@article{De Sanctis_Soliman_Tzoulis_Daar_Di Maio_Fiscina_Kattamis_2021, title={GLUCOSE METABOLISM AND INSULIN RESPONSE TO ORAL GLUCOSE TOLERANCE TEST (OGTT) IN PREPUBERTAL PATIENTS WITH TRANSFUSION DEPENDENT Β-THALASSEMIA (TDT): A LONG-TERM RETROSPECTIVE ANALYSIS: Long-term retrospective analysis of glucose homeostasis in children with transfusion dependent β-thalassemia}, volume={13}, url={https://www.mjhid.org/mjhid/article/view/4623}, DOI={10.4084/MJHID.2021.051}, abstractNote={<p> </p> <p style="margin: 0cm 0cm 0pt; ; mso-layout-grid-align: none;"><span style="font-family: Times New Roman;"><strong><span lang="EN-GB" style="color: black; mso-ansi-language: EN-GB;"><span style="font-size: medium;">Background</span></span></strong><span lang="EN-GB" style="font-size: 11.5pt; mso-ansi-language: EN-GB;">:</span><span style="font-size: medium;"> <span lang="EN" style="mso-ansi-language: EN;">G</span><span lang="EN-GB" style="mso-ansi-language: EN-GB;">lucose dysregulation (GD), including prediabetes and diabetes mellitus (DM), is a common complication of transfusion dependent </span><span lang="EL" style="mso-ansi-language: EL; mso-fareast-font-family: MinionPro-Regular;">β</span><span lang="EN-GB" style="mso-ansi-language: EN-GB;">-thalassemia (TDT) patients. The prevalence increases with age and magnitude of iron overload, affecting a significant proportion of patients. The development of GD is frequently asymptomatic and therefore an early diagnosis, according to the international guidelines, requires an annual oral glucose tolerance test (OGTT) in all TDT patients aged ten years or older. </span></span></span></p> <p> </p> <p style="margin: 0cm 0cm 0pt; ; mso-layout-grid-align: none;"><span style="font-family: Times New Roman;"><strong><span lang="EN-GB" style="color: black; mso-ansi-language: EN-GB;"><span style="font-size: medium;">Purpose:</span></span></strong> <span lang="EN-GB" style="letter-spacing: 0.05pt; mso-ansi-language: EN-GB;"><span style="font-size: medium;">This retrospective study aims to evaluate the prevalence of GD in a homogenous population of prepubertal TDT patients and to enhance understanding of the pathogenesis and progression of glucose homeostasis in this group of patients. </span></span></span></p> <p> </p> <p style="margin: 0cm 0cm 0pt; ; mso-layout-grid-align: none;"><span style="font-family: Times New Roman;"><strong><span lang="EN-GB" style="mso-ansi-language: EN-GB;"><span style="font-size: medium;">Methods</span></span></strong><span lang="EN-GB" style="font-size: 11.5pt; mso-ansi-language: EN-GB;">: </span><span style="font-size: medium;"><span lang="EN-GB" style="color: black; mso-ansi-language: EN-GB;">A selected group of 28 </span><span lang="EN-GB" style="mso-ansi-language: EN-GB;">TDT patients was followed for at least 10.3 years (range: 10.3 - 28.10 years) from prepubertal age (mean </span></span><span lang="EN-GB" style="font-size: 11pt; mso-ansi-language: EN-GB;">11.0 </span><span lang="EN-US" style="font-size: 11pt; mso-ansi-language: EN-US;">±</span> <span lang="EN-GB" style="mso-ansi-language: EN-GB;"><span style="font-size: medium;">standard deviation</span></span> <span lang="EN-US" style="font-size: 11pt; mso-ansi-language: EN-US;">1.1 years)</span><span style="font-size: medium;"><span lang="EN-GB" style="mso-ansi-language: EN-GB;"> to adulthood (28.7 </span><span lang="EN-US" style="mso-ansi-language: EN-US;">±</span><span lang="EN-GB" style="mso-ansi-language: EN-GB;"> 3.7 years). Glucose tolerance and insulin response to OGTT were assessed, and indices of β-cell function, insulin sensitivity and insulin secretion were calculated. </span></span></span></p> <p> </p> <p style="margin: 0cm 0cm 0pt; ; mso-layout-grid-align: none;"><span style="font-family: Times New Roman;"><strong><span lang="EN-GB" style="mso-ansi-language: EN-GB;"><span style="font-size: medium;">Results</span></span></strong><span lang="EN-GB" style="font-size: 11.5pt; mso-ansi-language: EN-GB;">:</span><span style="font-size: medium;"> <span lang="EN-GB" style="color: black; mso-ansi-language: EN-GB;">At baseline, 18 TDT patients had normal glucose tolerance (NGT) and 10 isolated impaired fasting glycaemia (IFG), according to the American Diabetes Association (ADA) criteria. Compared to 18 prepubertal healthy controls (mean </span><span lang="EN-US" style="mso-ansi-language: EN-US;">±</span></span><span lang="EN-GB" style="mso-ansi-language: EN-GB;"><span style="font-size: medium;"> SD <span style="color: black;">age: </span></span><span style="font-size: medium;">10.9 </span></span><span style="font-size: medium;"><span lang="EN-US" style="mso-ansi-language: EN-US;">±</span> <span lang="EN-US" style="mso-ansi-language: EN-US;">1.1 years), the fasting plasma glucose (</span><span lang="EN-GB" style="mso-ansi-language: EN-GB;">FPG), basal insulin level and </span><span lang="EN" style="mso-ansi-language: EN;">Homeostatic Model Assessment for Insulin Resistance (HOMA-IR) index</span> </span><span lang="EN-GB" style="mso-ansi-language: EN-GB;"><span style="font-size: medium;">were significantly higher in the group of TDT patients (p= 0.001, 0.01 and 0.012, respectively). At the last observation, 7/18 patients (38.8%) with NGT and 9/10 (90%) with IFG at baseline deteriorated; </span><span style="font-size: medium;">3 female patients developed type 2 DM (1 from the NGT group and 2 from the IFG group).</span></span><span style="font-size: medium;"><span lang="EN-GB" style="mso-ansi-language: EN-GB; mso-fareast-font-family: Swis721LtEU-Normal;"> Compared to adult controls, TDT patients with NGT had a reduced oral disposition index (DI)</span><span lang="EN-GB" style="mso-ansi-language: EN-GB;"> (p= </span></span></span><span style="font-size: medium;"><span lang="EN" style="font-family: Barlow; mso-ansi-language: EN;">0.006</span><span style="font-family: Times New Roman;"><span lang="EN-GB" style="mso-ansi-language: EN-GB;">), but no significant difference in HOMA-IR and Matsuda index. Conversely, </span><span lang="EN-US" style="mso-ansi-language: EN-US;">all insulin indices (HOMA-IR, MI and DI) but one [</span><span lang="EN-GB" style="mso-ansi-language: EN-GB; mso-fareast-font-family: Swis721LtEU-Normal;">insulinogenic index (</span><span lang="EN-US" style="mso-ansi-language: EN-US;">IGI)] were statistically different in TDT patients with GD compared to controls.</span> </span></span></p> <p> </p> <p style="margin: 0cm 0cm 0pt; ; mso-layout-grid-align: none;"><span style="font-size: medium;"><span style="font-family: Times New Roman;"><strong><span lang="EN-GB" style="mso-ansi-language: EN-GB;">Conclusion: </span></strong><span lang="EN-GB" style="mso-ansi-language: EN-GB;">This</span><span lang="EN" style="mso-ansi-language: EN;"> study shows a spectrum of disturbances in glucose homeostasis among TDT patients and that prepubertal patients with IFG are at higher risk for developing a deterioration of glucose metabolism.</span></span></span></p> <p> </p>}, number={1}, journal={Mediterranean Journal of Hematology and Infectious Diseases}, author={De Sanctis, Vincenzo and Soliman, Ashraf and Tzoulis, Ploutarchos and Daar, Shahina and Di Maio, Salvatore and Fiscina, Bernadette and Kattamis, Christos}, year={2021}, month={Aug.}, pages={e2021051} }