Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency

International Journal of Pediatrics
Gülsüm Alkanİsmail Reisli

Abstract

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder (PID) that typically presents with hypogammaglobulinemia and impaired antibody production. This study aimed to promote the awareness of CVID, whose clinical spectrum is quite broad. The demographic, clinical, and laboratory characteristics of 12 children (seven males and five females) with CVID were analyzed retrospectively. The patients were diagnosed using the diagnostic criteria of the European Society for Primary Immunodeficiencies. The median disease onset age was 7.2 ± 4.1 years, and the mean diagnosis age was 11.6 ± 3.7 years. The diagnosis delay was 4.3 ± 2.6 years, and the parental consanguinity rate was 75%. Most patients presented with recurrent infections, including upper respiratory tract infections (n = 8), lower respiratory tract infections (n = 9), and gastroenteritis (n = 5). In addition, growth retardation (n = 9) and bronchiectasis (n = 5) were common comorbidities. Two patients presented with autoimmune thrombocytopenia and anemia, and one patient exhibited lung empyema. All the patients had immunoglobulin G deficiencies. CVID is a heterogeneous disease, so the diagnosis is frequently delayed. In the CVID patients with pulmonary c...Continue Reading

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Citations

Dec 14, 2019·Pediatric Pulmonology·Nasim RamziGholamreza Azizi
Dec 24, 2021·European Journal of Pediatrics·Aleksandra Szczawinska-PoplonykMonika Wiczuk-Wiczewska

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Methods Mentioned

BETA
exome sequencing

Software Mentioned

Statistical Package for the Social Sciences

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