2,452 results match your criteria Thalassemia Imaging


Neuroimaging Abnormalities in Patients With Nontransfusion-dependent Thalassemia.

J Pediatr Hematol Oncol 2019 Feb 12. Epub 2019 Feb 12.

Pediatric Hematology Oncology Unit, Advanced Pediatric Centre.

Independence from regular transfusions is the hallmark of nontransfusion-dependent thalassemia (NTDT). However, the associated complications need anticipation and screening. One such complication is a hypercoagulable state predisposing to development of thrombosis. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001432DOI Listing
February 2019

N-terminal-pro-b-type natriuretic peptide levels and cardiac hemosiderosis in adolescent β-thalassemia major patients.

Ann Pediatr Cardiol 2019 Jan-Apr;12(1):32-37

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

Background: Iron-induced cardiomyopathy remains the leading cause of mortality in patients with β-thalassemia major. Iron overload cardiomyopathy, which may be reversible through iron chelation, is characterized by early diastolic dysfunction. Amino-terminal pro-brain natriuretic peptide (NT-proBNP) is a sensitive biomarker of diastolic dysfunction. Read More

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http://dx.doi.org/10.4103/apc.APC_49_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343373PMC
February 2019

Pantoprazole reduces serum ferritin in patients with thalassemia major and intermedia: A randomized, controlled study.

Therapie 2019 Jan 14. Epub 2019 Jan 14.

Cancer research center and department of pharmacology, Semnan university of medical sciences, 35198 Semnan, Iran. Electronic address:

Aim: Complications due to iron overload exert a problematic situation in patients with thalassemia. Proton pump inhibitors (PPIs) like pantoprazole are effective agents to reduce acid gastric acid secretion and perhaps to interrupt iron absorption in conditions with increased iron absorption. Our purpose was to study effects of pantoprazole addition to chelators on iron levels in patients with thalassemia major and intermedia. Read More

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http://dx.doi.org/10.1016/j.therap.2018.11.013DOI Listing
January 2019

Evaluation of osseous changes in dental panoramic radiography of thalassemia patients using mandibular indexes and fractal size analysis.

Oral Radiol 2019 Jan 24. Epub 2019 Jan 24.

Medicine Faculty, Department of Biostatistics and Medical Informatics, Istanbul Medeniyet University, Istanbul, Turkey.

Objective: This study aimed to evaluate the radiomorphometric indexes and fractal dimension (FD) on dental panoramic radiography (DPR) of Thalassemia Major (TM) patients.

Methods: In 59 TM patients and in 59 healthy control subjects, mandibular cortical index (MCI), mandibular cortical width (MCW), panoramic mandibular index (PMI), and FD of four different regions (FD1-4) were evaluated and compared using DPRs.

Results: The distribution of MCI in TM patients was similar to control subjects (p > 0. Read More

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http://dx.doi.org/10.1007/s11282-019-00372-7DOI Listing
January 2019
4 Reads

The effect of vitamin D pathway genes and deferasirox pharmacogenetics on liver iron in thalassaemia major patients.

Pharmacogenomics J 2019 Jan 17. Epub 2019 Jan 17.

Department of Medical Sciences, University of Turin, Amedeo di Savoia Hospital, 10149, Turin, Italy.

Monitoring and treating iron overload is crucial in transfusion-dependent thalassaemia patients. Liver stiffness measurement by transient elastography and T2* magnetic resonance imaging represent non-invasive ways to evaluate the adequacy of the iron chelation treatment. We explored the role of single nucleotide polymorphisms involved in vitamin D metabolism, transport and activity, and in deferasirox metabolism on liver iron burden parameters. Read More

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http://dx.doi.org/10.1038/s41397-019-0071-7DOI Listing
January 2019
3 Reads

Relationship between liver iron concentration determined by R2-MRI, serum ferritin, and liver enzymes in patients with thalassemia intermedia.

Blood Res 2018 Dec 17;53(4):314-319. Epub 2018 Dec 17.

Department of Endocrinology, Al-Elwiya Pediatric Teaching Hospital, Al-Rusafa Health Directorate, Baghdad, Iraq.

Background: Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI.

Methods: In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Read More

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http://dx.doi.org/10.5045/br.2018.53.4.314DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300676PMC
December 2018

Hepatocellular Carcinoma in β-Thalassemia Patients: Review of the Literature with Molecular Insight into Liver Carcinogenesis.

Int J Mol Sci 2018 Dec 17;19(12). Epub 2018 Dec 17.

Division of Hematology/Oncology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut 11-0236, Lebanon.

With the continuing progress in managing patients with thalassemia, especially in the setting of iron overload and iron chelation, the life span of these patients is increasing, while concomitantly increasing incidences of many diseases that were less likely to show when survival was rather limited. Hepatocellular carcinoma (HCC) is a major life-threatening cancer that is becoming more frequently identified in this population of patients. The two established risk factors for the development of HCC in thalassemia include iron overload and viral hepatitis with or without cirrhosis. Read More

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http://dx.doi.org/10.3390/ijms19124070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6321074PMC
December 2018
2 Reads

Sports Injuries: Diagnosis, Prevention, Stem Cell Therapy, and Medical Sport Strategy.

Adv Exp Med Biol 2018 Dec 12. Epub 2018 Dec 12.

Cell Therapy and Regenerative Medicine Research Center, Endocrinology and Metabolism Molecular-Cellular Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.

Sports injuries diagnosis, prevention, and treatment are the most important issues of sports medicine. Fortunately, sports injuries are often treated effectively, and people with damage recover and return to the sport in a satisfactory condition. Meanwhile, many sports injuries and complications can be prevented. Read More

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http://link.springer.com/10.1007/5584_2018_298
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http://dx.doi.org/10.1007/5584_2018_298DOI Listing
December 2018
8 Reads

Cardioprotective Effects of Spirulina Therapy in Children With Beta-Thalassemia Major.

J Pediatr Hematol Oncol 2018 Dec 7. Epub 2018 Dec 7.

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

Introduction: Cardiac failure in children with Thalassemia major (TM) is the major cause of death due to iron overload. Spirulina is a micro alga with proven anti-inflammatory, antioxidant and may have a cardioprotective effect.

Aim Of The Work: The aim of this study was to evaluate the possible beneficial effect of spirulina supplement in TM children. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001380DOI Listing
December 2018
2 Reads

Long-term improvement in cardiac magnetic resonance in β-thalassemia major patients treated with deferasirox extends to patients with abnormal baseline cardiac function.

Am J Hematol 2019 Mar 11;94(3):312-318. Epub 2018 Dec 11.

Department of Woman, Child and General and Specialized Surgery, Università degli Studi della Campania Luigi Vanvitelli, Naples, Italy.

The management of iron overload in thalassemia has changed dramatically since the implementation of magnetic resonance imaging, which allows detection of preclinical iron overload and prevention of clinical complications. This study evaluated the effect of deferasirox (DFX), the newest once-daily oral chelator, on cardiac function, iron overload and cardiovascular events over a longer follow up in a "real world" setting. Longitudinal changes in cardiac magnetic resonance T2*, cardiac function parameters and cardiovascular clinical events were assessed in a cohort of 98 TM patients exposed to DFX for a mean of 6. Read More

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http://dx.doi.org/10.1002/ajh.25370DOI Listing
March 2019
2 Reads

Noninvasive assessment and risk factors of liver fibrosis in patients with thalassemia major using shear wave elastography.

Hematology 2019 Dec 19;24(1):183-188. Epub 2018 Nov 19.

e Department of Radiology and Molecular Imaging , Sultan Qaboos University Hospital Sultan Qaboos University , Muscat , Oman.

Objectives: This study aimed to estimate the prevalence of liver fibrosis and assess the risk factors for developing significant liver fibrosis in patients with Thalassemia Major (TM).

Methods: All patients with TM over the age of 10 years were included in the study.

Results: A total of 94 eligible patients underwent 2-D SWE. Read More

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http://dx.doi.org/10.1080/10245332.2018.1540518DOI Listing
December 2019
3 Reads

Imaging features of thalassaemia.

Br J Radiol 2018 Nov 14:20180658. Epub 2018 Nov 14.

1 University of the West Indies, St Augustine Campus , St Augustine , Trinidad.

This article highlights the range of osseous findings that can be encountered as well as the imaging features of extramedullary haematopoiesis. As iron overload remains a major cause of morbidity and mortality in these disorders, we also discuss the MRI evaluation of hepatic and cardiac hemosiderosis, to aid in the optimization of iron chelation therapy. Future imaging use will be dictated by evolving clinical needs, such as in screening for emerging morbidities, including hepatic fibrosis and hepatocellular carcinoma. Read More

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https://www.birpublications.org/doi/10.1259/bjr.20180658
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http://dx.doi.org/10.1259/bjr.20180658DOI Listing
November 2018
12 Reads

Beta-Thalassemia Minor Manifesting as Proliferative Retinopathy.

Ophthalmic Surg Lasers Imaging Retina 2018 Oct;49(10):e161-e164

Beta-thalassemia (β-thalassemia) minor is characterized by a mutation in one of the two β-globin genes (HBB) that produce the β-globin chains in the hemoglobin molecule. Although other hemoglobinopathies have been frequently associated with retinal disease, there are limited reports of retinal pathology with β-thalassemia minor. We report two patients with β-thalassemia minor presenting with decreased vision, vitreous hemorrhage, and proliferative retinopathy. Read More

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https://www.healio.com/doiresolver?doi=10.3928/23258160-2018
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http://dx.doi.org/10.3928/23258160-20181002-22DOI Listing
October 2018
14 Reads

Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation.

Pediatr Blood Cancer 2019 Feb 21;66(2):e27503. Epub 2018 Oct 21.

Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait.

Background: There are conflicting reports on the role of hydroxyurea (HU) in the pathogenesis of avascular necrosis of the femoral head (AVNFH) in patients with sickle cell disease (SCD).

Procedure: The present study is a prospective cohort study of Kuwaiti children with SCD who were treated with HU. They had magnetic resonance imaging of the hips before starting HU and at regular intervals during a follow-up period, ranging from 1 to 15 years. Read More

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http://doi.wiley.com/10.1002/pbc.27503
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http://dx.doi.org/10.1002/pbc.27503DOI Listing
February 2019
15 Reads

Multicenter validation of the magnetic resonance T2* technique for quantification of pancreatic iron.

Eur Radiol 2018 Oct 18. Epub 2018 Oct 18.

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

Objectives: To assess the transferability of the magnetic resonance imaging (MRI) multislice multiecho T2* technique for pancreatic iron overload assessment.

Methods: Multiecho T2* sequences were installed on ten 1.5-T MRI scanners of the three main vendors. Read More

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http://link.springer.com/10.1007/s00330-018-5783-6
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http://dx.doi.org/10.1007/s00330-018-5783-6DOI Listing
October 2018
10 Reads

MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine.

Br J Haematol 2018 Dec 18;183(5):783-795. Epub 2018 Oct 18.

Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Napoli, Italy.

We prospectively assessed the efficacy of deferasirox versus deferiprone or desferrioxamine as monotherapy in thalassaemia major (TM) patients by magnetic resonance imaging (MRI). We selected the patients enrolled in the Myocardial Iron Overload in Thalassaemia network who received only one chelator between two MRIs (deferasirox = 235, deferiprone = 142, desferrioxamine = 162). Iron overload was measured by T2* technique and biventricular function by cine images. Read More

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http://doi.wiley.com/10.1111/bjh.15595
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http://dx.doi.org/10.1111/bjh.15595DOI Listing
December 2018
14 Reads

Evaluation of Iron Overload Between Age Groups Using Magnetic Resonance Imaging and Its Correlation with Iron Profile in Transfusion-dependent Thalassemia.

Acta Med Indones 2018 Jul;50(3):230-236

Pediatric Hematology-Oncology Division, Department of Child Health, Faculty of Medicine, Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

Background: routine blood transfusion in transfusion-dependent-thalassemia (TDT) causes iron accumulation in various organ. Serum markers of iron overload, serum ferritin and transferrin saturation, are sensitive but not specific. MRI T2-star (T2*) is valuable for detecting iron level in organs. Read More

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July 2018
5 Reads

Hepatic Dysfunction in a Child Post Bone Marrow Transplantation.

J Clin Exp Hepatol 2018 Sep 5;8(3):327-331. Epub 2018 May 5.

Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi, India.

A 10-year-old female child, a known case of Beta-Thalassemia major with superimposed chronic Hepatitis C Viral (HCV) infection, underwent bone marrow transplantation and presented, 7 months later, with worsening hepatic functions and skin rash. Considering the wide variety of possible etiologies, she was evaluated and later confirmed as a case of hepatic dysfunction secondary to HCV related hepatic injury with concomitant chronic graft versus host disease (dermatological involvement only). Read More

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http://dx.doi.org/10.1016/j.jceh.2018.04.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6175742PMC
September 2018
3 Reads

Fludarabine-Induced Posterior Reversible Encephalopathy Syndrome in a Pediatric Patient With β-Thalassemia: Case Report and Literature Review.

Clin Neuropharmacol 2018 Nov/Dec;41(6):224-229

Pediatric Neurology Unit, Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia.

Posterior reversible encephalopathy syndrome is a disorder of reversible subcortical vasogenic brain edema in the context of different diseases or exposure to cytotoxic drugs such as fludarabine. We present the case of a pediatric patient with β-thalassemia who develops a fludarabine-induced posterior reversible encephalopathy while he received an induction regimen to achieve an allogenic hematopoietic cell transplantation. The clinical presentation consists in altered mental state, headache, status epilepticus, visual disturbance, and hypertension. Read More

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http://dx.doi.org/10.1097/WNF.0000000000000309DOI Listing
December 2018
2 Reads

Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature.

BMC Ophthalmol 2018 Sep 12;18(1):246. Epub 2018 Sep 12.

Department of Ophthalmology, Medical School, University of Patras, 265 04, Patras, Greece.

Background: Deferoxamine (DFO) is one of the most commonly used chelation treatments for transfusional hemosiderosis. Pattern dystrophies constitute a distinct entity of retinal disorders that has been occasionally identified in association with deferoxamine.

Case Presentation: We report two cases of bilateral macular pattern dystrophy in transfusion dependent patients undergoing chronic chelation therapy with deferoxamine due to thalassemias. Read More

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http://dx.doi.org/10.1186/s12886-018-0911-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134579PMC
September 2018
5 Reads

Noninvasive Prediction of IDH1 Mutation and ATRX Expression Loss in Low-Grade Gliomas Using Multiparametric MR Radiomic Features.

J Magn Reson Imaging 2019 Mar 8;49(3):808-817. Epub 2018 Sep 8.

Department of Radiology, Huashan Hospital, Fudan University, Shanghai, P.R. China.

Background: Noninvasive detection of isocitrate dehydrogenase 1 mutation (IDH1(+)) and loss of nuclear alpha thalassemia/mental retardation syndrome X-linked expression ((ATRX(-)) are clinically meaningful for molecular stratification of low-grade gliomas (LGGs).

Purpose: To study a radiomic approach based on multiparametric MR for noninvasively determining molecular status of IDH1(+) and ATRX(-) in patients with LGG.

Study Type: Retrospective, radiomics. Read More

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http://doi.wiley.com/10.1002/jmri.26240
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http://dx.doi.org/10.1002/jmri.26240DOI Listing
March 2019
9 Reads

Serum ferritin is not a reliable predictor to determine iron overload in thalassemia major patients post-hematopoietic stem cell transplantation.

Eur J Haematol 2018 Dec 25;101(6):791-797. Epub 2018 Oct 25.

Department for Children and Adolescents, Division for Stem Cell Transplantation and Immunology, University Hospital Frankfurt, Frankfurt am Main, Germany.

Objective: Iron overload (IO) in transfusion-dependent anemia persists after hematopoietic stem cell transplantation (HSCT) and can cause long-term organ damage. In many studies, the diagnosis of IO before and after HSCT is based on serum ferritin (SF) levels rather than on assessment of liver iron concentration (LIC) by MRI or SQUID.

Method: In a retrospective multicenter study, we analyzed the concordance for indication of iron depletion therapy and correlation between LIC and SF of 36 thalassemia patients after HSCT. Read More

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http://doi.wiley.com/10.1111/ejh.13169
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http://dx.doi.org/10.1111/ejh.13169DOI Listing
December 2018
4 Reads

Perfusion MRI grading diffuse gliomas: Impact of permeability parameters on molecular biomarkers and survival.

Neurocirugia (Astur) 2019 Jan - Feb;30(1):11-18. Epub 2018 Aug 22.

Department of Radiology, Neuroradiology Section, Universitary Hospital 12 de Octubre, Madrid, Spain.

Background And Purpose: Our objectives were: (1) compare dynamic susceptibility-weighted (DSC) and dynamic contrast-enhanced (DCE) permeability parameters, (2) evaluate diagnostic accuracy of DSC and DCE discriminating high- and low-grade tumors, (3) analyze relationship of permeability parameters with overall (OS) and progression-free survival (PFS) and (4) assess differences in high-grade tumors classified according to molecular biomarkers.

Materials And Methods: 49 patients with histologically proved diffuse gliomas underwent DSC and DCE imaging. Parametric maps of cerebral blood volume (CBV), CBV-leakage corrected, volume transfer coefficient (Ktrans), fractional volume of the extravascular extracellular space (EES) (Ve), fractional blood plasma volume (Vp) and rate constant between EES and blood plasma (Kep) were calculated. Read More

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http://dx.doi.org/10.1016/j.neucir.2018.06.004DOI Listing
August 2018
3 Reads

Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major.

Indian Pediatr 2018 07;55(7):573-575

Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Objective: To evaluate the efficacy of prolonged deferiprone monotherapy in patients with b-thalassemia major.

Methods: This cross-sectional study included 40 patients (age range 9 to 38 years) with thalassemia major receiving deferiprone for ≥5 years. Serum ferritin, and myocardial iron concentration (MIC) and liver iron concentration (LIC) assessed by T2*MRI were recorded. Read More

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July 2018
1.014 Impact Factor

Syncopal Episodes of Arrhythmogenic Right Ventricular Cardiomyopathy in a Patient with Pre-existing Seizure Disorder.

Cureus 2018 Jun 7;10(6):e2760. Epub 2018 Jun 7.

Cardiology Fellowship, Lehigh Valley Health Network, Allentown, USA.

Arrhythmogenic right ventricular cardiomyopathy (ARVC), is a heritable condition that is an important, and under-recognized cause of sudden cardiac death. Microscopically, it is represented by fibrofatty replacement of myocardium involving the right ventricular inflow area, apex, and infundibulum. Common clinical manifestations of ARVC include palpitations, syncope, chest pain, dyspnea, and sudden cardiac death. Read More

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http://dx.doi.org/10.7759/cureus.2760DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080733PMC
June 2018
3 Reads

Administration of Intravenous Zoledronic Acid Every 3 Months vs. Annually in β-thalassemia Patients with Low Bone Mineral Density: a Retrospective Comparison of Efficacy.

Med Arch 2018 Jun;72(3):170-173

Young Researcher and Elite Club, Islamic Azad University, Kazerun Branch, Kazerun, Iran.

Introduction: The benefit of annual administration of zoledronic acid in the management of thalassemia-associated osteoporosis is unknown.

Aim: The aims of this study were to evaluate the efficacy of treatment with two different dosing regimens of IV zoledronic acid (annually versus every 3 months) for increasing low bone mineral density (BMD) in patients with osteoporosis associated with β-thalassemia as annually and 3-monthly on bone density in patients.

Materials And Methods: This retrospective, single-center study analyzed patients' clinical records and bone density measurements. Read More

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http://dx.doi.org/10.5455/medarh.2018.72.170-173DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6021158PMC
June 2018
10 Reads

Can Tc-labeled RBC-equilibrium radionuclide angiocardiography be an adjunct imaging biomarker for marrow hyperplasia in beta-thalassemia major patients?

J Nucl Cardiol 2018 Jul 23. Epub 2018 Jul 23.

Department of Nuclear Medicine, Nehru Hospital, Post Graduate Institute of Medical Education and Research, Sector-12, Chandigarh, 160012, India.

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http://dx.doi.org/10.1007/s12350-018-1380-yDOI Listing
July 2018
2 Reads

Predicting factors for liver iron overload at the first magnetic resonance in children with thalassaemia major.

Blood Transfus 2018 Jun 26:1-6. Epub 2018 Jun 26.

Rare Blood Cell Disease Unit, "Cardarelli" Hospital, Naples, Italy.

Background: Transfusion dependency determines iron overload in thalassaemia major, with devastating complications. Significant liver iron overload has been observed from early childhood and we aimed to evaluate factors that could predict liver iron overload at the first magnetic resonance imaging (MRI).

Materials And Methods: All transfusion-dependent children who underwent MRI to assess iron overload were retrospectively studied. Read More

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http://dx.doi.org/10.2450/2018.0092-18DOI Listing
June 2018
5 Reads

Complete blood picture with skeletal and visceral changes in patients with thalassemia major.

Int J Health Sci (Qassim) 2018 Jul-Aug;12(4):3-10

Department of Medicine, Sindh Medical College, Dow University of Health Sciences, Karachi, Pakistan.

Objective: The objective of this to find the prevalence of skeletal and visceral changes in thalassemics and its relationship with variation in blood components.

Methodology: This is a cross-sectional study conducted in tertiary care hospital in Karachi among patients diagnosed with thalassemia major who require regular blood transfusions.

Result: Among 200 individuals, 95 were females, whereas 105 were males. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6040856PMC
July 2018
2 Reads

Intracranial immature teratoma invading the nasal cavity mimicking olfactory neuroblastoma: A case report.

Medicine (Baltimore) 2018 Jul;97(28):e11527

Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University.

Rationale: Primary intracranial immature teratoma accounts for majority of congenital central nervous system germ-cell tumors, but it is extremely rare in patients older than 15 years.

Patient Concerns: A 27-year-old woman was referred to our hospital for headache, nasal congestion, and decreased olfactory sensation. Imaging showed a mass measuring approximately 5 cm × 4 cm in the right frontal lobe, which also filled the right nasal cavity. Read More

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http://dx.doi.org/10.1097/MD.0000000000011527DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6076187PMC
July 2018
16 Reads
5.723 Impact Factor

Comparison of Deferiprone to Deferasirox and Deferoxamine to Cardiac and Hepatic T2* MRI in Thalassemia Patients: Evidence-based Case Report.

Acta Med Indones 2018 Apr;50(2):168-176

Faculty of Medicine University of Indonesia - Cipto Mangunkusumo Hospital..

Background: there are currently three iron chelator readily available for patients Indonesia; deferiprone/DFP (branded as Ferriprox), deferasirox/DFX (branded as Exjade) and deferoxamine/DFO (branded as Desferal). This study aims to determine which iron chelator is the most efficient in reducing cardiac and hepatic iron overload (measured by means of T2* MRI).

Methods: journal search with determined MeSH term was done in PubMed and Scopus. Read More

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April 2018
12 Reads

The effect of desferrioxamine chelation versus no therapy in patients with non transfusion-dependent thalassaemia: a multicenter prospective comparison from the MIOT network.

Ann Hematol 2018 Oct 21;97(10):1925-1932. Epub 2018 Jun 21.

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

We prospectively assessed by magnetic resonance imaging (MRI) the advantages of desferrioxamine (DFO) with respect to the absence of chelation therapy in non transfusion-dependent thalassaemia (NTDT) patients. We considered 18 patients non-chelated and 33 patients who received DFO alone between the two MRI scans. Iron overload was assessed by the T2* technique. Read More

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http://dx.doi.org/10.1007/s00277-018-3397-3DOI Listing
October 2018
11 Reads

Clinical utility of endocrine markers predicting myocardial siderosis in transfusion dependent thalassemia major.

Pediatr Blood Cancer 2018 Oct 12;65(10):e27285. Epub 2018 Jun 12.

Department of Pediatrics and Child Health, Aga Khan University, Karachi, Pakistan.

Background: Endocrinopathy due to iron overload is the most common morbidity whereas myocardial siderosis causing toxic cardiomyopathy is the leading cause of mortality among patients with transfusion dependent thalassemia major (TDTM). If detected early, this can be treated with aggressive chelation. T2* cardiac magnetic resonance imaging (CMR) guided chelation protocols are now the gold standard but have limited availability in low and middle-income countries. Read More

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http://doi.wiley.com/10.1002/pbc.27285
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http://dx.doi.org/10.1002/pbc.27285DOI Listing
October 2018
12 Reads

Emphysematous osteomyelitis caused by in beta thalassemia major.

J Postgrad Med 2019 Jan-Mar;65(1):41-43

Department of Paediatrics, Nanavati Super Speciality Hospital, Vile Parle West, Mumbai, Maharashtra, India.

There have been various cases of salmonella osteomyelitis reported in sickle cell anemia. We present a case of emphysematous osteomyelitis caused by Salmonella typhi in a 29-year-old beta thalassemia major patient. Diagnosis of emphysematous osteomyelitis was confirmed by computed tomography and magnetic resonance imaging, and culture of pus drained during surgical debridement confirmed the causative microorganism, Salmonella typhi. Read More

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http://dx.doi.org/10.4103/jpgm.JPGM_689_17DOI Listing
June 2018
5 Reads

Prenatal thoraco-amniotic chest drain insertion to manage a case of fetal hydrops secondary to .

BMJ Case Rep 2018 Jun 4;2018. Epub 2018 Jun 4.

Fetal Medicine Centre, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.

Lymphoedema-distichiasis is an inherited autosomal dominant disorder of the lymphatic system. Rarely, it is associated with fetal hydrops; the risk and severity of which increases with successive generations. The causative gene is a member of the forkhead transcription factor family (). Read More

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http://dx.doi.org/10.1136/bcr-2017-223902DOI Listing
June 2018
2 Reads

Cytomegalovirus Pneumonitis in a Patient with Homozygous β-Thalassemia and Splenectomy.

Jpn J Infect Dis 2018 Sep 31;71(5):370-372. Epub 2018 May 31.

Department of Internal Medicine, University Hospital of Heraklion.

Cytomegalovirus (CMV) rarely causes disease in immunocompetent individuals but may cause severe disease in immunocompromised patients. We report the case of a young woman who had undergone multiple transfusions and splenectomy for homozygous β-thalassemia. She presented with prolonged fever and respiratory distress. Read More

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https://www.jstage.jst.go.jp/article/yoken/71/5/71_JJID.2018
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http://dx.doi.org/10.7883/yoken.JJID.2018.039DOI Listing
September 2018
9 Reads

Renal iron deposition by magnetic resonance imaging in pediatric β-thalassemia major patients: Relation to renal biomarkers, total body iron and chelation therapy.

Eur J Radiol 2018 Jun 9;103:65-70. Epub 2018 Apr 9.

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

Background: The reciprocal of multiecho gradient-echo (ME-GRE) T2* magnetic resonance imaging (MRI) R2*, rises linearly with tissue iron concentration in both heart and liver. Little is known about renal iron deposition in β-thalassemia major (β-TM).

Aim: To assess renal iron overload by MRI and its relation to total body iron and renal function among 50 pediatric patients with β-TM. Read More

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http://dx.doi.org/10.1016/j.ejrad.2018.04.007DOI Listing
June 2018
4 Reads
2.369 Impact Factor

Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood.

Pediatr Blood Cancer 2018 Sep 24;65(9):e27228. Epub 2018 May 24.

School of Public Health, University of Memphis, Memphis, Tennessee.

Background: Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease-modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime. Read More

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http://dx.doi.org/10.1002/pbc.27228DOI Listing
September 2018
12 Reads

Fetal lung size after thoracoamniotic shunting reflects survival in primary fetal hydrothorax with hydrops.

J Obstet Gynaecol Res 2018 Jul 23;44(7):1216-1220. Epub 2018 May 23.

Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan.

Aim: To assess the role of lung size and abnormal Doppler findings in the umbilical artery (UA) in determining the outcomes of fetuses with primary fetal hydrothorax (FHT) associated with hydrops who underwent thoracoamniotic shunting (TAS).

Methods: This was a retrospective study at a single center. We included cases of primary FHT with hydrops who underwent TAS at our hospital between 2004 and 2016. Read More

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http://dx.doi.org/10.1111/jog.13657DOI Listing
July 2018
4 Reads

Label-Free Optical Marker for Red-Blood-Cell Phenotyping of Inherited Anemias.

Anal Chem 2018 Jun 4;90(12):7495-7501. Epub 2018 Jun 4.

Institute of Applied Sciences and Intelligent Systems, ISASI, "E. Caianiello" , CNR , Via Campi Flegrei 34 , 80078 Pozzuoli , NA , Italy.

The gold-standard methods for anemia diagnosis are complete blood counts and peripheral-smear observations. However, these do not allow for a complete differential diagnosis as that requires biochemical assays, which are label-dependent techniques. On the other hand, recent studies focus on label-free quantitative phase imaging (QPI) of blood samples to investigate blood diseases by using video-based morphological methods. Read More

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http://dx.doi.org/10.1021/acs.analchem.8b01076DOI Listing
June 2018
8 Reads

Outcome of fetuses with congenital parvovirus B19 infection: systematic review and meta-analysis.

Ultrasound Obstet Gynecol 2018 Nov;52(5):569-576

Women's Health and Perinatology Research Group, Department of Clinical Medicine, Faculty of Health Sciences, UiT - The Arctic University of Norway, Tromsø, Norway.

Objective: To explore the outcome of fetuses affected by congenital parvovirus B19 (PB19) infection, with or without signs of hydrops on ultrasound.

Methods: PubMed, EMBASE and CINAHL databases were searched for studies reporting on prenatal diagnosis and outcome of fetal PB19 infection. The outcomes explored were miscarriage, perinatal death (PND), intrauterine death, neonatal death, spontaneous resolution of hydrops or fetal anemia, need for intrauterine transfusion (IUT), resolution of hydrops or anemia after transfusion, fetal loss following transfusion, abnormal brain scan after birth and abnormal neurodevelopmental outcome. Read More

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http://dx.doi.org/10.1002/uog.19092DOI Listing
November 2018
9 Reads
3.853 Impact Factor

Deferoxamine-induced electronegative ERG responses.

Doc Ophthalmol 2018 May 16. Epub 2018 May 16.

Department of Ophthalmology, Columbia University, New York, NY, USA.

Purpose: To report a case of deferoxamine-induced retinopathy characterized by electroretinography (ERG), optical coherence tomography angiography (OCT-A), and other multimodal imaging.

Methods: This is an observational case report of one patient. Full-field ERG was performed. Read More

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http://dx.doi.org/10.1007/s10633-018-9640-3DOI Listing
May 2018
4 Reads

Unusual case of iron overload with cancer-mimicking abdominal splenosis.

BMJ Case Rep 2018 May 16;2018. Epub 2018 May 16.

Department of Medicine, Unit of Internal Medicine, Azienda Ospedaliera Universitaria Integrata Verona, University of Verona, Verona, Italy.

A 48-year-old man, former alcohol abuser and drug addicted, was referred to our tertiary referral centre for iron disorders because of marked hyperferritinaemia. His clinical history revealed chronic hepatitis C, ß-thalassaemia trait and post-traumatic splenectomy at age of 22. MRI-estimated liver iron content was markedly elevated, while first-line genetic test for haemochromatosis was negative. Read More

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http://dx.doi.org/10.1136/bcr-2017-223410DOI Listing
May 2018
5 Reads

Growth and Endocrine Function in Tunisian Thalassemia Major Patients.

Mediterr J Hematol Infect Dis 2018 1;10(1):e2018031. Epub 2018 May 1.

Pediatric Immuno-Hematology Unit, Bone Marrow Transplantation Center Tunis, Tunis, Tunisia.

β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. Read More

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http://dx.doi.org/10.4084/MJHID.2018.031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5937976PMC
May 2018
10 Reads

Evaluating Liver Fibrosis by Acoustic Radiation Force Impulse in Thalassemia.

Indian J Pediatr 2018 Nov 11;85(11):1037-1039. Epub 2018 May 11.

Jankharia Imaging and Diagnostics Centre, Girgam, Mumbai, India.

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http://dx.doi.org/10.1007/s12098-018-2692-2DOI Listing
November 2018
19 Reads
0.920 Impact Factor

Arrested pneumatization of the sphenoid sinus mimicking skull base tumours: MRI prevalence in children with haematologic diseases.

Authors:
Taner Arpaci

Int J Neurosci 2018 May 22:1-4. Epub 2018 May 22.

a Department of Radiology , Acibadem University, Acibadem Adana Hospital , Adana , Turkey.

Objective: Arrested pneumatization of the sphenoid sinus is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of arrested pneumatization of the sphenoid sinus in paediatric patients with haemotologic diseases like sickle cell anaemia, thalassemia and leukemia.

Materials And Methods: One hundred and eight paediatric patients (43 girls, 65 boys; age range: 4-18 years; median age: 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging of the head and neck between 2010 and 2017 in a single institution were included. Read More

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https://www.tandfonline.com/doi/full/10.1080/00207454.2018.1
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http://dx.doi.org/10.1080/00207454.2018.1473397DOI Listing
May 2018
8 Reads

Better Renal Resistive Index Profile in Subjects with Beta Thalassemia Minor.

Med Princ Pract 2018 3;27(4):367-371. Epub 2018 May 3.

Department of Radiology, Numune Training and Research Hospital, Adana, Turkey.

Objective: Beta thalassaemia minor is a common genetic disorder without any characteristic symptoms except mild anemia. It is found to be associated with some cardiovascular risk factors such as insulin resistance and diabetes mellitus. The renal resistive index (RRI) is a measure of renal arterial resistance to blood flow. Read More

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http://dx.doi.org/10.1159/000489780DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167719PMC
January 2019

Contrast enhancement predicting survival in integrated molecular subtypes of diffuse glioma: an observational cohort study.

J Neurooncol 2018 Sep 17;139(2):373-381. Epub 2018 Apr 17.

Institute of Neuropathology, Department of Pathology and Neuropathology, University Hospital Tübingen, Eberhard Karls University, Tübingen, Germany.

Introduction: To assess the predictive value of magnetic resonance imaging (MRI) gadolinium enhancement as a prognostic factor in the 2016 World Health Organization Classification of Tumors of the Central Nervous System integrated glioma groups.

Methods: Four-hundred fifty patients with histopathologically confirmed glioma were retrospectively assessed between 07/1997 and 06/2014 using gadolinium enhancement, survival, and relevant prognostic molecular data [isocitrate dehydrogenase (IDH); alpha-thalassemia/mental retardation syndrome X-linked (ATRX); chromosome 1p/19q loss of heterozygosity; and O6-methylguanine DNA methyltransferase (MGMT)]. The Kaplan-Meier method was used to assess univariate survival data. Read More

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http://dx.doi.org/10.1007/s11060-018-2872-yDOI Listing
September 2018
7 Reads

Assessment of liver and cardiac iron overload using MRI in patients with chronic anemias in Latin American countries: results from ASIMILA study.

Hematology 2018 Oct 17;23(9):676-682. Epub 2018 Apr 17.

j Hospital Universitario , Monterrey , Mexico.

Objectives: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia.

Methods: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled. Iron overload was assessed using MRI. Read More

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http://dx.doi.org/10.1080/10245332.2018.1461292DOI Listing
October 2018
35 Reads