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2.
Immunol Res ; 67(4-5): 358-367, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31515711

RESUMO

Jeffrey Modell Foundation centers' network activities in Central and Eastern Europe (JMF CEE) have contributed to the development of care for patients with primary immunodeficiencies. On the data continuously collected from individual centers in participating countries since 2011, we demonstrate a steady improvement in a number of aspects concerning complex care for patients with primary immunodeficiencies. The presented data show an improvement of awareness about these rare diseases across the whole Central and Eastern European region, an increase in newly diagnosed patients as well as genetically confirmed cases, earlier establishment of diagnosis, and improved access to clinical treatment. We also present an active patient involvement that is reflected in the expansion of patient organization centers and their activities. The cooperation within the JMF CEE network has also contributed to greater international exposure of participating centers and further to the gradual development of research activities in the rapidly evolving field of primary immunodeficiencies. The improvement of all important aspects of the complex field of primary immunodeficiencies within the JMF CEE network documents the strength and advantages of the joint and coordinated networking.


Assuntos
Doenças da Imunodeficiência Primária/epidemiologia , Europa Oriental/epidemiologia , Feminino , Humanos , Masculino , Doenças da Imunodeficiência Primária/diagnóstico
3.
Hamostaseologie ; 28 Suppl 1: S52-4, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18958340

RESUMO

Clinical expression of inadequately treated haemophilia is dominated by orthopedic complications, requiring invasive or non-invasive interventions. OBJECTIVE: In Romania, with under dosed and late introduced "on demand" substitution, we aimed at highlighting the experience of orthopedic treatment and its outcome. PATIENTS, METHODS: Single center retrospective analysis regarding orthopedic interventions and their outcomes was conducted on 59 hemophilia A, B, and von Willebrand disease patients, between 2002 and 2007. RESULTS: The majority of interventions, invasive (60.71%) and non-invasive (39.28%), were elective, only two being emergencies. Postoperative functional evolution after synovectomies was good in 68.28%, fair in 24.39%, satisfactory in 7.31%. Results of 33 non-invasive (extensive releases) procedures were very good in 27.27%, good in 63.63%, poor in 9.09%. DISCUSSION, CONCLUSIONS: The important number and complexity of orthopedic interventions are proving the precarious musculoskeletal state in persons with hemophilia, demonstrating the need of improving substitution, at least with discontinue prophylaxis in patients with severe forms.


Assuntos
Hemartrose/complicações , Hemartrose/cirurgia , Hemofilia A/complicações , Procedimentos Ortopédicos , Anestesia Geral , Hemartrose/fisiopatologia , Humanos , Dor/etiologia , Dor/fisiopatologia , Amplitude de Movimento Articular , Sistema de Registros , Estudos Retrospectivos , Romênia , Sinovectomia
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