THIS PAPER is a discussion of present concepts in diagnosis of cystic fibrosis of the pancreas. Such concepts should develop from an examination of the definition of this disease. Cystic fibrosis is now known as a disease which appears as focal involvement of various exocrine glands in the body, with its principal symptomatic manifestations in two main anatomic areas. These two areas are the gastrointestinal tract where symptoms are exhibited as more frequent, foul-smelling and bulky stools, and the respiratory tract where involvement is shown principally as cough, wheezing or respiratory distress.
PARTICULAR CLINICAL FEATURES
My purpose here is not to discuss the well-recognized picture, but rather to emphasize some of the clinical characteristics. The clinical picture has widened in its range because of improved diagnostic tests such as determination of electrolytes in sweat and greater awareness by physicians. As a result, there are now known to be patients who show more or less gastrointestinal or respiratory involvement. On the one hand, there is an enlarging group of patients who have principally respiratory involvement and very little gastrointestinal disorder. These patients, of course, are frequently confused with patients with other chronic pulmonary disease or asthma. On the other hand, there is now a small group of patients with gastrointestinal symptoms and very little respiratory disease. These patients often are confused with those having chronic diarrhea or celiac disease.
There are a few points which might be emphasized as clinically diagnostic characteristicis. Cystic fibrosis is often associated with the diarrheal group of diseases, but it is not truly a diarrheal disease in the sense of more fluid bowel movements with acute exacerbations. The stools are bulky, foul-smelling and unformed movements, usually passed three to four times a day. A very large appetite has been constantly emphasized, but among a large enough group of such children one is impressed that this is not a constant symptom.
- Copyright © 1959 by the American Academy of Pediatrics