title: Diabetes in Patients with ß-thalassemia or other Hemoglobinopathies - Analysis from the DPV Database.
abstract: 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.
authors.affiliation: Department of Pediatrics, Klinikum rechts der Isar, Technische Universität München, Munich, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Warncke
authors.affiliation: Department of Pediatric and Adolescent Medicine, University Children's Hospital Cologne, Cologne, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Konrad
authors.affiliation: Department of Pediatrics and Adolescents, Laboratory of Hemoglobinopathy, University of Ulm, Ulm, Germany.
authors.forename: E
authors.initials: E
authors.lastname: Kohne
authors.affiliation: Department of Pediatrics, Children's Hospital Wilhelmstift, Hamburg, Germany.
authors.forename: E
authors.initials: E
authors.lastname: Hammer
authors.affiliation: Department of Pediatrics, Goethe University Frankfurt, Frankfurt, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Ohlenschläger
authors.affiliation: Department of Pediatrics, Klinikum Bremen Nord, Bremen, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Herrlinger
authors.affiliation: Department of Pediatrics, Kaiser-Franz-Josef-Spital mit Gottfried von Preyer'schem Kinderspital, Wien, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Jäger
authors.affiliation: Central Institute for Biomedical Technology, ZIBMT, University of Ulm, Ulm, Germany.
authors.forename: R W
authors.initials: RW
authors.lastname: Holl
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D015897:Comorbidity; D003430:Cross-Sectional Studies; D048909:Diabetes Complications / Q000453:epidemiology; D003922:Diabetes Mellitus, Type 1 / Q000453:epidemiology*; D003924:Diabetes Mellitus, Type 2 / Q000453:epidemiology*; D005260:Female; D006453:Hemoglobinopathies / Q000453:epidemiology*; D006801:Humans; D008297:Male; D012042:Registries; D055815:Young Adult; D017086:beta-Thalassemia / Q000453:epidemiology*