Abstract
Background: Idiopathic fibrosing alveolitis (IFA) in children is a rare chronic progressive pulmonary disease, which represents a heterogeneous group of respiratory disorders that are mostly chronic and associated with high morbidity, mortality.
Methods: The study had included the clinical data of 10 children (8 girls, 2 boys, mean age 10.7±1.7years) with IFA diagnosis. Lung function exploring was performed by spirometry “Minato Autospiro”, Japan.
Aim: Evaluation of IFA functional respiratory disorders in children.
Results: IFA is a rare chronic progressive lung disease with severe functional impact on the child's health. IFA's typical features in these children are cough, dyspnea, tachypnea, in some cases, cyanosis, disnutrition, nail-clubbing. HRCT pulmonary detected the changes characterized by the presence of abnormal basal bilateral ground-glass opacities, “honeycomb” cysts type, “syndrome ring”, thickening of subpleural reticular basal septum. Respiratory function in these children is marked by severe restrictive disorders (FVC – 53.92±7.7%, FEV1 – 54.38±9.3%) and obstructive disorders (FEF25-75 – 40.78±10.8%, PEF – 49.47±8.9%). Respiratory failure, pulmonary hypertension, heart failure are major complications in these children that determine prognosis and evolution of IFA.
Conclusion: Idiopathic fibrosing alveolitis is a severe pulmonary disease, which evolves with specific clinical signs such as dry cough, severe progressive respiratory failure associated with poor nutrition, pulmonary hypertension. Research of lung function in children with pulmonary fibrosis alveolitis demonstrate severe restrictive disorders associated with airflow limitation and bronchial conductivity disorders.
- Copyright ©the authors 2016
