LITZMAN, Jiří, Tomáš FREIBERGER, Dana BARTOŇKOVÁ, Marcela VLKOVÁ, Vojtěch THON and Jindřich LOKAJ. Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy. J Paediatr Child Health. vol. 39, No 4, p. 274-277. ISSN 1034-4810. 2003.
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Basic information
Original name Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy
Name in Czech Menngitida jako časná manifetace defcitu C2
Authors LITZMAN, Jiří (203 Czech Republic, guarantor), Tomáš FREIBERGER (203 Czech Republic), Dana BARTOŇKOVÁ (203 Czech Republic), Marcela VLKOVÁ (203 Czech Republic), Vojtěch THON (203 Czech Republic) and Jindřich LOKAJ (203 Czech Republic).
Edition J Paediatr Child Health, 2003, 1034-4810.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30102 Immunology
Country of publisher Czech Republic
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 0.879
RIV identification code RIV/00216224:14110/03:00024056
Organization unit Faculty of Medicine
UT WoS 000183179000007
Keywords (in Czech) deficience komplementu; C2 složka komplementu
Keywords in English complement deficiency; C2 complement component; immunoglobulin deficiency; meningitis
Tags C2 complement component, complement deficiency, immunoglobulin deficiency, meningitis
Changed by Changed by: prof. MUDr. Jiří Litzman, CSc., učo 403. Changed: 2/4/2010 08:14.
Abstract
Objective: Although frequently asymptomatic, C2 complement component deficiency may lead to severe pyogenic infections or lupus-like illness. Here we describe infectious manifestations in infancy and childhood in our C2 deficient patients. Methodology: Retrospective study of clinical manifestation in 3 patients. C2 deficiency was proved both by undetectable serum C2 level and typical homozygous 28bp deletion of C2 gene. Results: All patients were hospitalised at least once by the age of 12 months, each had one episode of meningitis in infancy, one also had arthritis with septicemia. Infections of the respiratory tract were the causes of other hospitalisations. Two patients also suffered from frequent mild respiratory tract infections, in both decreased IgA and IgG2 or IgG3 levels were recorded. Conclusion: Our observation point to an early manifestation of C2 deficiency within the first year of life, with meningitis as the most severe complication. The severity of immunodeficiency may be influenced by concomitant deficiencies of immunoglobulin isotypes.
Abstract (in Czech)
Meningitida je časnou manifestací defeitu C2, popis 3 případů.
Links
NI5220, research and development projectName: Imunitní reaktivita u hereditárního angioedému
Investor: Ministry of Health of the CR, Project Immunological reactivity in patients with hereditary angioedema
NI5558, research and development projectName: Molekulárně genetická analýza u pacientů s hereditárním angioedémem
Investor: Ministry of Health of the CR, Molecular Genetic Analysis in Patients with Hereditary Angioedema
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