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Article Type

Original Study

Abstract

Objectives The aim of the study was to perform a retrospective investigation describing the clinical and immunological spectrums of common variable immunodeficiency (CVID) at a single center during the period from January 2006 through to June 2013. Backgrounds CVID is the most common primary immunodeficiency. The inheritance of CVID is variable; the majority of cases are sporadic, but familial patterns of inheritance are seen in ~10-20% of cases. The hallmark of CVID is reduced serum levels of serum immunoglobulins, IgG, IgA, and or IgM, which leads to recurrent infections. CVID also has noninfectious manifestations such as autoimmunity, granulomatous disease, and malignancy. Patients and methods We reviewed the medical records of patients who had been diagnosed with CVID at the Immunology Clinic at Queen Rania Children«SQ»s Hospital from January 2006 through to June 2013. Collected data included clinical presentation, demographics, associated autoimmune features, allergies, complications, mortality, and immunological workup at the time of diagnosis. Results The total number of patients was 17 [12 (71%) male and five (29%) female patients]; the median age at presentation was 5.1 years. Infections were the most common presentation; pneumonia was the most frequent at 71%, followed by sinusitis in 59% of patients. Cytopenias were the most frequent autoimmune association; they were reported in one-third of the patients. Lymphoproliferation was noted in 29%; two of them were due to Epstein-Barr virus infection. Bronchiectasis was diagnosed in four (24%) patients. Fourteen of 17 patients had immunoglobulin IgG, IgM, and IgA levels 2 SD below the mean for age, whereas three patients had low IgG and IgA and normal IgM levels. Conclusion Although our study was limited by its retrospective nature and it did not represent the entire population of CVID patients in our country, it emphasizes the importance for awareness of these disorders to improve CVID outcomes.

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