Multiple Breath Washout for Early Assessment of Pulmonary Complications in Patients With Primary Antibody Deficiencies: An Observational Study in Pediatric Age.

Background: In primary antibody deficiencies (PADs), pulmonary complications are the main cause of morbidity, despite immunoglobulin substitutive therapy, antibiotic treatment of exacerbations, and respiratory physiotherapy. Current Italian recommendations for surveillance of PADs respiratory complications include an annual assessment of spirometry and execution of chest high-resolution computed tomography (HRCT) every 4 years. Objective: This study aimed to evaluate the effectiveness of the lung clearance index (LCI) as an early marker of lung damage in patients with PADs. LCI is measured by multiple breath washout (MBW), a non-invasive and highly specific test widely used in patients with cystic fibrosis (CF). Methods: Pediatric patients with PADs (n = 17, 10 male, 7 female, and age range 5-15 years) underwent baseline assessment of lung involvement with chest HRCT, spirometry, and multiple breath nitrogen washout. Among them, 13 patients were followed up to repeat HRCT after 4 years, while performing pulmonary function tests annually. Their baseline and follow-up LCI and forced expiratory volume at 1 s (FEV1) values were compared, taking HRCT as the gold standard, using logistic regression analysis. Results: Lung clearance index [odds ratio (OR) 2.3 (confidence interval (CI) 0.1-52) at baseline, OR 3.9 (CI 0.2-191) at follow-up] has a stronger discriminating power between altered and normal HRCT rather than FEV1 [OR 0.6 (CI 0.2-2) at baseline, OR 1.6 (CI 0.1-13.6) at follow-up]. Conclusion: Within the context of a limited sample size, LCI seems to be more predictive of HRCT alterations than FEV1 and more sensitive than HRCT in detecting non-uniform ventilation in the absence of bronchiectasis. A study of a larger cohort of pediatric patients followed longitudinally in adulthood is needed to challenge these findings.

Longitudinal Assessment of Patients With Cystic Fibrosis Lung Disease With Multivolume Noncontrast MRI and Spirometry.

BACKGROUND: MRI has been suggested as a radiation-free imaging modality to investigate early structural alterations and regional functional impairment in cystic fibrosis (CF) lung disease. PURPOSE/HYPOTHESIS: To compare functional and morphological MRI changes over the course of the disease to changes in spirometry. STUDY TYPE: Longitudinal retrospective study. POPULATION: Twenty patients with CF lung disease (at baseline, age = 16.5 (13.3-20.6) years, forced expiratory volume in 1 second (as % of predicted [%pred]) FEV1 = 71 (59-87) %pred, forced expiratory flow at 25-75% of forced vital capacity FEF25-75 = 39 (25-63) %pred. FIELD STRENGTH/SEQUENCE: 1.5T / T2 -weighted HASTE; T2 -weighted TSE-PROPELLER; T2 -weighted bSSFP; T1 -weighted 3D GRE. ASSESSMENT: Nonenhanced chest MRI and spirometry were retrospectively collected over a 3-year period from the initial recruitment visit. Images acquired at end-inspiration and end-expiration were registered by software using the optical flow method to measure expiratory-inspiratory differences in MR signal-intensity (Δ1 H-MRI). Measures of CF functional impairment were defined from Δ1 H-MRI: Δ1 H-MRI median, Δ1 H-MRI quartile coefficient of variation (QCV), and percent low-signal-variation volume (LVV). MR images were also evaluated by three readers using a CF-specific scoring system. STATISTICAL TESTS: Spearman correlation analysis, Spearman rank correlation analysis, linear mixed-effect model analysis, intraclass correlation coefficient. RESULTS: Functional imaging parameters and total morphological score correlated with all spirometric measures, as did subscores of bronchial wall thickening/bronchiectasis, mucus plugging, and consolidation. Overall, the percent change of Δ1H-MRI median correlated with the percent change of FEV1 (ΔFEV1 , r = 0.41, P < 0.01) and the percent change of FEF25-75 (ΔFEF25-75%, r = 0.38, P < 0.01). The percent change of LVV correlated with ΔFEV1 (r = -0.47, P < 0.001) and ΔFEF25-75 (r = -0.50, P < 0.001). DATA CONCLUSION: These preliminary results suggest that nonenhanced multivolume MRI may provide a feasible tool to regionally map early pulmonary alterations for longitudinal evaluation of CF lung disease, without exposing the patients to ionizing radiation. LEVEL OF EVIDENCE: 3T TECHNICAL EFFICACY STAGE: 5.

The NeoAPACHE Study Protocol I: Assessment of the Radiographic Pulmonary Area and Long-Term Respiratory Function in Newborns With Congenital Diaphragmatic Hernia.

In newborns with congenital diaphragmatic hernia (CDH), the radiographic lung area is correlated with functional residual capacity (FRC) and represents an alternative method to estimate lung hypoplasia. In a cohort of newborn CDH survivors, we retrospectively evaluated the relationship between radiographic lung area measured on the 1st day of life and long-term respiratory function. As a secondary analysis, we compared radiographic lung areas and respiratory function between patients undergoing fetal endoscopic tracheal occlusion (FETO) and patients managed expectantly (non-FETO). Total, ipsilateral, and contralateral radiographic areas were obtained by tracing lung perimeter as delineated by the diaphragm and rib cage, excluding mediastinal structures and herniated organs. Tidal volume (VT), respiratory rate (RR), and their Z-Scores when compared to the norm were collected from pulmonary function tests (PFTs) performed at 12 ± 6 months of age. Linear regression analyses using the absolute Z-Score values for each parameter were performed. In CDH survivors, an increase in total and ipsilateral lung area measured at birth was related to a reduction in the absolute Z-Score for VT in PFTs (p = 0.046 and p = 0.023, respectively), indicating a trend toward an improvement in pulmonary volumes and VT normalization. Radiographic lung areas were not significantly different between FETO and non-FETO patients, suggesting a volumetric lung increase due to prenatal intervention. However, the mean Z-Score value for RR was significantly higher in the FETO group (p < 0.001), probably due to impaired diaphragmatic motility in the most severe cases. Further analyses are necessary to better characterize the role of the radiographic pulmonary area in the prognostic evaluation of respiratory function in patients with CDH. Clinical Trial Registration: This trial was registered at ClinicalTrials.gov with the identifier NCT04396028.

Thoracoscopic Resection of Congenital Lung Malformation: Looking for the Right Preoperative Assessment.

INTRODUCTION: Consensus on the best postnatal radiological evaluation of congenital lung malformations (CLMs) is still lacking. In recent years, the interest on magnetic resonance imaging (MRI) has grown, but its role is still unknown. AIM: The aim of the study was to identify the best preoperative diagnostic assessment for CLM. MATERIALS AND METHODS: All patients with a prenatal suspicion of CLM between January 2014 and February 2018 were studied. Asymptomatic newborns underwent MRI, during spontaneous sleep without contrast. Patients with a positive MRI were scheduled for computed tomography (CT) within the fourth month of life. Thoracoscopic resection was performed in cases with a pathological CT. MRI, CT, and surgical findings were compared based on dimension, localization, and features of the CLM using the Cohen's kappa test (K). RESULTS: A total of 20 patients were included (10 males). No difference was found in the diameter and site of the lesions always localized in the same side (K = 1) and in the same pulmonary lobe (K = 1). Infants who underwent thoracoscopic resection included: three congenital pulmonary airway malformations (CPAMs), five extralobar and eight intralobar sequestrations (bronchopulmonary sequestrations [BPSs]), three bronchogenic cysts, and one congenital emphysema. The concordance between MRI and CT and between radiological investigations and pathology was satisfactory for the greatest part of the studied variables. MRI showed sensitivity of 100%, specificity of 82%, positive predictive value of 50% and negative predictive value of 100% for CPAM and 77, 100, 100, and 80% for BPS, respectively. CONCLUSION: MRI proved to be a reliable diagnostic investigation for CLM with high sensitivity and specificity. Early MRI in spontaneous sleep without contrast and preoperative contrast CT scan is a valuable preoperatory assessment.