TY - CHAP M1 - Book, Section TI - Cystic Fibrosis A1 - Iqbal, Sabah F. A1 - Pillai, Dinesh A1 - Brown, Kathleen M. A1 - Klein, Bruce L. A2 - Tenenbein, Milton A2 - Macias, Charles G. A2 - Sharieff, Ghazala Q. A2 - Yamamoto, Loren G. A2 - Schafermeyer, Robert PY - 2019 T2 - Strange and Schafermeyer's Pediatric Emergency Medicine, 5e AB - Cystic fibrosis (CF) is the most common life-limiting autosomal recessive disease among Caucasians in the United States.Most patients with CF have the classic triad of manifestations: chronic pulmonary disease, malabsorption due to pancreatic insufficiency, and elevated concentrations of sweat chloride and sodium.There is considerable individual variation in the clinical manifestations, severity, and course of the disease.In a patient known to have CF, the most common reason for presenting to the emergency department (ED) is for a pulmonary exacerbation.Gastrointestinal (GI) complications include malabsorption, obstruction, and hematemesis.In this era of universal newborn screening, it is rare for a patient to present with undiagnosed CF. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/04 UR - accessemergencymedicine.mhmedical.com/content.aspx?aid=1155296902 ER -