Modern Pediatrics. Ukraine. (2025).3(147): 47-54. doi: 10.15574/SP.2025.3(147).4754
Pochynok T. V., Chernyshov V. P., Gorobets N. I., Trembovetska Yu. B.
Bogomolets National Medical University, Kyiv, Ukraine

For citation: Pochynok TV, Chernyshov VP, Gorobets NI, Trembovetska YuB. (2025). Features of the course of primary selective immunoglobulin A deficiency in children. Modern Pediatrics. Ukraine. 3(147): 47-54. doi: 10.15574/SP.2025.3(147).4754.
Article received: Jan 28, 2025. Accepted for publication: Apr 08, 2025.

Immunoglobulin A is a key component of mucosal immunity and plays a crucial role in protecting the mucous membranes of the respiratory, gastrointestinal, and genitourinary systems. Selective IgA deficiency is the most common primary immunodeficiency in children, yet it often remains undiagnosed due to mild or absent clinical symptoms. However, the presence of IgA deficiency in children with recurrent infections can significantly influence the course of disease and may require adjustments in clinical management.
Aim – to determine the prevalence of selective IgA deficiency in children with frequent respiratory infections and to analyze their clinical features.
Materials and methods. A total of 98 children (50 girls and 48 boys), aged 4-14 years, with frequent respiratory infections were examined. Serum IgA levels were measured to detect selective deficiency. The study was conducted in accordance with the ethical standards of the Declaration of Helsinki, and informed parental consent was obtained for each participant.
Results. Selective IgA deficiency was identified in 19 children (11 boys and 8 girls), accounting for 18.6% of the study population. These patients more frequently presented with recurrent infections and had a more severe course of disease.
Conclusions. Although selective IgA deficiency is asymptomatic in most cases, it may be associated with the development of severe pathological conditions in children, including recurrent respiratory infections, allergic disorders, and autoimmune diseases. Detecting selective IgA deficiency in pediatric practice is essential for early diagnosis and timely management of related conditions. Pediatricians and immunologists should aim to identify the pathophysiological links between secretory IgA deficiency and various clinical manifestations to develop individualized monitoring and treatment strategies for affected children.
The study was conducted in accordance with the principles of the Declaration of Helsinki. The study protocol was approved by the Local Ethics Committee for participant. Informed consent was obtained from patients.
The authors declare no conflict of interest.
Keywords: children, immunodeficiency, selective immunoglobulin A deficiency, recurrent respiratory infections.

References

1. Aghamohammadi A, Abolhassani H, Biglari M, Abolmaali S, Moazzami K, Tabatabaeiyan M et al. (2011). Analysis of switched memory B cells in patients with IgA deficiency. Int Arch Allergy Immunol. 156(4): 462-468. https://doi.org/10.1159/000323903; PMid:21832837

2. Arima H, Koirala S, Nema K, Nakano M, Ito H, Poudel KM et al. (2022). High prevalence of rheumatoid arthritis and its risk factors among tibetan highlanders living in Tsarang, Mustang district of Nepal. J Physiol Anthropol. 41(1): 12. https://doi.org/10.1186/s40101-022-00283-3; PMid:35366946 PMCid:PMC8976395

3. Caviglia GP, Garrone A, Bertolino C, Vanni R, Bretto E, Poshnjari A et al. (2023). Epidemiology of Inflammatory Bowel Diseases: a Population Study in a Healthcare District of North-West Italy. J Clin Med. 12(2): 641. https://doi.org/10.3390/jcm12020641; PMid:36675570 PMCid:PMC9860659

4. Cinicola BL, Pulvirenti F, Capponi M, Bonetti M, Brindisi G, Gori A et al. (2022). Selective IgA Deficiency and Allergy: a fresh look to an Old Story. Medicina. 58(1): 129. https://doi.org/10.3390/medicina58010129; PMid:35056437 PMCid:PMC8781177

5. Ferreira AR, Di Meglio A, Pistilli B, Gbenou AS, El-Mouhebb M, Dauchy S et al. (2019). Differential impact of endocrine therapy and chemotherapy on quality of life of breast cancer survivors: a prospective patient-reported outcomes analysis. Annals of oncology. 30(11): 1784-1795. https://doi.org/10.1093/annonc/mdz298; PMid:31591636

6. Hammarström L, Vorechovsky I, Webster D. (2000). Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 120(2): 225-231. https://doi.org/10.1046/j.1365-2249.2000.01131.x; PMid:10792368 PMCid:PMC1905641

7. Kim JH, Jang JH, Lee SH, Yang EM, Jang SH, Jung KS et al. (2021). Specific antibody deficiency in adult patients with IgG or IgG subclass deficiency. Allergy Asthma Immunol Res. 13: 271-283. https://doi.org/10.4168/aair.2021.13.2.271; PMid:33474861 PMCid:PMC7840874

8. Lee H, Kovacs C, Mattman A, Hollander Z, Chen V, Ng R et al. (2022). The impact of IgG subclass deficiency on the risk of mortality in hospitalized patients with COPD. Respir Res. 23: 141. https://doi.org/10.1186/s12931-022-02052-3; PMid:35641962 PMCid:PMC9158163

9. Lehman HK, Yu KOA, Towe CT, Risma KA. (2022). Respiratory infections in patients with primary immunodeficiency. J Allergy Clin Immunol Pract. 10: 683-691. https://doi.org/10.1016/j.jaip.2021.10.073; PMid:34890826

10. Magen E, Blum I, Waitman DA, Kahan N, Forer B. (2021). Autoimmune inner ear disease among patients with selective IgA Deficiency. Audiol Neurotology. 26(2): 127-134. https://doi.org/10.1159/000509577; PMid:33311024

11. Makary CA, Behnke J, Peppers B, Ramadan HH. (2022). Outcome of immunoglobulin replacement therapy in adults with rhinosinusitis. Laryngoscope. 132: 732-736. https://doi.org/10.1002/lary.29694; PMid:34146351

12. Maltsev DV. (2016). Isolated IgA deficiency: epidemiology, clinic, diagnosis, treatment. Clinical immunology, allergology, infectology. 1(90): 5-15.

13. Mella MA, Lavrinienko A, Akhi R, Hindström R, Nissinen AE, Wang C et al. (2023). Compensatory IgM to the Rescue: Patients with Selective IgA Deficiency Have Increased Natural IgM Antibodies to MAA-LDL and No Changes in Oral Microbiota. Immunohorizons. 5(4): 170-181. https://doi.org/10.4049/immunohorizons.2100014; PMid:33893180

14. Morawska I, Kurkowska S, Bębnowska D, Hrynkiewicz R, Becht R, Michalski A et al. (2021). The epidemiology and clinical presentations of atopic diseases in selective IgA deficiency. J Clin Med. 10(17): 3809. https://doi.org/10.3390/jcm10173809; PMid:34501259 PMCid:PMC8432128

15. Nabavizadeh SH, Karimi MH, Esmaeilzadeh H, Attarhoseini M, Askarisarvestani A. (2021). The prevalence and clinical manifestations of IgA deficiency among blood donors at transfusion centers in Shiraz, Southern Iran. Am J Clin Experimental Immunol. 10(4): 112.

16. Pavelescu ML, Dijmărescu I, Dinulescu A, Păsărică AS, Burtescu C, Lavric CE et al. (2024). Selective immunoglobulin A deficiency and recurrent infections in children: how deep is this issue? Journal of Pediatric and Neonatal Individualized Medicine (JPNIV). 13(1): e130110.

17. Patrawala M, Cui Y, Peng L, Fuleihan RL, Garabedian EK, Patel K et al. (2020) Pulmonary disease burden in primary immune deficiency disorders: data from USIDNET registry. J. Clin. Immunol. 40: 340-349. https://doi.org/10.1007/s10875-019-00738-w; PMid:31919711

18. Rawla P, Killeen RB, Joseph NI. (2024). Selective IgA Deficiency. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; Jan-.

19. Quinn J, Modell V, Orange JS, Modell F. (2022). Growth in diagnosis and treatment of primary immunodeficiency within the global Jeffrey Modell Centers Network. Allergy Asthma Clin Immunol. 18: 19. https://doi.org/10.1186/s13223-022-00662-6; PMid:35246253 PMCid:PMC8896271

20. Thalhammer J, Kindle G, Nieters A, Rusch S, Seppänen MRJ, Fischer A et al. (2021). Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations. J Allergy Clin Immunol. 148: 1332-134118.

21. Vivarelli E, Matucci A, Bormioli S, Parronchi P, Liotta F, Cosmi L et al. (2021) Effectiveness of low-dose intravenous immunoglobulin therapy in minor primary antibody deficiencies: a 2-year real-life experience. Clin Exp Immunol. 205: 346-353. https://doi.org/10.1111/cei.13629; PMid:34061980 PMCid:PMC8374220

22. Vo Ngoc DT, Krist L, van Overveld FJ, Rijkers GT. (2017). The long and winding road to IgA deficiency: causes and consequences. Expert Rev Clin Immunol. 13(4): 371-382. https://doi.org/10.1080/1744666X.2017.1248410; PMid:27776452

23. Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A. (2017). Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management. Scand J Immunol. 85(1): 3-12. https://doi.org/10.1111/sji.12499; PMid:27763681