View Full Chapter Figures Only Tables Only Videos Only Print Share Email Send Email Your Name (required) Example: John Doe Email Address (required) Please enter a valid sender email address. Example: email@example.com CC Me Recipient Email Address (required) Separate multiple email address with semi-colons (up to 5). Subject Subject for your email. Message (Maximum characters: 1,000) Send Cancel Copyright Paragraph Text Copyright Line Text Get Citation Citation AMA Citation Doherty GM. Doherty G.M. Doherty, Gerard M.Cystic Fibrosis. In: Doherty GM. Doherty G.M. Ed. Gerard M. Doherty.eds. Quick Answers Surgery. New York, NY: McGraw-Hill; 2010. http://accesssurgery.mhmedical.com/content.aspx?bookid=853&Sectionid=49662057. Accessed January 16, 2017. MLA Citation Doherty GM. Doherty G.M. Doherty, Gerard M.. "Cystic Fibrosis." Quick Answers Surgery. Doherty GM. Doherty G.M. Ed. Gerard M. Doherty. New York, NY: McGraw-Hill, 2010, http://accesssurgery.mhmedical.com/content.aspx?bookid=853&Sectionid=49662057. Download citation file: RIS (Zotero) EndNote BibTex Medlars ProCite RefWorks Reference Manager © Copyright Tools Search Book Top Return Clip Search Book Cystic Fibrosis + Essential Features + • Serious congenital disorder, autosomal recessive disorder• Most common mutation is deletion of amino acid in position 508 (Phe)• Defect in chloride transport, results in more NaCl absorption in the airway• Defect occurs in apocrine sweat glands tracheobronchial tree, pancreas, GI tract• Airway secretions are low in volume and high in viscosity• Mucoid plugs form and are rubbery, semisolid, gray to greenish yellow in color resulting in impaction• Often history of recurrent upper respiratory tract infection, fever, and chest pain ++ Epidemiology + • 1 in 25 whites heterozygous carrier• 1 in 2000 homozygous• Single most common mutation characterized + Clinical Findings ++ Symptoms and Signs + • Recurrent respiratory infection• Fever• Chest pain• Meconium ileus in newborn, meconium in terminal ileum causes obstruction ++ Laboratory Findings + • Positive sweat test (NaCl in sweat) + Diagnostic Considerations ++ Rule Out + • Bronchogenic carcinoma• Bronchiectasis• Abscess• Bacterial pneumonia• Lipoid pneumonia• Pulmonary eosinophilic granuloma• Löffler syndrome + Work-up + • Chloride sweat test• Pilocarpin iontophoresis (NaCl concentrations exceeding 60 mEq/L) + Treatment and Management ++ Surgery + • Double lung transplant ++ Indications + • End stage pulmonary disease ++ Complications + • Bronchitis, bronchiectasis• Pulmonary fibrosis• Emphysema• Lung abscess• Complication of transplant: Chronic bronchitis obliterans major obstacle and cause of eventual transplant failure ++ Prognosis + • 1-year survival, 85%• 5-year survival, 50% + Resources ++ References ++Mak GZ et al: T-tube ileostomy for meconium ileus: four decades of experience. J Pediatr Surg 2000;35:349. [PubMed: 10693694] ++Glasscoe CA. Quittner AL. Psychological interventions for people with cystic fibrosis and their families. Cochrane Database of Systematic Reviews. 2008;(3):CD003148. ++Moran F. et al. Non-invasive ventilation for cystic fibrosis. Cochrane Database of Systematic Reviews. 2007;(4):CD002769.