Abstract:
Primary immunodeficiencies (PIDs) are genetic diseases of the immune system,
with quantitative and/or qualitative defects of one or more of its components, being
affected: the development, function, and/or morphology of the immune system.
Respiratory manifestations in IDP are represented by a varied spectrum, representing
the most frequent cause of morbidity (63-88%), but also of mortality (30-65%). Timely
diagnosis and appropriate therapy can improve or at least slow down the progression of
these complications
Aim: Considering the increased frequency of infections among patients with
primary immunodeficiencies, it was proposed to evaluate respiratory infections and
pathogens involved in pediatric primary immunodeficiencies.
Methods: 14 children with primary immunodeficiencies (42.8% with primary
immunodeficiencies due to antibody deficiency and 57.2% with combined primary
immunodeficiencies) were included in the study. The diagnosis of PID was confirmed
by the evaluation of serum IgA, IgM, IgG by the ELISA method in the immunology
laboratory of IMC Chisinau, the determination of lymphocytes and lymphocyte
subpopulations by lymphocyte immunophenotyping, genetic research. The frequency of
infections was determined by analysis of laboratory charts, and oropharyngeal smear
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and sputum bacteriology and subsequent culture were performed to identify the
pathogens involved.
Results: In the studied group, pneumonia was present in 49.2% of patients with
combined immunodeficiency, in the case of patients with humoral link damage,
pneumonia was more common among those with variable common immunodeficiency
(84%), hyperIgM (81%) and disease Bruton (62%). In their etiology, following the
culture of sputum and oropharyngeal secretions, the following were determined: St.
Aureus 38.4% (95%CI 13.8-68.4%); Str beta haemolytic 46.1% (95% CI 19.2-74.8%);
Cl. Pneumoniae 30.7% (95%CI 9-61.4%); Str. Pyogenes 53.8% (95%CI 25.1-80.7%);
Candida white. 38.4% (95%CI 13.8-68.4%); P. aeruginosa 15.3% (95%CI 1.9-45.4%);
H. Influenzae 30.7% (95%CI 9-61.4%); Moraxella cath. 23.8% (95%CI 5-53.8%); P.
jirovecii 7.6% (95%CI 0.1-36%);
Respiratory infections in immunodeficient patients are usually severe, persistent,
caused by unusual, atypical, or opportunistic microorganisms, and recurrent compared
to infections in non-IPD patients.
Conclusion: Primary immunodeficiencies are predominantly rare diseases with
clinical and immunological polymorphism. Severe IDPs in children evolve with
pulmonary infections with polyresistant and opportunistic germs, which determine
major risks of unfavorable developments. Early diagnosis, preventive measures, and
immunoglobulin replacement treatments reduce the risks of acquiring infections with
polyresistant germs and improve the prognosis and quality of life of patients.
