Immunoglobulins in ataxia-telangiectasia: Evidence for IgG4 and IgA2 subclass deficiencies

Rivat-Peran, Liliane; Buriot, D; Salier, Jean‐Philippe; Rivat, C.; Dumitresco, S.M.; Griscelli, C

doi:10.1016/0090-1229(81)90166-5

Cited by 36 publications

(13 citation statements)

References 15 publications

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“…Normal IgG levels have been observed in AT patients, 5,6,8 in agreement with our findings. Controversial results have been described for IgG subclasses in these patients.…”

Section: Discussionsupporting

confidence: 93%

“…6,8 IgA levels less than 7 mg/dl were detected in 50% of our patients, more often associated with an increase in IgG and/or IgM levels and with a small number of CD4 cells (data not shown). Elevated IgM levels have been observed in AT patients, 8 sometimes mimicking the hyperviscosity syndrome, suggesting a disorder in the maturation and differentiation of B lymphocytes. The association between IgA deficiency and unresponsiveness to polysaccharide antigens has been described.…”

Section: Discussionmentioning

confidence: 58%

“…Controversial results have been described for IgG subclasses in these patients. [6][7][8] However, IgG2 in subnormal concentration does not necessarily reflect immunodeficiency. 14 StrayPedersen et al found a positive relationship between antibody levels to pneumococcus and IgG2.…”

Section: Discussionmentioning

confidence: 99%

“…4,5 Despite normal IgG levels, 80% of AT patients have IgG2 deficiency; the remaining 20% do not have a defined pattern. [6][7][8] The production of viral and bacterial antibodies and antigens may be deficient. The Age (years) Polysaccharide antigens and ataxia-telangiectasia Guerra-Maranhão MC et al…”

Section: Introductionmentioning

confidence: 99%

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Resposta a antígenos polissacarídicos em pacientes com ataxia-telangiectasia

Guerra-Maranhão¹,

Costa‐Carvalho²,

Nudelman³

et al. 2006

J. Pediatr. (Rio J.)

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Objective: To analyze the production of antibodies to polysaccharide antigens in patients with ataxiatelangiectasia. Patients and methods:We used the ELISA technique to measure the levels of IgG antibodies to serotypes 1, 3, 5, 6B, 9V and 14 of Streptococcus pneumoniae in 14 patients with ataxia-telangiectasia before and after immunization with 23-valent polysaccharide vaccine. Adequate response to individual polysaccharide can be defined as a postimmunization antibody titer equal to or greater than 1.3 µg/ml or as a minimum fourfold increase over the baseline (preimmunization) value.Results: Six (43%) patients showed an absent response to all serotypes analyzed. Four patients showed adequate response to only one serotype, one patient to two serotypes, two patients to three serotypes and only one patient to four out of six serotypes analyzed. No patient had adequate response to all serotypes tested. Postimmunization pneumococcus IgG levels were higher than preimmunization levels to all serotypes analyzed, except for serotype 3. In spite of this, the mean postimmunization levels were lower than 1.3 µg/ml in all serotypes, except for serotype 14. Mean increment was less than four in all serotypes analyzed. Conclusion:Our results suggest that patients with ataxia-telangiectasia are at a high risk of having an impaired response to pneumococcus, which may be one of the causes of recurrent sinopulmonary infections in these patients.J Pediatr (Rio J). 2006;82(2):132-6: Ataxia-telangiectasia, Streptococcus pneumoniae, pneumococcus, polysaccharide, immunodeficiency, humoral immunity, antibody.

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“…Normal IgG levels have been observed in AT patients, 5,6,8 in agreement with our findings. Controversial results have been described for IgG subclasses in these patients.…”

Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Resposta a antígenos polissacarídicos em pacientes com ataxia-telangiectasia

Guerra-Maranhão¹,

Costa‐Carvalho²,

Nudelman³

et al. 2006

J. Pediatr. (Rio J.)

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“…Apesar de apresentarem níveis normais de IgG, 80% dos pacientes com AT apresentam deficiência de subclasse IgG2; para as demais, não há padrão definido [6][7][8] . A produção de anticorpos a antígenos virais e bacterianos pode estar deficiente.…”

Section: Introductionunclassified

Response to polysaccharide antigens in patients with ataxia-telangiectasia

Guerra-Maranhão¹,

Costa‐Carvalho²,

Nudelman³

et al. 2006

J Pediatr (Rio J)

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Fonte financiadora: FAPESP.Artigo submetido em 26.08.05, aceito em 09.11.05. AbstractObjective: To analyze the production of antibodies to polysaccharide antigens in patients with ataxia-telangiectasia. Patients and methods:We used the ELISA technique to measure the levels of IgG antibodies to serotypes 1, 3, 5, 6B, 9V and 14 of Streptococcus pneumoniae in 14 patients with ataxia-telangiectasia before and after immunization with 23-valent polysaccharide vaccine. Adequate response to individual polysaccharide can be defined as a postimmunization antibody titer equal to or greater than 1.3 µg/ml or as a minimum fourfold increase over the baseline (preimmunization) value.Results: Six (43%) patients showed an absent response to all serotypes analyzed. Four patients showed adequate response to only one serotype, one patient to two serotypes, two patients to three serotypes and only one patient to four out of six serotypes analyzed. No patient had adequate response to all serotypes tested. Postimmunization pneumococcus IgG levels were higher than preimmunization levels to all serotypes analyzed, except for serotype 3. In spite of this, the mean postimmunization levels were lower than 1.3 µg/ml in all serotypes, except for serotype 14. Mean increment was less than four in all serotypes analyzed. Conclusion:Our results suggest that patients with ataxiatelangiectasia are at a high risk of having an impaired response to pneumococcus, which may be one of the causes of recurrent sinopulmonary infections in these patients.J Pediatr (Rio J). 2006;82(2):132:6: Ataxia-telangiectasia, Streptococcus pneumoniae, pneumococcus, polysaccharide, immunodeficiency, humoral immunity, antibody. ResumoObjetivo: Estudar a produção de anticorpos a antígenos polissacarídicos em pacientes com ataxia-telangiectasia.Casuística e método: Utilizando a técnica de ELISA, determinamos os níveis de IgG aos sorotipos 1, 3, 5, 6B, 9V e 14 do pneumococo em 14 pacientes com ataxia-telangiectasia, antes e após a imunização com a vacina pneumocócica 23-valente. Resposta adequada a cada sorotipo foi definida como IgG > 1,3 µg/ml ou o incremento de quatro vezes dos níveis pós em relação aos pré-imunização.Resultados: Seis pacientes (43%) não responderam a todos os sorotipos analisados, quatro a apenas um sorotipo, um paciente a dois, dois a três e apenas um paciente a quatro dos seis sorotipos analisados. Nenhum paciente apresentou resposta adequada a todos os sorotipos testados. Os níveis de IgG ao pneumococo pós-imunização foram superiores aos pré-imunização para todos os sorotipos testados, exceto o 3. Apesar disso, os valores médios pós-imunização foram inferiores a 1,3 µg/ml para todos os sorotipos analisados, exceto o 14. A média de incremento da resposta foi inferior a quatro para todos os sorotipos analisados.Conclusão: Nossos resultados sugerem que pacientes com ataxiatelangiectasia têm grande risco de apresentar resposta inadequada ao pneumococo, o que pode ser uma das causas das infecções sinopulmonares de repetição.J Pediatr (Rio J). 2006;82(2):1...

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