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Glucocorticoids are administered to patients with cystic fibrosis for acute indications, such as bronchiolitis, 1 bronchial hyperreactivity, 2 and allergic bronchopulmonary aspergillosis, 3 as well as for long-term treatment of mild-to-moderate obstructive pulmonary disease, to reduce inflammation and improve lung function. 4 – 9 In two randomized clinical trials, patients with cystic fibrosis and mild-to-moderate lung disease who were treated with 1 or 2 mg of prednisone per kilogram of body weight on an alternate-day schedule over a period of two to four years had significantly better pulmonary function than such patients who were given placebo. 5 , 6 The benefits of long-term treatment with glucocorticoids in . . .

Cystic fibrosis, one of the most common life-threatening autosomal recessive disorders, can be difficult to diagnose, and its recognition is therefore often delayed. In 1995, the mean age at the time of diagnosis was 2.9 years in the United States. 1 At the time of diagnosis, many patients are malnourished or have chronic lung disease, 2 , 3 and some have hypoproteinemia with edema (acute kwashiorkor), vitamin E deficiency with hemolytic anemia, or severe hyponatremia, hypochloremia, and dehydration. 4 – 6 In fact, a recent analysis of data from the National Cystic Fibrosis Foundation Registry showed that 44 percent of the patients who received a . . .

Using multivariate analysis of covariance to test hypotheses about the effects of sports and sexual behavior on a sample of 611 Western New York adolescents, this study concludes that athletic participation and gender interact to influence adolescent sexual outcomes. Female athletes report significantly lower rates of sexual activity than female nonathletes; male athletes report slightly (though not significantly) higher rates than male nonathletes. The gender-specific effect of sports on sexual behavior remains, net of the impacts of race, age, socioeconomic status, quality of family relations, and participation in other extracurricular activities. This paper introduces cultural resource theory to explain how athletic participation influences both traditional cultural scripts and exchange resources, which, in turn, condition the sexual bargaining process and its outcomes for adolescents.

Alcohol use increases throughout adolescence. Based on family socialization theory, it was hypothesized that family factors, particularly parental support and monitoring, would influence individual trajectories in the development of alcohol misuse. Six waves of data were analyzed, based on interviews with 506 adolescents in the general population of a northeastern metropolitan area. Using growth-curve longitudinal analysis, results show that parenting significantly predicts adolescents' initial drinking levels (intercepts) as well as their rates of increase in alcohol misuse (slope). This study provides evidence that effective parenting is an important factor in preventing alcohol misuse.

In this paper we examine the degree to which family cohesion buffers the effects of fathers' problem drinking at Time 1 (T1) on adolescent distress, deviance, and heavy drinking at Time 2 (T2), one year later. Data from a representative sample of 658 families were used to test the hypotheses. Mothers, fathers (if present), and adolescent children were interviewed in the home. Fathers who were present completed self-report scales measuring problem drinking. When fathers were not available, mothers' reports on fathers' drinking were used to measure fathers' problem drinking. Results from regression analysis indicate that after controlling for the effects of race, SES, age, gender, and family structure: (1) the more cohesion in the family and the fewer stressful events, the less distress, deviance, and heavy drinking shown by adolescents; (2) the father's problem drinking affects adolescent distress and deviance when cohesion is low; but as cohesion increases, the effects of the father's drinking are reduced. The findings support the hypothesis that cohesion in families buffers the effects of fathers' problem drinking on adolescents.

Little research has examined the relationship between work-family conflict and alcohol or cigarette use among women. Building on affect regulation theory and recent research on work-family conflict and negative emotions, this study tested a model relating work-family conflict to heavy alcohol use and cigarette use via domain-specific (i.e., job- and family-related) and general negative affect. Data were obtained through household interviews with a random sample of 366 employed mothers of adolescents. As hypothesized, work-family conflict was indirectly related to both heavy alcohol use and cigarette use via negative affect.

Yet, just as in the diagnosis of CF per se, traditional methods of PA identification (relying on microbiology) leave much to be desired in young children with CF. [...]more attention has focused once again on the potential diagnostic value of Pseudomonas serology. [...]60 of 88 patients (75%) with chronic PA infection expectorated sputum for culture. [...]the very group of young CF patients of greatest interest (and importance) for these studies is the most difficult to evaluate reliably as the \"gold standard\" (PA positive culture) is potentially unreliable.

In this article we first review theoretical controversies and methodological issues centering around tests of Olson's circumplex model, Beaver's system theory, and social support theory. We then derive hypotheses about the effects of cohesion and adaptability on family members' psychological functioning, behavior, and perceptions of family relationships. The dependent variables are depression, anxiety, identity diffusion, individuation, self-esteem, deviance, school misconduct, grades, marital agreement, and parent-child communication. Using random-digit-dial screening, we obtained a sample of 699 families in a large northeastern city. Instead of a curvilinear effect, as predicted by both Olson and Beavers, we find that cohesion has a direct linear relationship to positive outcomes, a finding consistent with social support theory. Consistent with Beavers' theory, we find that the more adaptability, the better the outcomes for girls, but not for other family members. The results are discussed in light of recent questions about the validity of the FACES III scale.

Abstract Although early diagnosis of cystic fibrosis (CF) can lead to nutritional benefits, there has been uncertainty about pulmonary outcomes. Using a randomized controlled trial with unique unblinding/surveillance, we evaluated patients with CF who received similar treatment after being assigned to an early diagnosis (screened) group or to a standard diagnosis (control) group. When the youngest patient was 7 years of age, we compared outcomes using pulmonary function data and quantitative chest radiology. In the screened group (56 patients), diagnosis was made at a younger age of 12.4 weeks, compared with the diagnosis in control group (47 control patients) at the age of 95.8 weeks, but included a significantly greater proportion of patients with ΔF508 genotypes and pancreatic insufficiency. The first chest radiograph showed significantly fewer abnormalities in the screened group; but, over time, the two groups converged, and after 10 years of age the screened patients showed worse chest X-ray scores associated with earlier acquisition of Pseudomonas aeruginosa. No differences were detected in any measure of pulmonary dysfunction, which was generally mild in each group. Although CF neonatal screening provides a potential opportunity for better pulmonary outcomes, it appears that respiratory infections and pancreatic status are the dominant factors in pulmonary prognosis.

Genetic testing of an Irish kindred identified an exonic nucleotide substitution c.1664T>C (p.Leu555Pro) in the MLH1 mismatch repair (MMR) gene. This previously unreported variant is classified as a “variant of uncertain significance” (VUS). Immunohistochemical (IHC) analysis and microsatellite instability (MSI) studies, genetic testing, a literature and online MMR mutation database review, in silico phenotype prediction tools, and an in vitro MMR activity assay were used to study the clinical significance of this variant. The MLH1 c.1664T>C (p.Leu555Pro) VUS co-segregated with three cases of classic Lynch syndrome-associated malignancies over two generations, with consistent loss of MLH1 and PMS2 protein expression on IHC, and evidence of the MSI-High mutator phenotype. The leucine at position 555 is well conserved across a number of species, and this novel variant has not been reported as a normal polymorphism in the general population. In silico and in vitro analyses suggest that this variant may have a deleterious effect on the MLH1 protein and abrogate MMR activity. Evidence from clinical, histological, immunohistochemical, and molecular genetic data suggests that MLH1 c.1664T>C (p.Leu555Pro) is likely to be the pathogenic cause of Lynch syndrome in this family.