See also Chapter 427 . Inherited Disorders of Surfactant Metabolism Jennifer A. Wambach Lawrence M. Nogee F. Sessions Cole Aaron Hamvas Pulmonary surfactant is a mixture of phospholipids and proteins synthesized, packaged, and secreted by alveolar type II pneumocytes (AEC2s) that line the distal…
Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by impaired ciliary function leading to diverse clinical manifestations, including chronic sinopulmonary disease, persistent middle ear effusions, laterality defects, and infertility. The estimated frequency of PCD is 1 in 12,000 to…
Cystic fibrosis (CF) is an inherited multisystem disorder of children and adults; it is the most common life-limiting recessive genetic trait among whites. Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, the primary defect, leads to a wide…
Lung infection that destroys the lung parenchyma, resulting in cavitations and central necrosis, can result in localized areas composed of thick-walled purulent material, called lung abscesses. Primary lung abscesses occur in previously healthy patients with no underlying medical disorders and…
Bronchiectasis is characterized by irreversible abnormal dilation and anatomic distortion of the bronchial tree and represents the common end stage of many nonspecific and unrelated antecedent events. Its incidence has been decreasing overall in industrialized countries, but it persists as…
Pleurisy is the inflammation of the pleura; it may be accompanied by an effusion. The most common cause of pleural effusion in children is bacterial pneumonia (see Chapter 428 ); heart failure (see Chapter 469 ), rheumatologic causes, and metastatic…
Hypersensitivity Pneumonia Kevin J. Kelly Michelle L. Hernandez Keywords hypersensitivity pneumonia (HP) extrinsic allergic alveolitis acute HP recurrent subacute HP chronic progressive HP pet birds thermophilic organisms Hypersensitivity pneumonia (HP), aptly called extrinsic allergic alveolitis because the inciting agent is almost uniformly…
Etiology Repeated aspiration of even small quantities of gastric, nasal, or oral contents can lead to recurrent bronchitis or bronchiolitis, recurrent pneumonia, atelectasis, wheezing, cough, apnea, and/or laryngospasm. Pathologic outcomes include granulomatous inflammation, interstitial inflammation, fibrosis, lipoid pneumonia, and bronchiolitis…
Aspiration Syndromes Aspiration of material that is foreign to the lower airway produces a varied clinical spectrum ranging from an asymptomatic condition to acute life-threatening events, such as occur with massive aspiration of gastric contents or hydrocarbon products. Other chapters…
Pulmonary edema is an abnormal fluid collection in the interstitium and air spaces of the lung resulting in oxygen desaturation, decreased lung compliance, and respiratory distress. The condition is common in the acutely ill child. Pathophysiology Although pulmonary edema is…