Pathogenic  treatment of cystic fibrosis: the first clinical case in Russia

Amelina, E.; Красовский, С. А.; Usacheva, M.; Крылова, Н. А.

doi:10.18093/0869-0189-2017-27-2-298-301

Cited by 5 publications

(1 citation statement)

References 5 publications

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“…На настоящий момент этот список можно дополнить реализацией программы трансплантации легких (с 2012 г. эта операция проведена 36 пациентам с МВ), внедрением в рутинную практику гастростомии и началом патогенети-ческого лечения. Необходимо отметить, что перечисленные выше современные методы были инициированы и внедрены под началом и при непосредственном участии сотрудников лаборатории муковисцидоза НИИ пульмонологии [5][6][7]. Но если такое многообещающее лечение, как патогенетическое, на конец 2018 года проведено всего лишь нескольким пациентам в России и никак существенно не повлияло на показатели статистики, то трансплантация легких и тесно связанная с ней ночная гипералиментация через гастростому уже сыграли свою роль в сохранении жизни нескольких десятков пациентов.…”

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The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018

Krasovskiy

Amelina

Gorinova

et al. 2018

Journal of Clinical Practice

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Background: Evaluation of the main clinical and functional indicators and their relationship with the treatment and diagnostic care at different time periods can contribute to the development of further effective medical strategies and globally lead to improved care for cystic fibrosis patients. Aim: An assessment of the dynamics of some clinical and functional indicators in adult CF patients, who have been observed ed Research Institute of Pulmonology during the last 15 years(2003–2018). Methods: The comparative analysis was performed on the data of adult patients with cystic fibrosis, observed at 31.12.2003 and at the end of October of 2018. The group of patients in 2003 consists of 80 and in 2018 — of 667 patients. The comparative analysis between the groups was carried out according to the following indicators: age, survival, lung function, nutritional status, proportion of infection with pathogenic microflora, identification of mutations in the cystic fibrosis gene and diagnosis in adult patients. Results: An increase in patient’s age, survival rate, the proportion of identified mutations in cystic fibrosis gene and infection of Burkholderia cepacia complex in the group of patients in 2018 was revealed. There were no differences in the state of lung function, nutritional status, the ratio of the living and dead patients and proportion of patients diagnosed in adulthood between groups. Conclusion: The progress in the treatment through the introduction of modern methods and therapeutic programs leads to improved survival and an increase in the number of adult patients with cystic fibrosis.

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Section: Discussionunclassified

The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018

Krasovskiy

Amelina

Gorinova

et al. 2018

Journal of Clinical Practice

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Тargeted therapy for CF patients with F508del/F508del genotype

Амелина

Красовский

Шумкова

et al. 2019

Pulʹmonologiâ (Mosk.)

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Targeted therapy for cystic fibrosis (CF) is a novel approach to CF treatment that can restore and potentiate CFTR channel activity. Lumacaftor/ivacaftor combination therapy is related to significant clinical and functional benefits in CF patients who are homozygous for F508del CFTR mutation. The authors described a case of effective treatment with lumacaftor/ivacaftor combination in a homozygous F508del CF patient with severe lung disease. This experience demonstrates an urgent need to make this pathogenic treatment available for patients with this genotype in Russia.

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Cystic Fibrosis: A Breakthrough in 21st-Century Therapy

Simonova

Gorinova

Chernevich

2020

RPJ

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The review presents new data on the latest advances in the treatment of cystic fibrosis, a rare genetic disease. The methods used were literature search in the Scopus, Web of Science, and EMBASE databases. The importance of a number of drugs prescribed for anti-inflammatory purposes (ibuprofen, azithromycin) is discussed, data from multicenter studies of new drugs are presented. The role of mucolytic agents and the need to develop new antibacterial compounds are shown. Particular attention in the review is given to the development of new targeted therapies for cystic fibrosis. The data of studies of ivacaftor, lumacactor, tezacactor in this category of patients are presented, as well as a spectrum of mutations of the cystic fibrosis gene in which these molecules can be prescribed. The experience of the use of correctors in adult patients with cystic fibrosis in the Russian Federation is described, the effectiveness and safety of the long-term use of these drugs are described.

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Pathogenic treatment of cystic fibrosis: the first clinical case in Russia

Cited by 5 publications

References 5 publications

The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018

The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018

Тargeted therapy for CF patients with F508del/F508del genotype

Cystic Fibrosis: A Breakthrough in 21st-Century Therapy

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