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Mutational Spectrum of the CFTR Gene in the Kazakhstan Population

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F22%3A10444546" target="_blank" >RIV/00064203:_____/22:10444546 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/22:10444546

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=PMHEIqMFbS" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=PMHEIqMFbS</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1007/s13312-022-2518-1" target="_blank" >10.1007/s13312-022-2518-1</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Mutational Spectrum of the CFTR Gene in the Kazakhstan Population

  • Original language description

    Objective To study the frequency and spectrum of CFTR gene variants in different ethnic groups of Kazakhstan. Methods We reviewed the records of 58 patients with cystic fibrosis. All the patients underwent molecular genetic analysis to reveal genotype-phenotype correlations. Results The median (IQR) age of the patients was 5.4 year (7 months, 18 year); 40% were diagnosed at the age of 5-10 year. The study identified 28 specific variants: p.Phe508del, the variant most common in the European population, was detected in 30 patients (51.7%). Variants other than p.Phe508del were revealed in 31% (21 patients). Conclusions We found a number of specific variants characteristic of the Kazakhstani population. A pronounced regression of disease symptoms was detected in patients with mild mutations; whereas in patients with severe mutations, therapy produced very little effect.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30101 - Human genetics

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2022

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Indian Pediatrics

  • ISSN

    0019-6061

  • e-ISSN

    0974-7559

  • Volume of the periodical

    59

  • Issue of the periodical within the volume

    5

  • Country of publishing house

    IN - INDIA

  • Number of pages

    4

  • Pages from-to

    380-383

  • UT code for WoS article

    000805198600006

  • EID of the result in the Scopus database

    2-s2.0-85130633781