Differences in expression of cystic fibrosis in blacks and whites

Article Abstract:

Cystic fibrosis (CF) is an inherited disorder that causes the exocrine glands to produce abnormally thick secretions, particularly in the pancreas, lungs and sweat glands, and results in overactivity of the nervous system. This disease occurs mostly in whites and is usually identified early in infancy or childhood. The gene for cystic fibrosis has been located on chromosome 7 and is believed to encode the CF conductance regular protein. One mutation of the CF conductance regular protein is responsible for 70 percent of the cases of CF in whites, but this defect occurs in only 37 percent of blacks having CF. Genetic study of American blacks with CF has shown that there are a number of genetic mutations, most of which are unique to this population. A study was undertaken to look for possible differences in expression of CF between 24 black and 48 white CF patients. Blacks more often had only respiratory symptoms (38 percent versus 10 percent of whites). Blacks had fewer hospitalizations due to breathing problems (2 versus 6.9), better lung function as evaluated by forced vital capacity, and higher chest X-ray scores (18.2 versus 14.4) than white patients. Rates of complications were similar except blacks had a higher incidence of peptic ulcer disease (13 percent vs 0 percent) and dehydration due to hyponatremia (low sodium; 21 vs 2 percent). Although not statistically significant, there was a trend toward longer survival among black patients. It is concluded that differences exist between black and white patients with CF that may be due to genetic differences between these two populations. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Demographic aspects, African Americans, Genetic aspects

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Cystic Fibrosis Foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers

Article Abstract:

The purpose of these guidelines is to promote a uniform level of care and teaching at cystic fibrosis (CF) centers, and to assure high-quality patient care. Cystic fibrosis is a hereditary disease causing secretion of thick mucus, most often in the pancreas, lungs and sweat glands. Diagnosis should be timely and accurate, based on clinical findings and laboratory results. Two sweat tests performed on different days are required to confirm the diagnosis. Newly diagnosed patients should be assessed for physical and emotional function, and education of the family and patient should begin. Families need help to cope with the emotional impact of the diagnosis. Ongoing patient monitoring and evaluation is essential to anticipate and treat the physical and social complications of CF. To attain optimum breathing status, a complete respiratory history and evaluation must be obtained. The nutritional complications of CF need to be anticipated and treated to maximize nutrition and growth. As patients mature, their treatment needs must be reevaluated; adult patients have different needs from pediatric patients. Adequate treatment facilities are important; an appropriate number of properly trained professionals need to be available at the cystic fibrosis centers. Educational activities of the CF center are of great help, and activities should be projected out into the community. (Consumer Summary produced by Reliance Medical Information, Inc.)

Standards, Diagnosis, Cystic Fibrosis Foundation

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Effect of pancreatic enzyme supplements on iron absorption

Article Abstract:

One-third to two-thirds of patients with cystic fibrosis have iron deficiency. It has been reported that patients with cystic fibrosis who had been receiving pancreatic enzyme supplements for at least one year showed impaired ability to absorb iron. In this study, 13 young adults with cystic fibrosis and 9 controls of similar ages were compared concerning their ability to absorb an oral iron supplement. A preparation of pancreatic enzymes was given to both the cystic fibrosis and control groups. There was significant impairment of the ability to absorb iron in both groups after the administration of the pancreatic enzymes; there had been no difference in iron absorption before the pancreatic enzymes were given. The mechanism by which pancreatic enzymes impair iron absorption is not known, but it appears that long-term use of pancreatic enzymes may contribute to iron deficiency. It is concluded that the iron status of cystic fibrosis patients should be monitored carefully. If supplemental iron is given, it should not be given around the time that pancreatic enzyme supplementation is given. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Physiological aspects, Iron deficiency anemia, Digestive enzymes

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