Disorders of Humoral Immunity in Children with IgG Subclass Deficiency and Recurrent Respiratory Infections

Abstract

Respiratory tract infections in children are one of the most common causes for medical consultations. When the infections are of recurring nature, they are a major reason for the diagnostics for primary immunodeficiency that is in about 65% of cases underlain by disorders of humoral immunity. This study seeks to retrospectively evaluate the history of recurrent respiratory tract infections in children with humoral disorders and the associations among deficiencies in the immune system components. We evaluated 394 children aged 3 months to 18 years. We found 49.5% (195 cases) of children with IgG deficiencies, all of whom had normal IgE levels. There were 8.4% (33 cases) of IgA deficiency, 7.4% (29 cases) of IgM insufficiency, and 4.1% (16 cases) of CD19+ cells deficiency. The elevated level of CD19+ cells was found in 27.7% (109 out of the 394 children). Immunoglobulin deficiencies often coexisted with a deficiency in another immunoglobulin class above outlined. There was an interdependence between IgA abnormality and IgG, IgG3, and IgG4 abnormalities as well as between IgM abnormality and IgG and IgG1 abnormalities. We conclude that respiratory tract infections in children are often underlain by a convergence of IgG with both IgA and IgM abnormal states. The physiopathological meaning of this convergence for the infection course and resulting functional respiratory changes remains elusive.

Serum Protein Electrophoretic in Children

“…Only one case of agammaglobulinemia has been recorded, in a 3-year old boy. In order to confirm that a child has humoral immunodeficiency, it is necessary to eliminate certain nephrotic symptoms and protein-losing enteropathies, hematological malignancies, and also the use of certain drugs, in particular immunosuppressants [28]. In our case, the only information we could retain about the child is that he has diffuse interstitial lung disease.…”

Paediatric immunoglobulin A deficiency revisited

“…The infections are frequently prolonged and recurrent with numerous contributing bacteria, especially Streptococcus pneumoniae, Haemophilus influenzae, and Staphylococcus, but also atypical and opportunistic ones such as Mycoplasma or Pseudomonas aeruginosa [41]. Infections may result from an impaired immune response to vaccination noted in IgA-deficient children and adults who have been administered an unconjugated polyvalent pneumococcal vaccine [43]. IgA deficiency lower than 0.007 g/l is an established risk factor for the incidence of pneumonia and may predispose affected individuals to pneumococcal infections of the RT, and it also implies rhinosinusitis and tonsillitis [44].…”

Syncitial virus respiratory infections in children – immunological aspects

“…A recent retrospective study has shown that immunoglobulin deficiencies are affecting different isotypes. Thus, RTIs in children determine abnormal levels of IgG, and also of IgA and IgM (Pasternak et al, 2018). When studying cytokine secretion in RTI occurred in children decreased levels of proinflammatory cytokines were found, mainly IFN-gamma, indicating the increased risk for developing RTI.…”