2,471 results match your criteria Thalassemia Imaging


Cardiac T2* magnetic resonance analysis of membranous interventricular septum in assessment of cardiac iron overload in pediatric thalassemia patients: A pilot study.

Indian J Radiol Imaging 2019 Jan-Mar;29(1):33-39

Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.

Background: Cardiac iron deposition in transfusion-dependent thalassemia patients is patchy in distribution.

Purpose: The purpose of this study is to assess the correlation between T2* matrices of membranous interventricular septum (MIVS) and T2* values of muscular interventricular septum (IVS) on magnetic resonance imaging (MRI) and to evaluate the relationship of myocardial T2* at these two locations with MRI-estimated liver iron concentrations (LIC) and electrocardiographic (ECG) parameters.

Material And Methods: MRI of heart and liver was performed in 16 consecutive pediatric patients of transfusion-dependent thalassemia major to calculate liver iron concentration and T2* time of membranous and muscular IVS. Read More

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http://www.ijri.org/text.asp?2019/29/1/33/255233
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http://dx.doi.org/10.4103/ijri.IJRI_395_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467041PMC
April 2019
1 Read

Impact of Genotype of Beta Globin Gene on Hepatic and Myocardial Iron Content in Egyptian Patients with Beta Thalassemia.

Indian J Hematol Blood Transfus 2019 Apr 8;35(2):284-291. Epub 2018 Nov 8.

2Radiodiagnosis Department, Zagazig University, Zagazig, Egypt.

Iron overload causes most of the mortality and morbidity associated with thalassemia. Excess iron deposits primarily in the liver, but once a threshold level is reached, iron loading may occur in other tissues such as the heart. Magnetic resonance imaging is a well established technique to noninvasively quantify myocardial and liver iron content. Read More

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http://link.springer.com/10.1007/s12288-018-1034-x
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http://dx.doi.org/10.1007/s12288-018-1034-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439044PMC
April 2019
1 Read

Therapeutic mechanism of combined oral chelation therapy to maximize efficacy of iron removal in transfusion-dependent thalassemia major - a pilot study.

Expert Rev Hematol 2019 Mar 28:1-8. Epub 2019 Mar 28.

d Division of Pediatric Hematology and Oncology , China Medical University Children's Hospital , Taichung , Taiwan.

Objectives: Three iron chelators are used to treat transfusion-dependent beta-thalassemia: desferrioxamine (DFO), deferasirox (DFX), and deferiprone (DFP). Compliance is low for DFO as it cannot be administered orally. Combined administration of DFP and DFX is orally available, however, the therapeutic mechanism is unknown. Read More

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http://dx.doi.org/10.1080/17474086.2019.1593823DOI Listing
March 2019
4 Reads

Prevalence of extramedullary hematopoiesis, renal cysts, splenic and hepatic lesions, and vertebral hemangiomas among thalassemic patients: a retrospective study from the Myocardial Iron Overload in Thalassemia (MIOT) network.

Ann Hematol 2019 Mar 20. Epub 2019 Mar 20.

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

We determined the prevalence of incidental extracardiac findings (IEF) at Magnetic Resonance Imaging (MRI) potentially related to anemia and hypoxia in age- and sex-matched populations (N = 318) with thalassemia major (TM) and thalassemia intermedia (TI) enrolled in the Myocardial Iron Overload in Thalassemia network. Overall, IEFs were detected in 33.3% and 25. Read More

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http://dx.doi.org/10.1007/s00277-019-03659-1DOI Listing

The experience of a tertiary unit on the clinical phenotype and management of hypogonadism in female adolescents and young adults with transfusion dependent thalassemia.

Acta Biomed 2019 Jan 23;90(1):158-167. Epub 2019 Jan 23.

Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.

Background: Transfusion-dependent β-thalassemia (TDT) is associated with several complications necessitating a multidisciplinary approach for diagnosis, treatment and follow-up. Hypogonadism in female TDT patients is one of the most common endocrine complications, requiring hormone replacement therapy (HRT) throughout reproductive life. Little is known about the balance of benefits versus risks of treatment with sex steroids. Read More

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http://www.mattioli1885journals.com/index.php/actabiomedica/
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http://dx.doi.org/10.23750/abm.v90i1.8143DOI Listing
January 2019
4 Reads

Spontaneous iliopsoas hematoma in a trasfusion dependent β-thalassemia patient with hypersplenism: a case report.

Acta Biomed 2018 Dec 4;90(1):107-111. Epub 2018 Dec 4.

Department of Medical Education Hamad Medical Corporation (HMC), Doha, Qatar.

A 27-year-old married man with transfusion dependent β-thalassemia (TDT) complaining low back pain due to a spontaneous iliopsoas hematoma is reported. A magnetic resonance imaging (MRI) confirmed the diagnosis.The patient was managed conservatively. Read More

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http://dx.doi.org/10.23750/abm.v90i1.7943DOI Listing
December 2018
1 Read

Extreme cardiac iron loading in transfusion-dependent thalassaemia major: cardiac T2* and T1 mapping guiding treatment.

Eur Heart J 2019 Mar 15. Epub 2019 Mar 15.

Department of Cardiovascular Imaging, Barts Heart Centre, St Bartholomew's Hospital, West Smithfield, London, UK.

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https://academic.oup.com/eurheartj/advance-article/doi/10.10
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http://dx.doi.org/10.1093/eurheartj/ehz137DOI Listing
March 2019
10 Reads

Cardiac involvement by CMR in different genotypic groups of thalassemia major patients.

Blood Cells Mol Dis 2019 Mar 7;77:1-7. Epub 2019 Mar 7.

Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy. Electronic address:

Beta thalassemia major (β-TM) displays a great deal of phenotypic heterogeneity, not fully investigated in terms of cause-effect. We aimed to detect if different genotypic groups could be related to different levels of cardiac impairment, evaluated by cardiovascular magnetic resonance (CMR). We considered 671 β-TM patients (age 30. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10799796183044
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http://dx.doi.org/10.1016/j.bcmd.2019.01.008DOI Listing
March 2019
5 Reads

No evidence of increased cerebrovascular involvement in adult neurologically-asymptomatic β-Thalassaemia. A multicentre multimodal magnetic resonance study.

Br J Haematol 2019 Mar 5. Epub 2019 Mar 5.

Dipartimento di Medicina e Chirurgia, Scuola Medica Salernitana, Sezione di Neuroscienze, Università di Salerno, Salerno, Italy.

Multi-factorial causes jeopardize brain integrity in β-thalassaemia. Intracranial parenchymal and vascular changes have been reported among young β-thalassaemia patients but conventional magnetic resonance imaging (MRI) findings are contradictory making early MRI and magnetic resonance angiography (MRA)/venography monitoring a matter of debate. This study prospectively investigated 75 neurologically asymptomatic β-thalassaemia patients (mean-age 35·2 ± 10·7 years; 52/75 transfusion-dependent; 41/75 splenectomised) using a 3T magnetic resonance scanner; clinical, laboratory and treatment data were also collected. Read More

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http://dx.doi.org/10.1111/bjh.15834DOI Listing
March 2019
1 Read

Headache: an important symptom possibly linked to white matter lesions in thalassaemia.

Br J Haematol 2019 May 5;185(3):541-548. Epub 2019 Mar 5.

Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.

Neurological manifestations are reported only occasionally in patients with thalassaemia and are given much less prominence than the complications related to anaemia and iron overload. White matter changes (WMCs) on magnetic resonance imaging (MRI) in patients with thalassaemia were first reported two decades ago but the significance of these lesions remains unclear. We studied the neurological and cognitive manifestations in 82 older patients with thalssaemia [25 Thalassaemia major (TM), 24 thalassaemia intermedia (TI) and 33 haemaglobin E β thalassaemia (EBT)] and 80 controls, and found that headaches were more common in thalassaemia patients (50/82, 61%) than in controls (18/80, 22·5%: P < 0·001). Read More

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http://dx.doi.org/10.1111/bjh.15825DOI Listing
May 2019
5 Reads

Wrist Deformity in Children and Adolescents with b-thalassemia on Oral Iron Chelation Therapy.

Indian Pediatr 2019 Jan;56(1):41-44

Department of Radiology, Nanavati Super Speciality Hospital, Vile Parle East, Mumbai, Maharashtra, India.

Objective: To describe a novel wrist deformity in b-thalassemia major patients, and their radiographic and magnetic resonance imaging findings.

Methods: 30 patients with b-thalassemia major who were noticed to have ulnar deviation at wrist joint were evaluated for previous history of medications, serum ferritin levels, presence of pain and swelling at the wrist joint, and the duration of iron chelation therapy. Radiographs of wrist and limited magnetic resonance imaging (MRI) sequences were obtained in 30 and 15 patients, respectively. Read More

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January 2019
5 Reads
1.014 Impact Factor

Diffusion kurtosis imaging histogram parameter metrics predicting survival in integrated molecular subtypes of diffuse glioma: An observational cohort study.

Eur J Radiol 2019 Mar 15;112:144-152. Epub 2019 Jan 15.

Department of Neuroradiology, University Hospital Tübingen, Eberhard Karls University, Tübingen, Germany.

Purpose: The aim of the study was to assess the predictive value of preoperatively assessed diffusion kurtosis imaging (DKI) metrics as prognostic factors in the 2016 World Health Organization Classification of Tumors of the Central Nervous System integrated glioma groups.

Material And Methods: Seventy-seven patients with histopathologically confirmed treatment-naïve glioma were retrospectively assessed between 08/2013 and 10/2017 using mean kurtosis (MK) and mean diffusivity (MD) histogram parameters from DKI, overall and progression-free survival, and relevant prognostic molecular data (isocitrate dehydrogenase, [IDH]; alpha-thalassemia/mental retardation syndrome X-linked, [ATRX]; chromosome 1p/19q loss of heterozygosity). Receiver operating characteristic (ROC) analysis was performed on metric variables to determine the optimal cutoff-values. Read More

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http://dx.doi.org/10.1016/j.ejrad.2019.01.014DOI Listing
March 2019
2 Reads

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
5 Reads
0.956 Impact Factor

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
1 Read

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, 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
3 Reads

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
12 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
10 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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https://synapse.koreamed.org/DOIx.php?id=10.5045/br.2018.53.
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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
5 Reads

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
3 Reads

Atypical Prenatal Ultrasound Presentation and Neuropathological Findings in a Neonate With Alpha Thalassemia Major: A Case Report.

Pediatr Dev Pathol 2019 Mar-Apr;22(2):166-170. Epub 2018 Dec 14.

5 Department of Obstetrics and Gynecology, University of Calgary, Calgary, Canada.

Alpha thalassemia major is a hemoglobinopathy caused by the inactivation or deletion of all 4 α-globin alleles. We describe a case of α-thalassemia major with atypical ultrasound and neuropathological findings. The mother had her first prenatal visit at 27 4/7 gestational weeks. Read More

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http://dx.doi.org/10.1177/1093526618817655DOI Listing
April 2019
1 Read

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
17 Reads

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

J Pediatr Hematol Oncol 2019 Apr;41(3):202-206

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
April 2019
6 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
6 Reads

Echocardiographic evaluation of prevalence of pulmonary hypertension in β-thalassemia major: A cross sectional study.

Pediatr Hematol Oncol 2018 Aug - Sep;35(5-6):322-330. Epub 2018 Nov 23.

c Oncology Research Center , Tabriz University of Medical Sciences , Tabriz , Iran.

Introduction: Pulmonary hypertension is a common complication associated with thalassemia syndromes and it may play an important role in the pathogenesis of right ventricle failure. The true prevalence of pulmonary hypertension in patients with thalassemia major remains unclear and has been reported to be between 2 and 79%.

Materials And Methods: In total, 70 patients with thalassemia major were initially examined. Read More

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http://dx.doi.org/10.1080/08880018.2018.1534914DOI Listing
March 2019
11 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
8 Reads

Imaging features of thalassaemia.

Br J Radiol 2019 Mar 14;92(1095):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
March 2019
18 Reads

Beta-Thalassemia Minor Manifesting as Proliferative Retinopathy.

Ophthalmic Surg Lasers Imaging Retina 2018 10;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
20 Reads

Hemochromatosis associated with cholelithiasis as a cause of hydrops fetalis and stillbirth: Prenatal diagnosis.

J Clin Ultrasound 2019 Jan 30;47(1):47-50. Epub 2018 Oct 30.

Department of Embryo-Fetopathology, Maternity and Neonatology Center, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.

Prenatal diagnosis of neonatal hemochromatosis (NH) is usually raised in front of fetal hepatomegaly and heterogeneous liver architecture. We describe a novel sonographic feature that may be associated with NH. Ultrasound demonstrated reticulonodular liver and distended gallbladder with multiple gallstones in a hydropic fetus. Read More

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http://dx.doi.org/10.1002/jcu.22653DOI Listing
January 2019
5 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
29 Reads

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

Eur Radiol 2019 May 18;29(5):2246-2252. 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
May 2019
17 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
27 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
5 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
3 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
11 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
16 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
6 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
6 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
7 Reads
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
7 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
11 Reads

Estimation of pancreatic R2* for iron overload assessment in the presence of fat: a comparison of different approaches.

MAGMA 2018 Dec 24;31(6):757-769. Epub 2018 Jul 24.

Fondazione CNR Regione Toscana "G. Monasterio", Via Moruzzi, 1, 56124, Pisa, Italy.

Objectives: To propose a method for estimating pancreatic relaxation rate, R2*, from conventional multi-echo MRI, based on the nonlinear fitting of the acquired magnitude signal decay to MR signal models that take into account both the signal oscillations induced by fat and the different R2* values of pancreatic parenchyma and fat.

Materials And Methods: Single-peak fat (SPF) and multi-peak fat (MPF) models were introduced. Single-R2* and dual-R2* assumptions were considered as well. Read More

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http://dx.doi.org/10.1007/s10334-018-0695-7DOI Listing
December 2018
1 Read

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
4 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
12 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
21 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
21 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
17 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
19 Reads