Abnormal glucose homeostasis in patients of HbEβ-thalassemia: Prevalence and possible pathogenesis using the Oxford HOMA model

Abstract

Objectives: Eβ-thalassemia, the most serious form of HbE syndromes, may develop pre-diabetes (PD) and diabetes mellitus (DM), together constituting abnormal glucose homeostasis (AGH) as an endocrinopathy. This study aims to assess AGH prevalence and pathogenesis in this thalassemia subtype. Material and Methods: A cross-sectional study was conducted at a tertiary care hospital from February 2017 to December 2018 (1.9 years). One hundred and four HbEβ-thalassemia patients were randomly selected aged ≥5 years, irrespective of transfusion requirement. AGH was diagnosed by the American Diabetes Association criteria. The patient’s history, relevant examination details, and parameters related to glucose homeostasis were studied. The homeostasis assessment (HOMA) model of Oxford University was used, and formulae were applied to calculate HOMA-insulin resistance (IR) or HOMA-β (β-cell function). Results: The status of glucose homeostasis was as follows: Normal glucose homeostasis tolerance 83/104(79.8%), PD 20/104(19.2%), and DM one(1%). The patient’s age, age of starting transfusions, and HOMA-IR were significantly related to AGH. AGH was inversely associated with the age of starting chelation, though not significant (P = 0.07). There was no statistical significance of AGH development, with transfusion dependence (P = 0.63), family history of DM (P = 0.42), hepatitis C (P = 0.36), and higher ferritin levels (800/1000/1500/1700 ng/ml) (P > 0.5)/HOMA-β (P > 0.5). Conclusion: HbEβ-thalassemia patients are prone to develop AGH including overt diabetes. It is related to the patient’s age, age of initiation, and duration of transfusion therapy. The likely mechanism of pathogenesis is IR, though pancreatic β-cell destruction may also be contributory.