Clinical Manifestations

CF manifests itself at birth in about 8 percent of cases through mechanical obstruction of the small intestine by the secretion of abnormally viscous mucus (meconium ileus). Symptoms of insufficient secretion of exocrine (noninsulin) digestive enzymes by the pancreas appear during the first year of life in 90 percent of cases. The development of such symptoms indicates that pancreatic function is less than 10 percent of normal; and the more severe the deficiency of pancreatic enzymes, the more severe the fecal excretion of undigested fat, usually as diarrhea. As much as 80 percent of dietary fat may be lost, thus partially explaining malnutrition. Loss of undigested nutrients can be corrected only partially by treatment with pancreatic enzyme tablets. Pulmonary disease is responsible for most of the debility and mortality. Onset occurs in the first 2 years of life in at least 75 percent of cases, and by the age of 6 years in most of the remaining cases. The initial pulmonary abnormality is obstruction of the small bronchi by abnormally thick mucus. Structural deterioration of the lungs results in part from this and is exacerbated by an increased susceptibility to infections. A small number of patients retain sufficient pancreatic function to maintain nearly normal digestion; such patients also tend to have fewer respiratory difficulties. The variability in severity is ex plained by the occurrence of several different mutations on the pathogenetic gene.

The sweat of a child with CF contains a concentration of sodium and chloride that is about five times greater than normal, although salt is not lost excessively by other routes. Determining the salt content of perspiration has become a basic diagnostic test. The propensity to become salt-depleted makes persons with CF particularly intolerant to heat. As a result of pulmonary and metabolic therapy, many CF patients are now living into reproductive age, and thus it has been found that CF men, but not CF women, are sterile. In spite of all efforts, few patients survive to age 40.

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