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2018 ; 2018
(ä): 3527480
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Evaluation of Clinical and Immunological Characteristics of Children with Common
Variable Immunodeficiency
#MMPMID29849668
Alkan G
; Keles S
; Reisli ?
Int J Pediatr
2018[]; 2018
(ä): 3527480
PMID29849668
show ga
BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency
disorder (PID) that typically presents with hypogammaglobulinemia and impaired
antibody production. OBJECTIVES: This study aimed to promote the awareness of
CVID, whose clinical spectrum is quite broad. METHODS: 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.
RESULTS: 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. CONCLUSION: CVID is a heterogeneous disease, so the diagnosis is
frequently delayed. In the CVID patients with pulmonary complications,
relationships were seen with the diagnosis delay, symptom onset age, and lung
infection prevalence. Overall, the early diagnosis and treatment of PIDs can
preclude life-threatening complications.