VANČÍKOVÁ, Zuzana, Tomáš FREIBERGER, W. VACH, M. TROJANEK, M. RIZZI and A. JANDA. X-linked Agammaglobulinemia in Community-acquired Pneumonia Cases Revealed by Immunoglobulin Level Screening at Hospital Admission. Klinische Pädiatrie. Stuttgart: Georg Thieme Verlag, 2013, vol. 225, No 6, p. 339-342. ISSN 0300-8630. doi:10.1055/s-0033-1354415.
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Basic information
Original name X-linked Agammaglobulinemia in Community-acquired Pneumonia Cases Revealed by Immunoglobulin Level Screening at Hospital Admission
Authors VANČÍKOVÁ, Zuzana (203 Czech Republic), Tomáš FREIBERGER (203 Czech Republic, guarantor, belonging to the institution), W. VACH (276 Germany), M. TROJANEK (203 Czech Republic), M. RIZZI (276 Germany) and A. JANDA (276 Germany).
Edition Klinische Pädiatrie, Stuttgart, Georg Thieme Verlag, 2013, 0300-8630.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30209 Paediatrics
Country of publisher Germany
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 1.904 in 2012
RIV identification code RIV/00216224:14740/13:00072825
Organization unit Central European Institute of Technology
Doi http://dx.doi.org/10.1055/s-0033-1354415
UT WoS 000327015800010
Keywords in English X-linked agammaglobulinemia; immunodeficiency; humoral immunity; community-acquired pneumonia; childhood
Tags ok, podil, rivok
Tags International impact, Reviewed
Changed by Changed by: Olga Křížová, učo 56639. Changed: 15. 4. 2014 10:14.
Abstract
In children with primary immunodeficiencies, the onset of symptoms precedes the diagnosis and the initiation of appropriate treatment by months or years. This delay in diagnosis is due to the fact that while these disorders are rare, some of the infections seen in immunodeficient patients are common. Defective antibody production represents the largest group among these disorders, with otitis, sinusitis and pneumonia as the most frequent initial manifestation. We performed a prospective study of humoral immunity in children hospitalized due to community-acquired pneumonia in tertiary care hospital. Out of 254 patients (131 boys, 123 girls, median age 4.5 years) recruited over 3 years, we found 2 boys (age 11 and 21 months) lacking serum immunoglobulins and circulating B cells. Subsequent genetic analysis confirmed diagnosis of X-linked agammaglobulinemia. Despite their immunodeficiency, the pneumonia was uncomplicated in both patients and did not call for immunological evaluation. However, the immunoglobulin screening at admission allowed for an early diagnosis of the immunodeficiency and timely initiation of immunoglobulin substitution, the key prerequisite for a favorable course of the disease. Conclusions: Simple and inexpensive immunoglobulin measurement during the management of hospitalized children with community-acquired pneumonia may help in early identification of patients with compromised humoral immunity and prevent serious complications.
Links
EE2.3.20.0045, research and development projectName: Podpora profesního růstu a mezinárodní integrace výzkumných týmů v oblasti molekulární medicíny
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