Cystic fibrosis revisited - a Review study
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F62690094%3A18470%2F17%3A50013388" target="_blank" >RIV/62690094:18470/17:50013388 - isvavai.cz</a>
Alternative codes found
RIV/00179906:_____/17:10369443
Result on the web
<a href="http://dx.doi.org/10.2174/1573406412666160608113235" target="_blank" >http://dx.doi.org/10.2174/1573406412666160608113235</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.2174/1573406412666160608113235" target="_blank" >10.2174/1573406412666160608113235</a>
Alternative languages
Result language
angličtina
Original language name
Cystic fibrosis revisited - a Review study
Original language description
Background: Cystic fibrosis (CF) is an incurable, chronic disease, which causes severe damages to respiratory and digestive tracts. It is the most common genetically inherited disease among caucasians. This disease is caused by defects in CF genes, the so-called mutations in cystic fibrosis transmembrane conductance regulator (CFTR) gene population. At present over 100,000 people suffer from this disease worldwide. Objective: The purpose of this review study is to describe the pathophysiology of CF and provide the latest information on its diagnosis and treatment therapies with respect to the improvement of patient's quality of life and emphasis on targeted specialized care. Method: The methodological approaches include a method of literature review of available sources exploring the issue of cystic fibrosis both from a global and specific perspective point of view. A search was performed in the databases PubMed, MEDLINE, Web of Science, Scopus, Springer and ScienceDirect. Furthermore, other sources cited in the analyzed studies were also examined. On the basis of evaluation of these literature sources, the research issue was explored. Results: The main benefits (e.g., specialized centres for the treatment of CF exist or a new breakthrough in the gene therapy of CF has been made) and limitations (e. g., comorbidity of CF, lifelong and costly treatment, or adverse impact on patient's and caregiver's quality of life) in the treatment of narcolepsy are highlighted. Conclusion: CF requires an integrated treatment approach in specialized CF centers, involving various factors contributing to a better patient's state of health in the form of relevant and well-balanced non-pharmacological and pharmacological therapies. In addition, further large scale clinical trials are needed in order to develop compounds that are aimed at the most common classes of CFTR.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30104 - Pharmacology and pharmacy
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2017
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Medicinal chemistry
ISSN
1573-4064
e-ISSN
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Volume of the periodical
13
Issue of the periodical within the volume
2
Country of publishing house
AE - UNITED ARAB EMIRATES
Number of pages
8
Pages from-to
102-109
UT code for WoS article
000402476700001
EID of the result in the Scopus database
2-s2.0-85013812138