Childhood Respiratory Conditions: Nonasthma Chronic Lung Disease.

Bronchopulmonary dysplasia (BPD) is a chronic lung disease that results from impaired lung development or lung injury from ventilatory support. It primarily is seen in infants born prematurely. Approximately 95% of infants with BPD had a low birth weight (ie, less than 1,500 g). This condition affects pulmonary function throughout the life span. Many children with BPD develop asthmalike symptoms with recurrent wheezing beginning in the preschool-aged years. Complications include pulmonary hypertension, tracheomalacia, glottic damage, sleep apnea, and more frequent and/or severe respiratory infections. Measures should be taken to prevent respiratory infections in these patients. Cystic fibrosis (CF) is caused by an autosomal recessive sequence variation in the CF transmembrane conductance regulator ( CFTR ) gene that results in mucus accumulation on cell surfaces. Mucus accumulation in the airways causes chronic cough, wheezing, recurrent infections, and progressive loss of lung function. Treatment includes clearance of mucus from the lungs and infection management. Complications and associated conditions include sinusitis, nutritional and gastrointestinal issues, dehydration, pancreatic insufficiency, and CF-related diabetes. All of these should be addressed. Most cases of CF are diagnosed via newborn screening and follow-up sweat tests. New CFTR modulators that improve CFTR protein function offer hope of improved longevity and quality of life for patients with specific CFTR sequence variants.