Neonatal screening provides the opportunity to prevent malnutrition in infants with cystic fibrosis.

No abstract

We conclude that CF neonatal screening does not have a significant impact on the reproductive behavior of most families and that prenatal diagnosis is not used by the majority of CF families. IRT/DNA testing experiences seem to affect attitudes about having more children, and some parents are confused about the implications of the results, even with genetic counseling. However, persistent concerns about the sweat test result are limited. Questions raised by this study confirm the need for more research regarding the process of genetic counseling and its impact on reproductive attitudes and behavior in the newborn screening setting.

ABSTRACT. Objective. This study was pursued as an extension of a randomized clinical investigation of neonatal screening for cystic fibrosis (CF). The project included assessment of respiratory secretion cultures for pathogens associated with CF. The objective was to determine whether patients diagnosed through neonatal screening and treated in early infancy were more likely to become colonized with Pseudomonas aeruginosa compared with those identified by standard diagnostic methods.Methodology. The design involved prospective cultures of respiratory secretions obtained generally by oropharyngeal swabs at least every 6 months and more often if clinically indicated. Patients were managed with a standardized evaluation and treatment protocol at the two Wisconsin certified CF centers; however, there were community and environmental variations associated with the follow-up period as described below.Results. Overall, there were no differences in acquisition of respiratory pathogens between the screened and the control (standard diagnosis) groups. Evaluation of the data between and within the two centers, however, revealed significant differences with earlier acquisition of P aeruginosa in the center with the following distinguishing characteristics: urban location; following patients with the standard US approach in which newly diagnosed, young children were interspersed with older CF patients; and where there were more opportunities for social interactions with other CF patients. The differences were confined to the screened group followed in the urban center in which the median pseudomonas-free survival period was 52 weeks contrasted with 289 weeks in the other center. In addition, assessment of data for the entire CF populations followed at the two centers revealed that the urban center showed a significantly higher prevalence of P aeruginosa colonization in patients between the ages of 3 and 9 years.Conclusions. These results present questions and generate hypotheses on risk factors for acquisition of P aeruginosa in CF and suggest that clinic exposures and/or social interactions may predispose such patients to pseudomonas infections. Pediatrics 1997;100(5). URL: http://www.pediatrics.org/cgi/content/full/100/5/e2; cystic fibrosis, Pseudomonas aeruoginosa, transmission, epidemiology, pulmonary disease.

Rhinovirus (RV) infections are important triggers of acute asthma symptoms in susceptible persons. To determine whether the presence of allergy is a risk factor for enhanced lower airway effects during RV infection, we experimentally infected (RV16) 18 volunteers with allergic rhinitis and 13 normal control subjects and measured the effects on the response of the lower airways to histamine. All subjects were successfully infected, as indicated by increased upper respiratory symptoms and RV16 cultured from nasal secretions. The change in histamine PD20(deltaPD20) caused by RV infection was significantly different in allergic subjects from that in nonallergic control subjects (deltaPD20 = -0.40 versus -0.03 log units, p = 0.04). This relationship was strengthened after adjusting for initial PD20 and FEV1 (mean deltaPD20 = -0.43 versus 0.01 log units, p < 0.01). The virus-induced deltaPD20 was also influenced by baseline lung function: there was a positive correlation between initial FEV1 and deltaPD20, and a weak but significant negative correlation between baseline PD20 and deltaPD20. These findings indicate that host factors such as allergy, baseline FEV1, and baseline PD20 influence the changes in lower airway physiology caused by RV infection and raise the possibility that these factors contribute to the increased lower airway effects of RV infection in subjects with asthma.

The objective of this study was to identify risk factors of significance for acquisition of Pseudomonas aeruginosa by children with cystic fibrosis (CF). Our working hypothesis is that exposure of infants and young children with CF to older, infected patients increases their risk for acquiring this organism. A special opportunity arose to study this question in detail, as we have been performing a randomized clinical trial of neonatal screening for CF throughout the state of Wisconsin during the period of 1985–1994. Patients were selected for this study based on either early identification through screening or diagnosis by standard methods. A longitudinal protocol employed at Wisconsin's two CF Centers includes routine cultures of respiratory secretions and collection of clinical, demographic, and activity information on patients and their families. Previous observations in our trial revealed that one center at an old hospital in an urban location showed a significantly shorter time to acquisition of P. aeruginosafor CF patients followed there. To study the center effect further, we performed statistical analyses using survival curves and stepwise regression analysis of all life history covariates available. The results of these analyses showed that the statistically significant correlations involve the following risk factors: (1) center and old hospital (r = 0.42); (2) center and original physician (r = 0.61); (3) center and exposure to pseudomonas‐positive patients (r = 0.29); and (4) population density and urban location (r = 0.49). The final statistical model demonstrated that increased risk due to aerosol use (odds ratio = 3.45, P = 0.014) and a protective effect associated with education of the mother (odds ratio = 0.81, P = 0.024) were the most significant factors for acquisition of P. aeruginosa. The previously observed center effect was confined to the 1985–1990 interval at the old hospital (odds ratio = 4.43, P < 0.001). We conclude that multiple factors are involved in increasing the risk of young children with CF to acquire P. aeruginosa, and that the observed center effect can best be explained by a combination of factors. These results suggest that facilities and methods used to care for young children with CF can significantly influence their likelihood of acquiring pseudomonas in the respiratory tract. Pediatr Pulmonol. 1998; 26:81–88. © 1998 Wiley‐Liss, Inc.

The authors investigate the postonset hospitalization rate and risk factors during 1987-1994 in Wisconsin, in a population-based, incidence cohort followed from diagnosis of Type 1 diabetes mellitus at ages 0-29 (n = 577). The overall rate was 8.9 +/- 0.60 (standard error) per 100 person-years of diabetes, whereof 5.7 was due to hyperglycemia, 1.9 to hypoglycemia, and 1.3 to other and undetermined causes. Major risk factors for hospitalization were longitudinally measured glycosylated hemoglobin level (rate ratio = 1.5 per 2% increase, 95% confidence interval 1.4-1.7), black/other race (rate ratio = 1.9, 95% confidence interval 1.0-3.6), diagnosis in a non-university-based setting (rate ratio = 1.9, 95% confidence interval 1.2-3.2), female sex (rate ratio = 1.5, 95% confidence interval 1.0-2.4 at age 11), age in males (rate ratio = 0.6, 95% confidence interval 0.4-0.8 per 5-year increase), and public or no insurance up to 18 months postdiagnosis (rate ratio = 2.2, 95% confidence interval 1.1-4.4). For individuals less than 18 years, "black/other race" was replaced in the model by "having other than two biologic parents in the home" (rate ratio = 2.0, 95% confidence interval 1.1-3.5). Hence, hospitalization is common in children, adolescents, and young adults with diabetes, primarily for problems with glycemic control.

Inhaled helium-oxygen improves the overall respiratory status of children with acute RSV lower respiratory tract infection. In patients with mild-to-moderate bronchiolitis (Clinical Asthma Scores of <6), the beneficial effects of helium-oxygen were most pronounced in children with the greatest degree of respiratory compromise.