The aim of this study was to determine the clinical conditions of patients with haemophilia within Europe as recommended by the European Commission. In this multicentre, cross-sectional, ambispective study, conducted within 21 European countries patients' clinical data were collected, amongst others haemophilia type, severity, treatment pattern, use of factor products, bleeding, orthopaedic joint scores and infections. A total of 1400 patients, 84.3% with haemophilia A and 15.7% with haemophilia B were enrolled by 42 centres between 2004 and 2006. Thereof, 417 were children (30.0%) and 983 were adults (70.0%). About 70% of patients had severe factor deficiency (<1%). More than half of the adults were carriers of chronic infections (12.6% HIV, 55.8% HCV), compared to only 3.8% children (no HIV, 2.9% HCV). Patients were grouped according to per capita amount of clotting factor used in patients' region of residence in 2005: region 1: >5 IU; region 2: 2-5 IU; region 3: <2 IU. Paediatric and adult patients in region 3 had median numbers of three and eight joint bleeds, respectively, with worse joint scores compared to region 1 with zero and one bleed. Prophylactic therapy was used in only 31.3% children and 8.9% adults with severe haemophilia in region 3 compared to 93.7% and 54.1%, respectively, in region 1. Statistical analysis revealed that residence in areas with low factor consumption/availability is the most prominent risk factor for joint disease. Access of European patients with haemophilia to optimal care with safe factor VIII concentrates is limited and depends on the region of residence.
AB - The aim of this study was to determine the clinical conditions of patients with haemophilia within Europe as recommended by the European Commission. In this multicentre, cross-sectional, ambispective study, conducted within 21 European countries patients' clinical data were collected, amongst others haemophilia type, severity, treatment pattern, use of factor products, bleeding, orthopaedic joint scores and infections. A total of 1400 patients, 84.3% with haemophilia A and 15.7% with haemophilia B were enrolled by 42 centres between 2004 and 2006. Thereof, 417 were children (30.0%) and 983 were adults (70.0%). About 70% of patients had severe factor deficiency (<1%). More than half of the adults were carriers of chronic infections (12.6% HIV, 55.8% HCV), compared to only 3.8% children (no HIV, 2.9% HCV). Patients were grouped according to per capita amount of clotting factor used in patients' region of residence in 2005: region 1: >5 IU; region 2: 2-5 IU; region 3: <2 IU. Paediatric and adult patients in region 3 had median numbers of three and eight joint bleeds, respectively, with worse joint scores compared to region 1 with zero and one bleed. Prophylactic therapy was used in only 31.3% children and 8.9% adults with severe haemophilia in region 3 compared to 93.7% and 54.1%, respectively, in region 1. Statistical analysis revealed that residence in areas with low factor consumption/availability is the most prominent risk factor for joint disease. Access of European patients with haemophilia to optimal care with safe factor VIII concentrates is limited and depends on the region of residence.
KW - Adult
KW - Humans
KW - Male
KW - Aged
KW - Middle Aged
KW - Aged, 80 and over
KW - Risk Factors
KW - Adolescent
KW - Quality of Life
KW - Young Adult
KW - Prospective Studies
KW - Child
KW - Cross-Sectional Studies
KW - Child, Preschool
KW - Retrospective Studies
KW - Health Services Accessibility
KW - Europe/epidemiology
KW - Blood Coagulation Factors/administration & dosage/economics
KW - Hemarthrosis/etiology
KW - Hemophilia A/complications/economics/epidemiology/therapy
KW - Hemophilia B/complications/economics/epidemiology/therapy
KW - Adult
KW - Humans
KW - Male
KW - Aged
KW - Middle Aged
KW - Aged, 80 and over
KW - Risk Factors
KW - Adolescent
KW - Quality of Life
KW - Young Adult
KW - Prospective Studies
KW - Child
KW - Cross-Sectional Studies
KW - Child, Preschool
KW - Retrospective Studies
KW - Health Services Accessibility
KW - Europe/epidemiology
KW - Blood Coagulation Factors/administration & dosage/economics
KW - Hemarthrosis/etiology
KW - Hemophilia A/complications/economics/epidemiology/therapy
KW - Hemophilia B/complications/economics/epidemiology/therapy
M3 - SCORING: Journal article
VL - 18
SP - 729
EP - 737
JO - HAEMOPHILIA
JF - HAEMOPHILIA
SN - 1351-8216
IS - 5
M1 - 5
ER -
