SummaryThis study was aimed at development of cystic fibrosis (CF) patient register for those living at Moscow and Moscow region. The CF patients living in Moscow region were found to have some clinical, genetic and bacteriological differences from CF patients living at the Western Europe. The median survival in 2003-2012 was 39.5 years. Aging of CF patients was associated to reduction in respiratory function, occurrence of more aggres sive respiratory pathogens, increase in respiratory and gastrointestinal complication rate. Key words: cystic fibrosis, register, age related features.
РезюмеЦелью исследования явилось создание регистра больных муковисцидозом (МВ), проживающих в Москве и Московской области. Пока зано, что больные МВ московского региона имеют ряд клинико генетических и микробиологических особенностей по сравнению с пациентами ряда западных стран. Медиана выживаемости за период 2003-2012 гг. составила 39,5 лет. Продемонстрировано, что взрос ление больного МВ сопровождается снижением респираторной функции, сменой микрофлоры дыхательных путей на более агрессив ную, нарастанием частоты осложнений со стороны органов дыхания и пищеварения. Ключевые слова: муковисцидоз, регистр, возрастные особенности.
A non-interventional prospective cohort study was conducted on adult patients with cystic fibrosis, who received mucolytic therapy with 7% NaCl solution combined with 0.1% hyaluronic acid (medical product Hyaneb) and 7% NaCl solution for 4 and 8 weeks. Materials and methods: A total of 24 CF patients over 18 years old were enrolled in the study. A comparative analysis of respiratory function, therapy satisfaction, efficacy and safety of treatment was conducted following 4 weeks of therapy in 7% NaCl Solution Group (n = 12) and MP Hyaneb Group (n = 12), and the dynamics of these parameters was assessed against the background of the use of MP Hyaneb for 8 weeks (n = 12). Results: At week 4 of treatment, a significantly smaller number of patients complained of nasal congestion, ears and or accumulation of mucus in the throat and chest, irritation of the throat and unpleasant taste in the MP Hyaneb Group. No significant differences in the onset and increase in the intensity of cough were achieved. No significant differences between the groups in terms of spirometry parameters were identified. Findings: MP Hyaneb showed the best tolerability compared to 7% NaCl solution in adult patients with cystic fibrosis. The use of MP Hyaneb reduces the frequency and severity of irritation of the pharyngeal mucosa, cough and nasal congestion following administration for one month.
Генетическому разнообразию больных муковисцидозом (МВ) в России посвящены единичные работы на ограниченной выборке боль ных. Цель. Выявление особенностей генетического профиля больных МВ в России по данным Национального регистра (2014). Мате риалы и методы. Данные пациентов с МВ (n = 2 131) из 74 регионов России, включенные в Национальный регистр больных МВ (2014).
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