Unformatted text preview: most common lethal inherited disorders among Caucasians (1 in 3,200) • One in 31 Americans (one in 28 Caucasians) is an unknowing, symptom-less heterozygous carrier of the defective gene • Prognosis: In 2005, the median survival age was 36.8 years, and slowly increasing. • Decreased mucociliary clearance of sputum leads to chronic endobronchial bacterial colonization, and… o Production of large amounts of sputum o Wheezing o Dyspnea o Limited exercise tolerance o Death • Pancreatic insufficiency in 85% of patients, leading to malabsorption of fat and malnutrition. • Also diabetes mellitus, bowel obstructions, arthritis, and infertility....
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- Spring '11
- sputum, extracellular connective tissue