Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children

Moschese, Viviana; Chini, L; Graziani, S; Sgrulletti, Mayla; Gallo, Vera; Matteo, Gigliola Di; Ferrari, Simona; Cesare, Silvia Di; Cirillo, Emilia; Pession, Andrea; Pignata, Claudio; Specchia, Fernando

doi:10.1007/s00431-018-3248-1

Cited by 29 publications

(25 citation statements)

References 49 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the group we studied, the level of deficiency did not affect the observed frequency of respiratory tract infections, similar results were obtained by Moschese et al [5]. Analysis of parental questionnaires carried out by Janzi et al [3] indicated an increased risk of pseudocroup at year 1 in the group of aSIgAD compared to pSIgAD and children with normal IgA serum; there were no significant differences in the occurrence of pneumonia, bronchitis, otitis media, and RSV-infections in early childhood.…”

Section: Discussionsupporting

confidence: 90%

“…In both groups, children suffered most often from asthma, allergic rhinitis, and food allergy, which concurs with earlier reports. Previous studies do not provide a definite answer on the risk of occurrence allergy in pSIgAD [3,5,17]; however, according to our results, the incidence of individual allergic disorders does not show differences between the variants of deficiency.…”

Section: Discussioncontrasting

confidence: 44%

“…The results of one prospective study showed that children with SIgAD were at increased risk for a higher number of respiratory infections, resulting in significantly lower pulmonary function, and the incidence was related to the variant of SIgAD [17]. Gastrointestinal infections, including those caused by Giardia lamblia, as well as urinary tract infections, are also reported in SIgAD, but probably due to the existence of compensatory mechanisms their percentage is not high [5,[18][19][20]. In our study, the incidence of gastrointestinal and urinary tract infection was noticeably lower than the incidence of sinopulmonary conditions and did not differ between the groups.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to "absolute" SIgAD (aSIgAD), "partial" SIgAD (pSIgAD) is characterised by IgA level 2SD below normal for age and is commonly considered as a mild variant of SIgAD, not requiring specialist care [5]. Recent studies reviewed the potential risk of significant symptoms in pSIgAD; however, the results obtained were not consistent.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Selective IgA deficiency – does partial mean negligible?

Tąpolska-Jóźwiak¹,

Szczawińska-Popłonyk²

2020

polp

View full text Add to dashboard Cite

Introduction: Selective immunoglobulin A deficiency (SIgAD) is the most common primary immunodeficiency in the Caucasian population. The current definition describes SIgAD as serum IgA level below 0.07 g/l in individuals of four years of age or older with normal immunoglobulin G and M levels, in whom other causes of hypogammaglobulinaemia have been excluded. Most patients with SIgAD are asymptomatic, but some of them suffer from recurrent infections, primarily involving the respiratory system, allergies, autoimmune disorders, and malignancies. In contrast to "absolute" SIgAD, the clinical relevance of partial SIgAD has not yet been determined. Aim of the study: To compare the clinical course of absolute and partial SIgAD in children. Material and methods: The study was conducted through a retrospective review of the medical records of 25 children with absolute and 33 children with partial SIgAD, aged between 4 and 18 years, for the occurrence of recurrent respiratory, urinary, and gastrointestinal tract infections, as well as allergy, autoimmunity, and malignancies. Results: Respiratory tract infections were the most common clinical manifestation in both variants of SIgAD and affected nearly two-thirds of patients with absolute and about three-quarters with partial SIgAD. Apart from autoimmunity in total, which occurred more often in children with absolute than partial SIgAD (44% vs. 12.12%, p = 0.006), no statistically significant differences were found between the incidence of the analysed conditions in both groups. Conclusions: Due to the similar clinical course of both variants of SIgAD, all symptomatic patients should receive specialist care and undergo systematic follow-up visits. In children with autoimmune diseases basic immunology screening should be considered.

show abstract

Section: Discussionsupporting

confidence: 90%

Section: Discussioncontrasting

confidence: 44%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Selective IgA deficiency – does partial mean negligible?

Tąpolska-Jóźwiak¹,

Szczawińska-Popłonyk²

2020

polp

View full text Add to dashboard Cite

show abstract

“…Absolute IgAD is defined as an undetectable serum IgA level, below 0.005 g/l, whereas partial IgAD signifies an IgA level that is two standard deviations below age-adjusted mean. Selective IgA deficiency (SIgAD) is the most prevalent primary immunodeficiency in Caucasian populations, defined by serum level of IgA < 0.007 g/l, with a prevalence of approximately 1 : 600 in the population [1,2]. In children, according to the current ESID recommendation, a definitive diagnosis of absolute IgA deficiency is estab-lished beyond the age of four years because of delayed maturation of this immunoglobulin isotype [1,2] and concomitantly, in young children below four years of age, the IgA serum level lower than 0.007 g/l has no practical and prognostic significance.…”

Section: Introductionmentioning

confidence: 99%

Paediatric immunoglobulin A deficiency revisited

Gałęcki¹,

Janas²,

Juzyk³

et al. 2019

polp

View full text Add to dashboard Cite

Immunoglobulin A (IgA) is the most abundant antibody isotype on mucosal surfaces, with predominantly neutralising non-activating functions, and which plays multifaceted roles in the immune system. IgA protects the host from pathogen invasion in the gastrointestinal and respiratory tracts, enhances diversification of microbiota, and maintains the intestinal homeostasis, exerting its twofold proinflammatory and anti-inflammatory functions by different immune cells and receptors. Therefore, IgA deficiency in children encompasses a broad spectrum of clinical phenotypes including infectious, allergic, autoimmune, and inflammatory conditions. In this review we also provide new insight into the genetic background and immunopathology of IgA deficiency in children.

show abstract