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Mukoviszidose und Diabetes

Cystic fibrosis and diabetes

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Zusammenfassung

Die Mukoviszidose, auch zystische Fibrose (CF) genannt, ist die häufigste letale autosomale Erkrankung in unseren Breiten. Sie befällt Lunge, Pankreas, Leber, Genitaltrakt, Schweißdrüsen und andere Organe, die exokrine Gewebe enthalten. Zu den Symptomen gesellen sich mit zunehmendem Alter der Patienten noch weitere Komorbiditäten. Die häufigste Komorbidität ist der CFRD (CF-assoziierter Diabetes). CFRD gehört in die Gruppe der Typ-3-Diabetes-Formen. Ab dem 10. Lebensjahr beginnt die Anzahl zu steigen und erreicht mit 25 Jahren eine Häufigkeit von 30% der CF-Patienten. Aus der unterschiedlichen Pathogenese (Insulinrestsekretion lange erhalten bei CFRD und in der Regel kein Über-, sondern eher Untergewicht) leiten sich auch die Besonderheiten der Behandlung ab. Die Ernährung soll weiterhin hochkalorisch und fettreich sein. Insulin ist die einzig geprüfte Therapie. Die Verwendung oraler Antidiabetika, die in der Praxis oft „off-label“ als Therapie eingesetzt werden, erfolgt zurzeit noch in klinischen Studien und ist somit nicht als geprüft anzusehen. Neueste Daten belegen eine Aufhebung der ehemals dargestellten negativen Effekte von CFRD auf das Überleben bei früher intensiver Therapie.

Abstract

Cystic fibrosis (CF) is the most frequent lethal autosomal disease among Caucasians. CF is a multi-organ disease involving lung, pancreas, liver, reproductive tract, sweat glands and other organs with exocrine tissue. With increasing age co-morbidities become more important. CFRD (CF-related diabetes) is the most frequent co-morbidity in CF. CFRD is a type 3 diabetes with some overlap to type 1 and type 2 diabetes. From the age of 10 onwards the numbers begin to increase, with an incidence of 30% seen at 25 years. Differences in the pathogenesis (long-standing rest-secretion of insulin in CFRD and generally underweight rather than overweight) are reflected in different diagnostic approaches and different treatment regimes. Nutrition is almost unrestricted in CFRD with high caloric intake as a major aim of treatment. Insulin is the only proven treatment. Oral anti-diabetic drugs should be used only in controlled clinical trails and not “off-label”. Very recent data demonstrate initial evidence that early and aggressive treatment results in the same median survival with/without CFRD in CF patients.

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Literatur

  1. Ballmann M (2007) Diagnostik der Zystischen Fibrose. Pneumologie 4:261–266

    Article  Google Scholar 

  2. Mohan K, Miller H, Dyce P et al (2009) Mechanisms of glucose intolerance in cystic fibrosis. Diabet Med 26(6):582–588

    Article  CAS  PubMed  Google Scholar 

  3. O’Riordan SM, Robinson PD, Donaghue KC et al (2009) Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes (Suppl 12):43–50

    Google Scholar 

  4. Bismuth E, Laborde K, Taupin P et al (2008) Glucose tolerance and insulin secretion, morbidity, and death in patients with cystic fibrosis. J Pediatr 152(4):540–545

    Article  CAS  PubMed  Google Scholar 

  5. Sims EJ, Green MW, Mehta A (2005) Decreased lung function in female but not male subjects with established cystic fibrosis-related diabetes. Diabetes Care 28(7):1581–1587

    Article  PubMed  Google Scholar 

  6. Milla CE, Billings J, Moran A (2005) Diabetes is associated with dramatically decreased survival in female but not male subjects with cystic fibrosis. Diabetes Care 28(9):2141–2144

    Article  PubMed  Google Scholar 

  7. Moran A, Pekow P, Grover P et al (2009) Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care 32(10):1783–1788

    Article  CAS  PubMed  Google Scholar 

  8. Nousia-Arvanitakis S, Galli-Tsinopoulou A, Karamouzis M (2001) Insulin improves clinical status of patients with cystic-fibrosis-related diabetes mellitus. Acta Paediatr 90(5):515–519

    Article  CAS  PubMed  Google Scholar 

  9. Milla CE, Warwick WJ, Moran A (2000) Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med 162:891–895

    CAS  PubMed  Google Scholar 

  10. Moran A, Dunitz J, Nathan B et al (2009) Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care 32(9):1626–1631

    Article  PubMed  Google Scholar 

  11. Lanng S, Hansen A, Thorsteinsson B et al (1995) Glucose tolerance in patients with cystic fibrosis: five year prospective study. BMJ 311(7006):655–659

    CAS  PubMed  Google Scholar 

  12. Holl RW, Buck C, Babka C et al (2000) HbA1c is not recommended as a screening test for diabetes in cystic fibrosis. Diabetes Care 23(1):126

    Article  CAS  PubMed  Google Scholar 

  13. Lanng S, Thorsteinsson B, Nerup J et al (1994) Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Paediatr 83(8):849–853

    Article  CAS  PubMed  Google Scholar 

  14. Mohan K, Miller H, Burhan H et al (2008) Management of cystic fibrosis related diabetes: a survey of UK cystic fibrosis centers. Pediatr Pulmonol 43(7):642–647

    Article  PubMed  Google Scholar 

  15. Moran A, Phillips J, Milla C (2001) Insulin and glucose excursion following premeal insulin lispro or repaglinide in cystic fibrosis-related diabetes. Diabetes Care 24(10):1706–1710

    Article  CAS  PubMed  Google Scholar 

  16. Onady GM, Langdon LJ (2006) Insulin versus oral agents in the management of Cystic Fibrosis Related Diabetes: a case based study. BMC Endocr Disord 6:4

    Article  PubMed  Google Scholar 

  17. O’Riordan SM, Hindmarsh P, Hill NR et al (2009) Validation of continuous glucose monitoring in children and adolescents with cystic fibrosis: a prospective cohort study. Diabetes Care 32(6):1020–1022

    Article  Google Scholar 

  18. Moran A (2009) Abnormal glucose tolerance in CF – when should we offer diabetes treatment? Pediatr Diabetes 10(3):159–161

    Article  PubMed  Google Scholar 

  19. Hameed S, Morton JR, Jaffe A et al (2009) Early glucose abnormalities in cystic fibrosis are preceded by poor weight gain. Diabetes Care [Epub ahead of print Nov 12]

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  1. Abteilung Pädiatrische Pneumologie und Neonatologie, Medizinische Hochschule Hannover, Carl Neuberg Str. 1, 30625, Hannover, Deutschland

    M. Ballmann

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Correspondence to M. Ballmann.

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Ballmann, M. Mukoviszidose und Diabetes. Diabetologe 6, 16–22 (2010). https://doi.org/10.1007/s11428-009-0437-6

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  • DOI: https://doi.org/10.1007/s11428-009-0437-6

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