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Cystic Fibrosis-Related Diabetes

Clare Lee, M.D., MHS, Sherita Golden, M.D., M.H.S.
Cystic Fibrosis-Related Diabetes is a topic covered in the Johns Hopkins Diabetes Guide.

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  • Cystic fibrosis (CF): an autosomal recessive disease, diagnosed if : (1) clinical symptoms consistent with CF in at least one organ system AND (2) evidence of CF transmembrane conductance regulator (CFTR) dysfunction, which can include elevated sweat chloride >60 mmol/L on 2 occasions, presence of one of two disease-causing mutations in the CFTR (most common mutation is delta F508), or abnormal nasal potential difference.
  • Clinical manifestations: respiratory tract disease, sinus disease, exocrine pancreatic insufficiency, diabetes, meconium ileus and distal ileal obstruction, biliary disease, infertility, musculoskeletal disorders (reduced bone mineral content, hypertrophic osteoarthropathy), nephrolithiasis and nephrocalcinosis, and recurrent venous thrombosis.
  • Cystic fibrosis-related diabetes (CFRD): distinct from types 1 or 2 diabetes, with evidence of beta cell dysfunction but conflicting evidence regarding insulin resistance. Because basal insulin secretion is retained, patients with CFRD are not ketosis prone.

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Last updated: June 5, 2015


Lee, Clare, and Sherita Golden. "Cystic Fibrosis-Related Diabetes." Johns Hopkins Diabetes Guide, 2015. Johns Hopkins Guide, www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Diabetes_Guide/547033/all/Cystic_Fibrosis_Related_Diabetes.
Lee C, Golden S. Cystic Fibrosis-Related Diabetes. Johns Hopkins Diabetes Guide. 2015. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Diabetes_Guide/547033/all/Cystic_Fibrosis_Related_Diabetes. Accessed July 23, 2019.
Lee, C., & Golden, S. (2015). Cystic Fibrosis-Related Diabetes. In Johns Hopkins Diabetes Guide. Available from https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Diabetes_Guide/547033/all/Cystic_Fibrosis_Related_Diabetes
Lee C, Golden S. Cystic Fibrosis-Related Diabetes [Internet]. In: Johns Hopkins Diabetes Guide. ; 2015. [cited 2019 July 23]. Available from: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Diabetes_Guide/547033/all/Cystic_Fibrosis_Related_Diabetes.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cystic Fibrosis-Related Diabetes ID - 547033 A1 - Lee,Clare,M.D., MHS AU - Golden,Sherita,M.D., M.H.S. Y1 - 2015/06/05/ BT - Johns Hopkins Diabetes Guide UR - https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Diabetes_Guide/547033/all/Cystic_Fibrosis_Related_Diabetes DB - Johns Hopkins Guide DP - Unbound Medicine ER -