Early human development
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Children's interstitial lung disease (ILD) includes a wide range of rare respiratory disorders associated with high morbidity and mortality. Genetic factors, systemic disease processes, nonspecific inflammatory or fibrotic patterns of repair seen in a number of clinical settings are involved in the ILD pathogenesis. ⋯ The diagnostic approach is based on a combination of history/physical examinations, imaging studies, pulmonary function testing, genetic testing, bronchoalveolar lavage (BAL) and in most cases an open lung biopsy. Although some disease types overlap with those seen in adults, in this review emphasis is placed on entities unique to the pediatric population focusing on clinical characteristics, histologic definitions, radiologic-pathologic correlation and therapeutic strategies.
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Non-invasive positive pressure ventilation is increasingly used in children both in acute and in chronic setting. Clinical data supporting safety, efficacy and limitations in children are growing. ⋯ Non-invasive ventilation can be applied at home. Its use at home requires appropriate diagnostic procedures, accurate titration of the ventilators, cooperative and educated families and careful, well-organized follow-up programs.