MULTI-ORGAN FAILURE WITH CONNECTIVE TISSUE DYSPLASIA IN A NEWBORN (CLINICAL CASE)
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Abstract
Skeletal dysplasia encompasses various developmental disorders of bone and cartilage that manifest as disproportionate shortening of the limbs and trunk in newborns. Many types of skeletal dysplasia are complicated by respiratory failure at birth or shortly thereafter, requiring intensive care and prolonged hospitalization. Respiratory complications in these infants are characterized by airway abnormalities, 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 the use of pulmonary function tests for early recognition and treatment of complications.
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