Clinical outcomes in U.S. infants with cystic fibrosis from 2001 to 2012

  title={Clinical outcomes in U.S. infants with cystic fibrosis from 2001 to 2012},
  author={Heather E Hoch and Marci K. Sontag and Sharon L Scarbro and Elizabeth Juarez-Colunga and Cindie McLean and Allison Kempe and Scott D. Sagel},
  journal={Pediatric Pulmonology},
  pages={1492 - 1497}
All 50 United States implemented newborn screening (NBS) for cystic fibrosis (CF) by 2010. The purpose of this study was to evaluate trends over the decade when NBS became universal to determine current rates of malnutrition, stunting, and infection rates in U.S. infants with CF. 
Outcomes of infants born during the first 9 years of CF newborn screening in the United States: A retrospective Cystic Fibrosis Foundation Patient Registry cohort study
The aims were to describe the outcomes of infants with CF born during the first 9 years of universal NBS, to form the basis of additional analyses and to plan quality improvement initiatives.
Lung function determinants and mortality of children and adolescents with cystic fibrosis in South Africa 2007‐2016
This study aimed to describe the change in lung function, nutritional status, and mortality of children with CF at a single center in Cape Town, South Africa, and identify factors associated with poor lung function and mortality.
Cystic fibrosis year in review 2018, part 1
This report summarizes research and cases related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies, inflammation and infection, epidemiology and the physiologic, and imaging assessment of disease.
Cystic fibrosis knowledge and practice among primary care physicians in southwest region, Saudi Arabia
  • A. Asseri
  • Medicine
    Journal of family medicine and primary care
  • 2020
The need for extensive educational programs for the PCPs in order to improve early recognition of CF and start the appropriate management is highlighted.
The Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis.
The decrease in prevalence of P. aeruginosa and S. aureus since 2000, coinciding with more aggressive therapeutic approach, has resulted in Aspergillus becoming the most commonly isolated pathogen in young children.
Shifting Landscape of Airway Infection in Early Cystic Fibrosis
  • J. Hoppe, S. Sagel
  • Medicine
    American journal of respiratory and critical care medicine
  • 2019
The epidemiology of lower airway infection in a modern cohort of almost 400 infants and young children diagnosed by newborn screening, followed at two Australian CF centers is reexamine, with Aspergillus as the most commonly detected pathogen from the lower airways among young children with CF.
Cystic fibrosis diagnosed by state newborn screening: Or is it?
The case of an infant born in Illinois with a positive cystic fibrosis newborn screening with an elevated immunoreactive trypsinogen and two genetic mutations identified (F508del/F508Del) highlights the importance of performing the confirmatory sweat test prior to making a diagnosis of cystic Fibrosis.
Prevention of chronic infection with Pseudomonas aeruginosa infection in cystic fibrosis.
An update on definitions of chronicity of infection, approaches to airway sampling to detect infection, strategies for Pseudomonas aeruginosa eradication, impact of cystic fibrosis transmembrane regualtor protein (CFTR) modulators and future challenges for clinical trials is provided.
Cystic fibrosis related diabetes: Nutrition and growth considerations.
The CFTR modulator therapy shows early promise for the improvement of growth and nutritional parameters in CF and CF Foundation Nutritional Guidelines are recommended for the nutritional management of CFRD.
Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition.
a Department of Women's and Children's Health, University of Liverpool, Liverpool, UK b Division of Paediatric Pulmonology, Children's Hospital, St Gallen, Switzerland c Paediatric Pneumonology and


Changing incidence of cystic fibrosis in Wisconsin, USA
Previous investigations of cystic fibrosis (CF) incidence in Massachusetts, Colorado, and Minnesota (USA) yielded contradictory results, particularly regarding allele p.Phe508del; the racial
Geographic variations in cystic fibrosis: An analysis of the U.S. CF Foundation Registry
This work sought to determine if regional differences exist in the distribution of demographic variables and patient disease characteristics in the U.S. that may play a role in differential CF outcomes.
Improved pulmonary and growth outcomes in cystic fibrosis by newborn screening
The goal was to determine if newborn screening results in improved longitudinal growth and maintenance of normal pulmonary function in cystic fibrosis patients.
Invasive pneumonia and septic shock in infants as a presentation of cystic fibrosis with vitamin‐deficiency
An aggressive treatment regimen including nutritional supplementation was initiated which resulted in improvement in their pulmonary status and no further recurrences.
Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis
The objective of this study was to assess the diagnostic accuracy of oropharyngeal (OP) cultures relative to simultaneous bronchoalveolar lavage (BAL) cultures in very young children with CF, and to
Improved survival in cystic fibrosis patients diagnosed by newborn screening compared to a historical cohort from the same centre
NBS for CF leads to better lung function, nutritional status and improved survival in screened patients in early adulthood.
Effects of Diagnosis by Newborn Screening for Cystic Fibrosis on Weight and Length in the First Year of Life
Male sex, pancreatic insufficiency, meconium ileus, histamine blocker use, and respiratory Pseudomonas aeruginosa infection were associated with lower weight or length during the first year, significantly lower among low-length infants.
Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis.
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 and whether clinic exposures and/or social interactions may predispose such patients to pseudomonas infections is investigated.
Baseline Characteristics and Factors Associated With Nutritional and Pulmonary Status at Enrollment in the Cystic Fibrosis EPIC Observational Cohort
The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young
Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.
These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF.