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Pediatr Pulmonol. 2008 Jul;43(7):642-7. doi: 10.1002/ppul.20831.

Management of cystic fibrosis related diabetes: a survey of UK cystic fibrosis centers.

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Regional Adult Cystic Fibrosis Unit, The Cardiothoracic Centre NHS Trust, Thomas Drive, Liverpool L143PE, United Kingdom.



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.


A questionnaire survey (regarding screening, diagnosis, treatment and monitoring of CFRD) sent to all 45 recognized CF centers (>50 patients) in the UK.


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.


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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