Primary Ciliary Dyskinesia
Abstract Primary ciliary dyskinesia (PCD) is one of an expanding collection of disorders collectively known as ciliopathies. A rare, inherited disease of the motile cilia, PCD is clinically characterized by…
Abstract Primary ciliary dyskinesia (PCD) is one of an expanding collection of disorders collectively known as ciliopathies. A rare, inherited disease of the motile cilia, PCD is clinically characterized by…
Abstract Primary immunodeficiency states are not uncommon disorders which can affect the innate or adaptive arms of the immune system. All of these diseases have the potential for adverse pulmonary…
Abstract The presence and significance of aspiration is a common consideration for children seen in “aerodigestive centers.” Aspiration, the passage of food or liquid below the larynx, can be acute…
Abstract This chapter highlights important pulmonary manifestations of the systemic inflammatory diseases of childhood. We focus on diseases that are either common in the pediatric rheumatology clinic or those that…
Abstract While there are areas of overlap, childhood interstitial lung diseases (ILD) frequently include differing entities and histologic patterns than those described in the literature focusing on adult ILD. The…
Abstract Diffuse alveolar hemorrhage most commonly refers to bleeding from the low-pressure pulmonary vascular bed and is a rare cause of chronic interstitial lung disease in children. Patients can present…
Abstract Disorders of the chest wall, spine, and respiratory muscles include an array of conditions which share respiratory function abnormalities consistent with restrictive lung disease. Depending on the cause, they…
Abstract This chapter examines a variety of immunologic-mediated lung diseases. Hypersensitivity pneumonitis is caused by exposure to a variety of antigens that elicit a Th1-mediated hypersensitivity response in the lungs….
Abstract Children with acquired immunodeficiency are at risk for many infectious and noninfectious pulmonary complications. This chapter will discuss such complications in children who have had solid organ transplantation, hematopoietic…
Abstract Over the past several decades, medical and technologic progress in the care of critically ill infants with extreme prematurity (i.e., gestational age < 28 weeks), multisystem congenital anomalies, airway…