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Title: PAS MEETING: Early Diagnosis Results In Greater Growth In CF Children
URL: http://www.pslgroup.com/dg/FB73E.htm
Doctor's Guide
May 4, 1999


MADISON, WI -- May 4, 1999 -- Early diagnosis of cystic fibrosis (CF) through neonatal screening and aggressive nutritional treatment produces significantly better growth in CF children, according to findings of a study led by University of Wisconsin Medical School dean Dr. Philip Farrell.

Farrell presented the results today at the Pediatric Academic Societies' annual meeting in San Francisco.

CF is the most common life-threatening recessive genetic disease among Caucasian children, occurring in about one in 3,500 births. The disease produces pancreatic insufficiency leading to malnutrition; chronic respiratory problems; and salt loss that can lead to fatal dehydration. According to the National CF Foundation Registry, 44 percent of patients newly diagnosed with CF in 1993 showed malnutrition serious enough to cause either wasting of body mass or stunted growth.

The state of Wisconsin in 1985 began a research program using a randomised clinical trial design in which all newborns in the state are potentially assessed for CF. The purpose of the program is to gauge the benefits, risks and costs of screening. The study, conducted by the Wisconsin CF Neonatal Screening Group and supported by both the National Institutes of Health and the CF Foundation, was designed to measure whether screening and early nutritional intervention offered benefits to patients. All patients diagnosed in the project were placed on high-calorie diets along with enzyme and vitamin supplements, if they had proven pancreatic digestive deficiency. Those identified by screening, however, received early treatment before severe malnutrition occurred.

A previous report based on a smaller sample of patients (and published in the New England Journal of Medicine) found that youngsters identified through screening and aggressively treated were both taller and heavier than those identified by their symptoms. The current findings are based on the entire group of more than 650,000 newborns screened from April 1985 through June 1994.

The research team found impressive differences between the screened group and the control group. Specifically, those in the screened group were taller, heavier and had larger head circumference at diagnosis and over 11 years of assessment compared to the control group.

Farrell said the findings suggest that severe malnutrition persists after deferred diagnosis of CF and that catching up may not be possible.

Other research is underway to determine if early malnutrition has had any detrimental effect on intellectual ability or quality of life. Farrell also said the occurrence and severity of CF lung disease is being studied in two populations of patients. Other researchers in the Wisconsin CF Neonatal Screening Group are investigating costs associated with screening, standard diagnosis methods, and treatment.

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