Konya’daki talasemi majörlü hastalarda bozulmuş glukoz toleransı ve diyabet prevalansı

Amaç: Bu çalışmanın amacı Konya, Selçuk Üniversitesi Meram Tıp Fakültesi Pediatrik Hematoloji Bölümü’nde izlenen beta-talasemi majörlü hastalardaki, diyabet ve bozulmuş glukoz tolerans sıklığını değerlendirmek ve beta-talasemi majöre eşlik eden şelasyon tedavisine uyum, diyabet için aile hikayesi ve hastaların demografik özelliklerinin diyabet patogenezindeki olası rolünü araştırmaktı. Yöntem: Talasemi majör tanısı ile izlenen yaş ortalaması 9.56 ± 5.59 (yıl) olan 51 hasta değerlendirmeye alındı. Hastalara oral glukoz tolerans testi uygulandı. 0., 30., 60., 90. ve 120. dakikalarda alınan kan örneklerinin sonuçları Dünya Sağlık Örgütü tanı kriterlerine göre yorumlandı. Bulgular: Talasemi majörlü hastalar arasında bozulmuş glukoz toleransı sıklığı % 10 (51 hastanın 5’i), diyabet sıklığı ise % 10 (51 hastanın 5’i) olarak bulundu. Ferritin düzeyi anormal glukoz toleranslı talasemili hastalarda normal glukoz toleranslı hastalara göre daha yüksekti ancak istatistiksel olarak anlamlı değildi. Anormal glukoz toleranslı hastaların hiçbirinde diyabet için aile hikayesi yoktu. Anormal glukoz toleranslı talasemik hastaların birinde HCV-RNA pozitif bulundu. Sonuç: Bu çalışma merkezimizdeki talasemi majörlü hastalarda bozulmuş glukoz toleransı ve diyabet prevalansının literatürdeki daha önce bildirilen sonuçlara benzer olduğunu göstermektedir. Talasemi majörlü hastaların tamamı sadece açlık glukozuyla doğrudan tanı almadığı için talasemik hastalarda anormal glukoz toleransının teşhisi için OGTT yapılmasını öneriyoruz. Yüksek serum ferritin konsantrasyonunun talasemi majörlü hastalarda anormal glukoz toleransı için bir risk faktörüdür.

The prevalence of impaired glucose tolerance and diabetes in patients with beta-thalassemia major in Konya

Objective: The aim of the study was to evaluate the prevalence of diabetes and impaired glucose tolerance in beta-thalassemia major patients who had been observed in the Pediatric Hematology Unit, Department of Pediatrics, Selcuk University, Meram Faculty of Medicine, Konya and to study the possible role of demographic characteristics of patients, family history of diabetes and compliance with iron-chelation therapy in the pathogenesis of diabetes associated with beta-thalassemia major. Methods: 51 patients with thalassemia major were chosen for this evaluation. Mean age was 9.56 ± 5.59 years. Oral glucose tolerance test was applied for the study group. Blood samples were taken at 0, 30, 60, 90, and 120 minutes and the results were interpreted according to the criteria published by World Health Organization. Results: The prevalence of impaired glucose tolerance was 10% (5 of 51) and that of diabetes was 10% (5 of 51) among patients with thalassemia major. The ferritin level was high in thalassemic patients with abnormal glucose tolerance compared to those with normal glucose tolerance; the level was not statistically significant. None patients with abnormal glucose tolerance had a positive family history of diabetes. HCV-RNA was found positive in one of thalassemic patients with abnormal glucose tolerance. Conclusion: This study shows that the prevalence of diabetes and impaired glucose tolerance in the patients with thalassemia major in our center were similar to results of previous reports in the literature. Because not all of the patients with thalassemia major could be correctly diagnosed by fasting glucose alone, we suggest that use OGTT for the diagnosed of abnormal glucose tolerance in thalassemic patients. High serum ferritin concentration is a risk factor of abnormal glucose tolerance in patients with thalassemia major.

___

  • 1. Arrigo T, Crisafulli G, Meo A, Sturiale M, Lombardo F, Miceli M, et al. Glucose tolerance, insulin secretion and peripheral sensitivity in thalassaemia major. J Pediatr Endocrinol Metab 1998; 11:863-6.
  • 2. Gamberini MR, Fortini M, Gilli G, Testa MR, De Sanctis V. Epidemiology and chelation therapy effects on glucose homeostasis in thalassaemic patients. J Pediatr Endocrinol Metab 1998; 11:867-9.
  • 3. De Sanctis V, Zurlo MG, Senesi E, Boffa C, Cavallo L, Di Gregorio F. Insulin dependent diabetes in thalassaemia. Arch Dis Child 1988; 63(1):58-62.
  • 4. Italian Working Group on Endocrine Complications in Non-endocrine Diseasee: Multicentre study on prevalence of endocrine complications in thalassemia major. Clin Endocrinol 1995; 42:581-6.
  • 5. Merkel PA, Simonson DC, Amiel SA, Plewe G, Sherwin RS, Pearson HA, et al. Insulin resistance and hyperinsulinemia in patients with thalassemia major treated by hypertransfusion. N Engl J Med 1988; 318:809-14.
  • 6. Zuppinger K, Molinari B, Hirt A, Imbach P, Gugler E, Tonz O, et al. Increased risk of diabetes mellitus in beta- thalassemia major due to iron overload. Helv Paediatr Acta 1979; 34):197-207.
  • 7. Pollack MS, Levine LS, Oberfield SE, Markenson AL. HLA-A, B, C, and DR antigen frequencies in relation to development of diabetes and variations in white cell antibody formation in highly transfused thalassemia patients. Transfusion 1982; 22:279-82.
  • 8. Pappas S, Donohue SM, Denver AE, Mohamed-AIi V, Goubet S, Yudkin JS. Glucose intolerance in thalassemia major is related to insulin resistance and hepatic dysfunction. Metabolism 1996; 45:652-7.
  • 9. Diabetes Epidemiology Collaborative Analysis of Diagnostic Criteria in Europe Study Group. Will new diagnostic criteria for diabetes mellitus change phenotype of Diabetes Epidemiology Collaborative Analysis of Diagnostic Criteria in Europe Study Group: glucose tolerance and mortality: comparison of WHO and American Diabetes Association diagnostic criteria. Lancet 1999; 354: 617–21.
  • 10. Yaprak I. Beta Talasemi Tanı ve Tedavisinde Güncel Yaklaşımlar. Sted 2004; 13:58-9.
  • 11. Canpolat N, Aydoğan G, Akçay A, Şalcıoğlu Z, Akıcı F, Kıyak A. B-Talasemi Major ve Glukoz Metabolizması Bozukluğu. SSK Tepecik Hast Derg 2004; 14(2):119-24.
  • 12. Dedeoğlu S, Işık K, Kut A, Timur Ç, Arslanoğlu İ, Ataözden E, et al. Talasemi Majorlu Çocuklarda Pankreas Beta Hücre Fonksiyon Bozukluğunda Demir Birikiminin İrdelenmesi. Göztepe Tıp Dergisi 1998; 13:13-7.
  • 13. Güler E, Patıroğlu T, Çaksen H, Özdemir MA, Kurtoğlu S, Kendirci M. Talasemi Majörlü Vakalarda Endokrin Komplikasyonların Değerlendirilmesi. Türk Pediatri Arşivi. 1999; 34:174-81.
  • 14. Gamberini MR, Fortini M, De Sanctis V, Gilli G, Testa MR. Diabetes mellitus and impaired glucose tolerance in thalassaemia major: incidence, prevalence, risk factors and survival in patients followed in the Ferrara Center. Ped Endocrinol Rev 2004; 2:285-91.
  • 15. De Sanctis V, Eleftheriou A, Malaventura C; Thalassaemia International Federation Study Group on Growth and Endocrine Complications in Thalassaemia.Prevalence of endocrine complications and short stature in patients with thalassaemia major: a multicenter study by the Thalassaemia International Federation (TIF).Pediatr Endocrinol Rev. 2004;2 Suppl 2:249-55.
  • 16. Erttmann R, Hausdorf G, Landbeck G. Pancreatic sonography in thalassemia major. Klin Padiatr 1983; 195:97-9.
  • 17. Dmochowski K, Finegood DT, Francombe W, Tyler B, Zinman B. Factors determining glucose tolerance in patients with thalassemia major. J Clin Endocrinol Metab 1993; 77:478-83.
  • 18. Zuppinger K, Molinari B, Hirt A, Imbach P, Gugler E, Tonz O, et al. Increased risk of diabetes mellitus in beta- thalassemia major due to iron overload. Helv Paediatr Acta 1979; 34:197-207.
  • 19. Dandona P, Hussain MAM, Varghese Z, Politis D, Flynn DM, Hoffbrand AV. Insulin resistance and iron overload. Ann Clin Biochem 1983; 20:77-9.
  • 20. Niederau C, Berger M, Stremmel W, Starke A, Strohmeyer G, Ebert R, et al. Hyperinsulinaemia in non-cirrhotic haemochromatosis: impaired hepatic insulin degradation? Diabetologia 1984; 26:441-4.
  • 21. Felig P, Wahren J. Symposium I: hormone-fuel interactions in normal and diabetic man. The liver as site of insulin and glucagon action in normal, diabetic and obese humans. Isr J Med Sci 1975; 11:528-39.
  • 22. Cario H, Holl RW, Debatin KM, Kohne E. Insulin sensitivity and beta-cell secretion in thalassaemia major with secondary haemochromatosis: assessment by oral glucose tolerance test. Eur J Pediatr 2003; 162:139-46.
  • 23. Dmochowski K, Finegood DT, Francombe W, Tyler B, Zinman B. Factors determining glucose tolerance in patients with thalassemia major. J Clin Endocrinol Metab 1993; 77:478-83.
  • 24. Ladis V, Theodorides C, Palamidou F, Frissiras S, Berdousi H, Kattamis C. Glucose disturbances and regulation with glibenclamide in thalassemia. Pediatr Endocrinol Metab 1998; 11 SuppI3:871-8.
  • 25. Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE, et al. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med 1994; 331:567-73.
  • 26. Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A, et al. Survival in medically treated patients with homozygous beta-thalassemia. N Engl J Med 1994; 331:574-8.
  • 27. Fernandez-Real JM, Ricart-Engel W, Arroyo E, Balanca R, Casamitjana-Abella R, Cabrero D, et al. Serum ferritin as a component of the insulin resistance syndrome. Diabetes Care 1998; 21:62-8.