Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency
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 de...
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| Format: | Article |
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Wiley
2018-01-01
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| Series: | International Journal of Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2018/3527480 |
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| author | Gülsüm Alkan Sevgi Keles İsmail Reisli |
| author_facet | Gülsüm Alkan Sevgi Keles İsmail Reisli |
| author_sort | Gülsüm Alkan |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-9f039fa3b3154fea94f73382c8d2c2d9 |
| institution | Kabale University |
| issn | 1687-9740 1687-9759 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Pediatrics |
| spelling | doaj-art-9f039fa3b3154fea94f73382c8d2c2d92025-02-03T05:48:22ZengWileyInternational Journal of Pediatrics1687-97401687-97592018-01-01201810.1155/2018/35274803527480Evaluation of Clinical and Immunological Characteristics of Children with Common Variable ImmunodeficiencyGülsüm Alkan0Sevgi Keles1İsmail Reisli2Department of Pediatric Infectious Diseases, Selcuk University Faculty of Medicine, Konya, TurkeyDepartment of Pediatric Allergy and Immunology, Necmettin Erbakan University Meram Faculty of Medicine, Konya, TurkeyDepartment of Pediatric Allergy and Immunology, Necmettin Erbakan University Meram Faculty of Medicine, Konya, TurkeyBackground. 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.http://dx.doi.org/10.1155/2018/3527480 |
| spellingShingle | Gülsüm Alkan Sevgi Keles İsmail Reisli Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency International Journal of Pediatrics |
| title | Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency |
| title_full | Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency |
| title_fullStr | Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency |
| title_full_unstemmed | Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency |
| title_short | Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency |
| title_sort | evaluation of clinical and immunological characteristics of children with common variable immunodeficiency |
| url | http://dx.doi.org/10.1155/2018/3527480 |
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