Cystic fibrosis
1978
Ekvall, Shirley
Cystic fibrosis (CF), believed to be an inborn error of metabolism, results in deficiencies of the pancreatic digestive enzymes in 80-85% of CF patients. In addition, the absorption of liposoluble vitamins varies, causing deficiencies of vitamins A, K, and E. The most useful laboratory aid in diagnosing CF is the determination of chloride content of sweat. Data indicate that 98% of CF children show sweat chloride levels in excess of 50 mEq/liter, a level rarely reached by normal children. Dietary treatment by administration of pancreatic enzyme tablets or powders just before a meal can lessen the digestive disturbance which occurs in CF patients, improving absorption of fat and protein. Treatment follow-up examinations are necessary; height, weight, and skinfold measurments should be taken during each visit. Tables on signs, symptoms, and clinical evaluation for diagnosis of CF patients are also discussed. (wz)
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