Cystic Fibrosis Related Diabetes Mellitus: Diagnostics and Therapy
Authors:
S. Koloušková; D. Zemková; Z. Šumník; M. Šnajderová; V. Skalická; J. Bartošová; J. Brázová
Authors‘ workplace:
Pediatrická klinika UK 2. LF a FN Motol, Praha
přednosta prof. MUDr. J. Lebl, CSc.
Published in:
Čes-slov Pediat 2008; 63 (2): 90-95.
Overview
Cystic fibrosis related diabetes (CFRD) is associated with higher morbidity and 6 times higher mortality. In females, it occurs 5 years earlier compared to males. After 18 years of age, prevalence of CFRD increases and ranges between 12 and 34%, after 30 years it affects 35–50% of all patients. Prospective studies demonstrated deterioration of nutritional status and pulmonary functions 2–4 years prior diabetes onset; therefore, an early recognition of CFRD is a prerequisite for a successful management of CF patients. Insulin therapy of CFRD may delay progression of pulmonary changes. Pulmonary functions do not deteriorate within 5 years after onset of insulin therapy, in parallel with improved nutritional status.
Screening of CFRD is based on oral glucose tolerance test (OGTT) after 10 years of age (golden standard). An additional intravenous glucose tolerance test (IVGTT) is provided in subjects with impaired glucose tolerance or diabetes mellitus to detect insulinopenia and to start individualised therapy. Regarding the prevalent finding of insulinopenia, an early insulin therapy is started as early as possible if necessary to prevent serious consequences of developing clinical diabetes on nutritional status and pulmonary functions.
Special nutritional team takes care of diabetic diet in CFRD. In these subjects, diet is based on different principles in comparison to other diabetes subtypes. The food energy content has to be substantially increased and carbohydrates only moderately regulated.
Any unexplained deterioration of nutritional status or pulmonary functions in CF patients should be suspected of CFRD.
Key words:
cystic fibrosis related diabetes, screening, therapy, insulin, OGTT, IVGTT
Sources
1. Abdul-Karim FW, Dahms BB, Velasca ME, et al. Islet of Langerhans in adolescents and adults with cystic fibrosis. Arch. Pathol. Lab. Med. 1986;110: 602–606.
2. Ahmad T, Melson R, Taylor R. Insulin sensitivity and metabolic clearance rate of insulin in cystic fibrosis. Metabolism 1994;43: 163–167.
3. Allen HF, Gay EC, Klingensmith GJ, Hamman RF. Identification and treatment of cystic fibrosis-related diabetes. Current Opinion in Endocrinology, Diabetes and Obesity1998;21: 943–948.
4. American Diabetes Association Clinical Practice Recommendations. Diabetes Care 1998; S1.
5. Andersen DH. Cystic fibrosis in the pancreas and its relationship to celiac disease. Am. J. Dis. Child. 1938;56: 344–399.
6. Cucinotta D, Nibali SC, Attigo T, et al. Beta cell function, peripheral sensitivity to insulin and islet cell autoimmunity in cystic fibrosis patients with normal glucose tolerance. Horm. Res. 1990; 34: 33–38.
7. Cuccinotta D, De Luca F, Scoglio R, et al. Factor affecting diabetes mellitus onset in cystic fibrosis: evidence from a 10-yaers follow-up study. Acta Pediatr. 1999;88: 389–393.
8. Cystic Fibrosis Foundation Patient Registry 1997. Annual data report. Bethesda, MD: Cystic Fibrosis Foundation, 1998.
9. Schepper J, Dab I, Derde MP, Loeb H. Oral glucose tolerance testing in cystic fibrosis: Correlations with clinical parameters and glycosylated haemoglobin determinations. Eur. J. Pediatr. 1991;150: 403–406.
10. Doershuk CF (ed). Cystic Fibrosis in the 20th Century. People, Events and Progress. Cleveland, Ohio: AM Publishing Ltd., 2001, XII.
11. Finkelstein SM, Wielinski CL, Elliot GR, et al. Diabetes mellitus associated with cystic fibrosis. J. Pediatr. 1988;112: 373–377.
12. Handwerger S, Roth J, Gorden P, et al. Glucose intolerance in cystic fibrosis. N. Engl. J. Med. 1969;281: 451–461.
13. Hardin DS, LeBlanc A, Lukenbaugh S, et al. Proteolysis associated with insulin resistance in cystic fibrosis. Pediatrics 1998;101: 433–437.
14. Hardin DS, LeBlanc A, Para L, Seilheimer DK. Hepatic insulin resistance and defects in substrate utilization in cystic fibrosis. Diabetes 1999;49: 1082–1087.
15. Hardin DS, LeBlanc A, Marschall G, Seilheimer DK. Mechanism of insulin resistance in cystic fibrosis. Am. J. Physiol. Endocrinol. Metab. 2001;281: 1022–1028.
16. Koloušková S, Zemková D, Bartošová J, Šumník Z, Vávrová V. Vývoj porušené glukózové tolerance a diabetu u pacientů s cystickou fibrózou. Čes.-slov. Pediat. 2003;58: 270–273.
17. Koloušková S, Zemková D, Bartošová J, Šumník Z, Vávrová V. Early insulin treatment in insulinopenic CF patients improves body composition and stabilizes pulmonary function. J. Cystic Fibrosis 2003;2: S78.
18. 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: 684–697.
19. Lanng S, Thorsteinsson B, Roder ME, et al. Pancreas and gut hormone responses to oral glucose and intravenous glucagon in cystic patients with normal, impaired and diabetic glucose tolerance. Acta Endocrinol. 1993;128: 207–214.
20. Lanng S, Thorsteinsson B, Nerup J, Koch C. Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Pediatr.1994;83: 849–853.
21. Lanng S, Hahnsen A, Thorsteinsson B, Nerup J, Koch C. Glucose tolerance in patients with cystic fibrosis: five year prospective study. Brit. Med. J. 1995;311: 655–659.
22. Lanng S, Thorsteinnson B, Nerup J, et al. Diabetes mellitus in cystic fibrosis: a ten-year prospective study. Diabetologia 2000;43: 107A.
23. Lohr M, Goerchen P, Nizze H, et al. CF associated islet changes may provide a basis for diabetes: a immunocytological and morphometrical study. Virchows Ach. f. pathol. Anat. u. Physiol. 1989;414: 179–185.
24. Meacham LR, McKean LP, Buchanan CN, et al. Selective preservation of somatostatin secretion in CF patients with diabetes. Pediatr. Pulmonol. 1991;Suppl 6: 333A.
25. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at base. Am. J. Respir. Crit. Care Med. 2000;162: 891–895
26. Moran A, Doherty L, Wang X, et al. Abnormal glucose metabolism in cystic fibrosis. J. Pediatr. 1998;133: 10–17.
27. Moran A, Hardin D, Rodman D, et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus. A consensus conference report. Diabetes Research and Clinical Practice 1999;45: 61–73.
28. Nousia-Arvanitakis S, Galli-Tsinopoulou A, Karamouzis M. Insulin improves clinical status of patients with cystic fibrosis related diabetes mellitus. Acta Pediatr. 2001;90: 515–519.
29. Reisman J, Corey M, Canny G, Levison H. Diabetes mellitus in patients with cystic fibrosis effect on survival. Pediatrics 1990;86: 374–377.
30. Report of the Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Diabetes Care 1997;20:1183–1197.
31. Wilmhurst EG, Soeldner JS, Holsclaw DS, et al. Endogenous and exogenous insulin response in patients with cystic fibrosis. Pediatrics 1975;55: 75–82.
Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2008 Issue 2
Most read in this issue
- Newborn Screening in the Czech Republic and in Europe
- Current Diagnostic Strategies and Overview of Preimplantation, Prenatal and Postnatal DNA Diagnostics of Cystic Fibrosis in the Czech Republic
- Associate Professor Věra Vávrová and History of Cystic Fibrosis
- Cystic Fibrosis Related Diabetes Mellitus: Diagnostics and Therapy