Source: Annual Congress 2007 - Paediatric respiratory epidemiology: rare diseases, infectious diseases and asthma
Disease area: Paediatric lung diseases

Abstract

The Russian registry of Cystic Fibrosis began collecting data on Russian cystic fibrosis patients in 2001. The information can be used in clinical work and research. The database of registry includes 463 parameters, which are grouped in 55 tables (438 parameters are dynamic). The registry consists of the following sections: personal data, anamnesis, data of out-patient examination (every 3 months), data of hospitalization. Now registry contains the information on 641 patients (340M/311F aged 1mth-34 yrs), 578 patients alive, 63(9,8%) died. 189 (29,5%) patient constantly live at Moscow and Moscow region.
The following data were received with the help of the registry: severe CF symptoms have 452 (70,5%) patients, 636 (99,2 %) have pancreatic insufficiency. The most often complications CF were liver cirrhosis-77(12%), chronic lung heart-48 (7,5%), diabetes mellitus-24 (3,7%) patients. Genotype analysis had been performed on 281(43,8%) of these patients: 187 (66,5%) carries delF508 mutation. The basic treatment includes pancreatic enzymes (100% patients), vitamins (100%), mucolytics (90%), UDCA (88%), rhDNase (30%). The Russian CF registry will allow to quickly analyze the information on all patients observed at the Russian CF centre, and also to exchange the information with the regional CF centers.

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Citations should be made in the following way:
T. Kapustina, N. Kashirskaia, N. Kapranov, A. Voronkova (Moscow, Russian Federation). The Russian registry of cystic fibrosis. Eur Respir J 2007; 30: Suppl. 51, 2353

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