Pairunyar NakavacharaWorarat KajchamapornJulaporn PooliamVip ViprakasitFaculty of Medicine, Siriraj Hospital, Mahidol University2020-01-272020-01-272019-01-01Pediatric Blood and Cancer. (2019)15455017154550092-s2.0-85077146030https://repository.li.mahidol.ac.th/handle/20.500.14594/52001© 2019 Wiley Periodicals, Inc. Background: Diabetes mellitus (DM) associated with iron overload has been reported among adults with transfusion-dependent thalassemia and those with non-transfusion-dependent thalassemia (NTDT), especially in β-thalassemia disease. However, little is known about glucose metabolism and how early its dysregulation can develop in α-thalassemia hemoglobin H (Hb H) disease, which is one of the most common types of NTDT worldwide. Procedure: We prospectively calculated glucose metabolism index in 40 patients (aged 10–25 years) with Hb H disease. Glucose metabolism data were compared between patients with deletional versus nondeletional Hb H, and between patients with normal versus abnormal insulin secretion/sensitivity. Results: Despite normal glucose tolerance in all patients, 52.5% had abnormal insulinogenic index indicating decreased β-cell insulin secretion. Patients with functional hemoglobin < 8 g/dL had significantly higher percentages of abnormal insulinogenic index. There was no significant difference in abnormal insulinogenic index between deletional and nondeletional Hb H. Conclusion: Decreased β-cell insulin secretion is highly prevalent among children and adolescents with Hb H disease, and it is associated with levels of functional anemia at baseline, but not with the type of Hb H disease. This result warrants heightened awareness among hematologists due to potentially increased risk of DM later in life.Mahidol UniversityMedicineArticleSCOPUS10.1002/pbc.28109