Deepthi Alapati1, Thomas H Shaffer2. 1. Department of Pediatrics, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Center for Pediatric Lung Research, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, United States. Electronic address: Deepthi.alapati@nemours.org. 2. Center for Pediatric Lung Research, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, United States; Temple University School of Medicine, Philadelphia, PA, United States.
Abstract
BACKGROUND: Skeletal dysplasia encompasses a variety of developmental disorders of the bone and cartilage that manifest as disproportionate shortening of limbs and trunk in the neonate. Many types of skeletal dysplasia are complicated by respiratory failure at or soon after birth and require intensive care and prolonged hospitalization. Respiratory complications in these infants are complex and are characterized by airway anomalies, restrictive lung disease due to a narrow and abnormally compliant chest wall, pulmonary hypoplasia, and central apnea. Appropriate management of these unique patients requires a clear understanding of the pathophysiology and use of pulmonary function tests for early recognition and management of complications. CONCLUSION: This review provides an overview of the underlying respiratory pathology and a practical guide to the newborn care provider for the diagnosis and management of respiratory complications in infants with skeletal dysplasia.
BACKGROUND:Skeletal dysplasia encompasses a variety of developmental disorders of the bone and cartilage that manifest as disproportionate shortening of limbs and trunk in the neonate. Many types of skeletal dysplasia are complicated by respiratory failure at or soon after birth and require intensive care and prolonged hospitalization. Respiratory complications in these infants are complex and are characterized by airway anomalies, restrictive lung disease due to a narrow and abnormally compliant chest wall, pulmonary hypoplasia, and central apnea. Appropriate management of these unique patients requires a clear understanding of the pathophysiology and use of pulmonary function tests for early recognition and management of complications. CONCLUSION: This review provides an overview of the underlying respiratory pathology and a practical guide to the newborn care provider for the diagnosis and management of respiratory complications in infants with skeletal dysplasia.
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