Early manifestation and recognition of C2 complement deficiency
in the form of pyogenic infection in infancy

J 2003

Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy

LITZMAN, Jiří; Tomáš FREIBERGER; Dana BARTOŇKOVÁ; Marcela VLKOVÁ; Vojtěch THON et al.

Základní údaje

Originální název

Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy

Název česky

Menngitida jako časná manifetace defcitu C2

Autoři

LITZMAN, Jiří; Tomáš FREIBERGER ; Dana BARTOŇKOVÁ; Marcela VLKOVÁ; Vojtěch THON a Jindřich LOKAJ

Vydání

J Paediatr Child Health, 2003, 1034-4810

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30102 Immunology

Stát vydavatele

Česká republika

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 0.879

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/03:00024056

Organizační jednotka

Lékařská fakulta

UT WoS

000183179000007

Klíčová slova česky

deficience komplementu; C2 složka komplementu

Klíčová slova anglicky

complement deficiency; C2 complement component; immunoglobulin deficiency; meningitis

Štítky

C2 complement component, complement deficiency, immunoglobulin deficiency, meningitis

Změněno: 2. 4. 2010 08:14, prof. MUDr. Jiří Litzman, CSc.

Anotace

ORIG CZ

V originále

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.

Česky

Meningitida je časnou manifestací defeitu C2, popis 3 případů.

Návaznosti

NI5220, projekt VaV

Název: Imunitní reaktivita u hereditárního angioedému

Investor: Ministerstvo zdravotnictví ČR, Imunitní reaktivita u hereditárního angioedému

NI5558, projekt VaV

Název: Molekulárně genetická analýza u pacientů s hereditárním angioedémem

Investor: Ministerstvo zdravotnictví ČR, Molekulárně genetická analýza u pacientů s hereditárním angioedémem

Citovat

LITZMAN, Jiří; Tomáš FREIBERGER; Dana BARTOŇKOVÁ; Marcela VLKOVÁ; Vojtěch THON a Jindřich LOKAJ. Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy. J Paediatr Child Health. 2003, roč. 39, č. 4, s. 274-277. ISSN 1034-4810.

@article{554910,
   author = {Litzman, Jiří and Freiberger, Tomáš and Bartoňková, Dana and Vlková, Marcela and Thon, Vojtěch and Lokaj, Jindřich},
   article_number = {4},
   keywords = {complement deficiency; C2 complement component; immunoglobulin deficiency; meningitis},
   language = {eng},
   issn = {1034-4810},
   journal = {J Paediatr Child Health},
   note = {complement deficiency, C2 complement component, immunoglobulin deficiency, meningitis},
   title = {Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy},
   volume = {39},
   year = {2003}
}

TY  - JOUR
ID  - 554910
AU  - Litzman, Jiří - Freiberger, Tomáš - Bartoňková, Dana - Vlková, Marcela - Thon, Vojtěch - Lokaj, Jindřich
PY  - 2003
TI  - Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy
JF  - J Paediatr Child Health
VL  - 39
IS  - 4
SP  - 274-277
EP  - 274-277
SN  - 10344810
N1  - complement deficiency, C2 complement component, immunoglobulin deficiency, meningitis
KW  - complement deficiency
KW  - C2 complement component
KW  - immunoglobulin deficiency
KW  - meningitis
N2  - 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.
ER  -

LITZMAN, Jiří; Tomáš FREIBERGER; Dana BARTOŇKOVÁ; Marcela VLKOVÁ; Vojtěch THON a Jindřich LOKAJ. Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy. \textit{J Paediatr Child Health}. 2003, roč.~39, č.~4, s.~274-277. ISSN~1034-4810.

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