Electrocardiographic Findings in Iranian Children with Severe Chronic Anemia

Gharib, Reza; Ayazi, Shahraini

doi:10.1177/000992287201101110

Cited by 4 publications

(2 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…syndromes has been conducted in Iran, and unfortunately, no systematic large-scale investigation has been performed to clarify the status of thalassemia in Iran [17,18].…”

Section: Discussionmentioning

confidence: 99%

Prevalence and Genetic Analysis of α- and β-Thalassemia and Sickle Cell Anemia in Southwest Iran

Nezhad

Choghakabodi

et al. 2018

JEGH

View full text Add to dashboard Cite

This prospective study assessed the prevalence and genetic analysis of α - and β -thalassemia and sickle cell anemia (SCA) in Southwest Iran. Hematological indices were measured in 17,581 couples living in Khuzestan Province, Southwest Iran. Individuals with mean corpuscular volume <80, mean corpuscular hemoglobin <27, hemoglobin A2 ≥3/5 were considered as β -thalassemia traits. Prevalence of minor β -thalassemia, α -thalassemia, SCA, iron deficiency anemia, and silent thalassemia were respectively identified in 995 (5.6%), 1169 (6.65%), 1240 (7.05%), 911 (5.18%), and 1134 (6.45%) individuals using a multiplex amplification refractory mutation system, and direct DNA sequencing of globin genes. Three codons IVS-II-1 (G → A; 26%; n = 13), IVS-I-1 (G → T; 16%; n = 8), and IVS-I-110 (G → A; 14%; n = 7) were the most frequent mutants and IVS-II-1 was the most common β -thalassemia mutation. Also, based on a gap-polymerase chain reaction assay, genotype frequencies of α -globin mutations were − α 3.7 kb (50%; n = 25), Med/ αα thal (12%; n = 6), and − α 4.2/ αα (10%; n = 5), which were the most frequent deletion mutants (72% in total). The most common deletion (50%) was − α 3.7 kb . Our data suggest that the population of Southwest Iran is at high risk of α - and β -thalassemia caused by these deletion mutants and SCA. Our findings will be useful for developing an efficient control program and genetic counseling.

show abstract

“…syndromes has been conducted in Iran, and unfortunately, no systematic large-scale investigation has been performed to clarify the status of thalassemia in Iran [17,18].…”

Section: Discussionmentioning

confidence: 99%

Prevalence and Genetic Analysis of α- and β-Thalassemia and Sickle Cell Anemia in Southwest Iran

Nezhad

Choghakabodi

et al. 2018

JEGH

View full text Add to dashboard Cite

show abstract

“…Electrocardiographic abnormalities have been well documented in both the pre- and postchelation era [32, 33]. New-onset electrocardiographic abnormalities are usually evident in β -TM patients with HF and may include electrocardiographic findings that suggest left-sided heart (Q1S3 pattern and extreme left-axis deviation) or right-sided heart involvement (S1Q3 pattern and right-axis deviation), T-wave inversion beyond lead V1, P-wave prolongation or abnormalities and a consistent decrease in QRS amplitude.…”

Section: Diagnostic Workup and Arrhythmic Risk Markersmentioning

confidence: 99%

Arrhythmias and Sudden Cardiac Death in Beta-Thalassemia Major Patients: Noninvasive Diagnostic Tools and Early Markers

Russo

Melillo

Papa

et al. 2019

Cardiology Research and Practice

View full text Add to dashboard Cite

Beta-thalassemias are a group of inherited, autosomal recessive diseases, characterized by reduced or absent synthesis of beta-globin chains of the hemoglobin tetramer, resulting in variable phenotypes, ranging from clinically asymptomatic individuals to severe anemia. Three main forms have been described: heterozygotes, homozygotes β+, and homozygotes β°. Beta-thalassemia major (β-TM), the most serious form, is characterized by an absent synthesis of globin chains that are essential for hemoglobin formation, causing chronic hemolytic anemia. Cardiac complications represent a leading cause of mortality in β-TM patients, although an important and progressive increase of life expectancy has been demonstrated after the introduction of chelating therapies. Iron overload is the primary factor of cardiac damage resulting in thalassemic cardiomyopathy, in which diastolic dysfunction usually happens before systolic impairment and overt heart failure (HF). Although iron-induced cardiomyopathy is slowly progressive and it usually takes several decades for clinical and laboratory features of cardiac dysfunction to manifest, arrhythmias or sudden death may be present without signs of cardiac disease and only if myocardial siderosis is present. Careful analysis of electrocardiograms and other diagnostic tools may help in early identification of high-risk β-TM patients for arrhythmias and sudden cardiac death.

show abstract

Electrocardiographic consequences of cardiac iron overload in thalassemia major

et al. 2011

View full text Add to dashboard Cite

Background Iron cardiomyopathy is a leading cause of death in transfusion dependent thalassemia major (TM) patients and MRI (T2*) can recognize preclinical cardiac iron overload, but, is unavailable to many centers. Design and Methods We evaluated the ability of 12-lead electrocardiography to predict cardiac iron loading in TM. 12-lead electrocardiogram and cardiac T2* measurements were performed prospectively, with a detectable cardiac iron cutoff of T2*less than 20 ms. Patients with and without cardiac iron were compared using two-sample statistics and against population norms using age and gender-matched Z-scores. Results 45/78 patients had detectable cardiac iron. Patients having cardiac iron were older and more likely female but had comparable liver iron burdens and serum ferritin. Increased heart rate (HR) and prolonged corrected QT interval (QTc) were present, regardless of cardiac iron status. Repolarization abnormalities were the strongest predictors of cardiac iron, including QT/QTc prolongation, left shift of T-wave axis, and interpretation of ST/T-wave morphology. Recursive partitioning of the data for females using T-axis and HR and for males using QT, HR and T-axis produced algorithms with AUROC’s of 88.3 and 87.1 respectively. Conclusions Bradycardia and repolarization abnormalities on 12-lead electrocardiography were the most specific markers for cardiac iron in thalassemia major. Changes in these variables may be helpful to stratify cardiac risk when cardiac MRI is unavailable. However, diagnostic algorithms need to be vetted on larger and more diverse patient populations and longitudinal studies are necessary to determine reversibility of the observed abnormalities.

show abstract

Electrocardiographic Findings in Iranian Children with Severe Chronic Anemia

Cited by 4 publications

References 14 publications

Prevalence and Genetic Analysis of α- and β-Thalassemia and Sickle Cell Anemia in Southwest Iran

Prevalence and Genetic Analysis of α- and β-Thalassemia and Sickle Cell Anemia in Southwest Iran

Arrhythmias and Sudden Cardiac Death in Beta-Thalassemia Major Patients: Noninvasive Diagnostic Tools and Early Markers

Electrocardiographic consequences of cardiac iron overload in thalassemia major

Contact Info

Product

Resources

About