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

Sanctis, Vincenzo De

doi:10.4084/mjhid.2016.058

Cited by 33 publications

(40 citation statements)

References 68 publications

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“…Disturbances of glucose homeostasis, ranging from mild glucose intolerance to overt diabetes mellitus, and hyperinsulinism were reported in young adult patients with thalassemia and have been attributed to iron overload, HCV infection, anemia, and chronic liver disease. 66,67 An acute effect of blood transfusion on insulin sensitivity and β-cell function in patients with thalassemia has been reported by Wankanit et al 68 Alcohol and Tobacco. Alcohol and iron are known prooxidants, and oxidative stress is known to play an essential role in the development of several diseases, including cancer.…”

Section: Pathophysiology Past and Recent Issuesmentioning

confidence: 94%

Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (Hcc) in Β-Thalassemias: Past, Present and Future Perspectives

Sanctis¹,

Soliman

Daar

et al. 2020

Mediterr J Hematol Infect Dis

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Due to the recent alarming increase in the incidence of hepatocellular carcinoma (HCC) in thalassemias, the aim of the present report is to review briefly the frequency, the major risk factors and the surveillance of HCC in β-thalassemias. Over the past 33 years, 153 cases of HCC were reported in patients with thalassemia, mainly in Italy, and Greece. Among HCV-infected patients additional factors promoting development of HCC, included: advanced age, male sex, chronic hepatitis B (CHB) coinfection, and iron overload. For early diagnosis of HCC sequential ultrasound screening is recommended especially for thalassemia patients with chronic hepatitis C (CHC), that coincide with (one or more) additional risk factors for HCC. Here we report also the preliminary data of thalassemic patients, above the age of 30 years, followed in 13 different centers. The total number of enrolled patients was 1,313 (males: 612 and 701 females). The prevalence of HCC in thalassemia major patients [characterized by transfusion-dependency (TDT)] and thalassemia intermedia [characterized by nontransfusion dependency (NTDT)] was 1.68 % and 1.98 % ,respectively The lowest age at diagnosis was 36 years in TDT and 47 years in NTDT patients.We hope that our review can be used to develop more refined and prospective analyses of HCC magnitude and risk in patients with thalassemia, and to define specific international guidelines to support clinicians for an early diagnosis and treatment of HCC in thalassemic patients.

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Section: Pathophysiology Past and Recent Issuesmentioning

confidence: 94%

Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (Hcc) in Β-Thalassemias: Past, Present and Future Perspectives

Sanctis¹,

Soliman

Daar

et al. 2020

Mediterr J Hematol Infect Dis

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“… 21 Because of the increased risk of glucose disturbances in β-TDT patients with iron overload, there are recommendations for annual evaluation of glycemic status in children over ten years. 22 …”

Section: Discussionmentioning

confidence: 99%

First-phase insulin response (FPIR) to intravenous glucose tolerance test (IVGTT), insulin sensitivity and long-term follow-up in ?- transfusion-dependent thalassemia (TDT) normoglycemic patients with reduced insulin secretion to oral glucose tolerance t

Sanctis¹,

Soliman

Daar

et al. 2021

Mediterr J Hematol Infect Dis

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Summary. Objective: To study the function of the endocrine pancreas in transfusion-dependent ?-thalassemia (?-TDT) patients with normal oral glucose tolerance test (OGTT) and hypoinsulinemia. Patients and methods: Seven ?-TDT patients (mean age 22.4 ± 4.2 years) with normal glucose tolerance test (NGT) and poor insulin response (hypoinsulinemia) to OGTT, not associated with ?-cell autoimmunity, were referred for a second opinion to an Italian Centre, part of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine (ICET-A). In this pilot study, the first-phase insulin response (FPIR), expressed as the sum of 1 and 3 minutes insulin, of ?-TDT patients to intravenous glucose tolerance test (IVGTT), was tested. Moreover, the long-term natural history was followed prospectively using an annual OGTT, with the aim of detecting any abnormality of glucose metabolism. Results: The FPIR value was between the 1st and 3rd percentile in two patients and between the 3rd and 10th percentile in five. After 43 ± 26 months (range 11 - 80 months) of follow-up, 2 patients developed impaired glucose tolerance (IGT), 3 both IGT and impaired fasting glucose (IFG) and two overt diabetes mellitus (DM). Interestingly, the patients who developed DM had, at baseline the lowest value of insulinogenic index (IGI, 0.08 and 0.25), defined as the ratio of the increment of plasma insulin to plasma glucose during the first 30 minutes after OGTT. Moreover, a significant correlation was found between the IGI at baseline and at follow-up in the patients who developed IGT with or without IFG (R= 0.927; P: 0.023). A significant reduction of Matsuda insulin sensitivity index (ISIM) and Insulin Secretion-Sensitivity Index-2 (ISSI-2) was documented in the study cohort at diagnosis of IFG, IGT and DM. There was a significant inverse correlation between ISSI-2 and area under the curve of plasma glucose (AUC-PG). Conclusions: These data demonstrated, for the first time, a progressive deterioration in glucose homeostasis in ?-TDT subjects with NGT and hypoinsulinemia. Thus, we consider that variations of insulin sensitivity could possibly have an impact on glucose tolerance in adult patients with TDT. Further investigations should focus on factors that might positively influence insulin sensitivity, including nutrition, drugs and physical activity.

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“…We included β‐TM patients with serum ferritin ≥1000 μg/L because this cutoff denotes iron‐overload and predisposes to complications. It has been linked to endocrine dysfunction and glucose abnormalities in several studies . We found that 70% of the 20 β‐TM patients with RBG ≥7.8 mmol/L had serum ferritin ≥2500 mg/dL, with a median serum ferritin of 4750 (2304.5‐6500) μg/L.…”

Section: Discussionmentioning

confidence: 99%

“…It has been linked to endocrine dysfunction and glucose abnormalities in several studies. [40][41][42] We found that 70% of the 20 β-TM patients with Abbreviations: AUC, area under the curve; β-TM, β-thalassemia major; FBG, fasting blood glucose; HOMA-IR, homeostatic model assessment insulin resistance; HBA1c, hemoglobin A1c; IQR, interquartile range; MAD, mean average deviation; PPBG: postprandial blood glucose; RBG: random blood glucose; . have abnormal glucose levels in the diabetic range and three had IGT.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of continuous glucose monitoring system for detection of alterations in glucose homeostasis in pediatric patients with β-thalassemia major

et al. 2018

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Background Disturbances of glucose metabolism are common in β‐thalassemia major (β‐TM). Aim This study was conducted to assess the pattern of glucose homeostasis in pediatric β‐TM patients comparing oral glucose tolerance test (OGTT) and continuous glucose monitoring system (CGMS). Methods Two‐hundred β‐TM patients were studied and those with random blood glucose (RBG) ≥7.8 mmol/L (140 mg/dL) were subjected to OGTT, insertion of CGMS and measurement of fasting C peptide, fasting insulin, and hemoglobin A1c (HbA1c). Results Twenty patients (10%) had RBG ≥ 7.8 mmol/L. Using OGTT, 6 out of 20 patients (30%) had impaired glucose tolerance (IGT) while 7 (35%) patients were in the diabetic range. CGMS showed that 7/20 (35%) patients had IGT and 13 (65%) patients had diabetes mellitus (DM); 10 of the latter group had HbA1c readings within diabetic range. The percentage of diabetic patients diagnosed by CGMS was significantly higher than that with OGTT (P = 0.012). Serum ferritin was the only independent variable related to elevated RBG. All β‐TM patients with DM were non‐compliant to chelation therapy. Conclusions The use of CGMS in the diagnosis of early glycemic abnormalities among pediatric patients with β‐TM appears to be superior to other known diagnostic modalities.

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

Cited by 33 publications

References 68 publications

Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (Hcc) in Β-Thalassemias: Past, Present and Future Perspectives

Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (Hcc) in Β-Thalassemias: Past, Present and Future Perspectives

First-phase insulin response (FPIR) to intravenous glucose tolerance test (IVGTT), insulin sensitivity and long-term follow-up in ?- transfusion-dependent thalassemia (TDT) normoglycemic patients with reduced insulin secretion to oral glucose tolerance t

Evaluation of continuous glucose monitoring system for detection of alterations in glucose homeostasis in pediatric patients with β-thalassemia major

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