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X-linked agammaglobulinemia caused by new mutation in BTK gene: a case report

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00209775%3A_____%2F14%3A%230000284" target="_blank" >RIV/00209775:_____/14:#0000284 - isvavai.cz</a>

  • Result on the web

    <a href="http://biomed.papers.upol.cz/pdfs/bio/2014/03/23.pdf" target="_blank" >http://biomed.papers.upol.cz/pdfs/bio/2014/03/23.pdf</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.5507/bp.2013.011" target="_blank" >10.5507/bp.2013.011</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    X-linked agammaglobulinemia caused by new mutation in BTK gene: a case report

  • Original language description

    Primary immunodeficiencies (PID) are becoming a recognized public health problem worldwide. The most important subgroup of these disordes are the antibody deficiencies. X-linked agammaglobulinaemia was the first described entity of this group and is characterised by early onset of recurrent bacterial infections, profound deficiency of all immunoglobulin isotypes and markedly reduced number of peripheral B.-lymphocytes. We report the case of a 10-year old boy with X-linked agammaglobulinaemia caused by apreviously non-described mutation in BTK gene with typical clinical presentation but delayed diagnosis. Following diagnosis, substitution therapy with intravenous immunoglobulins was started and the clinical status of the patient improved. We reported acase of X-linked agammaglobulineamia with delayed diagnosis despite the typical anamnestic signs for primary humoral immunodeficiency. The disease was caused by a previously non-reported mutationin in the BTK gene. Measurement of serum i

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    FN - Epidemiology, infection diseases and clinical immunology

  • OECD FORD branch

Result continuities

  • Project

  • Continuities

    V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Others

  • Publication year

    2014

  • Confidentiality

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

Data specific for result type

  • Name of the periodical

    Biomedical Papers of the Faculty of Medicine of Palacky University

  • ISSN

    1213-8118

  • e-ISSN

  • Volume of the periodical

    158

  • Issue of the periodical within the volume

    3

  • Country of publishing house

    CZ - CZECH REPUBLIC

  • Number of pages

    4

  • Pages from-to

    470-473

  • UT code for WoS article

    000345398500023

  • EID of the result in the Scopus database