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

作者：Warncke K; Konrad K; Kohne E; Hammer E; Ohlenschlaeger U; Herrlinger S; Jaeger A; Holl R W

来源：Klinische Padiatrie, 2016, 228(6-7): 307-312.

DOI：10.1055/s-0042-111691

• ss-Thalassemia
• sickle cell disease
• hemoglobinopathy
• diabetes mellitus
• metabolic control
• ss-Thalassamie
• Sichelzellerkrankung
• Hamoglobinopathie
• Diabetes mellitus
• metabolische Kontrolle

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.

• 出版日期2016-11