Diabetes in Patients with ß-thalassemia or other Hemoglobinopathies - Analysis from the DPV Database

Klin Padiatr. 2016 Nov;228(6-07):307-312. doi: 10.1055/s-0042-111691. Epub 2016 Nov 15.


Background: Diabetes mellitus is a common endocrinopathy in patients with thalassemia major, but the occurrence of hemoglobinopathies is rare in Germany and Western Europe. The longitudinal German-Austrian DPV (Diabetes Patienten Verlaufsdokumentation) registry allows a comprehensive characterization of this group of patients. Patients/methods: Patients from the DPV-registry aged<30 years with thalassemia major or other hemoglobinopathies were compared to patients with type 1 diabetes (T1D) and type 2 diabetes (T2D) using the statistical software SAS 9.4. Results: 94 patients (0.13% of patients) with hemoglobinopathies are registered in DPV. 82.4% of 17 patients with thalassemia major, 100% of 12 patients with sickle cell disease (SCD) and >90% of 65 patients with other hemoglobinopathies receive insulin treatment. In the majority of patients with thalassemia major, hemosiderosis is documented. Patients with thalassemia major developed diabetes at a median age of 14.6 [IQR 8.4-18.0] years (9.0 years [5.3-12.5] in T1D; 18.7 years [14.2-25.6] in TD2; both p<0.01). They show high HbA1c/fructosamine levels and frequent hypoglycemia, reflecting poor metabolic control. Conclusion: Diabetes in thalassemia major is probably caused by hemosiderosis due to polytransfusion, while patients with SCD/thalassemia minor are most likely affected by T1D. The high rate of hypoglycemia in patients with ß-thalassemia major may be caused by liver fibrosis and a lack of hepatic glycogen stores.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Comorbidity
  • Cross-Sectional Studies
  • Diabetes Complications / epidemiology
  • Diabetes Mellitus, Type 1 / epidemiology*
  • Diabetes Mellitus, Type 2 / epidemiology*
  • Female
  • Hemoglobinopathies / epidemiology*
  • Humans
  • Male
  • Registries
  • Young Adult
  • beta-Thalassemia / epidemiology*