2,855 results match your criteria Thalassemia Imaging

Recurrent spinal cord compression due to extramedullary hematopoiesis in thalassemia patient: Case report.

Medicine (Baltimore) 2022 Jun 24;101(25):e29334. Epub 2022 Jun 24.

Hematology and Oncology Department, Hamad Medical Corporation, Doha, Qatar.

Introduction: Spinal cord compression secondary to extramedullary hematopoiesis (EMH) is a rare condition. Variable treatment options have been reported with different efficacy and recurrence rate. Due to its rarity, no clear optimal management guidelines have been established yet. Read More

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Optical coherence tomography findings in patients with transfusion-dependent β-thalassemia.

BMC Ophthalmol 2022 Jun 24;22(1):279. Epub 2022 Jun 24.

Poostchi Ophthalmology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Structural ophthalmologic findings have been reported in patients with β-thalassemia due to chronic anemia, iron overload, and iron chelation therapy toxicity in few previous studies. We aimed to investigate structural ocular findings and their relationship with hematological parameters in patients with transfusion-dependent β-thalassemia (TDT).

Methods: In this cross-sectional study, from January 2018 to January 2019, 39 patients with TDT over the age of 18 participated. Read More

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Recent advances in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms.

World J Gastrointest Surg 2022 May;14(5):383-396

Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany.

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a rare group of tumors originating from neuroendocrine cells of the digestive system. Their incidence has increased over the last decades. The specific pathogenetic mechanisms underlying GEP-NEN development have not been completely revealed. Read More

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Dual gradient echo in-phase and out of phase sequences in assessment of hepatic iron overload in patients with beta-thalassemia, would be better?

Eur J Radiol 2022 Jun 15;154:110412. Epub 2022 Jun 15.

Department of radiodiagnosis, Faculty of medicine, Alexandria University, Egypt.

Purpose: To evaluate the diagnostic accuracy of the dual gradient-echo (GRE) in- and out-of-phase sequences as a quantitative tool for hepatic iron overload in comparison with MRI R2* relaxometry in paediatric patients with beta-thalassemia.

Method: Sixty-three patients with beta-thalassemia major (transfusion-dependent) or beta-thalassemia intermedia (transfusion- and non-transfusion-dependent) were referred from the paediatric department (haematology unit) to the radiology department at a university hospital. The paediatrician conducted a clinical examination for the studied group, assessed their laboratory data, conducted R2* relaxometry and dual gradient echo sequences to calculate R2* and relative signal intensity index at the axial mid-section of the liver, and studied their correlation. Read More

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Diffuse midline glioma with H3-K27M mutation: A rare case with GFAP-positive anucleate whorled patterns.

Medicine (Baltimore) 2022 Jun 17;101(24):e29448. Epub 2022 Jun 17.

Department of Pathology, The First Affiliated Hospital of China Medical University, Shenyang, P.R. China.

Introduction: Diffuse midline glioma with H3-K27M mutation is an infiltrative high-grade glioma, with predominantly astrocytic differentiation.

Patient Concerns: A 54-year-old Chinese woman presented with memory loss for a month and walking instability for 15 days.

Diagnosis: Magnetic resonance imaging showed a mass shadow of isometric T1 and slightly longer T2 with mild mixed signals in the third ventricle of the suprasellar region. Read More

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Deep Learning Staging of Liver Iron Content From Multiecho MR Images.

J Magn Reson Imaging 2022 Jun 17. Epub 2022 Jun 17.

Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Background: MRI represents the most established liver iron content (LIC) evaluation approach by estimation of liver T2* value, but it is dependent on the choice of the measurement region and the software used for image analysis.

Purpose: To develop a deep-learning method for unsupervised classification of LIC from magnitude T2* multiecho MR images.

Study Type: Retrospective. Read More

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Iron overload disorders.

Hepatol Commun 2022 Jun 14. Epub 2022 Jun 14.

Liver Institute Northwest and Elson S. Floyd College of Medicine, Washington State University, Washington, USA.

Iron overload disorders represent a variety of conditions that lead to increased total body iron stores and resultant end-organ damage. An elevated ferritin and transferrin-iron saturation can be commonly encountered in the evaluation of elevated liver enzymes. Confirmatory homeostatic iron regulator (HFE) genetic testing for C282Y and H63D, mutations most encountered in hereditary hemochromatosis, should be pursued in evaluation of hyperferritinemia. Read More

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Relationship between pancreatic iron overload, glucose metabolism and cardiac complications in sickle cell disease: an Italian multicenter study.

Eur J Haematol 2022 Jun 11. Epub 2022 Jun 11.

Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Objectives: Evidence about the cross-talk between iron, glucose metabolism and cardiac disease is increasing. We aimed to explore the link of pancreatic iron by Magnetic Resonance Imaging (MRI) with glucose metabolism and cardiac complications (CC) in sickle cell disease (SCD) patients.

Methods: We considered 70 SCD patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Read More

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Extramedullary haematopoiesis presenting as an adnexal mass in a patient with β-thalassaemia.

BMJ Case Rep 2022 Jun 10;15(6). Epub 2022 Jun 10.

Department of Gynaecological Ultrasound and Prenatal Diagnostics, Women' Hospital, University Hospital of Basel, Basel, Switzerland.

Solid masses of the ovaries raise the suspicion of malignancy or metastasis and require histological diagnosis. Extramedullary haematopoesis (EMH) is a rare histological finding of a mass of the adnexa. The sonographic pattern of EMH has rarely been described in the literature. Read More

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Correlation between Myocardial Iron Overload Detected by CMRT2* and Left Ventricular Function Assessed by Tissue Doppler Imaging in Patients with Thalassemia Major.

J Cardiovasc Echogr 2022 Jan-Mar;32(1):17-22. Epub 2022 Apr 20.

Department of Biostatistics and Epidemiology, School of Health, Isfahan University of Medical Sciences, Isfahan, Iran.

Background: Iron overload and cardiac dysfunctions are common complications in patients with thalassemia major (TM). Different imaging methods can be used to detect ventricular dysfunction in these patients. In this study, we aim to understand the value of tissue Doppler imaging (TDI) in the detection of myocardial dysfunction in patients with TM who have been diagnosed with iron overload using cardiovascular magnetic resonance CMRT2*. Read More

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Symphysis morphology and mandibular alveolar bone thickness in patients with β-thalassemia major and different growth patterns.

Dental Press J Orthod 2022 23;27(2):e22205. Epub 2022 May 23.

Mashhad University of Medical Sciences, School of Dentistry, Dental Research Center (Mashhad, Iran).

Objective: The present study aimed to assess the morphology of symphysis and alveolar bone thickness (ABT) surrounding mandibular incisors in thalassemic patients, as compared to unaffected individuals.

Methods: This case-control study was conducted on lateral cephalograms of 60 thalassemic and 60 unaffected patients with Class II malocclusion seeking orthodontic treatment at Dental School, Shiraz University of Medical Sciences. The sample was divided into three subgroups including hyperdivergent, normodivergent, and hypodivergent, according to the Jarabak index. Read More

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A single center experience of prenatal parent-fetus trio exome sequencing for pregnancies with congenital anomalies.

Prenat Diagn 2022 06 20;42(7):901-910. Epub 2022 May 20.

Department for Women's Health, University Hospital of Tübingen, Tübingen, Germany.

Objectives: To examine the diagnostic yield of trio exome sequencing in fetuses with multiple structural defects with no pathogenic findings in cytogenetic and microarray analyses.

Methods: We recruited 51 fetuses with two or more defects, non-immune fetal hydrops or fetal akinesia deformation syndrome|or fetal akinesia deformation sequence (FADS). Trio exome sequencing was performed on DNA from chorionic villi samples and parental blood. Read More

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Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study.

Acta Biomed 2022 05 11;93(2):e2022022. Epub 2022 May 11.

Faculty of Medicine, Alexandria University.

Background And Aim Of The Work: Cardiac complications occur in patients with non-transfusion dependent thalassemia (NTDT). The study aimed to evaluate transfusion effect on systolic and diastolic cardiac function in young NTDT patients.  Methods:  Study design: Cohort study. Read More

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Evaluation of right and left ventricular function using speckle-tracking echocardiography in thalassemic patients.

Ann Pediatr Cardiol 2021 Oct-Dec;14(4):476-484. Epub 2022 Mar 25.

Department of Cardiology, Tanta University, Tanta, Egypt.

Background: Beta-thalassemia major is the most common chronic hemolytic anemia among children and adolescents across the world. Several studies have demonstrated that thalassemic patients who have preserved left ventricle systolic function could still have subtle systolic dysfunction. Among patients with beta-thalassemia, early detection of transfusion-induced myocardial iron loading and its intervention with aggressive chelation therapy may delay or reverse heart failure. Read More

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Report of a Rare Case of Beta-Thalassemia Major With Subperiosteal Hematomas.

Cureus 2022 Apr 2;14(4):e23770. Epub 2022 Apr 2.

Surgery Department, Hamad Medical Corporation, Doha, QAT.

Beta-thalassemia represents a range of hemoglobinopathies that are a consequence of an impairment in the synthesis of beta-globin chains. They result in different degrees of hemolysis and ineffective erythropoiesis, depending on the underlying mutations. They can lead to severe complications mainly resulting from anemia. Read More

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Left atrial deformation indices in β-thalassemia major patients.

Ann Hematol 2022 Jul 23;101(7):1473-1483. Epub 2022 Apr 23.

First Department of Cardiology, General Hospital of Athens Ippokrateio, National and Kapodistrian University of Athens, 114 Vasilissis Sofias Avenue, 11527, Attica, Athens, Greece.

The presence of atrial cardiomyopathy in β-thalassemia major (β-TM) patients complicates their clinical condition. The diagnosis is challenging even with cardiac magnetic resonance (CMR) imaging. Novel echocardiographic techniques are applied to increase the diagnostic yield. Read More

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Efficacy and Safety of Sinopharm Vaccine for SARS-CoV-2 and breakthrough infections in Iranian Patients with Hemoglobinopathies: A Preliminary Report.

Mediterr J Hematol Infect Dis 2022 1;14(1):e2022026. Epub 2022 Mar 1.

Coordinator of ICET-A Network (International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine) and Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.

Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic.

Aims Of The Study: Our aim was to investigate the efficacy and side effects of the Sinopharm vaccine in patients with hemoglobinopathies in Iran and the frequency of breakthrough infection after a full course of vaccination. Read More

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Transient Elastography of Liver and Serum Hyaluronic Acid Levels In Adult Transfusion Dependent Thalassemia Patients.

J Assoc Physicians India 2022 Apr;70(4):11-12

Lady Hardinge Medical College, New Delhi.

Iron overload occurs as a result of multiple blood transfusions and increased iron absorption in thalassemia patients. Iron deposition in liver results in liver stiffness and fibrosis. Non invasive methods including imaging and serum biomarkers have been introduced for assessment of liver fibrosis. Read More

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The use of hydroxyurea in the real life of MIOT network: an observational study.

Expert Opin Drug Saf 2022 Apr 17:1-8. Epub 2022 Apr 17.

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

Background: Hydroxyurea (HU) has been widely used in clinical practice to manage patients with non-transfusion dependent thalassemia (NTDT). Few data are available about the effects of its administration in Italian patients. We assessed hematological and non-hematological outcomes following short- and long-term exposure to HU. Read More

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Safety and Efficacy of the New Combination Iron Chelation Regimens in Patients with Transfusion-Dependent Thalassemia and Severe Iron Overload.

J Clin Med 2022 Apr 3;11(7). Epub 2022 Apr 3.

SSD Talassemia, Ospedale Pediatrico Microcitemico 'Antonio Cao', Via Jenner s.n., 09121 Cagliari, Italy.

The aim of this study is the evaluation of the safety and the efficacy of long-term combination therapy deferasirox plus desferrioxamine and deferasirox plus deferiprone in a large group of transfusion-dependent thalassemia patients with high values of serum ferritin and/or magnetic resonance, indicative of severe liver and cardiac iron accumulation. Sixteen adults with transfusion-dependent thalassemia were treated simultaneously with deferasirox plus desferrioxamine, while another 42 patients (seven children) were treated with deferasirox plus deferiprone. The hepatic and cardiac iron overload was assessed prior to treatment and then annually with magnetic resonance imaging, and the serum ferritin was measured monthly. Read More

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Mild-intensity physical activity prevents cardiac and osseous iron deposition without affecting bone mechanical property or porosity in thalassemic mice.

Sci Rep 2022 04 8;12(1):5959. Epub 2022 Apr 8.

Center of Calcium and Bone Research (COCAB), Faculty of Science, Mahidol University, Bangkok, 10400, Thailand.

Thalassemia causes anemia, ineffective erythropoiesis, bone loss and iron accumulation in several tissues, e.g., liver, bone and heart, the last of which leads to lethal cardiomyopathy and arrhythmia. Read More

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Osteonecrosis of Bilateral Distal Femurs in a Pregnant Patient Following Antenatal Betamethasone.

Cureus 2022 Mar 1;14(3):e22735. Epub 2022 Mar 1.

Rheumatology, Einstein Medical Center Philadelphia, Philadelphia, USA.

Corticosteroid therapy is a known risk factor for osteonecrosis, more commonly with chronic use and high cumulative dose. Osteonecrosis (avascular necrosis) has been described in pregnancy involving primarily the femoral head. To our knowledge, only rare cases of femoral meta diaphysis or knee osteonecrosis in pregnancy have been documented in the literature. Read More

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First trimester screening: Increased nuchal translucency or cystic hygroma?

Ultraschall Med 2022 04 5;43(2):111-114. Epub 2022 Apr 5.

Centre for Ultrasound and Prenatal Medicine, Frankfurt/Main, Germany.

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Neuroimaging Findings in Pediatric Patients with Thalassemia Major.

Hematol Rep 2022 Mar 21;14(1):54-60. Epub 2022 Mar 21.

Department of Radiology, Hatay State Hospital, Antakya 3106, Turkey.

Background: Cranial magnetic resonance imaging (MRI) studies about iron accumulation in children with thalassemia major are quite limited.

Aim: This study aimed to detect neurological findings with cranial MRIs in the pediatric patients with thalassemia major who did not develop any neurological complications.

Materials And Methods: Pediatric patients with thalassemia major who followed in the Pediatric Hematology Unit between 1 July 2017 and 1 January 2019 were included in the study. Read More

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Screening for glucose dysregulation in β-thalassemia major (β-TM): An update of current evidences and personal experience.

Acta Biomed 2022 03 14;93(1):e2022158. Epub 2022 Mar 14.

First Department of Paediatrics, National Kapodistrian University of Athens 11527, Greece.

Glucose dysregulation (GD) in patients with β-thalassemia major (β-TM) usually develops gradually. Prediabetes consists of two abnormalities, impaired fasting glucose (IFG) and impaired glucose tolerance (IGT), the latter detected by a standardized oral glucose tolerance test (OGTT). Diagnosis of prediabetes is essential for an early identification of high-risk individuals who will benefit from intensive iron chelation therapy and lifestyle modification. Read More

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GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study.

J Endocrinol Invest 2022 Jul 19;45(7):1439-1445. Epub 2022 Mar 19.

Section of Endocrinology, Geriatrics and Internal Medicine, Department of Medical Sciences, University of Ferrara, Via Fossato di Mortara 64/B, 44121, Ferrara, Italy.

Purpose: GH deficit (GHD) could represent an endocrine issue in ß-Thalassemia Major (ßTM) patients. GH/IGF-1 axis has not been extensively explored in ßTM adults, so far. We aim to assess GHD and IGF-1 deficiency prevalence in ßTM adult population, focusing on the relationship with liver disease. Read More

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Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review.

Ann Med 2022 12;54(1):764-774

Department of Medical Oncology/Hematology, National Centre for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

Introduction: Around 5% of the world's population is expected to have some degree and type of thalassaemia. Beta thalassaemia (BT) occurs due to a deficient production of the beta-globin chain of haemoglobin. Extramedullary haematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. Read More

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December 2022

Magnetic Resonance Imaging Quantification of the Liver Iron Burden and Volume Changes Following Treatment With Thalidomide in Patients With Transfusion-Dependent -Thalassemia.

Front Pharmacol 2022 18;13:810668. Epub 2022 Feb 18.

Department of Radiology, First Affiliated Hospital of Guangxi Medical University, Nanning, China.

Clinical trials have indicated that thalidomide could be used to treat thalassemia, but evidence of changes in liver iron burden and liver volume during thalidomide treatment is lacking. This study aimed to evaluate the liver iron burden and volume changes following thalidomide treatment in patients with transfusion-dependent -thalassemia. A total of 66 participants with transfusion-dependent -thalassemia were included in this prospective cohort study between January 2017 and December 2020. Read More

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February 2022

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease.

Int Med Case Rep J 2022 24;15:69-73. Epub 2022 Feb 24.

Department of Radiology, Mbarara University of Science and Technology, Mbarara City, South-Western Uganda, Uganda.

Background: The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Read More

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February 2022

Use of Deferasirox Film-Coated Tablets in Pediatric Patients with Transfusion Dependent Thalassemia: A Single Center Experience.

Biology (Basel) 2022 Feb 5;11(2). Epub 2022 Feb 5.

1st Pediatric Department, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece.

Thalassemic syndromes are characterized by clinical heterogenicity. For severe disease forms, lifelong blood transfusions remain the mainstay of therapy, while iron overload monitoring and adequate chelation treatment are required in order to ensure effective disease management. Compared to previous chelators, the new deferasirox film-coated tablet (DFX FCT) is considered to offer a more convenient and well-tolerated treatment scheme, aiming at better treatment-related and patient-related outcomes. Read More

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February 2022