“…Bronchiectasis is reported as an established disease complication in 18–68% of patients with CVID85 93–96 103 and 7–20% of patient cohorts with XLA,93 95 104 and is therefore a significant characteristic of these patient groups. Bronchiectasis appears to complicate isolated selective IgA deficiency relatively rarely,105–107 but may occur at greater frequency when IgA deficiency is part of, or evolves into, a more clinically significant and complex antibody deficiency disorder (specific antibody/IgG subclass deficiency or CVID) 108 109. In addition to these more common antibody deficiency disorders, there is an established and growing recognition of an important association between bronchiectasis and defects of specific antibody production 22 71 83 88–90.…”
“…Bronchiectasis is reported as an established disease complication in 18–68% of patients with CVID85 93–96 103 and 7–20% of patient cohorts with XLA,93 95 104 and is therefore a significant characteristic of these patient groups. Bronchiectasis appears to complicate isolated selective IgA deficiency relatively rarely,105–107 but may occur at greater frequency when IgA deficiency is part of, or evolves into, a more clinically significant and complex antibody deficiency disorder (specific antibody/IgG subclass deficiency or CVID) 108 109. In addition to these more common antibody deficiency disorders, there is an established and growing recognition of an important association between bronchiectasis and defects of specific antibody production 22 71 83 88–90.…”
“…This spectrum is reminiscent of programmed acquisition of serum Ig isotypes during ontogeny. Children first achieve adult levels of IgM around the age of 2 years, of IgG by 6 years old, and IgA during puberty [13]. In our previous studies, we showed increases in Ig levels to age-related normal values in IgA and IgG subclass deficient patients [19,21].…”
Clinical manifestations and immunoglobulin levels of the patients with Hyper-IgM type 4 phenotype recovered in late childhood at about 6 years of age. There was a transient CSR defect which was not observed in cases with transient hypogammaglobulinemia of infancy. Detection of a non-AID or non-UNG associated CSR defect in infancy should be confirmed later on since spontaneous recovery may occur.
“…Thus, at least a third of these neutropenia patients displayed IgG3 de®ciency according to today's norms (i.e. < 0.21 g L )1 ) [10,11], and altogether 47% according to the reference values earlier used (< 0.41 g L )1 ; when many patients were diagnosed [14]). However, IgG2 and IgG4 were normal in all patients.…”
IgG3 deficiency is common amongst chronic mild neutropenia patients, particularly in those with autoimmune background, but contributes not significantly to infection susceptibility.
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