2,610 results match your criteria Thalassemia Imaging


Prolonged Pyrexia: Kikuchi-Fujimoto Disease in a Patient With Hb H-Constant Spring Thalassemia.

Clin Med Insights Case Rep 2020 26;13:1179547620936424. Epub 2020 Jun 26.

Department of Haematology, Ampang Hospital, Ampang, Malaysia.

Introduction: Haemoglobin H (Hb H) disease is an alpha thalassemia characterised by either 3 alpha-globin gene deletions (deletional type) or 2 alpha-globin gene deletions with 1-point mutation (nondeletional type). Haemoglobin H-Constant Spring thalassemia is the most common Hb H disease in Asia. Kikuchi-Fujimoto disease (KFD) is an important cause of prolonged fever in thalassemia and is often self-limiting. Read More

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http://dx.doi.org/10.1177/1179547620936424DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7323278PMC

Association between Serum Vitamin D Level and Echocardiographic Abnormalities in Patients with Thalassemia Major.

Cardiovasc Hematol Disord Drug Targets 2020 Jun 30. Epub 2020 Jun 30.

Cardiovascular Research Center, Shahid Beheshti University of Medical Sciences, Tehran. Iran.

Objectives: Thalassemia major (TM) is one of the most common blood disorders with a high mortality rate due to cardiovascular disease. Vitamin D deficiency has been suggested to implicate in cardiac abnormalities. In this prospective study, we aimed to investigate the relationship between serum levels of vitamin D and tissue Doppler (TD) echocardiographic indices in thalassemia major patients. Read More

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http://dx.doi.org/10.2174/1871529X20666200701104931DOI Listing

Pseudoxanthoma elasticum in childhood in patient with β-thalassaemia.

Arch Soc Esp Oftalmol 2020 Jun 24. Epub 2020 Jun 24.

Departamento de Oftalmología, Hospital General, Valencia, España.

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterised by elastic tissue alterations and caused by mutations in a single gene, ABCC6, on chromosome 16p that includes manifestations that are predominantly cutaneous, ocular and cardiovascular. PXE-like lesions in association with β-thalassemia have previously been reported in the literature in patients with β-thalassaemia intermediate and major, being clinically indistinguishable from classic PXE. The case is presented of a 10-year-old boy with β-thalassaemia minor and characteristic lesions of PXE. Read More

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http://dx.doi.org/10.1016/j.oftal.2020.05.018DOI Listing

Myeloid diseases in the lung and pleura.

Semin Diagn Pathol 2020 Jun 12. Epub 2020 Jun 12.

Department of Pathology, UT Southwestern Medical Center, BioCenter EB3.234, 2330 Inwood Road, EB3.234, Dallas, TX 75390-9317, USA. Electronic address:

Myeloid diseases detected as primary or secondary lesions in the lung and pleura are rare. Clinical presentations and radiographic results may vary significantly depending on the nature of the diseases. The most common diseases associated with lung and pleura involvement are myeloid sarcoma/acute myeloid leukemia (AML) and extramedullary hematopoiesis (EMH). Read More

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http://dx.doi.org/10.1053/j.semdp.2020.06.002DOI Listing

Liver damage and sickle cell disease: genotype relationship.

Ann Hematol 2020 Jun 22. Epub 2020 Jun 22.

Unità di Medicina Generale, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35, 20122, Milan, Italy.

Sickle hepatopathy is a severe and not rare complication of sickle cell disease (SCD), showing mainly a cholestatic pattern. So far, no effective approaches to prevent or treat this condition have been recognized. We conducted a single-center observational study in 68 adult sickle cell patients, encompassing 17 with sickle cell anemia (SCA), 38 with sickle cell thalassemia (HbS/β-Thal), and 13 with HbSC disease. Read More

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http://dx.doi.org/10.1007/s00277-020-04113-3DOI Listing

Evaluation of the efficacy and safety of deferiprone compared with deferasirox in paediatric patients with transfusion-dependent haemoglobinopathies (DEEP-2): a multicentre, randomised, open-label, non-inferiority, phase 3 trial.

Lancet Haematol 2020 Jun;7(6):e469-e478

Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus, Valenzano, Italy.

Background: Transfusion-dependent haemoglobinopathies require lifelong iron chelation therapy with one of the three iron chelators (deferiprone, deferasirox, or deferoxamine). Deferasirox and deferiprone are the only two oral chelators used in adult patients with transfusion-dependent haemoglobinopathies. To our knowledge, there are no randomised clinical trials comparing deferiprone, a less expensive iron chelator, with deferasirox in paediatric patients. Read More

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http://dx.doi.org/10.1016/S2352-3026(20)30100-9DOI Listing

Longitudinal follow-up of patients with thalassaemia intermedia who started transfusion therapy in adulthood: a cohort study.

Br J Haematol 2020 May 19. Epub 2020 May 19.

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

We longitudinally evaluated the effects of regular blood transfusions (BTs), in the real-life context of the Myocardial Iron Overload in Thalassaemia network, in patients with thalassaemia intermedia (TI). We considered 88 patients with TI (52 females) who started regular BTs after the age of 18 years. Magnetic resonance imaging was used to quantify iron overload and biventricular function. Read More

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http://dx.doi.org/10.1111/bjh.16753DOI Listing

The corrected QT interval prolongation in adolescents with cardiac iron overload β-thalassemia major.

Turk J Pediatr 2020 ;62(2):267-273

Department of Child Health, Faculty of Medicine Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

Background And Objectives: Iron-induced cardiomyopathy remains the leading cause of mortality in β-thalassemia major patients. The T2* magnetic resonance imaging (MRI) technique is the gold standard for iron load detection, yet it is expensive and not widely available especially in the developing countries. Some previous studies showed that QTc interval could be used as an early detection of cardiac iron overload. Read More

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http://dx.doi.org/10.24953/turkjped.2020.02.013DOI Listing
January 2020

The role of MRA in pediatric sickle cell disease with normal transcranial Doppler imaging velocities.

J Stroke Cerebrovasc Dis 2020 Jul 10;29(7):104864. Epub 2020 May 10.

Pediatric Neuroradiology and Image Guided Therapy, Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto M5G 1H4, ON, Canada.

Purpose: The purpose of this study was to determine the additional information provided by Magnetic Resonance Angiography (MRA) in pediatric sickle cell disease (SCD) patients with normal Transcranial Doppler imaging (TCDI) examinations.

Methods: This cohort study included all pediatric SCD patients over an 18-year period who had no history of stroke and had normal TCDI examinations and subsequently underwent MRA. Routine TCDI inclusive of time-averaged mean of maximum velocities (TAMMV) were assesses and compared with tortuosity on MRA and silent infarct on MRI. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2020.104864DOI Listing
July 2020
1.993 Impact Factor

Ears of the Lynx Magnetic Resonance Imaging Sign.

Ann Neurol 2020 Jul 29;88(1):16-17. Epub 2020 May 29.

Department of Radiology, Orthopedic Research Center, Faculty of Medicine, Mazandaran University of Medical Science, Sari, Iran.

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http://dx.doi.org/10.1002/ana.25766DOI Listing

Biochemical and imaging markers in patients with thalassaemia.

Hellenic J Cardiol 2020 May 6. Epub 2020 May 6.

1(st) Cardiology Department, Aristotle University of Thessaloniki, AHEPA University Hospital, Thessaloniki, Greece.

Beta-thalassaemia is a genetic disease with different clinical aspects, which can lead to heart failure with a multifactorial mechanism. Over the last years, growing interest has been reported for biomarkers that may help in the diagnosis, staging and prognosis of heart disease at an early stage, in patients with beta-thalassaemia. This review will highlight the current clinical value of cardiac biomarkers in patients with beta-thalassaemia and the ongoing research for a possible expanded future use. Read More

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http://dx.doi.org/10.1016/j.hjc.2020.04.012DOI Listing

Liver Iron Content Determination Using a Volumetric Breath-Hold Gradient-Echo Sequence With In-Line R * Calculation.

J Magn Reson Imaging 2020 May 7. Epub 2020 May 7.

Medical Center, Clinic for Pediatric and Adolescent Medicine, Ulm University, Ulm, Germany.

Background: Liver iron overload is a serious condition occurring in patients requiring blood transfusions (eg, in thalassemia and different forms of anemia) or with dysfunctional iron resorption, since there is no physiological mechanism to excrete iron. Above a certain level of iron concentration, chelation therapy is indicated. To monitor therapy success, liver iron content should be assessed regularly. Read More

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http://dx.doi.org/10.1002/jmri.27185DOI Listing

Evaluation of the relationship between splenic iron overload and liver, heart and muscle features evident on T2*-weighted magnetic resonance imaging.

Adv Clin Exp Med 2020 Apr;29(4):475-480

Department of Radiology, Medical School, Dicle University, Diyarbakir, Turkey.

Background: Splenic iron overload is the most common clinical condition in patients with thalassemia. However, few studies of the effects of splenectomy have been published.

Objectives: To evaluate the relationship between splenic iron overload and liver, heart and muscle features visible in T2*-weighted magnetic resonance imaging, and to investigate the effects of splenectomy on these tissues in patients with beta-thalassemia major (TM). Read More

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http://dx.doi.org/10.17219/acem/116758DOI Listing

Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors.

Ann Hematol 2020 Jun 4;99(6):1209-1215. Epub 2020 May 4.

U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.

The phenotype/genotype relationship of patients with transfusion-dependent thalassaemia (TDT) is particularly complex and variable, thus generating different levels of severity and of annual transfusion volume (ATV). In this study, we explored the role and the contribution of several factors potentially involved in determining mean ATV in a cohort of TDT patients which have been followed since long time. We collected data on one-hundred and twenty-seven patients with transfusion-dependent β-thalassaemia followed at Rare Blood Cell Disease Unit, AORN Cardarelli Hospital. Read More

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http://dx.doi.org/10.1007/s00277-020-04047-wDOI Listing

Diffusion kurtosis imaging combined with molecular markers as a comprehensive approach to predict overall survival in patients with gliomas.

Eur J Radiol 2020 Jul 23;128:108985. Epub 2020 Apr 23.

Department of Radiology, Qilu Hospital of Shandong University, Jinan, China. Electronic address:

Purpose: The purpose of this study was to explore the usefulness of diffusion kurtosis imaging (DKI) and molecular markers in predicting the prognosis of glioma patients.

Method: Fifty-one patients with gliomas were examined by conventional MRI and DKI at 3.0 T before operation. Read More

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http://dx.doi.org/10.1016/j.ejrad.2020.108985DOI Listing

Consequences of parenteral iron-dextran loading investigated in minipigs. A new model of transfusional iron overload.

Blood Cells Mol Dis 2020 Jul 19;83:102440. Epub 2020 Apr 19.

Biomedical Research Laboratory, Aalborg University Hospital, Aalborg, Denmark; Department of Cardiothoracic Surgery, Aalborg University Hospital, Aalborg, Denmark.

Patients with blood transfusion-dependent anemias develop transfusional iron overload (TIO), which may cause cardiosiderosis. In patients with an ineffective erythropoiesis, such as thalassemia major, common transfusion regimes aim at suppression of erythropoiesis and of enteral iron loading. Recent data suggest that maintaining residual, ineffective erythropoiesis may protect from cardiosiderosis. Read More

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http://dx.doi.org/10.1016/j.bcmd.2020.102440DOI Listing

Comparison of global strain values of myocardium in beta-thalassemia major patients with iron load using specific feature tracking in cardiac magnetic resonance imaging.

Int J Cardiovasc Imaging 2020 Jul 28;36(7):1343-1349. Epub 2020 Apr 28.

Department of Radiology, Imam Reza Hospital, Tabriz University of Medical Sciences, Tabriz, Iran.

Thalassemia defined a spectrum of diseases characterized by reduced or absent production of one of the globin chains of hemoglobin. High iron deposition in the myocardium may cause functional impairment even before any changes in left ventricular (LV) ejection fraction. These impairments may appear as changes in strain values. Read More

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http://dx.doi.org/10.1007/s10554-020-01835-3DOI Listing

Single-cell O exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells.

Proc Natl Acad Sci U S A 2020 May 22;117(18):10067-10078. Epub 2020 Apr 22.

Department of Physiology, Anatomy & Genetics, University of Oxford, OX1 3PT Oxford, England;

Disorders of oxygen transport are commonly attributed to inadequate carrying capacity (anemia) but may also relate to inefficient gas exchange by red blood cells (RBCs), a process that is poorly characterized yet assumed to be rapid. Without direct measurements of gas exchange at the single-cell level, the barriers to O transport and their relationship with hematological disorders remain ill defined. We developed a method to track the flow of O in individual RBCs by combining ultrarapid solution switching (to manipulate gas tension) with single-cell O saturation fluorescence microscopy. Read More

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http://dx.doi.org/10.1073/pnas.1916641117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7211990PMC

Biventricular Reference Values by Body Surface Area, Age, and Gender in a Large Cohort of Well-Treated Thalassemia Major Patients Without Heart Damage Using a Multiparametric CMR Approach.

J Magn Reson Imaging 2020 Apr 20. Epub 2020 Apr 20.

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

Background: Cardiac MRI plays a critical role in the management of thalassemic patients. No accurate biventricular reference values are available.

Purpose: To establish the ranges for normal left ventricular (LV) and right ventricular (RV) volumes and ejection fraction (EF) and LV mass normalized to body surface area (BSA), age, and gender in a large cohort of well-treated beta-thalassemia major (β-TM) patients without heart damage using a multiparametric MRI. Read More

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http://dx.doi.org/10.1002/jmri.27169DOI Listing

American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults.

Blood Adv 2020 Apr;4(8):1554-1588

Evidence-Based Practice Center, Mayo Clinic, Rochester, MN.

Background: Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan.

Objective: These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD.

Methods: The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019001142DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189278PMC

Tilt-table Echocardiography Unmasks Early Diastolic Dysfunction in Patients With Hemoglobinopathies.

J Pediatr Hematol Oncol 2020 Apr 13. Epub 2020 Apr 13.

Division of Pediatric Cardiology.

Individuals with hemoglobinopathy (sickle cell anemia and thalassemia major) are at risk for cardiac complications such as heart failure and cardiomyopathy. Diastolic dysfunction is known to precede systolic dysfunction in many cardiac diseases. This study sought to determine whether changes in left atrial (LA) function during manipulation of cardiac preload by tilt-table echocardiography can unmask subclinical diastolic dysfunction in pediatric patients with hemoglobinopathies. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001799DOI Listing

Cardiac Iron Overload by MRI in Children With B-Thalassemia Major and its Correlation With Cardiac Function by Echocardiography.

J Pediatr Hematol Oncol 2020 Apr 2. Epub 2020 Apr 2.

Department of Pediatrics, Tanta University Hospital, Tanta, Egypt.

Background: Serial echocardiography is strongly recommended in asymptomatic B-thalassemia major (TM) patients for early detection of subtle cardiac dysfunction. T2*magnetic resonance imaging (MRI) is a noninvasive measurement of myocardial iron burden. Yet, it is not always available in many centers. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001786DOI Listing

Associations of serum sclerostin with bone mineral density, markers of bone metabolism and thalassaemia characteristics in adult patients with transfusion-dependent beta-thalassaemia.

Ann Med 2020 May - Jun;52(3-4):94-108. Epub 2020 Mar 26.

Clinic of Clinical Hematology, University Hospital "Sv. Georgi", Plovdiv, Bulgaria.

To assess serum sclerostin in transfusion-dependent beta-thalassaemia patients versus healthy controls and to examine its associations with bone mineral density, bone metabolism markers and beta thalassaemia alterations. Sixty-two transfusion-dependent beta-thalassaemia (TDßT) patients and 30 healthy controls were evaluated for serum sclerostin, osteocalcin, beta-cross laps, osteoprotegerin and serum level of receptor activator of nuclear factor kappa-Β ligand (sRANKL). Bone mineral density was measured at the lumbar spine and femoral neck. Read More

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http://dx.doi.org/10.1080/07853890.2020.1744708DOI Listing

ATRX/EZH2 complex epigenetically regulates FADD/PARP1 axis, contributing to TMZ resistance in glioma.

Theranostics 2020 10;10(7):3351-3365. Epub 2020 Feb 10.

Beijing Neurosurgical Institute, Capital Medical University, Beijing 100050, China.

: Glioma is the most common primary malignant brain tumor in adults. Chemoresistance of temozolomide (TMZ), the first-line chemotherapeutic agent, is a major issue in the management of patients with glioma. Alterations of alpha thalassemia/mental retardation syndrome X-linked (ATRX) gene constitute one of the most prevalent genetic abnormalities in gliomas. Read More

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http://dx.doi.org/10.7150/thno.41219DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053195PMC
February 2020
7.827 Impact Factor

Absence of T1 Hyperintensity in the Brain of High-risk Patients After Multiple Administrations of High-dose Gadobutrol for Cardiac Magnetic Resonance.

Clin Neuroradiol 2020 Mar 17. Epub 2020 Mar 17.

MRI Unit, Fondazione G. Monasterio CNR-Regione Toscana, Area della Ricerca S. Cataldo, 1 Moruzzi Street, 56124, Pisa, Italy.

Purpose: A prospective study was conducted to evaluate signal changes in the dentate nucleus, globus pallidus, pons, and thalamus (normalized to the deep cerebellum white matter) in T1-weighted magnetic resonance (MR) images after serial injections of gadobutrol in patients with thalassemia without neurological lesions.

Methods: In this study three groups were scanned at both 1.5 T and 3 T: 15 thalassemia patients transfused and chelated with ≥4 gadobutrol administrations at a high dose (0. Read More

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http://dx.doi.org/10.1007/s00062-020-00897-zDOI Listing

Characteristics and Prognosis of Hepatocellular Carcinoma in Multi-Transfused Patients with β-Thalassemia. Experience of a Single Tertiary Center.

Mediterr J Hematol Infect Dis 2020 1;12(1):e2020013. Epub 2020 Mar 1.

2 Department of Medicine, National and Kapodistrian University of Athens, Medical School, Hippokration General Hospital of Athens.

Background/aim: The incidence of hepatocellular carcinoma (HCC) in patients with transfusion dependent thalassemia (TDT) has been increasing, where viral hepatitis and iron overload are the two established HCC risk factors. The aim of this study was to investigate the etiological factors of HCC development and to evaluate the possible factors associated with survival in our cohort of TDT patients with HCC.

Methods: Records of patients with TDT diagnosed with HCC from 2008 to 2018 were reviewed. Read More

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http://dx.doi.org/10.4084/MJHID.2020.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7059739PMC

The planimetric Grothoff's criteria by cardiac magnetic resonance can improve the specificity of left ventricular non-compaction diagnosis in thalassemia intermedia.

Int J Cardiovasc Imaging 2020 Jun 21;36(6):1105-1112. Epub 2020 Feb 21.

U.O.C. Risonanza Magnetica, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi 1, 56124, Pisa, Italy.

We differentiated the left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to a negative remodeling in thalassemia intermedia (TI) patients applying linear and planimetric criteria and comparing the cardiovascular magnetic resonance (CMR) findings. CMR images were analyzed in 181 TI patients enrolled in the Myocardial Iron Overload in Thalassemia Network and 27 patients with proved LVNC diagnosis. The CMR diagnostic criteria applied in TI patients were: a modified linear CMR Petersen's criterion based on a more restrictive ratio of diastolic NC/C > 2. Read More

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http://dx.doi.org/10.1007/s10554-020-01797-6DOI Listing

Increased Bone Marrow F-Choline Uptake in a Patient with Hepatocellular Carcinoma and Thalassemia Intermedia

Authors:
Luca Filippi

Mol Imaging Radionucl Ther 2020 Feb;29(1):45-48

Santa Maria Goretti Hospital, Clinic of Nuclear Medicine, Latina, Italy

A 57-year-old male with history of thalassemia intermedia and hepatocellular carcinoma underwent a positron emission tomography/computed tomography (PET/CT) scan with F-choline before radioembolization procedure with Y-microspheres. The PET/CT scan with F-choline demonstrated highly increased tracer incorporation within a gross lesion in the hepatic dome coupled with diffuse activity in bone marrow, this latter aspect was probably due to the compensatory hematopoiesis stimulation induced by chronic hemolysis. This pattern of skeletal F-choline uptake should be considered as a peculiar PET/CT finding in thalassemic patients. Read More

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http://dx.doi.org/10.4274/mirt.galenos.2019.03064DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7057729PMC
February 2020

CMR for myocardial iron overload quantification: calibration curve from the MIOT Network.

Eur Radiol 2020 Jun 12;30(6):3217-3225. Epub 2020 Feb 12.

MRI Unit, Fondazione G. Monasterio CNR-Regione Toscana, Area della Ricerca S. Cataldo, Via Moruzzi, 1, 56124, Pisa, Italy.

Objectives: R2* cardiac magnetic resonance (CMR) allows the non-invasive measurement of myocardial iron. We calibrated cardiac R2* values against myocardial tissue-measured iron concentration by using a segmental approach and we assessed the iron distribution.

Methods: Five hearts of thalassemia patients were donated after death/transplantation to the CoreLab of the Myocardial Iron Overload in Thalassemia Network. Read More

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http://dx.doi.org/10.1007/s00330-020-06668-1DOI Listing

The rapidly advancing Class 2 CRISPR-Cas technologies: A customizable toolbox for molecular manipulations.

J Cell Mol Med 2020 Mar 10;24(6):3256-3270. Epub 2020 Feb 10.

Key Laboratory for Regenerative Medicine, Ministry of Education, School of Biomedical Sciences, Faculty of Medicine, CUHK-GIBH Joint Research Laboratory on Stem Cells and Regenerative Medicine, The Chinese University of Hong Kong, Hong Kong SAR, China.

The CRISPR-Cas technologies derived from bacterial and archaeal adaptive immune systems have emerged as a series of groundbreaking nucleic acid-guided gene editing tools, ultimately standing out among several engineered nucleases because of their high efficiency, sequence-specific targeting, ease of programming and versatility. Facilitated by the advancement across multiple disciplines such as bioinformatics, structural biology and high-throughput sequencing, the discoveries and engineering of various innovative CRISPR-Cas systems are rapidly expanding the CRISPR toolbox. This is revolutionizing not only genome editing but also various other types of nucleic acid-guided manipulations such as transcriptional control and genomic imaging. Read More

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http://dx.doi.org/10.1111/jcmm.15039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7131926PMC

Relation Between Cardiac T2* Values and Electrocardiographic Parameters in Children With Transfusion-dependent Thalassemia.

J Pediatr Hematol Oncol 2020 Feb 6. Epub 2020 Feb 6.

Departments of Pediatrics, Division of Pediatric Hematology Oncology.

Background/objectives: Cardiac T2* magnetic resonance imaging (MRI) is the gold standard to determine myocardial iron overload. As availability of Cardiac T2* is not uniform across developing nations, our strategy was to identify a more accessible and cost effective tool to assess myocardial iron accumulation. As children with transfusion-dependent thalassemia also experience various electrocardiographic abnormalities, we performed electrocardiography (ECG) as well as Cardiac T2* MRI on all children registered in our thalassemia unit. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001734DOI Listing
February 2020

Comparison of diastolic function in children with transfusion dependent beta thalassemia major by tissue and conventional doppler imaging indices and its correlation with serum ferritin levels.

Turk J Pediatr 2019 ;61(2):250-256

Departments of Pediatrics, Manipal Academy of Higher Education, Manipal, Karnataka.

Nanjegowda CK, Kamath SP, Kamath P, Shah TD, Kulkarni V, Lashkari HP, Baliga BS. Comparison of diastolic function in children with transfusion dependent beta thalassemia major by tissue and conventional doppler imaging indices and its correlation with serum ferritin levels. Turk J Pediatr 2019; 61: 250-259. Read More

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http://dx.doi.org/10.24953/turkjped.2019.02.014DOI Listing

Systematic Literature Review of the Burden of Disease and Treatment for Transfusion-dependent β-Thalassemia.

Clin Ther 2020 02 24;42(2):322-337.e2. Epub 2019 Dec 24.

Department of Pediatrics, Weill Cornell Medicine, New York, NY, USA.

Purpose: β-Thalassemia is an inherited blood disorder characterized by reduced or no production of adult hemoglobin. Systematic identification of the burden of β-thalassemia with contemporary treatments is lacking in published literature. Thus, a gap exists in understanding the baseline burden on which to assess future treatments. Read More

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http://dx.doi.org/10.1016/j.clinthera.2019.12.003DOI Listing
February 2020

Diffuse leptomeningeal glioneuronal tumor in a Chinese adult: a novel case report and review of literature.

Acta Neurol Belg 2020 Apr 24;120(2):247-256. Epub 2019 Dec 24.

Department of Functional Neurosurgery, The Brain Hospital Affiliated to Nanjing Medical University, Nanjing, 210029, China.

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare glioma tumor classified by the World Health Organization as a central nervous system tumor in 2016. DLGNT is most common in children and adolescents but is rare in adults. A 25-year-old male patient was admitted due to recurrent seizures. Read More

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http://dx.doi.org/10.1007/s13760-019-01262-9DOI Listing

Cardiac T * mapping: Techniques and clinical applications.

J Magn Reson Imaging 2019 Dec 14. Epub 2019 Dec 14.

University of Groningen, Groningen, The Netherlands.

Cardiac T * mapping is a noninvasive MRI method that is used to identify myocardial iron accumulation in several iron storage diseases such as hereditary hemochromatosis, sickle cell disease, and β-thalassemia major. The method has improved over the years in terms of MR acquisition, focus on relative artifact-free myocardium regions, and T * quantification. Several improvement factors involved include blood pool signal suppression, the reproducibility of T * measurement as affected by scanner hardware, and acquisition software. Read More

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http://dx.doi.org/10.1002/jmri.27023DOI Listing
December 2019

Rhenium-188 radiosynovectomy for chronic haemophilic synovitis: Evaluation of its safety and efficacy in haemophilic patients.

Haemophilia 2020 Jan 9;26(1):142-150. Epub 2019 Dec 9.

Hemophilia-Thalassaemia Center of Mashhad (Sarvar Clinic), Mashhad University of Medical Sciences, Mashhad, Iran.

Introduction: Radiocolloids labelled with less costly and more accessible radionuclides such as rhenium-188 are of interest to developing countries compared with those labelled with rhenium-186 and yttrium-90.

Aim: This study was aimed to evaluate the efficacy and safety of radiosynovectomy using rhenium-188 in patients with chronic haemophilic synovitis and recurrent hemarthrosis.

Methods: In this quasi-experimental prospective study, 20 haemophilic patients were evaluated at preinjection, and at 1, 3, 6 and 12 months after injection. Read More

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http://dx.doi.org/10.1111/hae.13880DOI Listing
January 2020
2.603 Impact Factor

Compressive Dorsal Myelopathy Secondary to Extramedullary Hematopoiesis in a Thalassemic Patient.

Case Rep Neurol Med 2019 17;2019:5827626. Epub 2019 Oct 17.

Department of Neurology, Ibn Sina Hospital, Kuwait.

Background: Extramedullary hematopoiesis (EMH) is one of the rare causes of spinal cord compression (SCC). It results from noncancerous proliferation of hematopoietic tissue outside the bone marrow as a compensatory mechanism for ineffective erythropoiesis. It occurs in the paraspinal area in 11-15% of thalassemic patients in intermediate and severe cases causing a paraspinal compressive mass. Read More

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http://dx.doi.org/10.1155/2019/5827626DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855030PMC
October 2019

Co-transplantation of Human Fetal Mesenchymal and Hematopoietic Stem Cells in Type 1 Diabetic Mice Model.

Front Endocrinol (Lausanne) 2019 6;10:761. Epub 2019 Nov 6.

Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.

Cell therapy can overcome the limitation of conventional treatments (including different medications and β cell replacement) for type 1 diabetes. Based- on several studies human fetal mesenchymal and hematopoietic stem cells are ideal candidates for stem cell therapy. On the other hand, co-transplantation of them can improve their effects. Read More

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http://dx.doi.org/10.3389/fendo.2019.00761DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856665PMC
November 2019

Left Ventricular Regional Function in Children with Beta Thalassemia with No Cardiac Manifestations (Four-Dimensional Echocardiographic Study).

Indian J Hematol Blood Transfus 2019 Oct 22;35(4):750-757. Epub 2019 Mar 22.

2Ministry of Health, Cairo, Egypt.

Early detection of myocardial dysfunction is essential for the management of patients with thalassemia. Four-dimensional echocardiography imaging technique may be useful for detecting subclinical cardiovascular disease. To evaluate the 4-dimensional echocardiographic strain in children with beta thalassemia major with no cardiac manifestation and correlate it with other echocardiographic parameters. Read More

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http://dx.doi.org/10.1007/s12288-019-01117-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6825061PMC
October 2019

Cardiac and hepatic siderosis in myelodysplastic syndrome, thalassemia and diverse causes of transfusion-dependent anemia: the TIMES study.

Hemasphere 2019 Jun 4;3(3):e224. Epub 2019 Jun 4.

Monash Medical Centre, Melbourne, Australia.

The significant morbidity and mortality associated with iron overload can be reduced by effective iron chelation. Magnetic resonance imaging (MRI) provides accurate and reproducible iron load assessment. The aim of this epidemiological study was to assess the prevalence and severity of cardiac and hepatic siderosis by MRI and to evaluate the impact of MRI on clinical management in patients with transfusion-dependent anemia and non-transfusion-dependent thalassemia (NTDT). Read More

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http://dx.doi.org/10.1097/HS9.0000000000000224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6746020PMC

Brain iron content in systemic iron overload: A beta-thalassemia quantitative MRI study.

Neuroimage Clin 2019 25;24:102058. Epub 2019 Oct 25.

Department of Medicine, Surgery and Dentistry, Scuola Medica Salernitana, University of Salerno, Baronissi, Salerno 84081, Italy; Department of Diagnostic Imaging, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", Scuola Medica Salernitana, Salerno 84131, Italy.

Objective: Multisystem iron poisoning is a major concern for long-term beta-thalassemia management. Quantitative MRI-based techniques routinely show iron overload in heart, liver, endocrine glands and kidneys. However, data on the brain are conflicting and monitoring of brain iron content is still matter of debate. Read More

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http://dx.doi.org/10.1016/j.nicl.2019.102058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6849415PMC
October 2019

Prevalence of left ventricular diastolic dysfunction by cardiac magnetic resonance imaging in thalassemia major patients with normal left ventricular systolic function.

BMC Cardiovasc Disord 2019 11 6;19(1):245. Epub 2019 Nov 6.

Division of Cardiology, Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.

Background: The leading cause of mortality of thalassemia major patients is iron overload cardiomyopathy. Early diagnosis with searching for left ventricular diastolic dysfunction before the systolic dysfunction ensued might yield better prognosis. This study aimed to define the prevalence of the left ventricular diastolic dysfunction (LVDD) in thalassemia major patients with normal left ventricular systolic function and the associated factors. Read More

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http://dx.doi.org/10.1186/s12872-019-1235-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6833207PMC
November 2019

Serum ferritin levels and irregular use of iron chelators predict liver iron load in patients with major beta thalassemia: a cross-sectional study.

Croat Med J 2019 Oct;60(5):405-413

Farzad Kompani, Department of Pediatrics, Tehran University of Medical Sciences, Children's Medical Center, Keshavarz Blvd, Tehran 14194, Iran,

Aim: To determine whether serum ferritin, liver transaminases, and regularity and type of iron chelation protocol can be used to predict liver iron load as assessed by T2* magnetic resonance imaging (MRI) in patients with beta thalassemia major (TM).

Methods: This cross-sectional study, conducted from March 1, 2014 to March 1, 2015, involved 90 patients with beta TM on regular packed red blood cell transfusion. Liver and cardiac iron load were evaluated with T2* MRI. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6852133PMC
October 2019

Global longitudinal strain as an Indicator of cardiac Iron overload in thalassemia patients.

Cardiovasc Ultrasound 2019 Nov 4;17(1):24. Epub 2019 Nov 4.

Department of Cardiovascular Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background And Objective: Cardiac involvement due to iron overload is the most common cause of morbidity and mortality in patients with thalassemia, and many patients remain asymptomatic until the late stages. Therefore, early detection of heart problems in such patients at subclinical stages can improve the prognosis of these patients. We investigated the role of speckled tracking (SI) and tissue Doppler echocardiography (TDI) in early detection of iron overload in these patients. Read More

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http://dx.doi.org/10.1186/s12947-019-0174-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6829819PMC
November 2019
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Immobile Tricuspid Valve: Incidental Finding in a Case of Terminal Cardiomyopathy Due to Thalassemia Major.

Authors:
Erman Cilsal

Arq Bras Cardiol 2019 10;113(3):438-443. Epub 2019 Oct 10.

Adana City Education and Research Hospital, Adana - Turkey.

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http://dx.doi.org/10.5935/abc.20190195DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6882387PMC

Evaluation of the effects of hemoglobinopathies on the mandible with fractal dimension analysis.

Niger J Clin Pract 2019 Oct;22(10):1435-1440

Department of Dentomaxillofacial Radiology, Faculty of Dentistry, Hatay Mustafa Kemal University, Hatay, Turkey.

Aims: The aim of this study was to evaluate the mandibular bone changes on panoramic radiographs of thalassemia and sickle cell anemia (SCA) patients using fractal dimension (FD) analysis and to compare the findings obtained from healthy individuals.

Materials And Methods: A total of 90 panoramic radiographs (from 30 patients with thalassemia, 30 patients with SCA and 30 healthy individuals) were assessed to measure FD, mandibular cortical width (MCW), panoramic mandibular index (PMI) in the mandible. Three region of interests (ROI) of angulus, corpus, and interdental bone were created. Read More

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http://dx.doi.org/10.4103/njcp.njcp_196_19DOI Listing
October 2019
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Marital status and paternity in patients with Transfusion- Dependent Thalassemia (TDT) and Non Transfusion-Dependent Thalassemia (NTDT): an ICET - A survey in different countries.

Acta Biomed 2019 09 6;90(3):225-237. Epub 2019 Sep 6.

Quisisana Hospital, Ferrara.

Background: More than five decades ago, thalassemia major (TDT) was fatal in the first decade of life. Survival and quality of life have improved progressively thanks to the implementation of a significant advance in diagnostic and therapeutic methods, consisting mainly of a frequent transfusion program combined with intensive chelation therapy. Improvement also includes imaging methods used to measure liver and cardiac iron overload. Read More

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http://dx.doi.org/10.23750/abm.v90i3.8586DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7233736PMC
September 2019
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Detection of Extramedullary Hematopoietic Tissue in a Patient with Beta-Thalassemia Major on Tc99m-Sestamibi Parathyroid Scintigraphy.

Indian J Nucl Med 2019 Oct-Dec;34(4):324-325

Department of Nuclear Medicine, University Hospital of Larissa, Thessaly, Greece.

A 50-year-old man with beta-thalassemia major underwent Tc-99m sestamibi parathyroid scintigraphy due to elevated parathyroid hormone and calcium serum levels. Single-photon emission computed tomography imaging of neck and thorax revealed a parathyroid adenoma, as well as increased tracer uptake in a paraspinal region in the right hemithorax, where X-ray and computed tomography of the thorax had shown previously a mass compatible with extramedullary hematopoietic tissue. Read More

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http://dx.doi.org/10.4103/ijnm.IJNM_115_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6771193PMC
October 2019
1 Read