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160 Follow-up of Two Children with Very Low IgE from Birth
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  1. Helena Tesari Crnković,
  2. Andrea Šimić Klarić,
  3. Marijana Tomić Rajić,
  4. Vlado Drkulec,
  5. Romana Gjergja Juraški,
  6. Mirjana Turkalj
  1. General County Hospital Požega, Faculty of Medicine, J.J. Strossmayer University of Osijek

Abstract

Background Immunoglobulin E (IgE) is best known for its association with allergic diseases. Very low IgE may be a marker for other immunodeficiencies. Deficiency or low IgE is defined with IgE concentration lower than 2.5 IU/ml. A majority of studies suggest a prevalence of 1-2.6 %.

The clinical significance of an isolated low IgE („selective IgE deficiency’) is unknown and is not currently included in the consensus classification of Primary Immunodeficiency Diseases (PIDD). Low IgE is suggestive of a primary humoral immunodeficiency, like Common variable immunodeficiency (CVID). In recent studies a connection between low serum IgE and low levels of one or more classes of immunoglobulins are found. The children’s group was characterized with a higher prevalence of asthma.

Both groups (children and adults) had significantly higher prevalence of chronic sinusitis, otitis media, autoimmune, and oncological diseases than controls. In our case report two cases of very low IgE are presented. Both of them have been followed up from birth.

Case presentation: a cohort of 72 children has been followed up from birth up to 10 years of age. In two of them a very low serum IgE was found. Serum IgE was assessed at birth from cord blood, and reassessed at 1, 2 and 10 years of age with specific IgE (sIgE) for standard palette of food and inhalant allergens. At 10 years of age skin prick test, measurement of fractional exhaled nitric oxide (FeNO) and spirometry have been conducted.

The girl had an unmeasurable level of IgE in cord blood, and also at 1 and 2 years, and her IgE at 10 years was 2.0 IU/ml. The boy’s IgE in cord blood, and at 1, 2 and 10 years was 0.11, 1.0, 8.2 and 0.1 IU/ml, respectively.

They both had symptoms characteristic of asthma and allergic rhinoconjuctivitis throughout their childhood, with negative sIgE at 1, 2 and 10 years of age and no evidence of type I hypersensitivity. Their other immunoglobulin (IgG, IgM, and IgA) levels at 10 years was within reference range for age, and they showed no symptoms suggestive of immunodeficiency or autoimmunity.

Conclusion here we report of two cases with selective IgE deficiency in children who had been followed up from birth. Very low serum IgE may serve as a marker of immune dysregulation and autoimmunity, and should trigger appropriate investigation (immunoglobulin quantification). The children will be closely monitored.

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