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  • 1
    Electronic Resource
    Electronic Resource
    Health status and health-related quality of life of children with haemophilia from six West European countries (2004)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 10 (2004), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  A multicentre, international, cross-sectional study was carried out in the frame of field testing of the first haemophilia-specific quality-of-life (QoL) questionnaire (Haemo-QoL). The aim of this paper is to describe health status and health care and their impact on QoL in haemophilic children in Western Europe. Children aged 4–16 years with severe haemophilia without inhibitors were enrolled by 20 centres in France, Germany, Italy, the Netherlands, Spain and the United Kingdom. Clinical information was collected by the physicians with a medical documentation form. Health-related QoL (HRQoL) of children was assessed with Haemo-QoL, available for three age groups. Clinical data were available in 318 patients, 85.5% with haemophilia A. The mean age at first bleeding was 11 months, at first joint bleed 25 months. Functional joint impairments were found in 11.3%. Prophylaxis treatment was given to 66.7% of children in whom breakthrough bleeds occurred 0.4 times a month compared to 1.1 bleeds in children receiving on-demand treatment. A significantly higher factor consumption was found only in the two younger age groups of prophylaxis patients compared to on-demand patients. HRQoL was satisfactory in this cohort: young children were impaired mainly in the dimension ‘family’ and ‘treatment’, whereas older children had higher impairments in the so-called ‘social’ dimensions, such as ‘perceived support’ and ‘friends’. Health care of children in Western Europe is progressively improving with a large diffusion of home treatment and prophylaxis. This provides a high level of health status and HRQoL, being better in haemophilic adolescents on prophylaxis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1355-0691.2004.00876.x
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  • 2
    Electronic Resource
    Electronic Resource
    Severe protein C deficiency and aseptic osteonecrosis of the hip joint: a case report (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S159 
    Details
    ISSN: 1432-1076
    Keywords: Key words Protein C deficiency ; Aseptic osteonecrosis ; Thrombophilia ; Hip joint
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Homozygous congenital protein C deficiency is accompanied by severe thrombophilia. Thrombotic events can be reduced in number and severity by lifelong oral anticoagulation therapy. A 4-year-old boy was first diagnosed as having severe congenital homozygous protein C deficiency during the neonatal period when purpura fulminans occurred as the first manifestation of thrombosis. From this time he had been treated with phenprocoumon (INR 3.5–4.5). During an infection of the upper respiratory tract he developed signs of a new episode of purpura fulminans (INR 2.6). Additional anticoagulation therapy with LMW heparin (Clexane®) and protein C concentrate was given while the oral anticoagulation therapy was continued. On the third day of this episode the boy suffered from pain of unknown origin. MRI of the abdomen and of the pelvis revealed nontraumatic osteonecrosis of the hip joint. After a few weeks of immobilisation no special treatment was necessary. One year later he was able to walk with no problem. Conclusion Aseptic osteonecrosis of the hip joint is associated with a variety of disorders including vascular thrombosis and haemorrhage. Especially young children and handicapped patients with thrombophilia and pain of unknown origin are suspected to have thrombosis in atypical regions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014345
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  • 3
    Electronic Resource
    Electronic Resource
    Clinical relevance of genetic risk factors for thrombosis in paediatric oncology patients with central venous catheters (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S143 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We prospectively evaluated the clinical relevance of genetic risk factors of thrombosis in 137 paediatric patients with solid tumours or leukaemia/lymphoma. The factor V G1691A (FV-L), the prothrombin G20210A (FII-L) and the homozygous MTHFR variant were examined. In addition, protein C, protein S and antithrombin (AT) deficiency were evaluated in patients with ALL or thrombosis. The inter-group incidence of risk factors and thrombotic events was compared. 73 of the 137 patients had ALL and 64 another form of leukaemia, lymphoma or a solid tumour. They were treated according to the established paediatric tumour protocols ALL-BFM, NHL-BFM, COSS, CWS and others. All patients had central venous lines. No patient received heparin or any other anticoagulant. Endpoints of the study were thrombosis, regular completion of chemotherapy or death. Incidence of mutations in the whole group: FV-L (7.3% heterozygous, 0.7% homozygous); FII-L (2.9% heterozygous, no homozygotes); MTHFR (51.8% heterozygous, 10.9% homozygous). Ten patients (7.3%), 6 with ALL and 4 with solid tumours, developed thrombosis. 4 of the 6 patients with ALL and thrombosis (67%) but only 21% of ALL patients without thrombosis had a genetic risk factor (P 〈 0.013, χ2). No genetic defect was found in the 4 patients with other malignancies and thrombosis,. However, besides a tumour, these patients had additional exogenous risk factors including diabetes insipidus and hemiparesis. Conclusion Genetic mutations appear to be additional risk factors for the development of thrombosis in patients with ALL. In contrast, these mutations do not appear to be relevant risk factors for thrombosis in the small number of children with other malignant diseases reported here. This difference may be due to asparaginase and corticosteroids being used in ALL but not in solid tumour protocols.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014341
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