Background: Granulomatous lymphocytic interstitial lung disease (GLILD) has been increasingly recognized in children affected with primary immunodeficiencies (PIDs). In this study, we aimed to better characterize the spectrum of pediatric PIDs coexisting with GLILD including clinical and immunological predictors, thoracic imaging findings, and histopathologic features. Methods: We respectively reviewed records of six representative cases of children, three of them affected with common variable immunodeficiency (CVID) and three with syndromic immunodeficiencies, in whom a diagnosis of GLILD was established based on clinical, radiological, and histopathologic findings. Clinical and immunological predictors for GLILD were also analyzed in the patients studied. Results: All the children with GLILD had a history of autoimmune phenomena, organ-specific immunopathology, and immune dysregulation. Defective B-cell maturation and deficiency of memory B cells were found in all the children with GLILD. The radiological and histopathological features consistent with the diagnosis of GLILD, granulomatous disease, and lymphoid hyperplasia, were accompanied by chronic airway disease with bronchiectasis in children with CVID and syndromic PIDs. Conclusions: Our study shows that both CVID and syndromic PIDs may be complicated with GLILD. Further studies are required to understand the predictive value of coexisting autoimmunity and immune dysregulation in the recognition of GLILD in children with PIDs.

Noninvasive and easy-to-use tools to monitor airway inflammation in asthma are needed to maintain disease control, particularly in pediatric population. The aim of the study was to evaluate exhaled breath temperature (EBT) in pediatric respiratory clinic setting. We evaluated 37 children and adolescents with asthma (5-17 years; median: 11 years). The patients were followed up in stable condition and during exacerbations (paired observations in n = 19 subjects). We evaluated medication use, EBT, fractional exhaled nitric oxide (FeNO), spirometry and atopic status of patients. EBT was significantly higher in children with asthma exacerbation {entire group: median [interquartile range (IQR)]: 32.3 [1.1]°C vs. 33.8 [1.7]°C; p < 0.001 and mean ± SD: 33.1 ± 1.0°C vs. 33.6 ± 1.1°C; p = 0.038 for paired observations}. Significant correlation was observed between EBT and FeNO in the entire group (r = 0.22; p = 0.03). No difference was observed in EBT median values in atopic and non-atopic subjects in the entire group (median [IQR]: 32.6 [1.6] vs. 32.7 [2.0]; p = 0.88) and in subgroups. There was no difference in EBT values in patients receiving systemic or inhaled glucocorticosteroids (p = 0.45 and 0.83). There was no significant correlation between EBT and body or room temperature. The only significant predictor of exacerbation in logistic regression model was EBT {aOR = 2.4; 95% [confidence interval (CI)]: 1.4-4.1}. ROC analysis demonstrated applicability of EBT as a marker of asthma exacerbation in children (AUC = 0.748; p < 0.001; cut-off = 33.3°C; sensitivity: 64.3%; specificity: 82.1%). We suggest that EBT may serve as marker and predictor of asthma exacerbation in children. EBT follow-up may be useful in asthma monitoring in children and adolescents.

In recent years, questionnaires were published in English to assess the quality of life of patients with PCD (Primary Ciliary Diskinesia) for adults, adolescents aged 13–17 years, and children aged 6–12 years and their caregivers. This study aimed to prepare Polish versions of the questionnaires and validate them in specific age groups with the participation of Polish patients with PCD. The individual questionnaires were translated and discussed with the involvement of the creator of the original questionnaire in English. Patients completed the questionnaires according to their affiliation with one of the groups. Validation was based on internal consistency analysis (Cronbach’s alpha coefficient and split-half reliability) and test–retest reliability (intraclass correlation coefficient—ICC). The internal consistency of all questionnaires was from moderate to very good (Cronbach’s alpha 0.67–0.91, split-half reliability 0.53–0.95). The consistency of the measurements showed excellent repeatability (ICC 0.67–0.91). The surveyed Polish PCD patients rated their quality of life quite well (63–77%). QOL questionnaires for patients with PCD can be used routinely during each medical check-up as a simple tool to provide the doctor with an indication of the effectiveness of treatment and the impact of the disease on the patient’s quality of life.

Introduction. Spontaneous pneumomediastinum is a very rare condition in children. Nevertheless it should be considered in the differential diagnosis in patients who present with chest pain and dyspnoe.Aim. The aim of our study was to describe clinical presentation, management and outcomes of the paediatric patients with spontaneous pneumomediastinumMaterial and Methods. This was a retrospective analysis of the charts of all the patients who were admitted to the Department of Pneumonology, Paediatric Allergy and Clinical Immunology in a ten year period from 01.01.2011 till 31.12.2020 in whom spontaneous pneumomediastinum was diagnosed.Results. There were 11 children (7 females) with spontaneous pneumomediastinum. The median age of the children was 11 years (range 3 to 17.5 years). Most of the children presented to the hospital with chest pain, three children complained of the neck swelling and four children developed dyspnoe. Three children with the primary spontaneous pneumomediastinum had a history of physical exercise prior to the onset of symptoms. The secondary spontaneous pneumomediastinum occurred in two children with asthma and 4 children with pneumonia. Genetic material of human Bocavirus was identified in 3 cases. In 81.8% of children pneumomediastinum was accompanied by subcutaneous emphysema and in one case, in a child with severe pneumonia and respiratory insufficiency caused by Bocavirus with pneumorrhachis. In 10 children computed tomography was performed, bronchoscopy in 4 and esophagoscopy in two children. There was no evidence of esophageal rupture or bronchial tree rupture in any of our patients. Three children with pneumonia and pneumomediastinum developed respiratory insufficiency, two of these were treated with mechanical ventilation and one with High Flow Nasal Cannula oxygen therapy. All the children received oxygen. In one child surgical procedure was performed and the drain was inserted into mediastinal space in order to decompress it. Outcome was favourable in all children. Mean time to recovery was 10.6 ± 1.2 days. There was no recurrence of symptoms in any of our patients.Conclusions. Spontaneous pneumomediastinum in most cases is a benign condition, sporadically however it may progress rapidly, leading to respiratory insufficiency and warrant invasive management.