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Projects / Programmes source: ARIS

SYSTEMIC IMMUNE-MEDIATED DISEASES IN CHILDREN AND ADOLESCENTS

Research activity

Code Science Field Subfield
3.01.00  Medical sciences  Microbiology and immunology   

Code Science Field
B660  Biomedical sciences  Pediatrics 
Keywords
Pediatric antiphospholipid syndrome, Juvenile scleroderma, Vaccination, Mechanisms of immune tolerance, Inherited immune deficiencies
Evaluation (rules)
source: COBISS
Researchers (17)
no. Code Name and surname Research area Role Period No. of publicationsNo. of publications
1.  19209  PhD Aleš Ambrožič  Microbiology and immunology  Researcher  2008 - 2011  219 
2.  19258  PhD Tadej Avčin  Human reproduction  Head  2008 - 2011  471 
3.  20256  PhD Bojana Avguštin  Psychiatry  Researcher  2008 - 2011  67 
4.  13023  PhD Tadej Battelino  Medical sciences  Researcher  2008 - 2011  1,230 
5.  30144  Maja Čamernik    Technical associate  2009 - 2011  15 
6.  15657  PhD Maruša Debeljak  Oncology  Researcher  2008 - 2011  247 
7.  30143  Mateja Hren    Technical associate  2009 - 2011 
8.  10972  PhD Janez Jazbec  Oncology  Researcher  2008 - 2011  320 
9.  19257  PhD Lidija Kitanovski  Oncology  Researcher  2008 - 2011  155 
10.  29593  Gašper Markelj  Microbiology and immunology  Junior researcher  2010 - 2011  81 
11.  05325  PhD Darja Paro  Neurobiology  Researcher  2008 - 2011  351 
12.  07530  PhD Zvonka Rener-Primec  Neurobiology  Researcher  2008 - 2011  209 
13.  26481  PhD Snežna Sodin Šemrl  Microbiology and immunology  Researcher  2008 - 2011  319 
14.  28571  PhD Nataša Toplak  Microbiology and immunology  Researcher  2008 - 2011  176 
15.  20128  PhD Alenka Trampuš Bakija  Cardiovascular system  Researcher  2008 - 2011  127 
16.  29810  Tina Vesel  Microbiology and immunology  Researcher  2008 - 2011  65 
17.  28940  MSc Simona Eva Žitnik  Microbiology and immunology  Researcher  2008 - 2011 
Organisations (2)
no. Code Research organisation City Registration number No. of publicationsNo. of publications
1.  0312  University Medical Centre Ljubljana  Ljubljana  5057272000  77,334 
2.  1620  University Psyhiatric Hospital  Ljubljana - Polje  1191004000  3,457 
Abstract
Systemic immune-mediated diseases are one of the most common chronic illnesses of children and an important cause of short- and long-term disability. In the last decade, there have been dramatic advances in understanding the function of immune system, however, morbidity and mortality still remain serious threats for children with several systemic immune-mediated diseases. In addition to the complications that can occur in adults, specific concerns such as those related to growth, development, childhood vaccinations and quality of life characterize the disease in pediatric patients. The proposed research project will focus on the following systemic immune-mediated diseases in children and adolescents: 1. Pediatric antiphospholipid syndrome Based on literature data and personal experience obtained on treating children with elevated antiphospholipid antibodies (aPL), we believe that there are some important differences in pathophysiology and clinical spectrum of the APS related to the age at onset of the disease. Investigations in the present research proposal are designed to elucidate factors specific to children and to examine the clinical significance and pathogenic mechanisms of aPL in the pediatric population. 2. Juvenile scleroderma There are no accurate data on the long-term outcome in JLS patients treated with different protocols. In our study, we plan to evaluate the outcome in a large group of patients with JLS to determine impact of treatment on the long term outcome of JLS. 3. Autoimmune responses after vaccination In the proposed study we will evaluate safety and efficacy of influenza vaccination in a large group of healthy adults and in children with various systemic autoimmune diseases on immunosupressive medications. 4. Mechanisms of immune tolerance and autoimmune responses in pediatric patients on specific immunotherapy We will compare results of basophil activation test between pediatric patients with good clinical response and those with poor clinical response on treatment with specific immunotherapy. Since there are no accurate data on the induction of autoimmune phenomena during the process of specific immunotherapy, we plan to periodically assess also possible induction of disease-specific autoantibodies. 5. Inherited immune deficiencies In the proposed study we plan to investigate clinical and genetic characteristics of Central and South-Eastern European patients with chronic granulomatous disease.
Significance for science
Our research project was focused on systemic immune-mediated diseases which are one of the most common chronic disorders in pediatric population. Our studies were clinically focused with two main objectives: - development of better diagnostic ability for early identification of patients with different systemic immune-mediated diseases - recognition of patients with systemic-immune mediated disease that have worse long-term outcome and impact of treatment on the long-term outcome The research project provided data from a large cohort of pediatric patients with antiphospholipid syndrome. We reported original information on clinical presentation, laboratory features, and outcomes of pediatric APS. In addition, we identified some important differences when compared with antiphospholipid syndrome in adult patients. In a large prospective cohort study evaluating autoimmune responses following annual influenza vaccination we demonstrated that influenza vaccination did not increase the percentage of positive autoantibodies in the general healthy adult population and that newly synthesized autoantibodies after the influenza vaccination had no apparent clinical significance. Within a network of pediatric immunology centers in eastern and central European countries we performed analyses of clinical and genetic features in large cohorts of patients with rare primary immunodeficiency diseases including chronic granulomatous disease, autoinflammatory diseases, X-linked agammaglobulinemia and Wiskott-Aldrich syndrome.
Significance for the country
The research proposal was clinically focused and was aimed to improvement of health care including development of new clinical and laboratory diagnostic protocols in children with systemic immune-mediated diseases. We identified new clinical, laboratory and genetic features in children with systemic immune-mediated diseases that will contribute to the improved clinical care and will have impact on the long-term outcome of these patients. The results of our research project were presented at several international meetings and published in 16 articles in peer-reviewed journals and 3 book chapters.
Most important scientific results Annual report 2008, 2009, final report, complete report on dLib.si
Most important socioeconomically and culturally relevant results Annual report 2008, 2009, final report, complete report on dLib.si
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