Pleuropulmonary Blastoma Megan K. Dishop Claire Langston Pleuropulmonary blastoma, a relatively recently recognized childhood malignancy, is an embryonal tumor of the lung, pleura, or mediastinum derived from the thoracic splanchnopleural…
Pulmonary Hypoplasia Megan K. Dishop Pulmonary hypoplasia is a decrease in weight and/or volume of the lung due to impaired growth in utero. It is identified in approximately 14% of…
Acinar Dysplasia Megan K. Dishop Claire Langston This condition is an exceedingly rare but quite striking form of primary pulmonary hypoplasia with arrest of lung development at an immature stage…
Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins Megan K. Dishop Claire Langston Alveolar capillary dysplasia with misalignment of pulmonary veins is an increasingly recognized cause of persistent pulmonary hypertension…
Genetic Disorders of Surfactant Metabolism (SFTPB, SFTPC, and ABCA3 Mutations) Megan K. Dishop The genetic disorders of surfactant metabolism are a group of diseases caused by mutations in genes affecting…
Primary Ciliary Dyskinesia Megan K. Dishop Primary ciliary dyskinesia (PCD) is a disorder of immotile or abnormally motile cilia resulting in symptoms of chronic recurrent respiratory infection, sinusitis, and otitis…
Cystic Fibrosis Megan K. Dishop Claire Langston Cystic fibrosis is the major cause of severe chronic lung disease in children. It also is the principal reason for lung transplantation in…
Pulmonary Interstitial Glycogenosis Megan K. Dishop Pulmonary interstitial glycogenosis (PIG) is a form of interstitial widening in infant lungs, typically seen below the age of 6 months. This entity was…
Neuroendocrine Cell Hyperplasia of Infancy (Persistent Tachypnea of Infancy) Megan K. Dishop Claire Langston Neuroendocrine cell hyperplasia of infancy (NEHI) is the pathologic correlate of the clinical syndrome persistent tachypnea…
Tissue Heterotopia and Related Abnormalities Megan K. Dishop Claire Langston Although rare, a variety of heterotopic tissues in the lung have been described, including striated muscle, adrenal tissue, thyroid, pancreas,…
