scholarly journals Anti-Thyroid Peroxidase Antibodies and Male Gender Are Associated with Diabetes Occurrence in Patients with Beta-Thalassemia Major

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Giovanni M. Pes ◽  
Francesco Tolu ◽  
Maria P. Dore

Background. Intensive transfusion schedule and iron-chelating therapy prolonged and improved quality of life in patients withβ-thalassemia (β-T) major. However, this led to an increased risk of developing impaired glucose tolerance or diabetes. In this study we analyzed variables associated with the occurrence of impaired glucose tolerance or diabetes in patients withβ-T major.Methods. 388 Sardinian patients were included. Age, gender, duration of chelation therapy, body mass index, and markers of pancreatic and extrapancreatic autoimmunity were analyzed.Results. Multiple logistic regression analysis showed that anti-thyroid peroxidase (TPO) antibodies (Ab) (OR = 3.36;p=0.008) and male gender (OR = 1.98;p=0.025) were significantly associated with glucose impairment, while the other variables were not. Ferritin levels were significantly higher in TPOAb positive compared to TPOAb negative patients (4870 ± 1665μg/L versus 2922 ± 2773μg/L;p<0.0001).Conclusions. In patients withβ-T major a progressive damage of insulin-producing cells due to secondary hemosiderosis appears to be the most reasonable mechanism associated with glucose metabolism disorders. The findings need to be confirmed with additional well designed studies to address the question of whether TPOAb may have a role in the management of these patients.


Circulation ◽  
2020 ◽  
Vol 141 (Suppl_1) ◽  
Author(s):  
Anshul Saxena ◽  
Muni Rubens ◽  
Venkataraghavan Ramamoorthy ◽  
Sankalp Das ◽  
Chintan B Bhatt ◽  
...  

Introduction: Cardiometabolic syndrome consists of a cluster of metabolic dysfunctions such as impaired glucose tolerance, insulin resistance, dyslipidemia, central adiposity, and hypertension. According to the latest estimates, globally, nearly 25% of all adults have cardiometabolic syndrome. Both cardiometabolic syndrome and cancer pathophysiology commonly involve inflammation and oxidative stress. The objective of this systematic review was to evaluate existing evidences that support the association between cardiometabolic syndrome and risk of developing cancer. Methods: We searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials and Scopus for relevant articles published from the database inception until October 2019. We followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines for this review. Using the Oxford Center for Evidence-Based Medicine guidelines individual studies were evaluated. A total of 59 articles were included in this study. Results: Our review showed that cardiometabolic syndrome was associated with increased risk for colorectal, hepatic, endometrial, breast, and bladder cancers. These associations showed variations for sex and geographical locations. For example, the associations were stronger for pancreatic and rectal cancers among women. The strength of these associations was also stronger for sex specific cancers such as breast and endometrial cancers. Studies on European populations showed that these associations were stronger for colorectal cancer among women. However, one study showed that presence of cardiometabolic syndrome contributed protective effects to prostate cancer among American men. In general, strongest associations were found for colorectal cancer among both men and women and hepatic cancer among men. Among cardiometabolic factors, impaired glucose tolerance and central adiposity were the greatest contributors towards increased risk for cancers. Conclusion: Given these results, there should be greater focus on primary prevention to identify and treat cardiometabolic risk factors. In addition, patients with greater cardiometabolic risk factors should be screened earlier and more frequently for cancers. A number of gender and geographical gaps identified in this review could be targeted for improvements as per the goals of 2030 sustainable development initiatives. Future studies should consider cardiometabolic syndrome and cancer together and develop effective interventions for decreasing the incidence and morbidity associated with both the conditions.



2019 ◽  
Vol 12 (1) ◽  
pp. 76-83 ◽  
Author(s):  
Uni Gamayani ◽  
Ni Luh Meidha Dini Lestari ◽  
Ahmad Rizal Ganiem ◽  
Ramdan Panigoro

Background: Children with beta-thalassemia major may suffer from working memory impairment. For a more refined understanding of this issue, we assessed working memory function in beta-thalassemia children and evaluated its influence on academic achievement and quality of life. Methods: This was a cross-sectional study involving 60 beta-thalassemia children aged 8-12 years. All participants underwent a working memory assessment using the digit span and were interviewed using academic achievement and Indonesian version of PedsQL 4.0 questionnaires. Working memory in beta-thalassemia children significantly influences their quality of life, both directly (β=0.32) and indirectly, through their academic achievement (β=0.639). Longer duration of transfusion (p=0.01) is significantly related to poorer working memory, while lower hemoglobin level (p=0.81) and higher ferritin level (p=0.24) are not significantly associated with working memory. Conclusion: We concluded that working memory influences the quality of life in beta-thalassemia children, both directly and indirectly, through their academic achievement.



Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3551-3551
Author(s):  
Alessia Pepe ◽  
Laura Pistoia ◽  
Saveria Campisi ◽  
Roberto Sarli ◽  
Piera Giovangrossi ◽  
...  

Background. A preliminary study involving 59 patients demonstrated that pancreatic iron assessed by magnetic resonance imaging (MRI) was the strongest overall predictor of glucose dysregulation in thalassemia major (TM) patients. Aim. In the present multicenter study we explored systematically the link between pancreatic iron and glucidic metabolism in a large cohort of TM patients. Methods. We considered 705 TM patients (372 F, mean age 37.00±9.95 years) enrolled in the E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project. T2* measurements were performed over pancreatic head, body and tail and global value was the mean. The pattern of disturbances of glucose metabolism was assessed by means of the oral glucose tolerance test (OGTT). Results. According to OGTT results, 546 patients (77.4%) had normal glucose tolerance (NGT), 14 (2.0%) had isolated impaired fasting glucose (IFG), 29 (4.1%) had impaired glucose tolerance (IGT), and 116 (16.5%) had diabetes mellitus (DM). None of the 85 patients (12.1%) without pancreatic iron overload (global pancreas T2*≥26 ms) had IGT or DM (Figure 1). The 84.6% of patients with NGT had pancreatic iron overload. The global pancreatic T2* values were significantly higher in patients with NGT than in patients with DM (13.58±11.08 ms versus 8.09±4.72 ms, P&lt;0.0001). Receiver operator characteristic (ROC) analysis showed that a global pancreas T2*&lt;13.73 ms was the optimal cutoff for predicting an abnormal OGTT, with an area under the curve (AUC) of 0.62. Global pancreas T2* values showed a weak significant correlation with insulin values (R=0.160; P=0.002) and homeostasis model assessment-insulin resistance (HOMA-IR) index (R=0.122; P=0.019). Conclusion. A normal global pancreas T2* value has a negative predictive value of 100% for IGT and DM. The low specificity of pancreatic iron overload for glucose dysregulation seems to support the hypothesis that a latency time is need before pancreatic iron burden could give impaired glucose tolerance and overt diabetes mellitus. Figure 1 Disclosures Pepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.



2019 ◽  
Vol 6 (1) ◽  
pp. 121-138
Author(s):  
Amal Ahmed Khalil ◽  
Mohamed Mahmoud Sarhan ◽  
Nagwa Rizk Mohammed ◽  
Neveen Moheb Mohammed Gomah


2021 ◽  
pp. 23-25
Author(s):  
Sudipta Bera ◽  
Tapobrata Biswas ◽  
Anuradha Sinha ◽  
Anindya Adhikari

Background: Hemoglobinopathies are one of the major health burden globally including India and Southeast Asian region. This study was carried out to nd out the prevalence of β-thalassemia, HbS, HbD, HbE as well as identication of asymptomatic carriers who have an increased risk of having a child with thalassemia in the western part of West Bengal. Materials and methods: In this retrospective cohort study, the data was collected from all newly registered patients with anemia, referred from different outdoor patients in a tertiary care hospital, attending thalassemia clinic over a period of 3 years from January2018 to December2020. Detailed clinical history was taken and blood samples collected are tested with Sysmex automated blood cell counter for red cell indices. Diagnosis of hemoglobinopathy was done by G8 HPLC (high performance liquid chromatography) Analyzer by TOSOH Bioscience. Results: A total 2297 cases were studied during the study period. The age of the patients ranged between 6 months to 60 years. Higher percentage of the study sample was at age of more than 10 years (93.8%). Among 2297 cases, 477 cases showed abnormal Hb fractions on HPLC. The following Hb abnormalities detected were following: β (beta) thalassemia trait 13.2%, β thalassemia major 2.3% followed by sickle cell trait 2.17%, HbE trait 1.8%, HbS disease 0.21%, Hb E disease 0.13%, hereditary persistence of fetal hemoglobin (HPFH) traits 0.17% and Hb D trait 0.08% along with HbE-β-thalassemia 0.30% and HbS-β-thalassemia 0.26%. Conclusion: Among the hemoglobinopathies, β-thalassemia trait (12.9%) is prevalent in western part of Bengal.



Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3848-3848
Author(s):  
Anupam Sachdeva ◽  
Satya Prakash Yadav ◽  
Subash C. Arya ◽  
Virender K. Khanna ◽  
Archana D. Arya

Abstract Abnormalities of the glucose metabolism are well documented in patients with Thalassemia Major who are frequently transfused and receiving therapy for chelation, due to excess iron deposition in the pancreas. The incidence of abnormalities in the glucose metabolism increase with age, with peak incidence between 16–20 years. The Indian (Asian) population is genetically predisposed to developing type 2 diabetes mellitus which is an additional risk factor for our Thalassemic population. Chelation is suboptimal in most of the patients due to economic reasons and ignorance. Impaired glucose tolerance (IGT) usually precedes the development of frank diabetes mellitus and intensive chelation in those with impaired glucose tolerance test may delay/prevent the onset of diabetes mellitus. Hence it is important to know the glyco-metabolic status of these children. At our Thalassemia endocrinology clinic, glucose tolerance test (GTT) is performed routinely in all subjects with Thalassemia major who have not already developed diabetes to identify the “at risk” population.GTT is performed by drawing a baseline fasting sample for blood glucose, oral glucose was given in a dose of 1.75mg/kg upto a maximum of 75 gms. Blood glucose level is measured 2 hours after oral glucose. According to the WHO criteria, Fasting plasma glucose between 110–126mg/dl is classified as impaired fasting and above 126mg/dl as diabetes. 2-hour plasma glucose value between 140–200mg/dl is classified as impaired glucose tolerance and above 200 mg/dl as diabetes. The purpose of this study was to analyze the status of the glucose metabolism of children and young adults with Thalassemia major who were attending our Thalassemia endocrinology clinic and to compare the factors affecting subjects with an abnormal glucose metabolism with those who have a normal glucose metabolism. The parameters compared were: effect of mean S. ferritin levels, age of onset of chelation and genetic predisposition. Retrospective analysis of our case records was done to determine the prevalence of diabetes and impaired glucose tolerance in children and young adults between 13 and 25 years of age. Of the 33 subjects evaluated, 16 out of 33 (48.5%) subjects had an abnormality of the glucose metabolism. 14/33 subjects (42.4%) had developed diabetes mellitus and 2 had an impaired GTT. Of the 16 affected subjects 9 were males and 7 were females (M:F = 1.28:1). The mean serum ferritin for this group was 5464ng/ml, 5503ng/ml for the diabetic group and 5425 for those with impaired GTT. (Range 2523–10904ng/ml). History of diabetes in a first or second degree relative was positive in 9 subjects(56.25%), negative in 2 and unknown in 5 subjects. Average age of onset of chelation was 8 years in this group. Oral glucose tolerance test was normal in 17/33(51.5%) subjects of which 10 were males and 7 were females (1.42:1). Average serum ferritin was 4747.4ng/ml in the group with a normal glucose tolerance. (1600–8294ng/ml). Family history of diabetes in a first or second degree relative was positive in 8 subjects(47%), negative in 4 and unknown in 5 subjects. Average age of onset of chelation was 6.5 years in the group with normal glucose metabolism. In conclusion of the 33 subjects evaluated, 48.5% had an abnormal glucose metabolism.



Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3344-3344 ◽  
Author(s):  
Lorenzo Giovanni Mantovani ◽  
Luciana Scalone ◽  
Simona Ravera ◽  
Diana Rofail ◽  
Maria Domenica Cappellini ◽  
...  

Abstract Background Patients with beta Thalassemia Major (TM) require life-long blood transfusions, which often cause iron overload that may increase patients’ morbidity and mortality. Iron Chelation Treatment (ICT), aimed to reduce iron overload, is based on 8–12 hour infusions of Deferoxamine (DFO) for 5–7 days/week, and/or Deferiprone (L1) orally administered. Current ICT can be related to low satisfaction, low compliance, and potentially negative consequences on clinical effectiveness, patients’ wellbeing and on healthcare costs. Aims: To investigate the Health-Related Quality-of-Life (HRQoL) of TM patients and their satisfaction with ICT. Methods: The Italian-THAlassemia-Cost-&-Outcomes-Assessment (ITHACA) was a naturalistic multicentre study conducted to evaluate costs, HRQoL, compliance and treatment satisfaction in TM patients undergoing ICT for at least 3 years, enrolled at Italian Thalassemia Care Centers. HRQoL was measured in >14 years old patients with 2 generic instruments: EQ-5D; Short Form-36 (SF-36). To measure satisfaction >12 years old patients received a 28-item instrument consisting of 4 domains: ‘perceived effectiveness’, ‘acceptance’, ‘burden’, and ‘side effects’. Each domain scored from from 1 (very dissatisfied) to 5 (very satisfied). Results Based on 126 patients: median age 29.4 years (12.3–48.5), 49.6% male. At enrolment 48.0% were using DFO, 33.6% L1, 18.4% were treated with DFO+L1. 86.5% of patients had at least one TM-related complication, 13.5% changed treatment regimen at least once in a median of 11.6 months before enrolment. With EQ-5D profile patients reported moderate problems with ‘mobility’ (9.1%), ‘self care’ (0.8%), ‘usual activities’ (23.5%), moderate or severe ‘pain/discomfort’ (60.5%) and ‘anxiety/depression’ (39.5%). Mean EQ-5D-Visual Analogue Scale was 73.0 (30–100). The SF-36 Physical Component Summary mean(SD) score was 47.7(8.4), while the mean score estimated in the Italian general population comparable for age and sex is 53.3; the Mental Component Summary mean(SD) score in TM patients was 45.1(8.8), while the Italian general population mean score was 47.7. Mean satisfaction scores were 4.29 (perceived effectiveness), 3.37 (acceptance), 3.87 (burden), and 3.57 (side effects). Simple linear regression analysis showed that satisfaction with burden (r2=12.6%, p<0.0001), side effects (r2=12.3%, p<0.0001) and acceptance(r2=11.3%, p=0.0001), are positively associated with the adherence item ‘never thinking about stopping medication’ (p<0.0001 in both cases). Conclusions: TM patients on ICT have impaired levels of physical and mental HRQoL. Therapies improving patients’ satisfaction and compliance to ICT may have positive consequences not only on clinical effectiveness but also on overall patients’ well-being.



2018 ◽  
Vol 2018 ◽  
pp. 1-16 ◽  
Author(s):  
Meicen Zhou ◽  
Shuli He ◽  
Fan Ping ◽  
Wei Li ◽  
Lixin Zhu ◽  
...  

Objective. To investigate the association of polymorphisms in uncoupling protein 2 (UCP2) and peroxisome proliferator-activated receptor (PPARγ) with glucolipid metabolism in Chinese Han population. Methods. Five hundred eighty-nine subjects were divided into normal glucose tolerance (NGT) group (n=198) and abnormal glucose tolerance group (n=358). HbA1c, blood lipid profile, plasma glucose, and insulin were determined. Insulin sensitivity (HOMA-IR and Matsuda index (ISIM)) and insulin secretion indexes (HOMA-β, early and total phase disposition index) were evaluated. Eight potential functional SNPs in UCP2 and 7 in PPARγ were selected. SNPs were genotyped on Sequenom MassARRAY platform. Results. The GG genotype of rs2920502 in PPARγ was associated with decreased risk of impaired glucose tolerance (G allele: OR: 0.818, 95%CI: 0.526–0.969, P=0.042; GG: OR: 0.715, 95%CI: 0.527–0.97, P=0.031). The TT genotype of rs3856806 in PPARγ was associated with increased risk of impaired glucose tolerance (T allele: OR: 1.46, 95%CI: 1.055–2.017, P=0.022; TT: OR: 1.58, 95%CI: 1.104–2.761, P=0.032). The GG genotype of rs2920502 in PPARγ had better blood glucose and increased insulin secretion and had lower HOMA-IR than GC/CC genotypes. Conclusion. It probably could prevent insulin resistance in early stage by classifying the genotype of rs649446 and rs7109266 in UCP2. The GG genotype of rs2920502 in PPARγ had a decreased risk for diabetes. The TT genotype of rs3856806 in PPARγ had an increased risk for diabetes.



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