Introduction: Cystic fibrosis related diabetes (CFRD), a poor prognostic factor in cystic fibrosis (CF), is an increasing problem and guidelines regarding its management have recently been published. However, the evidence base for CFRD screening and diagnosis is not comprehensive and its current management in the UK is unknown. We therefore conducted a questionnaire survey of all recognized UK CF centers to assess clinical practice and determine adherence to these recent recommendations.
Methods: A questionnaire survey (regarding screening, diagnosis, treatment and monitoring of CFRD) sent to all 45 recognized CF centers (>50 patients) in the UK.
Results: Completed questionnaires were returned by 37 centers (82%). Although 35 (95%) centers screened patients annually for CFRD, 12 (34%) used a single investigation whereas the remaining 23 (66%) used two or more methods. As regards diagnosis only 11 (30%) used the recommended combination of oral glucose tolerance test (OGTT) and serial glucose monitoring, with 18 (49%) using OGTT alone. Insulin was the preferred treatment of choice in 36 (97%) centers, and pediatric centers were less likely to use oral hypoglycemic agents (4/17 vs. 9/16; chi(2) = 3.6, P < 0.05). 29 (78%) centers carried out an annual diabetes review and in 34 (92%) patients were cared for jointly between the CF and endocrinology teams. Glycosylated hemoglobin and serial glucose monitoring were the most common investigations used to monitor glycemic control.
Conclusions: This survey highlights the disparities in the management of CFRD with regards to screening and diagnostic practice, and poor adherence to national guidelines.
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http://dx.doi.org/10.1002/ppul.20831 | DOI Listing |
Because of the lengthening of their life-expectancy, more people with cystic fibrosis (CF) now pursue parenthood. To explore the experience of parenting while having CF, 18 French parents with CF were interviewed (including 12 mothers and 9 participants with a lung transplant). A thematic analysis of the interview transcripts was conducted.
View Article and Find Full Text PDFJ Clin Transl Endocrinol
December 2024
Division of Endocrinology Diabetes and Metabolism, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
Cystic fibrosis-related diabetes (CFRD) is the most common non-pulmonary comorbidity in people with cystic fibrosis (CF). Current guidelines recommend insulin therapy as the treatment of choice for people with CFRD. In the past, obesity and overweight were uncommon in individuals with CF.
View Article and Find Full Text PDFRespir Med Case Rep
November 2024
Infectious and Tropical Diseases Unit, Careggi University Hospital, Florence, Italy.
Cystic fibrosis (CF)-related central (CNS) and peripheral nervous system (PNS) disorders have not yet been fully described. We report the first case of post-infective neuromuscular hyperexcitability syndrome in a 23-year-old male patient with CF and pulmonary exacerbation. CNS radiological investigations were unremarkable and no autoantibodies were detected.
View Article and Find Full Text PDFLung
January 2025
Mother and Child Department, Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Purpose: The study evaluated the effects of elexacaftor/tezacaftor/ivacaftor (ETI) therapy in people with cystic fibrosis (pwCF) and a clinical history of Aspergillus fumigatus (AF) infection.
Methods: This prospective cohort study included pwCF who initiated ETI therapy and had received antifungal treatment in the preceding five years due to allergic bronchopulmonary aspergillosis (ABPA group) or other AF-related clinical manifestations (AF group). A control group of pwCF with no prior respiratory cultures positive for AF was also included.
Epidemiology
December 2024
Department of Public Health, Policy and Systems, University of Liverpool, Liverpool, UK.
Background: Children with cystic fibrosis (CF) from socioeconomically deprived areas have poorer growth, worse lung function, and shorter life expectancy than their less-deprived peers. While early growth is associated with lung function around age 6, it is unclear whether improving early growth in the most deprived children reduces inequalities in lung function.
Methods: We used data from the UK CF Registry, tracking children born 2000-2010 up to 2016.
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