Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the Cystic Fibrosis Registry.

The international literature shows that the demography of cystic fibrosis (CF) is changing, with patients increasingly surviving into adulthood. As they age, patients with CF become more susceptible to specific non-pulmonary chronic diseases. In this study, adult data from the CF Registry of Ireland (CFRI) was used to determine the prevalence and associated features of these diseases. 104 (25.7%) adults had diabetes versus 13 (2.9%) children (p < 0.001). Liver disease was present in 47 (11.6%) adults and 26 (5.7%) children (p = 0.002). 173 (42.7%) adults had bone disease versus 25 (5.5%) children (p < 0.001). Adults with one non-pulmonary chronic disease, for example liver disease, were more likely to have another (p = 0.002), those with diabetes and bone disease had a higher number of hospital admissions in the last 12 months (p < 0.001 for both) and higher rates of depression (p = 0.046 and p = 0.049, respectively). These results highlight a number of challenges for the Irish healthcare system.

[1]  Steven R. Martin,et al.  Epidemiology of liver disease in cystic fibrosis: a longitudinal study. , 2004, Journal of hepatology.

[2]  Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome , 2002, Hepatology.

[3]  C. Combescure,et al.  The Prevalence of Osteoporosis, Osteopenia, and Fractures Among Adults with Cystic Fibrosis: A Systematic Literature Review with Meta-Analysis , 2009, Calcified Tissue International.

[4]  J. Kouwenberg,et al.  Demographics of glucose metabolism in cystic fibrosis. , 2009, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[5]  H. Quinton,et al.  Validation and use of a parametric model for projecting cystic fibrosis survivorship beyond observed data: a birth cohort analysis , 2011, Thorax.

[6]  David Rodman,et al.  Cystic fibrosis adult care: consensus conference report. , 2004, Chest.

[7]  R. Lorini,et al.  Liver disease as risk factor for cystic fibrosis‐related diabetes development , 2007, Acta paediatrica.

[8]  P. Farrell,et al.  The prevalence of cystic fibrosis in the European Union. , 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[9]  Parinya Chamnan,et al.  Diabetes as a Determinant of Mortality in Cystic Fibrosis , 2009, Diabetes Care.

[10]  S. Freedman,et al.  Cystic fibrosis , 2009, The Lancet.

[11]  J. Stockman Epidemiology of Cystic Fibrosis-Related Diabetes , 2007 .

[12]  M. Rosenberg,et al.  Diagnosis of cystic fibrosis in the Republic of Ireland: epidemiology and costs. , 2007, Irish medical journal.

[13]  P. Merkel,et al.  Consensus statement: Guide to bone health and disease in cystic fibrosis , 2005 .

[14]  P. Merkel,et al.  Guide to bone health and disease in cystic fibrosis. , 2005, The Journal of clinical endocrinology and metabolism.

[15]  M. Wilschanski,et al.  Patterns of GI disease in adulthood associated with mutations in the CFTR gene , 2007, Gut.

[16]  C. Rand,et al.  The association between depression, lung function, and health-related quality of life among adults with cystic fibrosis. , 2007, Chest.

[17]  J. Jacquot,et al.  Bone disease in cystic fibrosis: what's new? , 2011, Joint, bone, spine : revue du rhumatisme.

[18]  P. Bye,et al.  Adults with cystic fibrosis: meeting the challenge! , 2003, Internal medicine journal.

[19]  F. Edenborough,et al.  Cystic fibrosis‐related diabetes , 2003, Diabetic medicine : a journal of the British Diabetic Association.

[20]  M. S. Kirkman,et al.  Epidemiology, Pathophysiology, and Prognostic Implications of Cystic Fibrosis–Related Diabetes , 2010, Diabetes Care.

[21]  A. Morabito,et al.  Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome , 2002, Hepatology.

[22]  D. Geddes,et al.  Clinical importance of cystic fibrosis-related diabetes. , 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.