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

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Gülsüm Alkan ◽  
Sevgi Keles ◽  
İsmail Reisli
Keyword(s):  
Respiratory Tract ◽  
Common Variable Immunodeficiency ◽  
Respiratory Tract Infections ◽  
Disease Onset ◽  
Infection Prevalence ◽  
Diagnosis Delay ◽  
Upper Respiratory Tract ◽  
Onset Age ◽  
Tract Infections ◽  
Common Variable

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.

Infección por rinovirus: Necesidad de estudios anatomopatológicos y secuelas funcionales en niños

2021 ◽  
pp. 1-2
Author(s):  
Ma. del Rocío García-Olvera
Keyword(s):  
Respiratory Tract ◽  
Lower Respiratory Tract ◽  
Respiratory Tract Infections ◽  
Likelihood Method ◽  
Local Alignment ◽  
Infection Prevalence ◽  
Upper Respiratory Tract ◽  
Lower Respiratory Tract Infections ◽  
Alignment Tool ◽  
Tract Infections

Rhinoviruses (RVs) are increasingly implicated not only in mild upper respiratory tract infections, but also in more severe lower respiratory tract infections; however, little is known about species diversity and viral epidemiology of RVs among the infected children. Therefore, we investigated the rhinovirus (RV) infection prevalence over a 2-year period, compared it with prevalence patterns of other common respiratory viruses, and explored clinical and molecular epidemiology of RV infections among 590 children hospitalized with acute respiratory infection in north-western and central parts of Croatia. For respiratory virus detection, nasopharyngeal and pharyngeal flocked swabs were taken from each patient and subsequently analyzed with multiplex RT-PCR. To determine the RV species in a subset of positive children, 50UTR in RV-positive samples has been sequenced. Nucleotide sequences of referent RV strains were retrieved by searching the database with Basic Local Alignment Tool and used to construct alignments and phylogenetic trees using MAFFT multiple sequence alignment tool and the maximum likelihood method, respectively. In our study population RV was the most frequently detected virus, diagnosed in 197 patients (33.4%), of which 60.4% was detected as a monoinfection. Median age of RV-infected children was 2.25 years, and more than half of children infected with RV (55.8%) presented with lower respiratory tract infections. Most RV cases were detected from September to December, and all three species co-circulated during the analyzed period (2017–2019). Sequence analysis based on 50UTR region yielded 69 distinct strains; the most prevalent was RV-C (47.4%) followed by RV-A (44.7%) and RV-B (7.9%). Most of RV-A sequences formed a distinct phylogenetic group; only strains RI/HR409–18 (along with a reference strain MF978777) clustered with RV-C strains. Strains belonging to the group C were the most diverse (41.6% identity among strains), while group B was the most conserved (71.5% identity among strains). Despite such differences in strain groups (hitherto undescribed in Croatia), clinical presentation of infected children was rather similar. Our results are consistent with newer studies that investigated the etiology of acute respiratory infections, especially those focused on children with lower respiratory tract infections, where RVs should always be considered as potentially serious pathogens.

scholarly journals Predictive Factors for and Complications of Bronchiectasis in Common Variable Immunodeficiency Disorders

2022 ◽  
Author(s):  
Johannes M. Sperlich ◽  
Bodo Grimbacher ◽  
Veronika Soetedjo ◽  
Sarita Workman ◽  
Siobhan O. Burns ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lung Function ◽  
Respiratory Tract ◽  
Common Variable Immunodeficiency ◽  
Respiratory Tract Infections ◽  
Forced Expiratory Volume ◽  
Serum Immunoglobulin ◽  
Tract Infections ◽  
Common Variable

AbstractBronchiectasis is a frequent complication of common variable immunodeficiency disorders (CVID). In a cohort of patients with CVID, we sought to identify predictors of bronchiectasis. Secondly, we sought to describe the impact of bronchiectasis on lung function, infection risk, and quality of life. We conducted an observational cohort study of 110 patients with CVID and an available pulmonary computed tomography scan. The prevalence of bronchiectasis was 53%, with most of these patients (54%) having mild disease. Patients with bronchiectasis had lower median serum immunoglobulin (Ig) concentrations, especially long-term IgM (0 vs 0.25 g/l; p < 0.01) and pre-treatment IgG (1.3 vs 3.7 g/l; p < 0.01). CVID patients with bronchiectasis had worse forced expiratory volume in one second (2.10 vs 2.99 l; p < 0.01) and an annual decline in forced expiratory volume in one second of 25 ml/year (vs 8 ml/year in patients without bronchiectasis; p = 0.01). Patients with bronchiectasis also reported more annual respiratory tract infections (1.77 vs 1.25 infections/year, p = 0.04) and a poorer quality of life (26 vs 14 points in the St George’s Respiratory Questionnaire; p = 0.02). Low serum immunoglobulin M concentration identifies patients at risk for bronchiectasis in CVID and may play a role in pathogenesis. Bronchiectasis is relevant because it is associated with frequent respiratory tract infections, poorer lung function, a greater rate of lung function decline, and a lower quality of life.

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