Contemporary Perspectives on Pediatric Diffuse Lung Disease
Department of Pediatric Radiology, Texas Children's Hospital, Baylor College of Medicine, 6701 Fannin Street, Houston, TX 77030, USA. Radiologic Clinics of North America
(Impact Factor: 1.98).
09/2011; 49(5):847-68. DOI: 10.1016/j.rcl.2011.06.004
It is much more challenging in children than in adults to obtain computed tomography images of the lung parenchyma at optimal lung volumes without motion artifact. Some of the more common forms of diffuse lung disease in adults rarely occur in children, and several forms of diffuse lung disease are unique to children. Recognition of these differences has led to the development of a new classification scheme for pediatric diffuse lung disease. Knowledge of this classification and recognition of characteristic imaging findings of specific disorders will lead to accurate diagnosis and guide appropriate treatment of children with diffuse lung disease.
Available from: Joaquim Piqueras
Available from: ncbi.nlm.nih.gov
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ABSTRACT: The pathologic classification of diffuse lung disease in children and adolescents has undergone revision in recent years in response to rapid developments and new discoveries in the field. A number of important advancements have been made in the last 10 years including the description of new genetic mutations causing severe lung disease in infants and children, as well as the description of new pathologic entities in infants. These recently described entities, including ABCA3 surfactant disorders, pulmonary interstitial glycogenosis, and neuroendocrine cell hyperplasia of infancy, are being recognized with increasing frequency. This review will include brief discussion of the etiology and pathogenesis of the major groups of diffuse lung disease in children. Histopathologic features are discussed for each of the major categories of diffuse lung disease in children, beginning with the genetic, developmental, and alveolar growth disorders common in infancy, followed by brief discussion of airway diseases, immunologic diseases, and pulmonary vascular diseases seen more commonly in older children. A protocol for handling pediatric wedge lung biopsies is also discussed, which optimizes the diagnostic yield of lung biopsies in this population.
Available from: Mateja Cernelc-kohan
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ABSTRACT: Advances in genetics and clinical diagnostics, along with recently described clinical entities and refined classification schemes, have improved our understanding of diffuse and interstitial lung diseases in children. This review presents recent updates in these disorders in the context of systemic inflammatory conditions.
Classification of childhood diffuse lung disease (DLD) using adult paradigms is not useful. Distinct clinical-pathologic entities exist in children. Infants are more likely to present with genetic and developmental disorders, and older children with inflammatory and immune-mediated conditions. A combination of clinical evaluation, high-resolution computed tomography scanning, pulmonary function testing and serology, with bronchoscopy and surgical lung biopsy in selected cases, is most useful in the evaluation of DLD in the context of rheumatologic conditions. Common causes of DLD, such as infection, especially in the setting of immunodeficiency, must be ruled out. Optimal therapy for specific disorders will require careful analysis of data from national registries. Emerging use of biomarkers and high-throughput molecular analysis will yield novel insight into these disorders.
In the setting of known or suspected rheumatologic disorders, diagnosis and management of DLD are challenging, and require close collaboration among rheumatologists, pulmonologists, and other specialists.
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