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Harrison's Principles of Internal Medicine, 18e
| Part 11. Disorders of the Respiratory System > Section 2. Diseases of the Respiratory System > |
Chapter 259. Cystic Fibrosis
Sections: Cystic Fibrosis: Introduction, Pathogenesis, Clinical Features, Diagnosis, Further Readings.
Topics Discussed: cystic fibrosis.
Excerpt:"Cystic fibrosis (CF) is a monogenic disorder that presents as a multisystem disease. The first signs and symptoms typically occur in childhood, but about 5% of patients in the United States are diagnosed as adults. Due to improvements in therapy, >46% of patients are now adults ( 18 years old) and 16.4% are past the age of 30. The median survival is >37.4 years for patients with CF; thus, CF is no longer only a pediatric disease, and internists must be prepared to recognize and treat its many complications. CF is characterized by chronic bacterial infection of the airways that leads to bronchiectasis and bronchiolectasis, exocrine pancreatic insufficiency and intestinal dysfunction, abnormal sweat gland function, and urogenital dysfunction...."
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