This work contains the results of a research of the dynamic processes in the “bridge-train” system while passenger trains move over a bridge structure (overpass) in high-speed. The article presents the methodology of mathematic modelling, and the basic differential equations of the studied system elements motion are provided. Also there is a description of dynamic interaction of the bridge-train system numerical model based on the FEM. In general, taking into account in the design scheme of the “bridge” system not only spans, but also piers with a foundation, it is possible to more accurately determine the values of the bridge natural frequencies, which is a key factor in assessing the dynamic response of a structure when passing a high-speed train.
BackgroundRegisters are an effective tool for tracing the dynamics of patients with rare pathologies.ObjectiveOur aim was to examine the demographic, clinical and genetic features of children with Gaucher disease in Russia.MethodsWe held a retrospective survey of the paediatric register data with regard to children suffering from Gaucher disease. The period of data accounting was from 2006 to 2016. Results: 115 children with Gaucher disease aged from 3 months to 17 years (the median age of diagnosis is 5 years) were registered; 62 of them (53.9%) are girls. The prevalence of the disease was 0.32 cases for 1 00 000 children. 95 (82,6%) children had Gaucher disease type 1; 6 (5.2%) — type 2 and 14 (12.2%) — type 3. The highest morbidity was in Central (27; 23,5%) and Volga (27; 23,5%) Federal Districts; and the least — in the Far East (3; 2.6%). At the time of diagnosis all the patients had splengomegaly. The genotype and phenotype correlations in 90 children with Gaucher disease were as follows: in case of type 1 (n=77) there are 21 (27.3%) patients with N370S/L444P genotype and 12 (15.6%) — N370S/other mutation; among 13 children with neuropathic forms (type 2 and type 3) there are 9 (62.9%) children with L444P/L444P genotype and 3 (23,1%) had L444P/D409H. The rest of genotypes were presented by other mutations, 13 of which were revealed for the first time. The p.W223R (p.W184R) mutation is specific for Russian patients. Enzyme replacement therapy was carried out for 109 patients (94,8%): in 105 (96,3%) children (Gaucher disease type 1 and type 3) with imiglucerase and in 4 (3.7%) children with type 1 — with velaglucerase alfa. Pathogenetic therapy stops the main symptoms in most patients.ConclusionThe paediatric Gaucher disease registry allows to systemize the data concerning the disease course in children and optimising the approaches to its monitoring in Russia.
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