[Pulmonary XXXX in Russia: the first results of the national register].

@entity1 @entity101 @entity28 @entity12584 @entity1066 @entity120 @entity7855 @entity460 @entity299
AIM: To investigate the demographic and clinical characteristics of @entity1 with @entity120 ( @entity120 ) and @entity120 ( @entity120 ), regions of their residence, the specific features of diagnosis and drug therapy, and the @entity1 ' survival within the framework of a multicenter open-label prospective study. SUBJECTS AND METHODS: The study enrolled @entity1 over 18 years of age with @entity120 classified as Group 1 ( @entity120 ) and Group 4 ( @entity120 ). The follow-up was made in 9 expert centers of Russia on January 1 to February 28, 2014. The data of @entity1 with @entity120 and @entity120 were electronically entered at the register website: www.pul-hyp.medibase.ru. RESULTS: The study included 242 @entity1 (183 @entity1 and 59 @entity1 ) from 44 regions of the Russian Federation. @entity120 of different etiologies was present in 79.3%, including 43.8% with idiopathic @entity120 , 28.5% with @entity299 -associated @entity120 , 6.2% with diffuse connective tissue-associated @entity120 , 0.8% with hereditary @entity120 ; 20.7% were diagnosed with @entity120 . The @entity1 ' mean age at the inclusion in the register was 43.2    15.8 years; the median @entity120 duration was 4.4 years (1.4 to 10.6%). The distance covered during a 6-minute walk test was 395.2?111.1 m; the Borg dyspnea index was 3.4    1.4 scores and the functional class was 2.5    0.8. After right cardiac catheterization, the mean pulmonary artery pressure was 53.1    19.5 mm Hg; cardiac output, 3.8    1.2 l/ min; cardiac index, 2.1    0.6 l/min/m2; pulmonary vascular resistance, 1105    677.6 dyn  sec/cm5. A positive acute pharmacological test was seen in 16.3% of the @entity1 . The most common comorbidity was erosive @entity1066 of the gastrointestinal tract (23.5%), @entity101 (16.9%), and @entity28 (16.5%). 66% of the @entity1 received @entity120 -specific therapy: @entity7855 (51.6%), @entity12584 (20.9%), or iloprost (7%); 7.4% of the @entity1 were observed within the framework of clinical trials, 17.7% received combined therapy for @entity120 : 16.3 and 1.4% had dual- or triple-component therapy, respectively; 55.8% of the @entity1 took @entity460 antagonists, 61.4% had diuretics, 85.6% had antithrombotic drugs. The survival rates were 98% in the first year of the follow-up and 90.1% by the end of 2013. CONCLUSION: The formation of a national register of @entity1 with @entity120 and @entity120 makes it possible to introduce current approaches to diagnosing and treating the @entity1 of this category and to evaluate the efficiency and quality of rendered medical care and will contribute to the estimation of required health care volumes and the expenditure of public health resources.
