Wisconsin cystic fibrosis chest radiograph scoring system: validation and standardization for application to longitudinal studies.

This study was designed to achieve a final modeling, validation, and standardization plan for the Wisconsin cystic fibrosis (CF) chest radiographic scoring system. Sixty chest radiographs were selected to reflect a range of severity of lung pathology in children with CF. Seven experienced volunteer raters (three radiologists and four pediatric pulmonologists) from five institutions were recruited to evaluate and score the films. Analysis of scores revealed that the subcomponents of the Wisconsin system showed considerable variation from rater to rater, but reliability assessment indicated satisfactory Cronbach's alpha coefficients (0.83-0.90) among the seven raters. It was found that an additive method of total score computation is significantly more reliable (P < 0.05) than either the original multiplicative model or the traditional Brasfield scoring system. Comparison of radiologists and pulmonologists revealed a marked, systematic difference in scoring with the former group being more conservative in interpretation of abnormalities than the pulmonologists, and some of the raters showing very limited sensitivity. Quantitative chest radiology applied to children with cystic fibrosis studied long-term in longitudinal research projects requires the careful use of sensitive scoring methods and careful selection and training of multiple raters. This is particularly important since pulmonologists and radiologists can differ systematically in interpreting/scoring abnormalities.

Health risk taking and human immunodeficiency virus risk in collegiate female athletes.

Risky health behaviors of female intercollegiate varsity athletes and their nonathlete peers were compared. Five hundred seventy-one female university students (109 athletes and 462 nonathlete peers) at two midwestern universities completed a self-administered, anonymous questionnaire during team meetings or class sessions. Each athlete was matched with two nonathlete controls of similar age, ethnicity, and class year to test for dichotomous outcome variables. A human immunodeficiency virus (HIV) risk scale was developed to identify factors associated with increased HIV risk for all of the female participants. Measures of alcohol and other drug use were associated with HIV risk. The athletes were found to engage in significantly fewer risk-taking behaviors than the nonathletes and to be at less risk for HIV. High levels of risk behaviors generally indicated the need for increased efforts to change risky behaviors in all college women.

Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group.

BACKGROUND: Many patients with cystic fibrosis are malnourished at the time of diagnosis. Whether newborn screening and early treatment may prevent the development of a nutritional deficiency is not known. METHODS: We compared the nutritional status of patients with cystic fibrosis identified by neonatal screening or by standard diagnostic methods. A total of 650,341 newborn infants were screened by measuring immunoreactive trypsinogen on dried blood spots (from April 1985 through June 1991) or by combining the trypsinogen test with DNA analysis (from July 1991 through June 1994). Of 325,171 infants assigned to an early-diagnosis group, cystic fibrosis was diagnosed in 74 infants, including 5 with negative screening tests. Excluding infants with meconium ileus, we evaluated nutritional status for up to 10 years by anthropometric and biochemical methods in 56 of the infants who received an early diagnosis and in 40 of the infants in whom the diagnosis was made by standard methods (the control group). Pancreatic insufficiency was managed with nutritional interventions that included high-calorie diets, pancreatic-enzyme therapy, and fat-soluble vitamin supplements. RESULTS: The diagnosis of cystic fibrosis was confirmed by a positive sweat test at a younger age in the early-diagnosis group than in the control group (mean age, 12 vs. 72 weeks). At the time of diagnosis, the early-diagnosis group had significantly higher height and weight percentiles and a higher head-circumference percentile (52nd, vs. 32nd in the control group; P=0.003). The early-diagnosis group also had significantly higher anthropometric indexes during the follow-up period, especially the children with pancreatic insufficiency and those who were homozygous for the deltaF508 mutation. CONCLUSIONS: Neonatal screening provides the opportunity to prevent malnutrition in infants with cystic fibrosis.

Substance use and other health risk behaviors in collegiate athletes.

OBJECTIVES: To (a) determine the prevalence of alcohol and other drug use, and health risk behaviors among a general university population; and (b) compare health risk-taking behaviors between genders, and varsity athletes and their non-athlete peers. DESIGN: Descriptive survey of multiple health risk behaviors, including physical, mental health, alcohol and other drug, and sexual risk taking. SETTING: Two large midwestern universities. PARTICIPANTS: A convenience sample of 86% of 1,210 eligible students (271 athletes and 775 nonathlete peers) completed a self-administered, anonymous questionnaire during team meetings or class sessions. INTERVENTION: None. MAIN OUTCOME MEASURES: Differences between gender and athlete status were assessed using Cochran-Mantel-Haenszel statistics for the following variables, determined by questionnaire responses: physical risk, mental health, alcohol and other drug use, and sexual behaviors. RESULTS: Common risk behaviors in the entire collegiate sample included riding in a car with a driver who was under the influence of alcohol, driving and swimming under the influence of alcohol, binge drinking, and low rate of condom use for all types of sexual intercourse. Risk-taking behaviors varied by gender, with men showing more risk behaviors than women, except for suicide and sexual behaviors. Male athletes had a higher prevalence of risk behaviors than their male nonathlete counterparts, in contrast to female athletes, who had fewer risk behaviors than their female nonathlete counterparts. CONCLUSION: In contrast to previous studies, results of the present study show, when results are stratified by gender, that not all athletes engage more frequently than nonathletes in high risk behaviors. Results suggest that educational and early intervention strategies to decrease risk may need to be tailored according to gender and athletic status.

Sweat chloride concentrations in infants homozygous or heterozygous for F508 cystic fibrosis.

OBJECTIVE: To determine whether an adequate volume of sweat could be obtained routinely from infants younger than 6 weeks old and to evaluate sweat chloride levels in infants with known genotype statuses, including heterozygote carriers for cystic fibrosis (CF). METHODOLOGY: Infants were evaluated using pilocarpine iontophoresis and measurement of sweat volume and chloride concentration. The majority of these infants were referred because of newborn screening test results positive for CF based on immunoreactive trypsinogen analysis. DNA analyses for the 3-base pair deletion at codon 508 of the CF transmembrane regulator gene (F508 mutation) were performed whenever possible, and patients with CF were categorized by genotype. RESULTS: Sweat tests were performed successfully (>/-50 mg of sweat) in 99.3% of the infants tested, and there was no difference in the proportion of unsuccessful tests in infants younger than or older than 6 weeks of age. The normal mean +/- SD sweat chloride was 10.6 +/- 5.2 mEq/L (95% confidence interval, 9.9-11.3). Patients with CF who are F508 homozygotes or F508 compound heterozygotes or who have two other non-F508 mutant alleles were shown to have similar sweat chloride levels, with mean values of 99.9, 98.8, and 96.6 mEq/L, respectively. The group of infants who were found to be CF (F508) heterozygote carriers, when compared with the healthy group, had mildly but significantly increased sweat chloride concentrations, with a mean +/- SD of 14.9 +/- 8.4 mEq/L (95% confidence interval, 13.4-16.4). CONCLUSIONS: Quantitative pilocarpine iontophoresis can be used successfully in infants younger than 6 weeks of age who are undergoing routine diagnostic evaluations to follow up newborn screening test results that are positive for CF. The upper limit of normal sweat chloride in infants should be revised to 40 mEq/L (mean + 3 SD of the CF heterozygote carrier group). CF heterozygote carrier infants with one F508 mutant allele show phenotypic manifestations of CF, including subclinical elevations of sweat chloride.

Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis.

OBJECTIVE: To assess the tolerance and acceptability of a nutrition supplement in patients with cystic fibrosis (CF), to monitor changes in dietary intake, and to evaluate nutritional status. DESIGN: Subjects were their own controls for this 3-month, prospective, open study. Acceptability and tolerance questionnaires and 3-day food records were completed at baseline and monthly intervals. Compliance and nutritional status were also assessed. SETTING: This study was conducted at the University of Wisconsin Hospital and Clinics Cystic Fibrosis Center, Madison. SUBJECTS: Patients with CF older than 4 years of age were recruited during clinic or hospital visits if they met specific weight or growth criteria (n = 19). INTERVENTION: Subjects were asked to consume the supplement at a maximum of 30% their estimated daily energy requirements. MAIN OUTCOME MEASURES: Responses to acceptability ratings of and tolerance questions about the supplement were obtained along with anthropometric data and biochemical measurements of serum albumin, plasma retinol, alpha-tocopherol, and fatty acid levels. STATISTICAL ANALYSES PERFORMED: Data were analyzed using Minitab and Statistical Analysis Software. Paired and unpaired t tests and nonparametric sign tests were used, as well as regression and Pearson correlations. A significance level of .05 was used for all tests. RESULTS: All subjects tolerated the supplement, although 12 reported mild symptoms of fullness, nausea, and/or bloating, which were resolved when intake was distributed throughout the day. Mean compliance was 69% of recommended intake. Weight gain in children was strongly correlated with compliance (r = .98). Linoleic acid intake increased significantly (P = .0003) as did plasma linoleic acid in the phospholipid fraction (P = .03). CONCLUSION: The supplement studied would be a beneficial addition to the supplementation choices available to patients with CF.