| Titre : | A national web-based register for noninvasive positive pressure ventilation in children (Poster) |
| contenu dans : | |
| Auteurs : | Collectif ; Mahloul M ; Leboucher T ; Vibert JF ; Cohen E ; Clément A ; Fauroux B |
| Type de document : | Article |
| Année de publication : | 2009 |
| Pages : | p. 53 |
| Langues: | Anglais |
| Résumé : | Context National registers on rare diseases give useful epidemiological data but they are limited by their feasibility, exhaustiveness, and cost. Our national reference center for rare lung diseases (RespiRare) developed, within a national programon rare diseases, a web-based system that consists in a secured and real-time patient medical record, including medical visits, hospitalisations, and all medical investigations. Eight accredited secondary centers distributed over the French territory have the same system and are connected with the national center. Systems have been developed for interstitial lung disease (e-Pi), primary ciliary dyskinesia, (e-Cil) lung malformation (e-Dev), and chronic respiratory insufficiency (e-R). Collection of biological data for research is possible. Results Six months after the start of the program, 82 children treated with long term noninvasive positive pressure ventilation (NPPV),mean age 10.4 ± 4.6 years (range 0.6 to 21.2 years), have been included in the e-R registry. The patients presented with neuromuscular disease (n=33), upper airway obstruction (n=27), lung disease (n=8), or other conditions (n=14). Seventy one (87%) patients were treated with pressure-targeted NPPV while 11 (14%) were treated with volume-targeted NPPV. The mean age at start of NPPV was 8.1 ± 4.4 years (range 1 month to 20 years). Conclusions To our knowledge, this web based medical system is the first national register developed for children with chronic respiratory insufficiency. The easy accessible, on line collection of real-time data on a nation-wide basis will improve the knowledge of the natural history of rare diseases, the effect of clinical and therapeutic management, and will allow genotype / phenotype correlation analyses. |
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