J Pediatr Infect Dis 2022; 17(03): 148-154
DOI: 10.1055/s-0042-1748761
Original Article

Inborn Errors of Immunity among Egyptian Children with Recurrent Acute Otitis Media

Mohammed Abd Elsameea
1   Department of Otorhinolaryngology, Faculty of Medicine, Sohag University, Sohag, Egypt
,
Mohammed Abd-Elkader
1   Department of Otorhinolaryngology, Faculty of Medicine, Sohag University, Sohag, Egypt
,
Eman M. Fahmy
2   Department of Pediatrics, Faculty of Medicine, Sohag University, Sohag, Egypt
,
2   Department of Pediatrics, Faculty of Medicine, Sohag University, Sohag, Egypt
,
Mostafa A. ElTaher
1   Department of Otorhinolaryngology, Faculty of Medicine, Sohag University, Sohag, Egypt
› Author Affiliations
Funding None.

Abstract

Objective Our objective was to investigate the relative frequency and pattern of inborn errors of immunity (IEIs) among Egyptian children with recurrent acute otitis media (rAOM).

Methods This was a cross-sectional study that included children from the age of 6 months to 16 years with rAOM. Those with structural, functional, and environmental risk factors were excluded. Enrolled children underwent thorough clinical, otorhinolaryngological, and immunological evaluation, including hematological counting, quantitative immunoglobulins assay, lymphocytic flow cytometric immunophenotyping, CH50, and phagocytic function tests.

Results The study included 69 children with rAOM (44 boys and 25 girls; median age 30 months). IEIs were identified in 14 children, including transient hypogammaglobulinemia (three cases), selective IgA deficiency (three cases), agammaglobulinemia (two cases), common variable immunodeficiency (two cases), and one case for each of congenital neutropenia, Chediak–Higashi syndrome, hyper IgM syndrome, and Griscelli syndrome. Parental consanguinity and history of unexplained/infection-related siblings' deaths were significantly associated with IEIs (p = 0.018 and 0.003, respectively). AOM and related complications were more frequent among IEI cases (p = 0.018 and 0.032, respectively). IEI cases had lower levels of hemoglobin (10.7 ± 2.80 vs. 12.3 ± 1.64 g/dL; p = 0.002), IgG (203 [78–1,370] vs. 708 [42.3–1,509] mg/dL; p = 0.000), and IgA (24.3 [3–310] vs. 80 [15.6–305] mg/dL; p = 0.009) compared with non-IEI cases.

Conclusion The current study identified IEIs in one-fifth of children with rAOM, most of which were predominately antibody deficiencies. An immunological workup for rAOM is particularly important in the presence of certain indicators for IEIs, provided that other more common risk factors are excluded.

Supplementary Material



Publication History

Received: 04 January 2022

Accepted: 29 March 2022

Article published online:
30 May 2022

© 2022. Thieme. All rights reserved.

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  • References

  • 1 Suzuki HG, Dewez JE, Nijman RG, Yeung S. Clinical practice guidelines for acute otitis media in children: a systematic review and appraisal of European national guidelines. BMJ Open 2020; 10 (05) e035343
  • 2 Setchanova L, Stancheva I, Popova D, Alexandrova A, Mitov I. Bacterial spectrum of acute otitis media in bulgarian children during the 10-valent pneumococcal conjugate vaccine era. J Pediatr Infect Dis 2020; 15 (03) 135-143
  • 3 Tipton C, Honsinger K, Harris M, Malhotra P. Acute otologic infections in pediatric patients. J Pediatr Infect Dis 2019; 14 (02) 052-062
  • 4 Marengo R, Ortega Martell JA, Esposito S. Paediatric recurrent ear, nose and throat infections and complications: can we do more?. Infect Dis Ther 2020; 9 (02) 275-290
  • 5 Schilder AGM, Chonmaitree T, Cripps AW. et al. Otitis media. Nat Rev Dis Primers 2016; 2 (01) 16063
  • 6 Tangye SG, Al-Herz W, Bousfiha A. et al. Human inborn errors of immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol 2020; 40 (01) 24-64
  • 7 Bardou MLD, Pontarolli D, Grumach AS. Otitis media and inborn errors of immunity. Curr Allergy Asthma Rep 2020; 20 (10) 59
  • 8 Bjelac JA, Yonkof JR, Fernandez J. Differing performance of the warning signs for immunodeficiency in the diagnosis of pediatric versus adult patients in a two-center tertiary referral population. J Clin Immunol 2019; 39 (01) 90-98
  • 9 Abolnezhadian F, Saki N, Nikakhlagh S, Safavi E, Asar S. The relative frequency of primary immunodeficiency diseases in pediatric patients with recurrent sinusitis and otitis media. Electron J Gen Med 2019; 16 (01) em102
  • 10 Reda SM, Yousef TA, Elfeky RA, Sallam MT, Gaafar RA. Could recurrent otitis media predict primary antibody deficiencies in Egyptian children?. Egypt J Otolaryngol 2014; 30 (02) 82-87
  • 11 Aghamohammadi A, Moin M, Karimi A. et al. Immunologic evaluation of patients with recurrent ear, nose, and throat infections. Am J Otolaryngol 2008; 29 (06) 385-392
  • 12 Aghamohammadi A, Rezaei N, Yazdani R. et al; MENA-I. E. I. Study Group. Consensus Middle East and North Africa Registry on inborn errors of immunity. J Clin Immunol 2021; 41 (06) 1339-1351
  • 13 Galal N, Meshaal S, Elhawary R. et al. Patterns of primary immunodeficiency disorders among a highly consanguineous population: Cairo university pediatric hospital's 5-year experience. J Clin Immunol 2016; 36 (07) 649-655
  • 14 Lieberthal AS, Carroll AE, Chonmaitree T. et al. The diagnosis and management of acute otitis media. Pediatrics 2013; 131 (03) e964-e999
  • 15 Harmes KM, Blackwood RA, Burrows HL, Cooke JM, Harrison RV, Passamani PP. Otitis media: diagnosis and treatment. Am Fam Physician 2013; 88 (07) 435-440
  • 16 Rosenzweig S, Kobrynski L, Fleisher T. Laboratory evaluation of primary immunodeficiency disorders. In: Sullivan K, Stiehm E. eds. Stiehm's Immune Deficiencies: Inborn Errors of Immunity. 2nd ed.. Elsevier Inc.; 2020
  • 17 Grumach AS, Goudouris ES. Inborn errors of immunity: how to diagnose them?. J Pediatr (Rio J) 2021; 97 (Suppl. 01) S84-S90
  • 18 Lehman H, Hernandez-Trujillo V, Ballow M. Diagnosing primary immunodeficiency: a practical approach for the non-immunologist. Curr Med Res Opin 2015; 31 (04) 697-706
  • 19 Lo Stanley F. Reference intervals for laboratory tests and procedures. In: Kliegman R. St. Geme J, eds. Nelson Textbook of Pediatrics. 21st ed.. Elsevier; 2019
  • 20 Seidel MG, Kindle G, Gathmann B. et al; ESID Registry Working Party and collaborators. The European Society for Immunodeficiencies (ESID) Registry working definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract 2019; 7 (06) 1763-1770
  • 21 Pichichero ME. Immunologic dysfunction contributes to the otitis prone condition. J Infect 2020; 80 (06) 614-622
  • 22 Gámez G, Rojas JP, Cardona S. et al. Factors associated with Streptococcus pneumoniae nasopharyngeal carriage and antimicrobial susceptibility among children under the age of 5 years in the Southwestern Colombia. J Pediatr Infect Dis 2021; 16 (05) 205-215
  • 23 Karimi A, Isaiyan A, Aghamohammadi A. et al. Immunological evaluation of children with recurrent ear, nose, and throat (ENT) infections. Iran J Pediatr 2007; 17 (Suppl. 01) 5-13
  • 24 Church JA. Immunologic evaluation of the child with recurrent otitis media. Ear Nose Throat J 1997; 76 (01) 31-34 , 42
  • 25 Bazregari S, Azizi G, Tavakol M. et al. Evaluation of infectious and non-infectious complications in patients with primary immunodeficiency. Cent Eur J Immunol 2017; 42 (04) 336-341
  • 26 Reda SM, Afifi HM, Amine MM. Primary immunodeficiency diseases in Egyptian children: a single-center study. J Clin Immunol 2009; 29 (03) 343-351
  • 27 Al Ghwass MM, Halawa EF, Sabry SM, Ahmed D. Iron deficiency anemia in an Egyptian pediatric population: a cross-sectional study. Ann Afr Med 2015; 14 (01) 25-31
  • 28 Golz A, Netzer A, Goldenberg D, Westerman ST, Westerman LM, Joachims HZ. The association between iron-deficiency anemia and recurrent acute otitis media. Am J Otolaryngol 2001; 22 (06) 391-394
  • 29 Akcan FA, Dündar Y, Bayram Akcan H, Cebeci D, Sungur MA, Ünlü İ. The association between iron deficiency and otitis media with effusion. J Int Adv Otol 2019; 15 (01) 18-21
  • 30 Kumar V, Choudhry VP. Iron deficiency and infection. Indian J Pediatr 2010; 77 (07) 789-793
  • 31 Makary C, Chaiban R, Addicks B, Demirdag YY. Primary immunodeficiencies in patients with recurrent ear and sinus infections. J Allergy Clin Immunol 2013; 131 (02) AB68
  • 32 Abolhassani H, Azizi G, Sharifi L. et al. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immunol 2020; 16 (07) 717-732
  • 33 Seidel MG, Kindle G, Gathmann B. et al; ESID Registry Working Party and collaborators. The European Society for Immunodeficiencies (ESID) Registry working definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract 2019; 7 (06) 1763-1770
  • 34 Tangye SG, Al-Herz W, Bousfiha A. et al. Human inborn errors of immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol 2020; 40 (01) 24-64
  • 35 ESID Registry - Working definitions for clinical diagnosis of PID. Accessed December 24, 2021 at: https://esid.org/Working-Parties/Registry-Working-Party/Diagnosis-criteria