Acquisition ofPseudomonas aeruginosain Children With Cystic Fibrosis

Abstract

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.

Outcomes of Infants With Indeterminate Diagnosis Detected by Cystic Fibrosis Newborn Screening

“…Cystic fibrosis (CF) is an autosomalrecessive condition caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. 1 Early diagnosis of CF results in improved nutritional and cognitive outcomes, 2,3 and as of 2010 CF newborn screening (NBS) has been implemented in every state in the United States. 4 CF is one of the most common causes for a positive NBS test.…”

Pubertal Height Growth and Adult Height in Cystic Fibrosis After Newborn Screening

“…Anthropometric measurements were obtained according to a standardized protocol and performed by trained research nurses and dietitians. 7,23 Height and weight data at 2 to 18 years were used for the analyses. Age-and gender-specific z score/ percentile values for height and BMI were computed by using the 2000 Centers for Disease Control and Prevention (CDC) growth charts.…”

“…Pancreatic functional status -pancreatic sufficiency (PS) or pancreatic insuffiency (PI), was monitored as described 23 during the first 4 years of life, and the final determination occurred at age 4 years.…”

Newborn Screening for Cystic Fibrosis: Evaluation of Benefits and Risks and Recommendations for State Newborn Screening Programs—

“…Failure to thrive is particularly common in infants and young children. On the basis of data from the Wisconsin randomized controlled trial of newborn screening for CF during 1985-1994, the mean height-for-age Z-score at age 1 year among children without screening results is approximately -1.0, or 1 standard deviation (SD) below the reference mean (28). With the introduction of nutritional therapies (e.g., pancreatic enzyme supplements and a high-fat diet) after a diagnosis of CF with pancreatic insufficiency, the majority of children experience partial catch-up growth, and the mean height-for-age Z-score settles around -0.4 by age 2 years.…”

“…The Wisconsin CF Neonatal Screening Project randomly assigned neonates born in Wisconsin during 1985-1994 to either a screened or control group (28,76) (Table 3). Neonatal CF screening was performed on blood spots from all subjects, but positive results were reported only to families in the screened group.…”