Skip to main content

Advertisement

SpringerLink
Log in

Cystic Fibrosis Related Diabetes

  • Diabetes and Other Diseases-Emerging Associations (JJ Nolan, Section Editor)
  • Published:
Current Diabetes Reports Aims and scope Submit manuscript

Abstract

Improved life expectancy in cystic fibrosis (CF) has led to an expanding population of adults with CF, now representing almost 50 % of the total CF population. This creates new challenges from long-term complications such as diabetes mellitus (DM), a condition that is present in 40 %–50 % of adults with CF. Cystic fibrosis-related diabetes (CFRD) results from a primary defect of insulin deficiency and although sharing features with type 1 (DM1) and type 2 diabetes (DM2), it is a clinically distinct condition. Progression to diabetes is associated with poorer CF clinical outcomes and increased mortality. CFRD is not associated with an increased risk of cardiovascular disease and the prevalence of microvascular complications is lower than DM1 or DM2. Rather, the primary goal of insulin therapy is the preservation of lung function and optimization of nutritional status. There is increasing evidence that appropriate screening and early intervention with insulin can reverse weight loss and improve pulmonary function. This approach may include targeting postprandial hyperglycemia not detected by standard diagnostic tests such as the oral glucose tolerance test. Further clinical research is required to guide when and how much to intervene in patients who are already dealing with the burden of one chronic illness.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1 Suppl):1S–39.

    Article  PubMed  Google Scholar 

  2. McCormick J, Mehta G, Olesen HV, Viviani L, Macek M, Mehta A, et al. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010;375:1007–13.

    Article  PubMed  Google Scholar 

  3. Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32:1626–31. This paper reported data from a US CF center where early diagnosis and aggressive treatment of CFRD was associated with a significant narrowing of the gap in mortality between those CF patients with and without diabetes.

    Article  PubMed Central  PubMed  Google Scholar 

  4. Yung B, Noormohamed FH, Kemp M, Hooper J, Lant AF, Hodson ME. Cystic fibrosis-related diabetes: the role of peripheral insulin resistance and beta-cell dysfunction. Diabet Med. 2002;19:221–6.

    Article  CAS  PubMed  Google Scholar 

  5. Costa M, Potvin S, Hammana I, Malet A, Berthiaume Y, Jeanneret A, et al. Increased glucose excursion in cystic fibrosis and its association with a worse clinical status. J Cyst Fibros. 2007;6:376–83.

    Article  CAS  PubMed  Google Scholar 

  6. Marshall BC, Butler SM, Stoddard M, Moran AM, Liou TG, Morgan WJ. Epidemiology of cystic fibrosis-related diabetes. J Pediatr. 2005;146:681–7.

    Article  CAS  PubMed  Google Scholar 

  7. Lanng S, Hansen A, Thorsteinsson B, Nerup J, Koch C. Glucose tolerance in patients with cystic fibrosis: five year prospective study. BMJ. 1995;311:655–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Scheuing N, Holl RW, Dockter G, Fink K, Junge S, Naehrlich L, et al. Diabetes in cystic fibrosis: multicenter screening results based on current guidelines. PLoS ONE. 2013;8:e81545.

    Article  PubMed Central  PubMed  Google Scholar 

  9. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000;162(3 Pt 1):891–5.

    Article  CAS  PubMed  Google Scholar 

  10. O'Riordan SM, Robinson PD, Donaghue KC, Moran A. Management of cystic fibrosis-related diabetes. Pediatr Diabetes. 2008;9(4 Pt 1):338–44.

    Article  PubMed  Google Scholar 

  11. Finkelstein SM, Wielinski CL, Elliott GR, Warwick WJ, Barbosa J, Wu SC, et al. Diabetes mellitus associated with cystic fibrosis. J Pediatr. 1988;112:373–7.

    Article  CAS  PubMed  Google Scholar 

  12. Koch C, Rainisio M, Madessani U, Harms HK, Hodson ME, Mastella G, et al. Presence of cystic fibrosis-related diabetes mellitus is tightly linked to poor lung function in patients with cystic fibrosis: data from the European Epidemiologic Registry of Cystic Fibrosis. Pediatr Pulmonol. 2001;32:343–50.

    Article  CAS  PubMed  Google Scholar 

  13. Milla CE, Billings J, Moran A. Diabetes is associated with dramatically decreased survival in female but not male subjects with cystic fibrosis. Diabetes Care. 2005;28:2141–4.

    Article  PubMed  Google Scholar 

  14. Bremer AA. The use of insulin in cystic fibrosis-related diabetes: A philosophical difference from the use of insulin in Type 1 and Type 2 diabetes. Pediatr Pulmonol. 2012;47:1039–41.

    Article  PubMed  Google Scholar 

  15. Kerem E, Corey M, Kerem BS, Rommens J, Markiewicz D, Levison H, et al. The relation between genotype and phenotype in cystic fibrosis–analysis of the most common mutation (delta F508). N Engl J Med. 1990;323:1517–22.

    Article  CAS  PubMed  Google Scholar 

  16. Hartling SG, Garne S, Binder C, Heilmann C, Petersen W, Petersen KE, et al. Proinsulin, insulin, and C-peptide in cystic fibrosis after an oral glucose tolerance test. Diabetes Res. 1988;7:165–9.

    CAS  PubMed  Google Scholar 

  17. Moran A, Becker D, Casella SJ, Gottlieb PA, Kirkman MS, Marshall BC, et al. Epidemiology, pathophysiology, and prognostic implications of cystic fibrosis-related diabetes: a technical review. Diabetes Care. 2010;33:2677–83. A comprehensive review published at the same time as the CFRD consensus conference clinical care guidelines.

    Article  PubMed Central  PubMed  Google Scholar 

  18. Hardin DS, LeBlanc A, Lukenbough S, Seilheimer DK. Insulin resistance is associated with decreased clinical status in cystic fibrosis. J Pediatr. 1997;130:948–56.

    Article  CAS  PubMed  Google Scholar 

  19. Austin A, Kalhan SC, Orenstein D, Nixon P, Arslanian S. Roles of insulin resistance and beta-cell dysfunction in the pathogenesis of glucose intolerance in cystic fibrosis. J Clin Endocrinol Metab. 1994;79:80–5.

    CAS  PubMed  Google Scholar 

  20. Ahmad T, Nelson R, Taylor R. Insulin sensitivity and metabolic clearance rate of insulin in cystic fibrosis. Metabolism. 1994;43:163–7.

    Article  CAS  PubMed  Google Scholar 

  21. Moran A, Pyzdrowski KL, Weinreb J, Kahn BB, Smith SA, Adams KS, et al. Insulin sensitivity in cystic fibrosis. Diabetes. 1994;43:1020–6.

    Article  CAS  PubMed  Google Scholar 

  22. Blackman SM, Hsu S, Vanscoy LL, Collaco JM, Ritter SE, Naughton K, et al. Genetic modifiers play a substantial role in diabetes complicating cystic fibrosis. J Clin Endocrinol Metab. 2009;94:1302–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  23. Blackman SM, Hsu S, Ritter SE, Naughton KM, Wright FA, Drumm ML, et al. A susceptibility gene for type 2 diabetes confers substantial risk for diabetes complicating cystic fibrosis. Diabetologia. 2009;52:1858–65.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  24. Couce M, O'Brien TD, Moran A, Roche PC, Butler PC. Diabetes mellitus in cystic fibrosis is characterized by islet amyloidosis. J Clin Endocrinol Metab. 1996;81:1267–72.

    CAS  PubMed  Google Scholar 

  25. Minicucci L, Cotellessa M, Pittaluga L, Minuto N, d’Annunzio G, Avanzini MA, et al. Beta-cell autoantibodies and diabetes mellitus family history in cystic fibrosis. J Pediatr Endocrinol Metab. 2005;18:755–60.

    Article  CAS  PubMed  Google Scholar 

  26. Gottlieb PA, Yu L, Babu S, Wenzlau J, Bellin M, Frohnert BI, et al. No relation between cystic fibrosis-related diabetes and type 1 diabetes autoimmunity. Diabetes Care. 2012;35:e57.

    Article  PubMed Central  PubMed  Google Scholar 

  27. Sobrin L. Longitudinal validation of hemoglobin A(1c) criteria for diabetes diagnosis: risk of retinopathy. Diabetes. 2012;61:3074–5.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  28. Colagiuri S, Lee CM, Wong TY, Balkau B, Shaw JE, Borch-Johnsen K. Glycemic thresholds for diabetes-specific retinopathy: implications for diagnostic criteria for diabetes. Diabetes Care. 2011;34:145–50.

    Article  PubMed Central  PubMed  Google Scholar 

  29. Association AD. Diagnosis and classification of diabetes mellitus. Diabetes Care. 2011;33 Suppl 1:S62–9.

    Article  Google Scholar 

  30. Chance WW, Rhee C, Yilmaz C, Dane DM, Pruneda ML, Raskin P, et al. Diminished alveolar microvascular reserves in type 2 diabetes reflect systemic microangiopathy. Diabetes Care. 2008;31:1596–601.

    Article  PubMed Central  PubMed  Google Scholar 

  31. Kuziemski K, Pienkowska J, Slominski W, Specjalski K, Dziadziuszko K, Jassem E, et al. Role of quantitative chest perfusion computed tomography in detecting diabetic pulmonary microangiopathy. Diabetes Res Clin Pract. 2011;91:80–6.

    Article  CAS  PubMed  Google Scholar 

  32. Goldman MD. Lung dysfunction in diabetes. Diabetes Care. 2003;26:1915–8.

    Article  PubMed  Google Scholar 

  33. Pitocco D, Fuso L, Conte EG, Zaccardi F, Condoluci C, Scavone G, et al. The diabetic lung—a new target organ? Rev Diabet Stud. 2012;9:23–35.

    Article  PubMed Central  PubMed  Google Scholar 

  34. Klein OL, Krishnan JA, Glick S, Smith LJ. Systematic review of the association between lung function and Type 2 diabetes mellitus. Diabetes Med. 2010;27:977–87.

    Article  CAS  Google Scholar 

  35. Wood DM, Brennan AL, Philips BJ, Baker EH. Effect of hyperglycemia on glucose concentration of human nasal secretions. Clin Sci (Lond). 2004;106:527–33.

    Article  CAS  Google Scholar 

  36. Brennan AL, Gyi KM, Wood DM, Johnson J, Holliman R, Baines DL, et al. Airway glucose concentrations and effect on growth of respiratory pathogens in cystic fibrosis. J Cyst Fibros. 2007;6:101–9.

    Article  CAS  PubMed  Google Scholar 

  37. Philips BJ, Meguer JX, Redman J, Baker EH. Factors determining the appearance of glucose in upper and lower respiratory tract secretions. Intensive Care Med. 2003;29:2204–10.

    Article  PubMed  Google Scholar 

  38. Baker EH, Clark N, Brennan AL, Fisher DA, Gyi KM, Hodson ME, et al. Hyperglycemia and cystic fibrosis alter respiratory fluid glucose concentrations estimated by breath condensate analysis. J Appl Physiol. 2007;102:1969–75.

    Article  CAS  PubMed  Google Scholar 

  39. Philips BJ, Redman J, Brennan A, Wood D, Holliman R, Baines D, et al. Glucose in bronchial aspirates increases the risk of respiratory MRSA in intubated patients. Thorax. 2005;60:761–4.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  40. Garnett JP, Gray MA, Tarran R, Brodlie M, Ward C, Baker EH, et al. Elevated paracellular glucose flux across cystic fibrosis airway epithelial monolayers is an important factor for Pseudomonas aeruginosa growth. PLoS ONE. 2013;8:e76283.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  41. Hunt WR, Zughaier SM, Guentert DE, Shenep MA, Koval M, McCarty NA, et al. Hyperglycemia impedes lung bacterial clearance in a murine model of cystic fibrosis-related diabetes. Am J Physiol Lung Cell Mol Physiol. 2014;306:L43–9.

    Article  CAS  PubMed  Google Scholar 

  42. Esposito K, Nappo F, Marfella R, Giugliano G, Giugliano F, Ciotola M, et al. Inflammatory cytokine concentrations are acutely increased by hyperglycemia in humans: role of oxidative stress. Circulation. 2002;106:2067–72.

    Article  CAS  PubMed  Google Scholar 

  43. Waugh N, Royle P, Craigie I, Ho V, Pandit L, Ewings P, et al. Screening for cystic fibrosis-related diabetes: a systematic review. Health Technol Assess. 2012;16:iii–iv:1–179. A comprehensive and informative review of currently recommended and potentially more appropriate screening methods for CFRD.

    Google Scholar 

  44. Sterescu AE, Rhodes B, Jackson R, Dupuis A, Hanna A, Wilson DC, et al. Natural history of glucose intolerance in patients with cystic fibrosis: ten-year prospective observation program. J Pediatr. 2010;156:613–7.

    Article  PubMed  Google Scholar 

  45. Moran A, Hardin D, Rodman D, Allen HF, Beall RJ, Borowitz D, et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. Diabetes Res Clin Pract. 1999;45:61–73.

    Article  CAS  PubMed  Google Scholar 

  46. Moran A, Pekow P, Grover P, Zorn M, Slovis B, Pilewski J, et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009;32:1783–8. This study confirmed that insulin therapy improved CF-specific outcomes in CFRD without fasting hyperglycemia.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  47. Rolon MA, Benali K, Munck A, Navarro J, Clement A, Tubiana-Rufi N, et al. Cystic fibrosis-related diabetes mellitus: clinical impact of prediabetes and effects of insulin therapy. Acta Paediatr. 2001;90:860–7.

    Article  CAS  PubMed  Google Scholar 

  48. Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33:2697–708. The most up-to-date recommendations for clinical practice in CFRD.

    Article  PubMed Central  PubMed  Google Scholar 

  49. Holl RW, Buck C, Babka C, Wolf A, Thon A. HbA1c is not recommended as a screening test for diabetes in cystic fibrosis. Diabetes Care. 2000;23:126.

    Article  CAS  PubMed  Google Scholar 

  50. Lee KM, Miller RJ, Rosenberg FM, Kreisman SH. Evaluation of glucose tolerance in cystic fibrosis: comparison of 50-g and 75-g tests. J Cyst Fibros. 2007;6:274–6.

    Article  CAS  PubMed  Google Scholar 

  51. Mueller-Brandes C, Holl RW, Nastoll M, Ballmann M. New criteria for impaired fasting glucose and screening for diabetes in cystic fibrosis. Eur Respir J. 2005;25:715–7.

    Article  CAS  PubMed  Google Scholar 

  52. Godbout A, Hammana I, Potvin S, Mainville D, Rakel A, Berthiaume Y, et al. No relationship between mean plasma glucose and glycated hemoglobin in patients with cystic fibrosis-related diabetes. Diabetes Metab. 2008;34(6 Pt 1):568–73.

    Article  CAS  PubMed  Google Scholar 

  53. Wagener JS, McNeill GC, Taussig LM, Corrigan JJ, Lemen R. Ferrokinetic and hematologic studies in cystic fibrosis patients. Am J Pediatr Hematol Oncol. 1983;5:153–60.

    CAS  PubMed  Google Scholar 

  54. O'Riordan SM, Robinson PD, Donaghue KC, Moran A. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2009;10 Suppl 12:43–50.

    Article  PubMed  Google Scholar 

  55. Management of Cystic Fibrosis Related Diabetes Mellitus: Report of the UK Cystic Fibrosis Working Group. UK CF Trust; 2004.

  56. Park PJ, Griffin SJ, Duffy SW, Wareham NJ. The effect of varying the screening interval on false positives and duration of undiagnosed disease in a screening programme for type 2 diabetes. J Med Screen. 2000;7:91–6.

    Article  CAS  PubMed  Google Scholar 

  57. Dobson L, Sheldon CD, Hattersley AT. Conventional measures underestimate glycaemia in cystic fibrosis patients. Diabetes Med. 2004;21:691–6.

    Article  CAS  Google Scholar 

  58. Brodsky J, Dougherty S, Makani R, Rubenstein RC, Kelly A. Elevation of 1-hour plasma glucose during oral glucose tolerance testing is associated with worse pulmonary function in cystic fibrosis. Diabetes Care. 2011;34:292–5.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  59. Walshaw M. Routine OGTT screening for CFRD - no thanks. J Royal Soc Med. 2009;102 Suppl 1:40–4.

    Article  Google Scholar 

  60. Dobson L, Hattersley AT, Tiley S, Elworthy S, Oades PJ, Sheldon CD. Clinical improvement in cystic fibrosis with early insulin treatment. Arch Dis Child. 2002;87:430–1.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  61. Moreau F, Weiller MA, Rosner V, Weiss L, Hasselmann M, Pinget M, et al. Continuous glucose monitoring in cystic fibrosis patients according to the glucose tolerance. Horm Metab Res. 2008;40:502–6.

    Article  CAS  PubMed  Google Scholar 

  62. Lachin JM, Orchard TJ, Nathan DM, Group DER. Update on cardiovascular outcomes at 30 years of the diabetes control and complications trial/epidemiology of diabetes interventions and complications study. Diabetes Care. 2014;37:39–43.

    Article  PubMed  Google Scholar 

  63. Writing Team for the Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and Complications Research Group. Sustained effect of intensive treatment of type 1 diabetes mellitus on development and progression of diabetic nephropathy: the Epidemiology of Diabetes Interventions and Complications (EDIC) study. JAMA. 2003;290:2159–67.

    Article  Google Scholar 

  64. The Diabetes Control and Complications Trial Research Group. The effect of intensive treatment of diabetes on the development and progression of long-term complications in insulin-dependent diabetes mellitus. N Engl J Med. 1993;329:977–86.

    Article  Google Scholar 

  65. UK Prospective Diabetes Study (UKPDS) Group. Effect of intensive blood-glucose control with metformin on complications in overweight patients with type 2 diabetes (UKPDS 34). Lancet. 1998;352:854–65.

    Article  Google Scholar 

  66. Schwarzenberg SJ, Thomas W, Olsen TW, Grover T, Walk D, Milla C, et al. Microvascular complications in cystic fibrosis-related diabetes. Diabetes Care. 2007;30:1056–61.

    Article  PubMed  Google Scholar 

  67. Andersen HU, Lanng S, Pressler T, Laugesen CS, Mathiesen ER. Cystic fibrosis-related diabetes: the presence of microvascular diabetes complications. Diabetes Care. 2006;29:2660–3.

    Article  PubMed  Google Scholar 

  68. Onady GM, Stolfi A. Insulin and oral agents for managing cystic fibrosis-related diabetes. Cochrane Database Syst Rev. 2013;7, CD004730.

    PubMed  Google Scholar 

  69. Moran A, Phillips J, Milla C. Insulin and glucose excursion following premeal insulin lispro or repaglinide in cystic fibrosis-related diabetes. Diabetes Care. 2001;24:1706–10.

    Article  CAS  PubMed  Google Scholar 

  70. Grover P, Thomas W, Moran A. Glargine vs NPH insulin in cystic fibrosis related diabetes. J Cyst Fibros. 2008;7:134–6.

    Article  CAS  PubMed  Google Scholar 

  71. Hardin DS, Rice J, Rice M, Rosenblatt R. Use of the insulin pump to treat cystic fibrosis related diabetes. J Cyst Fibros. 2009;8:174–8.

    Article  CAS  PubMed  Google Scholar 

  72. Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326:1187–91.

    Article  CAS  PubMed  Google Scholar 

  73. Kerem E, Corey M, Stein R, Gold R, Levison H. Risk factors for Pseudomonas aeruginosa colonization in cystic fibrosis patients. Pediatr Infect Dis J. 1990;9:494–8.

    Article  CAS  PubMed  Google Scholar 

  74. Matel JL, Milla CE. Nutrition in cystic fibrosis. Semin Respir Crit Care Med. 2009;30:579–86.

    Article  PubMed  Google Scholar 

  75. Stack M, Mahony M, Boyle C. Obesity in childhood cystic fibrosis (Abstract 220). J Cyst Fibros. 2007;6(Suppl):S54.

  76. Kastner-Cole D, Palmer CN, Ogston SA, Mehta A, Mukhopadhyay S. Overweight and obesity in deltaF508 homozygous cystic fibrosis. J Pediatr. 2005;147:402–4.

    Article  PubMed  Google Scholar 

  77. Panagopoulou P, Fotoulaki M, Nikolaou A, Nousia-Arvanitakis S. Prevalence of malnutrition and obesity among cystic fibrosis patients. Pediatr Int. 2014;56:89–94.

    Article  CAS  PubMed  Google Scholar 

  78. Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB, et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97:872–7.

    Article  CAS  PubMed  Google Scholar 

  79. Coderre L, Fadainia C, Belson L, Belisle V, Ziai S, Maillhot G, et al. LDL-cholesterol and insulin are independently associated with body mass index in adult cystic fibrosis patients. J Cyst Fibros. 2012;11:393–7.

    Article  CAS  PubMed  Google Scholar 

  80. Collins S, Reynolds F. How do adults with cystic fibrosis cope following a diagnosis of diabetes? J Adv Nurs. 2008;64:478–87.

    Article  PubMed  Google Scholar 

  81. Dashiff C, Suzuki-Crumly J, Kracke B, Britton L, Moreland E. Cystic fibrosis-related diabetes in older adolescents: parental support and self-management. J Spec Pediatr Nurs. 2013;18:42–53.

    Article  PubMed  Google Scholar 

  82. Tierney S, Deaton C, Webb K, Jones A, Dodd M, McKenna D, et al. Isolation, motivation and balance: living with type 1 or cystic fibrosis-related diabetes. J Clin Nurs. 2008;17:235–43.

    Article  PubMed  Google Scholar 

  83. Aschner P, LaSalle J, McGill M. Global Partnership for Effective Diabetes M. The team approach to diabetes management: partnering with patients. Int J Clin Pract Suppl. 2007;157:22–30.

    Article  PubMed  Google Scholar 

  84. Swartz LM, Laffel LM. A teenage girl with cystic fibrosis-related diabetes, diabetic ketoacidosis, and cerebral edema. Pediatr Diabet. 2008;9(4 Pt 2):426–30.

    Article  CAS  Google Scholar 

  85. Eenkhoorn V, Van den Driessche A, Van Gaal L, Desager K, De Block C. Diabetic keto-acidosis as a presentation of cystic fibrosis-related diabetes: a case report. J Diabetes Complications. 2011;25:137–41.

    Article  PubMed  Google Scholar 

  86. Tierney S, Webb K, Jones A, Dodd M, McKenna D, Rowe R, et al. Living with cystic fibrosis-related diabetes or type 1 diabetes mellitus: a comparative study exploring health-related quality of life and patients’ reported experiences of hypoglycaemia. Chronic Illn. 2008;4:278–88.

    Article  PubMed  Google Scholar 

  87. Radike K, Molz K, Holl RW, Poeter B, Hebestreit H, Ballmann M. Prognostic relevance of hypoglycemia following an oral glucose challenge for cystic fibrosis-related diabetes. Diabetes Care. 2011;34:e43.

    Article  PubMed Central  PubMed  Google Scholar 

  88. van den Berg JM, Morton AM, Kok SW, Pijl H, Conway SP, Heijerman HG. Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD). J Cyst Fibros. 2008;7:515–9.

    Article  PubMed  Google Scholar 

  89. Yung B, Landers A, Mathalone B, Gyi KM, Hodson ME. Diabetic retinopathy in adult patients with cystic fibrosis-related diabetes. Respir Med. 1998;92:871–2.

    Article  CAS  PubMed  Google Scholar 

  90. Dobson L, Stride A, Bingham C, Elworthy S, Sheldon CD, Hattersley AT. Microalbuminuria as a screening tool in cystic fibrosis-related diabetes. Pediatr Pulmonol. 2005;39:103–7.

    Article  CAS  PubMed  Google Scholar 

  91. Guman-Wignot TM, Kaufman J, Holsclaw Jr DS, Schmoyer IR, Alhadeff JA. Analysis and HPLC fractionation of urine from patients with cystic fibrosis, chronic lung diseases and normal controls. Clin Biochem. 1989;22:377–83.

    Article  CAS  PubMed  Google Scholar 

  92. Lanng S, Thorsteinsson B, Nerup J, Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr. 1992;151:684–7.

    Article  CAS  PubMed  Google Scholar 

  93. Kerem E, Viviani L, Zolin A, Macneill S, Hatziagorou E, Ellemunter H, et al. Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry. Eur Respir J. 2014;43:125–33.

    Article  PubMed  Google Scholar 

  94. Alicandro G, Battezzati PM, Battezzati A, Speziali C, Claut L, Motta V, et al. Insulin secretion, nutritional status and respiratory function in cystic fibrosis patients with normal glucose tolerance. Clin Nutr. 2012;31:118–23.

    Article  CAS  PubMed  Google Scholar 

  95. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000;162(3 Pt 1):891–5.

    Article  CAS  PubMed  Google Scholar 

  96. Leclercq A, Gauthier B, Rosner V, Weiss L, Moreau F, Constantinescu AA, et al. Early assessment of glucose abnormalities during continuous glucose monitoring associated with lung function impairment in cystic fibrosis patients. J Cyst Fibros. 2013;S1569–993. [Epub ahead of print].

  97. Brennan AL, Gyi KM, Wood DM, Johnson J, Holliman R, Baines DL, et al. Airway glucose concentrations and effect on growth of respiratory pathogens in cystic fibrosis. J Cyst Fibros. 2007;6:101–9.

    Article  CAS  PubMed  Google Scholar 

  98. Mohan K, Israel KL, Miller H, Grainger R, Ledson MJ, Walshaw MJ. Long-term effect of insulin treatment in cystic fibrosis-related diabetes. Respiration. 2008;76:181–6.

    Article  CAS  PubMed  Google Scholar 

  99. Kolouskova S, Zemkova D, Bartosova J, Skalicka V, Sumnik Z, Vavrova V, et al. Low-dose insulin therapy in patients with cystic fibrosis and early-stage insulinopenia prevents deterioration of lung function: a 3-year prospective study. J Pediatr Endocrinol Metab. 2011;24(7–8):449–54.

    CAS  PubMed  Google Scholar 

  100. Mozzillo E, Franzese A, Valerio G, Sepe A, De Simone I, Mazzarella G, et al. One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements. Pediatr Diabetes. 2009;10:162–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Compliance with Ethics Guidelines

ᅟ

Conflict of Interest

Donal O’Shea and Jean O’Connell declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Department of Endocrinology, St. Vincent’s University Hospital, Elm Park, Dublin 4, Ireland

    Donal O’Shea & Jean O’Connell

Authors
  1. Donal O’Shea
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jean O’Connell
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jean O’Connell.

Additional information

This article is part of the Topical Collection on Diabetes and Other Diseases-Emerging Associations

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

O’Shea, D., O’Connell, J. Cystic Fibrosis Related Diabetes. Curr Diab Rep 14, 511 (2014). https://doi.org/10.1007/s11892-014-0511-3

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11892-014-0511-3

Keywords

Search

Navigation