Present International Projects in the Area of Diagnostics and Therapy of Cystic Fibrosis: Participation of Specialists from the Czech Republic into the Problem Solving
Authors:
J. Brázová 1; M. Macek ml. 2
Authors‘ workplace:
Pediatrická klinika UK 2. LF a FN Motol, Praha
přednosta prof. MUDr. J. Lebl, CSc.
1; Ústav biologie a lékařské genetiky UK 2. LF a FN Motol, Praha
přednosta prof. MUDr. M. Macek ml., DrSc.
2
Published in:
Čes-slov Pediat 2008; 63 (2): 106-112.
Overview
Cystic fibrosis (CF) is one of the most systematically studied severe and chronic monogenic disorders. Despite this fact, many pathophysiological mechanisms still remain to be elucidated in order to assure customised therapeutic strategies. Experts from all over the world have been investigating this complex disorder in order to understand its complex pathophysiology. In this regard, high numbers of cases are necessary for valid clinical studies. Therefore, the current trend in CF research is focused on international cooperation. Investigators from around the world cooperate within international projects pertaining to all aspects of CF, i.e. starting from the study of the basic CFTR gene defect, through all types of CF clinical trials, including analysis of relevant psychosocial issues. This article summarizes Czech participation in international projects related to CF.
Key words:
cystic fibrosis, ECORN-CF, EuroCareCF, ECFS, CF Europe, Rapsody, EuroGentest, CF Thematic Network, Snip2Chip, Micro2DNA
Sources
1. Bush A, Alton EWFW, Davies JC, et al. Cystic Fibrosis in the 21st Century. S. Karger AG, 2006.
2. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am. J. Respir. Crit. Care Med. 2003;168(8): 918–951.
3. Schoni MH. Macrolide antibiotic therapy in patients with cystic fibrosis. Swiss Med. Wkly. 2003;133(21–22): 297–301.
4. Malfroot A, Adam G, Ciofu O, et al. Immunisation in the current management of cystic fibrosis patients. J. Cyst. Fibros. 2005;4(2): 77–87.
5. Pier G. Application of vaccine technology to prevention of Pseudomonas aeruginosa infections. Expert Rev. Vaccines 2005;4(5): 645–656.
6. Lang AB, Rudeberg A, Schoni MH, et al. Vaccination of cystic fibrosis patients against Pseudomonas aeruginosa reduces the proportion of patients infected and delays time to infection. Pediatr. Infect. Dis. J. 2004;23(6): 504–510.
7. Zuercher AW, Imboden MA, Jampen S, et al. Cellular immunity in healthy volunteers treated with an octavalent conjugate Pseudomonas aeruginosa vaccine. Clin. Exp. Immunol. 2005;142(2): 381–387.
8. Piedra PA, Cron SG, Jewell A, et al. Immunogenicity of a new purified fusion protein vaccine to respiratory syncytial virus: a multi-center trial in children with cystic fibrosis. Vaccine 2003;21(19–20): 2448–2460.
9. Doring G, Elborn JS, Johannesson M, et al. Clinical trials in cystic fibrosis. J. Cyst. Fibros. 2007;6(2): 85–99.
Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2008 Issue 2
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