Background: There is growing recognition and understanding of the entities that cause interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD in older children and adults.Methods: A multidisciplinary panel was convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants under 2 years of age. Recommendations were formulated using a systematic approach. Outcomes considered important included the accuracy of the diagnostic evaluation, complications of delayed or incorrect diagnosis, psychosocial complications affecting the patient’s or family’s quality of life, and death.Results: No controlled clinical trials were identified. Therefore, observational evidence and clinical experience informed judgments. These guidelines: (1) describe the clinical characteristics of neonates and infants (<2 yr of age) with diffuse lung disease (DLD); (2) list the common causes of DLD that should be e...

/pdf/an-official-american-thoracic-society-clinical-practice-2ujvvcpf03.pdf

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

Rationale: Unrecognized airway infection and inflammation in young children with cystic fibrosis (CF) may lead to irreversible lung disease; therefore early detection and treatment is highly desirable.Objectives: To determine whether the lung clearance index (LCI) is a sensitive and repeatable noninvasive measure of airway infection and inflammation in newborn-screened children with CF.Methods: Forty-seven well children with CF (mean age, 1.55 yr) and 25 healthy children (mean age, 1.26 yr) underwent multiple-breath washout testing. LCI within and between-test variability was assessed. Children with CF also had surveillance bronchoalveolar lavage performed.Measurements and Main Results: The mean (SD) LCI in healthy children was 6.45 (0.49). The LCI was higher in children with CF (7.21 [0.81]; P < 0.001). The upper limit of normal for the LCI was 7.41. Fifteen (32%) children with CF had an elevated LCI. LCI measurements were repeatable and reproducible. Airway infection was present in 17 (36%) children wit...

https://www.atsjournals.org/doi/pdf/10.1164/rccm.201109-1631OC

Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index.

Rationale: Airway wall structure in preschoolers with severe recurrent wheeze is poorly described.Objectives: To describe airway wall structure and inflammation in preschoolers with severe recurrent wheeze.Methods: Flexible bronchoscopy was performed in two groups of preschoolers with severe recurrent wheeze: group 1, less than or equal to 36 months (n = 20); group 2, 36–59 months (n = 29). We assessed airway inflammation, reticular basement membrane (RBM) thickness, airway smooth muscle (ASM), mucus gland area, vascularity, and epithelial integrity. Comparisons were then made with biopsies from 21 previously described schoolchildren with severe asthma (group 3, 5–11.2 yr).Measurements and Main Results: RBM thickness was lower in group 1 than in group 2 (3.3 vs. 3.9 μm; P = 0.02), was correlated with age (P < 0.01; ρ = 0.62), and was higher in schoolchildren than in preschoolers (6.8 vs. 3.8 μm; P < 0.01). ASM area was lower in preschoolers than in schoolchildren (9.8% vs. 16.5%; P < 0.01). Vascularity wa...

/pdf/airway-remodeling-in-preschool-children-with-severe-31irtf95vh.pdf

Airway Remodeling in Preschool Children with Severe Recurrent Wheeze

Neonatal respiratory distress syndrome can progress to bronchopulmonary dysplasia (BPD), a serious pulmonary fibrotic disorder. Given the involvement of the extrinsic coagulation cascade in animal models of lung fibrosis, we examined its role in BPD. We observed a higher number of neutrophils expressing tissue factor (TF) in bronchoalveolar lavage fluid (BALF) from infants with BPD than from those with uncomplicated respiratory distress syndrome together with a parallel decrease in TF and connective tissue growth factor (CTGF) in BALF supernatants during the disease course. The involvement of coagulation in the fibrotic process associated with BPD was further evaluated by treating primary human colonic myofibroblasts with BALF supernatants from infants with BPD. These human colonic myofibroblasts demonstrated an enhanced C5a- and thrombin-dependent migration. Moreover, they expressed TF in an endothelin-1-dependent manner, with subsequent activation of the extrinsic coagulation cascade and CTGF production mediated by protease-activator receptor-1 signaling. These data provide a novel mechanism for the development of BPD and indicate that endothelin-1 signaling contributes to fibrosis by upregulating a TF/thrombin amplification loop responsible for CTGF production, and offer novel and specific therapeutic targets for pulmonary fibrotic disease.

Endothelin-1 Signaling Promotes Fibrosis In Vitro in a Bronchopulmonary Dysplasia Model by Activating the Extrinsic Coagulation Cascade

Background: Cysteinyl leukotrienes (CysLTs), including LTC4, LTD4 and LTE4, are pivotal mediators in the pathophysiology of asthma. Aim: To determine whether CysLT levels are increased in the lower airways of children with respiratory syncytial virus (RSV) bronchiolitis, as they are in asthmatic children, and to investigate a possible heterogeneity in CysLT levels in children with RSV bronchiolitis. Methods: Bronchoalveolar lavage (BAL) fluids were obtained from children with acute RSV bronchiolitis (n = 20), from children with acute asthma who had no identifiable virus infection (n = 16) and from control subjects (n = 14). BAL cell counts and differentials were determined, and the concentrations of CysLTs were measured by ELISA. Results: CysLT levels in the asthma (70.6 ± 52.7 pg/ml, p < 0.001) and bronchiolitis groups (21.9 ± 23.3 pg/ml, p < 0.05) were significantly higher than in the control group (8.7±5.2 pg/ml). Among bronchiolitis subjects, the eosinophil-positive subgroup (n = 6) showed significantly higher CysLT levels (49.0±26.7 pg/ml, p = 0.001) than the control group, but this was not observed in the eosinophil-negative subgroup (n = 14, 10.3±6.3 pg/ml, p = 0.47). Conclusion: CysLT levels are increased in the lower airways during RSV bronchiolitis, although their intensities are lower than those in acute asthma. Among bronchiolitis subjects, high CysLT producers could be distinguished from low CysLT producers by the presence of eosinophilia in BAL fluids, suggesting a pathophysiological heterogeneity in RSV bronchiolitis.

Increased levels of BAL cysteinyl leukotrienes in acute RSV bronchiolitis

ERS Task Force on bronchoalveolar lavage in children Members of the Task Force: J. de Blic and F. Midulla (co-chairmen), A. Barbato, A. Clement, I. Dab,

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ERS Task Force on bronchoalveolar lavage in children Members of the Task Force: J. de Blic and F. Midulla (co-chairmen), A. Barbato, A. Clement, I. Dab,