By George L. Ph.D., D.Sc. Clark
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If exocrine pancreatic deficiency is the sole clinical symptom in the presence of a normal sweat test, the symptoms may be due to pancreatitis or distal abdominal obstruction syndrome. In the two cases presented here, case 1 is an example of a classic CF presentation in which there is an elevated sweat test and family history supported by the identification of known gene mutations. Case 2 is more representative of an intermediate phenotype with a later onset. 3,4 Pathogenesis Cystic fibrosis manifests as an exocrine pancreatic disease that leads to abnormal functioning of the lungs, GI, and reproductive tracts.
Am. Rev. Respir. Dis. 121:217–23, 1980. FLETCHER , C. : The natural history of chronic airflow obstruction. Br. Med. J. 1:1645–8, 1977. THOMPSON , A. , ROBBINS , R. A. : Intraluminal airway inflammation in chronic bronchitis: Characterization and correlation with clinical parameters. Am. Rev. Respir. Dis. 140:1526–37, 1989. NATHAN , S. : Lung transplantation: disease-specific considerations for referral. Chest 127:1006–16, 2005. MILLER , BERGER , R. , MALTHANER , R. A. : Lung volume reduction surgery vs.
To explore possible causes for the pulmonary involvement, allergy testing was performed. Skin testing was positive for Aspergillosis and Alternaria, two environmental fungi. Previous laboratory data from an outside facility revealed an elevated sweat chloride of 95 mmol/L (0 –40 mmol/L), and a chest x-ray showed infiltration and mild bronchiectasis of the right upper lobe in addition to hyperinflation of both upper lobes with possible air trapping. His sweat chloride on the day of presentation was 99 mmol/L and sodium of 90 mmol/L.
Applied X-rays by George L. Ph.D., D.Sc. Clark