2,737 results match your criteria Thalassemia Imaging

The tailor-made treatment in a particular case of pulmonary hypertension in thalassaemia intermedia: a case report.

Eur Heart J Case Rep 2021 Jun 16;5(6):ytab193. Epub 2021 Jun 16.

Dyspnea Lab, Department Clinical Sciences and Community Health, University of Milan, Italy.

Background: Pulmonary hypertension (PH) is a haemodynamic condition, secondary to different causes. Thalassaemia may lead to PH of different origin and needs a comprehensive analysis to be correctly characterized and possibly treated.

Case Summary: We present a case study of a patient with a non-transfusion-dependent thalassaemia and a previous diagnosis of group 5 PH. Read More

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Correlation Between Serum Ferritin and Viral Hepatitis in Thalassemia Patients.

Hemoglobin 2021 Jun 14:1-5. Epub 2021 Jun 14.

Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Serum ferritin is an acute phase protein; importantly, its level is noticeably increased in response to iron overload and systemic inflammation. The iron overload status in thalassemia patients has been recognized as a potential way to measure liver iron concentration (LIC) levels using magnetic resonance imaging (MRI). The aim of this study was to investigate the effect of chronic viral hepatitis on the level of serum ferritin in patients with thalassemia. Read More

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Myocardial iron overload by cardiovascular magnetic resonance native segmental T1 mapping: a sensitive approach that correlates with cardiac complications.

J Cardiovasc Magn Reson 2021 Jun 14;23(1):70. Epub 2021 Jun 14.

Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Background: We compared cardiovascular magnetic resonance segmental native T1 against T2* values for the detection of myocardial iron overload (MIO) in thalassaemia major and we evaluated the clinical correlates of native T1 measurements.

Methods: We considered 146 patients (87 females, 38.7 ± 11. Read More

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How does iron deposition modify the myocardium? A feature-tracking cardiac magnetic resonance study.

Int J Cardiovasc Imaging 2021 Jun 8. Epub 2021 Jun 8.

Trauma Research Center, Rajaee (Emtiaz) Trauma Hospital, Shiraz University of Medical Sciences, Shiraz, Iran, Chamran Boulevard, Shiraz, Iran.

Iron-overload cardiomyopathy is the principal cause of mortality in thalassemia. Via feature-tracking cardiac magnetic resonance (FT-CMR), we investigated alterations in cardiac deformation with the progression in myocardial iron overload (MIO). We enrolled 154 patients with thalassemia (50. Read More

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Carvedilol improves left ventricular diastolic dysfunction in patients with transfusion-dependent thalassemia.

Ann Pediatr Cardiol 2021 Apr-Jun;14(2):152-158. Epub 2021 May 3.

Cardiac Electrophysiology Research and Training Center, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Background: Iron overload cardiomyopathy is the most common cause of death in patients with transfusion-dependent thalassemia.

Aim: The aim of this study was to determine the efficacy of carvedilol treatment in patients with transfusion-dependent thalassemia who had left ventricular diastolic dysfunction.

Methods: Eighteen patients with transfusion-dependent thalassemia who had left ventricular diastolic dysfunction were enrolled. Read More

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Quantitative T2* imaging of iron overload in a non-dedicated center - Normal variation, repeatability and reader variation.

Eur J Radiol Open 2021 24;8:100357. Epub 2021 May 24.

Department of Medical Physics, Faculty of Medicine and Health, Örebro University, Sweden.

Background: Patients with transfusion dependent anemia are at risk of complications from iron overload. Quantitative T2* magnetic resonance imaging (MRI) is the best non-invasive method to assess iron deposition in the liver and heart and to guide chelation therapy.

Purpose: To investigate the image quality and inter-observer variations in T2* measurements of the myocardium and the liver, and to obtain the lower limit of cardiac and hepatic quantitative T2* values in patients without suspicion of iron overload. Read More

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Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study.

Hematol Transfus Cell Ther 2021 May 9. Epub 2021 May 9.

Hospital Israelita Albert Einstein, São Paulo, SP, Brazil. Electronic address:

Introduction: Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. Read More

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Cerebral venous sinus thrombosis and aneurysm in a patient with double heterozygous beta-thalassemia major: A case report.

Medicine (Baltimore) 2021 May;100(21):e26082

Department of Neurology, The Third People's Hospital of Chengdu, The Affiliated Hospital of Southwest Jiaotong University, Chengdu, Sichuan Province, China.

Rationale: Thalassemia is an inherited disease associated with thromboembolic events (TEE) and cerebral artery disease. Here, we report a patient with beta-thalassemia presenting with intracerebral hemorrhage due to cerebral venous sinus thrombosis (CVST), and intracranial aneurysms were found after examination. We believe that it is very rare for this patient to have two kinds of cerebrovascular diseases. Read More

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Fat Embolism Syndrome in Sickle Cell β-Thalassemia Patient With Osteonecrosis: An Uncommon Presentation in a Young Adult.

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211012266

Ashland Bellefonte Cancer Center, Ashland, KY, USA.

Fat embolism syndrome is a relatively infrequent presentation in sickle cell thalassemia patients. It most commonly occurs in long bone fractures in the setting of trauma. However, nonorthopedic trauma and nontraumatic cases have been reported to contribute to fat embolism. Read More

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Bone Marrow Fat Distribution in Patients With β-Thalassemia: A Study Using Chemical Shift-Based Water-Fat MRI.

Acad Radiol 2021 May 12. Epub 2021 May 12.

Department of Biomedical Imaging, Faculty of Medicine, University of Malaya, Malaysia.

Rationale And Objectives: Molecular studies have shown the changes in bone marrow fat in relation to altered hematopoiesis. This study aims to investigate the changes in the bone marrow fat in patients affected by β-thalassemia by using chemical shift-encoded (CSE)-MRI.

Materials And Methods: Twenty-three subjects, comprising of six healthy (17-31 years old) and 17 β-thalassemia subjects (19-39 years old), were scanned using a multiecho fast low angle shot sequence (0. Read More

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The diagnostic accuracy of Artificial Intelligence-Assisted CT imaging in COVID-19 disease: A systematic review and meta-analysis.

Inform Med Unlocked 2021 6;24:100591. Epub 2021 May 6.

Health Research Institute, Thalassemia and Hemoglobinopathies Research Centre, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Artificial intelligence (AI) systems have become critical in support of decision-making. This systematic review summarizes all the data currently available on the AI-assisted CT-Scan prediction accuracy for COVID-19. The ISI Web of Science, Cochrane Library, PubMed, Scopus, CINAHL, Science Direct, PROSPERO, and EMBASE were systematically searched. Read More

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Multifocal Osteonecrosis in a 3-Year-Old Child With Sickle Beta Plus Thalassemia.

J Pediatr Hematol Oncol 2021 May 11. Epub 2021 May 11.

Department of Pediatrics, Division of Hematology/Oncology Division of Infectious Diseases Department of Medical Imaging, University of Arizona, Tucson, AZ.

Sickle beta+thalassemia is considered to be a mild form of sickle cell disease. However, some patients with mild disease can present with osteonecrosis. Here, we present a rare 3-year-old male who presented with acute pain, a baseline hemoglobin of 13 g/dL, who acutely developed multifocal osteonecrosis, and improved with partial exchange transfusion and hydroxyurea therapy. Read More

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A double-blind, controlled, crossover trial of amlodipine on iron overload status in transfusion dependent β-thalassemia patients.

Int J Clin Pract 2021 May 10:e14337. Epub 2021 May 10.

Student Research Committee, Mashhad University of Medical Sciences, Mashhad, Iran.

Background And Aim: This study examined whether administration of amlodipine could improve myocardial iron loading status in patients with transfusion dependent β-thalassemia (TDT), through a placebo-controlled, crossover study.

Methods: Amlodipine (5 mg, daily) or placebo were prescribed to all patients (n = 19) for 6 months, and after a 2-week washout period, patients were crossed over to the other group. The efficacy of amlodipine on iron loading was assessed by measuring myocardial T2*-weighted magnetic resonance imaging (MRI T2*, millisecond [ms]) and serum ferritin (ng/mL). Read More

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Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients.

Anemia 2021 15;2021:5581831. Epub 2021 Apr 15.

Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia.

Thalassemia is a genetic disease caused by disruption of globin chain synthesis leading to severe anemia and thus regular blood transfusion is necessary. However, there have been known transfusions-related consequences, including iron overload and multi-organ damage. The aims of this study were to evaluate liver and cardiac function in youth and adult transfusion-dependent Indonesian thalassemic patients and to assess its correlation with serum ferritin levels, as well as T2 magnetic resonance imaging (MRI). Read More

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Left ventricular systolic dyssynchrony index and endothelial dysfunction parameters as subclinical predictors of cardiovascular involvement in patients with beta-thalassemia major.

Echocardiography 2021 Jun 4;38(6):825-833. Epub 2021 May 4.

Department of Cardiology, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.

Objective: Cardiovascular iron load is the leading cause of morbidity and mortality in beta-thalassemia major (β-TM). However, many patients remain asymptomatic until the late stage. In this cross-sectional study, we investigated the role of three-dimensional (3D) echocardiography and endothelial dysfunction parameters in asymptomatic β-TM patients, and the relationship between these parameters and cardiac magnetic resonance imaging (MRI) T2* value. Read More

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The Importance of Cardiac T2* Magnetic Resonance Imaging for Monitoring Cardiac Siderosis in Thalassemia Major Patients.

Tomography 2021 Apr 18;7(2):130-138. Epub 2021 Apr 18.

Internal Medicine Department, Faculty of Medicine, Khon Kaen University, Khon Kaen 40000, Thailand.

Cardiac T2* magnetic resonance imaging (MRI) has recently attracted considerable attention as a non-invasive method for detecting iron overload in various organs in thalassemia major patients. This study aimed to identify the prevalence of cardiac siderosis in thalassemia major patients and evaluate cardiac T2* MRI for monitoring cardiac siderosis before and after patients receive iron chelation therapy and its relation to serum ferritin, left ventricular ejection fraction, and liver iron concentration. The information gathered would be used for the direct monitoring, detection, and treatment of complications early on. Read More

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Brain injury pathophysiology study by a multimodal approach in children with sickle cell anemia with no intra or extra cranial arteriopathy.

Haematologica 2021 04 22. Epub 2021 Apr 22.

Centre Hospitalier Intercommunal de Créteil, Service d'Imagerie Médicale, Créteil, France; AP-HP, Hôpital Universitaire Robert Debré, Service d'Imagerie Médicale, Paris.

Despite its high prevalence in children with sickle cell anemia (SCA), the pathophysiology of silent cerebral infarcts (SCIs) remains elusive. The main objective of this study was to explore the respective roles of major determinants of brain perfusion in SCA children with no past or current history of intracranial or extracranial vasculopathy. We used a multimodal approach based notably on perfusion imaging Arterial spin labelling (ASL) MRI and Near Infra-Red Spectroscopy (NIRS), as well as biomarkers reflecting blood rheology and endothelial activation. Read More

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Universal Gene Correction Approaches for β-hemoglobinopathies Using CRISPR-Cas9 and Adeno-Associated Virus Serotype 6 Donor Templates.

CRISPR J 2021 Apr;4(2):207-222

University Children's Hospital, Department of Pediatrics I, Hematology and Oncology, University of Tübingen, Germany; University of Tübingen, Tübingen, Germany.

Mutations in the human gene are the cause of β-hemoglobinopathies, one of the most common inherited single-gene blood disorders in the world. Novel therapeutic approaches are based on lentiviral vectors (LVs) or CRISPR-Cas9-mediated gene disruption to express adult hemoglobin (HbA), or to reactivate the completely functional fetal hemoglobin, respectively. Nonetheless, LVs present a risk of insertional mutagenesis, while gene-disrupting transcription factors (BCL11A, KLF1) involved in the fetal-to-adult hemoglobin switch might generate dysregulation of other cellular processes. Read More

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Evaluation of Corneal Epithelial Thickness and Dry Eye Disease Tests in Thalassemic Adolescents.

Clin Ophthalmol 2021 6;15:1425-1431. Epub 2021 Apr 6.

Department of Ophthalmology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Purpose: To assess dry eye disease (DED) in thalassemic adolescents by evaluating corneal epithelial thickness (CET) and various dry eye clinical tests and correlate them to tissue iron overload.

Methods: The study included 120 Beta-thalassemia patients (11 to 18 years) and 120 matched controls. CET maps were captured using anterior segment optical coherence tomography. Read More

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Dual retrotympanic aural mass.

BMJ Case Rep 2021 Apr 12;14(4). Epub 2021 Apr 12.

Otorhinolaryngology, University of Malaya, Kuala Lumpur, Malaysia.

High-riding jugular bulb (HRJB), although rare, may pose a challenge as it may be mistaken for other non-alarming condition, such as middle ear effusion. Patients with HRJB classically present with pulsatile tinnitus. We report a unique case of a 26-year-old patient with underlying beta thalassaemia who presented with a 2-month history of intermittent epistaxis and rhinorrhoea. Read More

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Evaluation of iron overload by cardiac and liver T2* in β-thalassemia: Correlation with serum ferritin, heart function and liver enzymes.

J Cardiovasc Thorac Res 2021 18;13(1):54-60. Epub 2021 Feb 18.

Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.

In this study, we aimed to assess the relationship of cardiac and hepatic T2* magnetic resonance imaging (MRI) values as a gold standard for detecting iron overload with serum ferritin level, heart function, and liver enzymes as alternative diagnostic methods. A total 58 patients with beta-thalassemia major who were all transfusion dependent were evaluated for the study. T2* MRI of heart and liver, echocardiography, serum ferritin level, and liver enzymes measurement were performed. Read More

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February 2021

Monitoring retinal pathology and cerebral injury in sickle cell disease using spectral-domain optical coherence tomography in pediatric patients.

Pediatr Blood Cancer 2021 Jul 31;68(7):e29028. Epub 2021 Mar 31.

Center for Cancer and Blood Disorders, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

Purpose: This study aimed to confirm the correlation between sickle cell disease (SCD) genotype and retinal damage identified by spectral-domain optical coherence tomography (SD-OCT), and examine a potential link between hypoxic ischemic injury in the retina and brain.

Methods: In this prospective, observational case series, 117 patients (56 males) aged 5-20 years with SCD (36 SC, 68 SS, eight Sβ+ thalassemia, five Sβ0 thalassemia) underwent ophthalmologic examination including funduscopy and SD-OCT imaging. Comparison of SCD genotypes and association between ocular findings and cerebrovascular disease (CVD) in subjects with SS/Sβ0 genotype were investigated. Read More

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A non-alcohol-related case of Madelung's disease: Challenging patient with progressive jugular vein distension.

Radiol Case Rep 2021 May 19;16(5):1183-1187. Epub 2021 Mar 19.

Department of Medical Surgical Sciences and Advanced Technologies, Radiology I Unit, University of Catania, Via Santa Sofia 78, 92123 Catania, Italy.

Madelung's disease or multiple symmetric lipomatosis (MSL) is a rare benign disease characterized by abnormal, multiple and symmetric fat depositions in the subcutaneous layer, involving head, neck, back, trunk and also upper and lower limbs. MSL may be related to alcohol abuse or metabolic disorders; it may be both silent or clinically manifest. We describe a case of a 48-yo man with β-thalassemia admitted to medicine department for neck swelling without fever or respiratory symptoms. Read More

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Novel retinal findings in β-thalassemia major: older age and higher ferritin level as the risk factors.

Graefes Arch Clin Exp Ophthalmol 2021 Mar 18. Epub 2021 Mar 18.

Department of Ophthalmology, Faculty of Medicine Universitas Indonesia - Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia.

Purpose: To investigate retinal changes in β-thalassemia major patients and identify their association with systemic risk factors.

Methods: In this prospective study, 120 β-thalassemia major patients received complete ophthalmic examinations (best-corrected visual acuity, contrast sensitivity, color vision, and indirect ophthalmoscopy) and retinal imaging using color fundus photography and fundus autofluorescence imaging. Patients were grouped according to the presence of thalassemia-related retinal changes. Read More

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Correlation between dynamic susceptibility contrast perfusion MRI and genomic alterations in glioblastoma.

Neuroradiology 2021 Mar 18. Epub 2021 Mar 18.

Department of Radiology, University of Minnesota Health, B-226 Mayo Memorial Building, MMC 292, 420 Delaware Street S.E., Minneapolis, MN, 55455, USA.

Purpose: To determine if dynamic susceptibility contrast perfusion MR imaging (DSC-pMRI) can predict significant genomic alterations in glioblastoma (GB).

Methods: A total of 47 patients with treatment-naive GB (M/F: 23/24, mean age: 54 years, age range: 20-90 years) having DSC-pMRI with leakage correction and genomic analysis were reviewed. Mean relative cerebral blood volume (rCBV), maximum rCBV, relative percent signal recovery (rPSR), and relative peak height (rPH) were derived from T2* signal intensity-time curves by ROI analysis. Read More

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Role of CMR feature-tracking derived left ventricular strain in predicting myocardial iron overload and assessing myocardial contractile dysfunction in patients with thalassemia major.

Eur Radiol 2021 Mar 15. Epub 2021 Mar 15.

Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, 110029, India.

Objective: Myocardial iron overload (MIO) in thalassemia major (TM) may cause subclinical left ventricular (LV) dysfunction which manifests with abnormal strain parameters before a decrease in ejection fraction (EF). Early detection of MIO using cardiovascular magnetic resonance (CMR)-T2* is vital. Our aim was to assess if CMR feature-tracking (FT) strain correlates with T2*, and whether it can identify early contractile dysfunction in patients with MIO but normal EF. Read More

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Pseudothrombocytosis in a Case of Beta-thalassaemia Intermedia Masquerading as Myeloproliferative Neoplasm.

Curr Med Imaging 2021 Mar 10. Epub 2021 Mar 10.

Department of Pathology, All India Institute of Medical Sciences, Jodhpur. India.

Finding of spurious thrombocytopenia is a common occurrence in clinical practice whereas pseudothrombocytosis is very uncommon event. Despite several technical advancements in automated haematology analysers, a careful peripheral smear examination remains standard examination in cases of discrepancy of platelet counts. We are presenting a case of beta thalassaemia intermedia who had pseudothrombocytosis, which was falsely labelled as myeloproliferative neoplasm before the patient visited our haematology clinic. Read More

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Relationship Between Pituitary Siderosis and Endocrinological Disorders in Pediatric Patients with Beta-Thalassemia.

Cureus 2021 Jan 23;13(1):e12877. Epub 2021 Jan 23.

Radiology, Dicle University School of Medicine, Diyarbakir, TUR.

Introduction Excess iron accumulation occurs mainly in organs such as reticuloendothelial cells, the pituitary gland, and the pancreas in beta-thalassemia because of blood transfusions. In the present study, it was aimed to investigate the relationship between T2* values on magnetic resonance imaging (MRI) and clinically diagnosed pituitary endocrinological disorders in children with thalassemia major. Methods This study enrolled patients diagnosed with beta-thalassemia at pediatric hematology outpatient clinics. Read More

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January 2021

Longitudinal effect of disease-modifying therapy on tricuspid regurgitant velocity in children with sickle cell anemia.

Blood Adv 2021 01;5(1):89-98

Department of Hematology, St Jude Children's Research Hospital, Memphis, TN.

Elevated tricuspid regurgitant velocity (TRV) ≥2.5 m/s is a predictor of disease severity in adults and children with sickle cell anemia (SCA), but how disease-modifying therapies (DMTs) affect this biomarker is incompletely understood. We investigated the effect of DMTs on TRV elevation in children. Read More

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January 2021