The ICET-A Recommendations for the Diagnosis and Management of Disturbances of Glucose Homeostasis in Thalassemia Major Patients

Overview

Journal Mediterr J Hematol Infect Dis

Specialty Infectious Diseases

Date 2016 Nov 23

PMID 27872738

Citations 24

Authors

Vincenzo De Sanctis

Ashraf T Soliman

Heba Elsedfy

Saif Al Yaarubi

Nicos Skordis

Doaa Khater

Mohamed El Kholy

Iva Stoeva

Bernadette Fiscina

Michael Angastiniotis

Shahina Daar

Christos Kattamis

Affiliations

Soon will be listed here.

Abstract

Iron overload in patients with thalassemia major (TM) affects glucose regulation and is mediated by several mechanisms. The pathogenesis of glycaemic abnormalities in TM is complex and multifactorial. It has been predominantly attributed to a combination of reduced insulin secretory capacity and insulin resistance. The exact mechanisms responsible for progression from norm glycaemia to overt diabetes in these patients are still poorly understood but are attributed mainly to insulin deficiency resulting from the toxic effects of iron deposited in the pancreas and insulin resistance. A group of endocrinologists, haematologists and paediatricians, members of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine (ICET-A) convened to formulate recommendations for the diagnosis and management of abnormalities of glucose homeostasis in thalassemia major patients on the basis of available evidence from clinical and laboratory data and consensus practice. The results of their work and discussions are described in this article.

Citing Articles

Pancreatic Volume in Thalassemia: Determinants and Association with Alterations of Glucose Metabolism.

Meloni A, Restaino G, Positano V, Pistoia L, Keilberg P, Santodirocco M Diagnostics (Basel). 2025; 15(5).

PMID: 40075815 PMC: 11899254. DOI: 10.3390/diagnostics15050568.

Risk Factors for Impaired Glucose Metabolism in Transfusion-Dependent Patients with β-Thalassemia: A Single-Center Retrospective Observational Study.

Venou T, Kyriakidis F, Barmpageorgopoulou F, Theodoridou S, Vyzantiadis A, Klonizakis P Hematol Rep. 2025; 17(1).

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Left ventricular diastolic and systolic functions by cardiac magnetic resonance in beta-thalassemia major: correlation with clinical findings and cardiac complications.

Meloni A, Saba L, Positano V, Taccori M, Pistoia L, De Marco E Int J Cardiovasc Imaging. 2025; .

PMID: 39928284 DOI: 10.1007/s10554-025-03352-7.

A Multicenter ICET-A Survey on Adherence to Annual Oral Glucose Tolerance Test (OGTT) Screening in Transfusion-Dependent Thalassemia (TDT) Patients - The Expert Clinicians' Opinion on Factors Influencing the Adherence and on Alternative Strategies....

De Sanctis V, Canatan D, Daar S, Kattamis C, Banchev A, Modeva I Mediterr J Hematol Infect Dis. 2025; 17(1):e2025008.

PMID: 39830799 PMC: 11740908. DOI: 10.4084/MJHID.2025.008.

Left and right atrioventricular coupling index in patients with beta-thalassemia major.

Meloni A, Saba L, Positano V, Pistoia L, Spasiano A, Putti M Int J Cardiovasc Imaging. 2024; 40(8):1631-1640.

PMID: 38775931 DOI: 10.1007/s10554-024-03146-3.

References

Loebstein R, Lehotay D, Luo X, Bartfay W, Tyler B, Sher G . Diabetic nephropathy in hypertransfused patients with beta-thalassemia. The role of oxidative stress. Diabetes Care. 1998; 21(8):1306-9. DOI: 10.2337/diacare.21.8.1306. View

. Standards of medical care in diabetes. Diabetes Care. 2004; 28 Suppl 1:S4-S36. View

Eslam M, Kawaguchi T, del Campo J, Sata M, Khattab M, Romero-Gomez M . Use of HOMA-IR in hepatitis C. J Viral Hepat. 2011; 18(10):675-84. DOI: 10.1111/j.1365-2893.2011.01474.x. View

Giardina P, Grady R . Chelation therapy in beta-thalassemia: an optimistic update. Semin Hematol. 2001; 38(4):360-6. DOI: 10.1016/s0037-1963(01)90030-7. View

Incorvaia C, Parmeggiani F, Mingrone G, Sebastiani A, De Sanctis V . Prevalence of retinopathy in diabetic thalassaemic patients. J Pediatr Endocrinol Metab. 1999; 11 Suppl 3:879-83. View

Weatherall D . The definition and epidemiology of non-transfusion-dependent thalassemia. Blood Rev. 2012; 26 Suppl 1:S3-6. DOI: 10.1016/S0268-960X(12)70003-6. View

Suvarna J, Ingle H, Deshmukh C . Insulin resistance and beta cell function in chronically transfused patients of thalassemia major. Indian Pediatr. 2006; 43(5):393-400. View

Tahara Y, Shima K . Kinetics of HbA1c, glycated albumin, and fructosamine and analysis of their weight functions against preceding plasma glucose level. Diabetes Care. 1995; 18(4):440-7. DOI: 10.2337/diacare.18.4.440. View

Soliman A, DeSanctis V, Yassin M, Elalaily R, Eldarsy N . Continuous glucose monitoring system and new era of early diagnosis of diabetes in high risk groups. Indian J Endocrinol Metab. 2014; 18(3):274-82. PMC: 4056122. DOI: 10.4103/2230-8210.131130. View

10.

De Sanctis V, Soliman A, Yassin M . Iron overload and glucose metabolism in subjects with β-thalassaemia major: an overview. Curr Diabetes Rev. 2013; 9(4):332-41. DOI: 10.2174/1573399811309040005. View

11.

De Sanctis V, Zurlo M, Senesi E, Boffa C, Cavallo L, Di Gregorio F . Insulin dependent diabetes in thalassaemia. Arch Dis Child. 1988; 63(1):58-62. PMC: 1779356. DOI: 10.1136/adc.63.1.58. View

12.

Noetzli L, Carson S, Nord A, Coates T, Wood J . Longitudinal analysis of heart and liver iron in thalassemia major. Blood. 2008; 112(7):2973-8. PMC: 2556627. DOI: 10.1182/blood-2008-04-148767. View

13.

Mangiagli A, Campisi S, De Sanctis V, Caruso Nicoletti M, Cardinale G, Galati M . Effects of acarbose in beta-thalassaemia major patients with normal glucose tolerance and hyperinsulinism. Pediatr Endocrinol Rev. 2006; 2 Suppl 2:272-5. View

14.

Noetzli L, Papudesi J, Coates T, Wood J . Pancreatic iron loading predicts cardiac iron loading in thalassemia major. Blood. 2009; 114(19):4021-6. PMC: 2774543. DOI: 10.1182/blood-2009-06-225615. View

15.

Khattab M, Eslam M, Sharwae M, Shatat M, Ali A, Hamdy L . Insulin resistance predicts rapid virologic response to peginterferon/ribavirin combination therapy in hepatitis C genotype 4 patients. Am J Gastroenterol. 2010; 105(9):1970-7. DOI: 10.1038/ajg.2010.110. View

16.

Noetzli L, Panigrahy A, Mittelman S, Hyderi A, Dongelyan A, Coates T . Pituitary iron and volume predict hypogonadism in transfusional iron overload. Am J Hematol. 2012; 87(2):167-71. DOI: 10.1002/ajh.22247. View

17.

De Sanctis V, Soliman A, Elsedfy H, Pepe A, Kattamis C, El Kholy M . Diabetes and Glucose Metabolism in Thalassemia Major: An Update. Expert Rev Hematol. 2015; 9(4):401-8. DOI: 10.1586/17474086.2016.1136209. View

18.

Hafez M, Youssry I, El-Hamed F, Ibrahim A . Abnormal glucose tolerance in beta-thalassemia: assessment of risk factors. Hemoglobin. 2009; 33(2):101-8. DOI: 10.1080/03630260902817131. View

19.

Mangiagli A, Italia S, De S, Campisi S . Impaired glucose homeostasis in young adult thalassemic patients: a pilot study with acarbose. J Pediatr Endocrinol Metab. 2002; 15(2):205-10. DOI: 10.1515/jpem.2002.15.2.205. View

20.

Zamani F, Razmjou S, Akhlaghpoor S, Eslami S, Azarkeivan A, Amiri A . T2* magnetic resonance imaging of the liver in thalassemic patients in Iran. World J Gastroenterol. 2011; 17(4):522-5. PMC: 3027020. DOI: 10.3748/wjg.v17.i4.522. View