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WS23.5 Airway infections with Pseudomonas aeruginosa and MRSA linked with increased odds of CF related diabetes
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Oral Presentations / Journal of Cystic Fibrosis 15 (2016) S1–S50
WS23.5 Airway infections with Pseudomonas aeruginosa and MRSA linked with increased odds of CF related diabetes A.M. Granchelli1 , T. Block1 , C. Kartsonaki2 , R.H. Keogh3 , D.R. Cox4 , F.R. Adler5,6 , T.G. Liou1 . 1 University of Utah, Internal Medicine, Salt Lake City, United States; 2 University of Oxford, Nuffield Department of Population Health, Oxford, United Kingdom; 3 London School of Hygiene and Tropical Medicine, Medical Statistics, London, United Kingdom; 4 Nuffield College, Statistics, Oxford, United Kingdom; 5 University of Utah, Math, Salt Lake City, United States; 6 University of Utah, Biology, Salt Lake City, United States Objective: Cystic Fibrosis related diabetes (CFRD) is a risk factor for mortality, with pathogenesis possibly related to susceptibility of CFTR deficient pancreatic b-cells to oxidative stress. Because airway infections may increase oxidative stress in CF patients, we evaluated their association with CFRD. Methods: We analyzed 23,622 patients with two or more consecutive years in the CF Foundation Patient Registry during 2003–2011. We excluded lung transplant patients and those with pre-existing diabetes. We identified infection as at least one culture positive during year t and predicted diagnoses of diabetes in year t+1. We performed c2 tests of association between infection and acquisition of diabetes, and used both univariate and multivariate generalized linear models (logistic regression) to identify clinical factors and infections associated with increased odds of developing diabetes. We corrected for lung function, nutrition status, pancreatic sufficiency, acute exacerbation history, and gender. We corrected for calendar year accounting for change in infection prevalence over time. Results: The odds of developing CFRD increased in patients with Pseudomonas aeruginosa (OR 1.25 [99% CI 1.13–1.37]) and MRSA (1.20 [1.09–1.32]). Infections with methicillin sensitive Staphylococcus aureus, Alcaligenes xylosoxidans, Stenotrophomonas maltophilia, Candida, or Aspergillus are neither positively or negatively associated with subsequent CFRD. Conclusions: Pseudomonas aeruginosa and MRSA airway infections in CF may contribute to the development of CFRD. This understanding emphasizes the need in patients with these infections for CFRD screening to prevent its complications. WS23.6 Patient perspectives on electronic access to registry health records: an Irish–Slovene online survey J. Rodman1 , U. Krivec1 , A. Jackson2 . 1 Department of Pulmonology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia; 2 The Cystic Fibrosis Registry of Ireland, Dublin, Ireland Background and Objectives: The potential use of a patient registry as a tool to support patient self-management has not yet been explored within the field of cystic fibrosis (CF). The aim of this survey was to evaluate the interest of CF patients in accessing their personal data which are available in the European CF Registry and giving them the opportunity to be involved in the development of potential future electronic applications that meet their preferences in supporting CF self-management. Methods: A secure, anonymised online survey which focused on the access of CF patients to medical health records was distributed to CF patients in Ireland (IE) and Slovenia (SI). Results: A total of 188 CF patients or parents/guardians of CF patients (147 from IE and 41 from SI) provided quantitative and qualitative data. 99.3% IE and 97% SI patients have Internet access at home, using it mostly daily. Mobile phones are mostly used in IE (81.5%) while PC or laptops in SI (94%). A high percentage of respondents (86.3% IE, 88% SI) would like to be able to refer to a summary of relevant health information after a hospital visit. 97.9% IE and 88% SI patients would be interested in logging into an online website or mobile application to see CF health records. They particularly stressed the importance of the security of data access. Conclusion: The results of our survey provide evidence that CF patients expect to gain benefit from self-management applications that provide secure electronic access to a summary of their own registry health records.
ePS01. Screening and Diagnosis ePS01.1 A 7-year review of CF newborn screening results from a UK regional laboratory K.D. Patterson1,2 , P. Goddard3 , M. Desai4 , W. Carroll2 , M.A. Preece3 , F.J. Gilchrist1,2 . 1 Keele University, Institute of Science and Technology for Medicine, Keele, United Kingdom; 2 Royal Stoke University Hospital, Academic Department of Child Health, Stoke on Trent, United Kingdom; 3 Birmingham Children’s Hospital, Department of Newborn Screening, Birmingham, United Kingdom; 4 Birmingham Children’s Hospital, Paediatric Respiratory Medicine, Birmingham, United Kingdom Objectives: Newborn screening (NBS) for cystic fibrosis (CF) was implemented across the whole of the United Kingdom by July 2007. The West Midlands Newborn Screening Laboratory is one of 16 in the UK and serves two tertiary paediatric CF centres. We report the CF NBS results from this laboratory. Methods: The CF NBS results from the West Midlands Newborn Screening Laboratory were reviewed from 1st November 2007 to 31st October 2014. Results: In total 507,608 babies were screened. 200 were referred to one of the tertiary CF centres as “CF Suspected”. 165 were subsequently confirmed as CF, giving a birth prevalence of 1 in 3076 live births and a positive predictive value of 82.5%. Of the 165 confirmed cases, 83 (50.3%) were homozygous and 63 (38.1%) were heterozygous for Phe508del. 101 carriers were identified. To date, 9 patients who had a negative CF NBS within this time period have been diagnosed with CF, one of which had meconium ileus. Conclusion: The birth prevalence of CF in the West Midlands is lower than the expected figure for the UK, which is 1 in 2381. This difference is likely to be due to two factors; the ethnic diversity of the West Midlands population and children with a negative newborn screen who are yet to be diagnosed with CF. In keeping with the aims of the UK CF NBS program the rates of carrier detection and false negatives were low. We now plan to review the case notes of all patients with a positive CF NBS to gain anthropological and microbiological data for the first 2 years of life. ePS01.2 Newborn screening for cystic fibrosis in Switzerland – Evaluation after 5 years M. Jurca1 , C.E. Kuehni1 , C.S. Rueegg2 , R. Fingerhut3 , S. Gallati4 , T. Torresani3 , M. Baumgartner3 , J. Barben5 , Swiss Cystic Fibrosis Screening Group. 1 University of Bern, Institute of Social and Preventive Medicine, Paediatric Epidemiology, Bern, Switzerland; 2 University of Lucerne, Department of Health Sciences and Health Policy, Lucerne, Switzerland; 3 University Children’s Hospital of Zurich, Swiss Newborn Screening Laboratory, Zurich, Switzerland; 4 University Hospital of Bern, Division of Human Genetics, Bern, Switzerland; 5 Children’s Hospital of Eastern Switzerland, Division of Pediatric Pulmonology, St. Gallen, Switzerland Objectives: Newborn screening (NBS) for cystic fibrosis (CF), based on immunoreactive trypsinogen (IRT)-DNA-IRT algorithm, was introduced in Switzerland in 2011. The program aims to detect all children with classic CF, but to avoid detecting children with “CF screen positive, inconclusive diagnosis” (CFSPID). Here we evaluate the test characteristics for NBS-CF in Switzerland over the past 5 years. Methods: We analysed data from the national CF screening database, including all children screened between January 2011 and November 2015. Children with positive screening results were referred to a CFcentre for further examination, including sweat test. We measured the number of referred children and of confirmed diagnoses. We also calculated specificity, sensitivity, positive and negative predictive values (PPV, NPV) of the screening procedure. Results: Out of 420,731 births within 5 years, 443 children were screened positive and referred to a CF-centre. 120 (27%) were diagnosed with CF, 18 (4%) had CFSPID, 302 (68%) children were CF negative, and 3
Oral Presentations / Journal of Cystic Fibrosis 15 (2016) S1–S50
WS23.5 Airway infections with Pseudomonas aeruginosa and MRSA linked with increased odds of CF related diabetes A.M. Granchelli1 , T. Block1 , C. Kartsonaki2 , R.H. Keogh3 , D.R. Cox4 , F.R. Adler5,6 , T.G. Liou1 . 1 University of Utah, Internal Medicine, Salt Lake City, United States; 2 University of Oxford, Nuffield Department of Population Health, Oxford, United Kingdom; 3 London School of Hygiene and Tropical Medicine, Medical Statistics, London, United Kingdom; 4 Nuffield College, Statistics, Oxford, United Kingdom; 5 University of Utah, Math, Salt Lake City, United States; 6 University of Utah, Biology, Salt Lake City, United States Objective: Cystic Fibrosis related diabetes (CFRD) is a risk factor for mortality, with pathogenesis possibly related to susceptibility of CFTR deficient pancreatic b-cells to oxidative stress. Because airway infections may increase oxidative stress in CF patients, we evaluated their association with CFRD. Methods: We analyzed 23,622 patients with two or more consecutive years in the CF Foundation Patient Registry during 2003–2011. We excluded lung transplant patients and those with pre-existing diabetes. We identified infection as at least one culture positive during year t and predicted diagnoses of diabetes in year t+1. We performed c2 tests of association between infection and acquisition of diabetes, and used both univariate and multivariate generalized linear models (logistic regression) to identify clinical factors and infections associated with increased odds of developing diabetes. We corrected for lung function, nutrition status, pancreatic sufficiency, acute exacerbation history, and gender. We corrected for calendar year accounting for change in infection prevalence over time. Results: The odds of developing CFRD increased in patients with Pseudomonas aeruginosa (OR 1.25 [99% CI 1.13–1.37]) and MRSA (1.20 [1.09–1.32]). Infections with methicillin sensitive Staphylococcus aureus, Alcaligenes xylosoxidans, Stenotrophomonas maltophilia, Candida, or Aspergillus are neither positively or negatively associated with subsequent CFRD. Conclusions: Pseudomonas aeruginosa and MRSA airway infections in CF may contribute to the development of CFRD. This understanding emphasizes the need in patients with these infections for CFRD screening to prevent its complications. WS23.6 Patient perspectives on electronic access to registry health records: an Irish–Slovene online survey J. Rodman1 , U. Krivec1 , A. Jackson2 . 1 Department of Pulmonology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia; 2 The Cystic Fibrosis Registry of Ireland, Dublin, Ireland Background and Objectives: The potential use of a patient registry as a tool to support patient self-management has not yet been explored within the field of cystic fibrosis (CF). The aim of this survey was to evaluate the interest of CF patients in accessing their personal data which are available in the European CF Registry and giving them the opportunity to be involved in the development of potential future electronic applications that meet their preferences in supporting CF self-management. Methods: A secure, anonymised online survey which focused on the access of CF patients to medical health records was distributed to CF patients in Ireland (IE) and Slovenia (SI). Results: A total of 188 CF patients or parents/guardians of CF patients (147 from IE and 41 from SI) provided quantitative and qualitative data. 99.3% IE and 97% SI patients have Internet access at home, using it mostly daily. Mobile phones are mostly used in IE (81.5%) while PC or laptops in SI (94%). A high percentage of respondents (86.3% IE, 88% SI) would like to be able to refer to a summary of relevant health information after a hospital visit. 97.9% IE and 88% SI patients would be interested in logging into an online website or mobile application to see CF health records. They particularly stressed the importance of the security of data access. Conclusion: The results of our survey provide evidence that CF patients expect to gain benefit from self-management applications that provide secure electronic access to a summary of their own registry health records.
ePS01. Screening and Diagnosis ePS01.1 A 7-year review of CF newborn screening results from a UK regional laboratory K.D. Patterson1,2 , P. Goddard3 , M. Desai4 , W. Carroll2 , M.A. Preece3 , F.J. Gilchrist1,2 . 1 Keele University, Institute of Science and Technology for Medicine, Keele, United Kingdom; 2 Royal Stoke University Hospital, Academic Department of Child Health, Stoke on Trent, United Kingdom; 3 Birmingham Children’s Hospital, Department of Newborn Screening, Birmingham, United Kingdom; 4 Birmingham Children’s Hospital, Paediatric Respiratory Medicine, Birmingham, United Kingdom Objectives: Newborn screening (NBS) for cystic fibrosis (CF) was implemented across the whole of the United Kingdom by July 2007. The West Midlands Newborn Screening Laboratory is one of 16 in the UK and serves two tertiary paediatric CF centres. We report the CF NBS results from this laboratory. Methods: The CF NBS results from the West Midlands Newborn Screening Laboratory were reviewed from 1st November 2007 to 31st October 2014. Results: In total 507,608 babies were screened. 200 were referred to one of the tertiary CF centres as “CF Suspected”. 165 were subsequently confirmed as CF, giving a birth prevalence of 1 in 3076 live births and a positive predictive value of 82.5%. Of the 165 confirmed cases, 83 (50.3%) were homozygous and 63 (38.1%) were heterozygous for Phe508del. 101 carriers were identified. To date, 9 patients who had a negative CF NBS within this time period have been diagnosed with CF, one of which had meconium ileus. Conclusion: The birth prevalence of CF in the West Midlands is lower than the expected figure for the UK, which is 1 in 2381. This difference is likely to be due to two factors; the ethnic diversity of the West Midlands population and children with a negative newborn screen who are yet to be diagnosed with CF. In keeping with the aims of the UK CF NBS program the rates of carrier detection and false negatives were low. We now plan to review the case notes of all patients with a positive CF NBS to gain anthropological and microbiological data for the first 2 years of life. ePS01.2 Newborn screening for cystic fibrosis in Switzerland – Evaluation after 5 years M. Jurca1 , C.E. Kuehni1 , C.S. Rueegg2 , R. Fingerhut3 , S. Gallati4 , T. Torresani3 , M. Baumgartner3 , J. Barben5 , Swiss Cystic Fibrosis Screening Group. 1 University of Bern, Institute of Social and Preventive Medicine, Paediatric Epidemiology, Bern, Switzerland; 2 University of Lucerne, Department of Health Sciences and Health Policy, Lucerne, Switzerland; 3 University Children’s Hospital of Zurich, Swiss Newborn Screening Laboratory, Zurich, Switzerland; 4 University Hospital of Bern, Division of Human Genetics, Bern, Switzerland; 5 Children’s Hospital of Eastern Switzerland, Division of Pediatric Pulmonology, St. Gallen, Switzerland Objectives: Newborn screening (NBS) for cystic fibrosis (CF), based on immunoreactive trypsinogen (IRT)-DNA-IRT algorithm, was introduced in Switzerland in 2011. The program aims to detect all children with classic CF, but to avoid detecting children with “CF screen positive, inconclusive diagnosis” (CFSPID). Here we evaluate the test characteristics for NBS-CF in Switzerland over the past 5 years. Methods: We analysed data from the national CF screening database, including all children screened between January 2011 and November 2015. Children with positive screening results were referred to a CFcentre for further examination, including sweat test. We measured the number of referred children and of confirmed diagnoses. We also calculated specificity, sensitivity, positive and negative predictive values (PPV, NPV) of the screening procedure. Results: Out of 420,731 births within 5 years, 443 children were screened positive and referred to a CF-centre. 120 (27%) were diagnosed with CF, 18 (4%) had CFSPID, 302 (68%) children were CF negative, and 3