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1.
Tomography ; 9(5): 1711-1722, 2023 09 11.
Artigo em Inglês | MEDLINE | ID: mdl-37736989

RESUMO

BACKGROUND: The E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project is an Italian Network assuring high-quality quantification of tissue iron overload by magnetic resonance imaging (MRI). We evaluated the impact of the COVID-19 pandemic on E-MIOT services. METHODS: The activity of the E-MIOT Network MRI centers in the year 2020 was compared with that of 2019. A survey evaluated whether the availability of MRI slots for patients with hemoglobinopathies was reduced and why. RESULTS: The total number of MRI scans was 656 in 2019 and 350 in 2020, with an overall decline of 46.4% (first MRI: 71.7%, follow-up MRI: 36.9%), a marked decline (86.9%) in the period March-June 2020, and a reduction in the gap between the two years in the period July-September. A new drop (41.4%) was recorded in the period October-December for two centers, due to the general reduction in the total amount of MRIs/day for sanitization procedures. In some centers, patients refused MRI scans for fear of getting COVID. Drops in the MRI services >80% were found for patients coming from a region without an active MRI site. CONCLUSIONS: The COVID-19 pandemic had a strong negative impact on MRI multi-organ iron quantification, with a worsening in the management of patients with hemoglobinopathies.


Assuntos
COVID-19 , Hemoglobinopatias , Sobrecarga de Ferro , Humanos , COVID-19/diagnóstico por imagem , Pandemias , Hemoglobinopatias/complicações , Hemoglobinopatias/diagnóstico por imagem , Sobrecarga de Ferro/diagnóstico por imagem , Imageamento por Ressonância Magnética
2.
Eur J Intern Med ; 114: 93-100, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37150716

RESUMO

BACKGROUND: No study has evaluated the effect of hepatitis C virus (HCV) infection on the wide spectrum of complications affecting patients with thalassemia. OBJECTIVES: This multicenter study prospectively assessed the relationship of HCV infection with diabetes mellitus and cardiovascular complications in patients with thalassemia major (TM). METHODS: We considered 1057 TM patients (539 females; 29.79±10.08 years) enrolled in the MIOT Network and categorized into 4 groups: negative patients (group 1a, N=460), patients who spontaneously cleared the virus within 6months (group 1b, N=242), patients who eradicated the virus after the treatment with antiviral therapy (group 2, N=102), and patients with chronic HCV infection (group 3, N=254). RESULTS: Group 1a and 1b were considered as a unique group (group 1). For both groups 1 and 3, a match 1:1 for age and sex with group 2 was performed. The effective study cohort consisted of 306 patients (three groups of 102 patients). During a mean follow-up of 67.93±39.20months, the group 3 experienced a significantly higher % increase/month in aspartate transaminase levels and left ventricular mass index than both groups 1 and 2. The changes in iron overload indexes were comparable among the three groups. Compared to group 1, the chronic HCV group showed a significantly higher risk of diabetes (hazard ratio-HR=5.33; p=0.043) and of cardiovascular diseases (HR=3.80; p=0.034). CONCLUSION: Chronic HCV infection is associated with a significant higher risk of diabetes mellitus and cardiovascular complications in TM patients and should be approached as a systemic disease in which extrahepatic complications increase the weight of its pathological burden.


Assuntos
Diabetes Mellitus , Cardiopatias , Hepatite C Crônica , Hepatite C , Talassemia , Talassemia beta , Feminino , Humanos , Talassemia beta/complicações , Talassemia beta/epidemiologia , Hepatite C/complicações , Talassemia/complicações , Hepatite C Crônica/complicações , Hepatite C Crônica/epidemiologia , Hepacivirus , Diabetes Mellitus/epidemiologia , Morbidade
3.
Int J Cardiovasc Imaging ; 39(5): 991-999, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36637709

RESUMO

PURPOSE: The left ventricular global function index (LVGFI) is a comprehensive marker of cardiac performance, integrating LV morphology with global function. We explored the cross-sectional association of LVGFI with myocardial iron overload (MIO), LV ejection fraction (LVEF), myocardial fibrosis, and heart failure (HF) in ß-thalassemia major (TM) patients. METHODS: We considered 1352 adult TM patients (708 females, 32.79 ± 7.16years) enrolled in the Myocardial Iron Overload in Thalassemia Network and 112 healthy subjects (50 females, 32.09 ± 6.08years). LVGFI and LVEF were assessed by cine images and MIO by multislice multiecho T2* technique. Replacement myocardial fibrosis was detected by late gadolinium enhancement technique. RESULTS: LVGFI and LVEF were significantly lower in patients with significant MIO (global heart T2*<20ms) than in patients without MIO and in healthy subjects but were comparable between TM patients without MIO and healthy subjects. In TM, LVGFI was significantly associated with LVEF (R = 0.733; p < 0.0001). Global heart T2* values were significantly associated with both LVGFI and LVEF, but the correlation with LVGFI was significantly stronger (p = 0.0001). Male sex, diabetes mellitus, significant MIO, and replacement myocardial fibrosis were the strongest predictors of LVGFI. Eighty-six patients had a history of HF and showed significantly lower global heart T2* values, LVEF, and LVGFI than HF-free patients. A LVGFI ≤ 44.9% predicted the presence of HF. The LVGFI showed a diagnostic performance superior to that of LVEF (area under the curve: 0.67 vs. 0.62; p = 0.039). CONCLUSION: In TM patients the LVGFI correlates with MIO and provides incremental diagnostic value for HF detection compared with LVEF.


Assuntos
Cardiomiopatias , Insuficiência Cardíaca , Sobrecarga de Ferro , Talassemia beta , Adulto , Feminino , Humanos , Masculino , Talassemia beta/complicações , Talassemia beta/diagnóstico , Estudos Transversais , Meios de Contraste , Valor Preditivo dos Testes , Gadolínio , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/complicações , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/etiologia , Função Ventricular Esquerda , Fibrose , Imageamento por Ressonância Magnética/métodos
4.
Radiol Med ; 127(11): 1199-1208, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36087241

RESUMO

PURPOSE: We evaluated the feasibility and reproducibility of bone marrow T2* values and established the lower limit of normal in a cohort of healthy subjects. We investigated the clinical correlates of bone marrow T2* values in patients with thalassemia major (TM). MATERIAL AND METHODS: Thirty healthy subjects and 274 consecutive TM patients (38.96 ± 8.49 years, 151 females) underwent MRI at 1.5T. An axial slice in the upper abdomen was acquired by a T2* gradient-echo multiecho sequence and the T2* value was calculated in a circular region of interest defined in the visible body of the first or second lumbar vertebra. In patients, also liver and heart T2* values were assessed. RESULTS: In healthy subjects bone marrow T2* values were independent of age and gender. The lower limit of normal for bone marrow T2* was 13 ms. In both healthy subjects and 30 randomly selected patients, the coefficient of variation for inter-operator-reproducibility was < 10%. TM patients exhibited significantly lower bone marrow T2* values than healthy subjects (7.47 ± 5.18 ms vs. 17.08 ± 1.89 ms; p < 0.0001). A pathological bone marrow T2* was detected in 82.8% of TM patients. In TM, the female sex was associated with reduced bone marrow T2* values. Bone marrow T2* values were inversely correlated with mean serum ferritin levels (R = -0.431; P < 0.0001) and hepatic iron load (R = - 0.215; P < 0.0001). A serum ferritin level > 536 ng/ml predicted the presence of a pathological bone marrow T2*. A positive correlation was found between bone marrow and heart T2* values (R = 0.143; P = 0.018). A normal bone marrow T2* showed a negative predictive value of 100% for cardiac iron. CONCLUSION: Bone marrow T2* measurements can be easily obtained using the same sequences acquired for liver iron quantification and may bring new insights into the pathophysiology of iron deposition; hence, they should be incorporated into clinical practice.


Assuntos
Sobrecarga de Ferro , Talassemia beta , Feminino , Humanos , Talassemia beta/diagnóstico por imagem , Talassemia beta/complicações , Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Ferritinas , Ferro , Sobrecarga de Ferro/diagnóstico por imagem , Fígado/patologia , Imageamento por Ressonância Magnética , Miocárdio/patologia , Valores de Referência , Reprodutibilidade dos Testes , Estudos de Casos e Controles
5.
Eur Heart J ; 43(26): 2482-2492, 2022 07 07.
Artigo em Inglês | MEDLINE | ID: mdl-34907420

RESUMO

AIMS: A tailored chelation therapy guided by magnetic resonance imaging (MRI) is a strategy to improve the prognosis in iron-loaded patients, in many cases still hampered by limited MRI availability. In order to address this issue, the Myocardial Iron Overload in Thalassemia (MIOT) network was established in Italy and we aimed to describe the impact of 10-year activity of this network on cardiac burden in thalassemia major (TM). METHODS AND RESULTS: Within the MIOT network, 1746 TM patients (911 females; mean age 31.2 ± 9.1 years) were consecutively enrolled and prospectively followed by 70 thalassemia and 10 MRI centres. Patients were scanned using a multiparametric approach for assessing myocardial iron overload (MIO), biventricular function, and myocardial fibrosis. At the last MRI scan, a significant increase in global heart T2* values and a significantly higher frequency of patients with no MIO (all segmental T2* ≥20 ms) were detected, with a concordant improvement in biventricular function, particularly in patients with baseline global heart T2* <20 ms. Forty-seven percentage of patients changed the chelation regimen based on MRI. The frequency of heart failure (HF) significantly decreased after baseline MRI from 3.5 to 0.8% (P < 0.0001). Forty-six patients died during the study, and HF accounted for 34.8% of deaths. CONCLUSION: Over 10 years, continuous monitoring of cardiac iron and a tailored chelation therapy allowed MIO reduction, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. A national networking for rare diseases therefore proved effective in improving the care and reducing cardiac outcomes of TM patients.


Assuntos
Sobrecarga de Ferro , Talassemia , Talassemia beta , Adulto , Feminino , Humanos , Ferro , Imageamento por Ressonância Magnética/métodos , Imagem Cinética por Ressonância Magnética/métodos , Miocárdio/patologia , Doenças Raras , Talassemia/complicações , Talassemia/patologia , Adulto Jovem , Talassemia beta/complicações , Talassemia beta/terapia
6.
Blood Transfus ; 19(2): 168-176, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33000750

RESUMO

BACKGROUND: The causes and effects of genotypic heterogeneity in beta-thalassemia major (ß-TM) have not been fully investigated. The aim of this multicentre study was to determine whether different genotype groups could predict the development of cardiovascular magnetic resonance abnormalities and cardiac complications. MATERIALS AND METHODS: We considered 708 ß-TM patients (373 females, age 30.05±9.47 years) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network. Data were collected from birth to the first cardiac magnetic resonance scan. Myocardial iron overload was assessed using a T2* technique. Biventricular function was quantified by cine images. Macroscopic myocardial fibrosis was evaluated by a late gadolinium enhancement technique. RESULTS: Three groups of patients were identified: ß+ homozygotes (n=158), ß+/ß° heterozygotes (n=298) and ß° homozygotes (n=252). Compared to ß+ homozygotes, the other two groups showed a significantly higher risk of myocardial iron overload and left ventricular dysfunction. We recorded 90 (13.0%) cardiac events: 46 episodes of heart failures, 38 arrhythmias (33 supraventricular, 3 ventricular and 2 hypokinetic) and 6 cases of pulmonary hypertensions. ß° homozygotes showed a significantly higher risk than ß+ homozygotes of arrhythmias and cardiac complications considered globally. DISCUSSION: Different genotype groups predicted the development of myocardial iron overload, left ventricular dysfunction, arrhythmias and cardiac complications in ß-TM patients. These data support the importance of genotype knowledge in the management of ß-TM patients.


Assuntos
Doenças Cardiovasculares/etiologia , Talassemia beta/complicações , Adulto , Doenças Cardiovasculares/diagnóstico por imagem , Doenças Cardiovasculares/genética , Feminino , Predisposição Genética para Doença , Humanos , Imageamento por Ressonância Magnética , Masculino , Prognóstico , Adulto Jovem , Talassemia beta/genética
7.
J Magn Reson Imaging ; 53(1): 61-70, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32311193

RESUMO

BACKGROUND: Cardiac MRI plays a critical role in the management of thalassemic patients. No accurate biventricular reference values are available. PURPOSE: To establish the ranges for normal left ventricular (LV) and right ventricular (RV) volumes and ejection fraction (EF) and LV mass normalized to body surface area (BSA), age, and gender in a large cohort of well-treated beta-thalassemia major (ß-TM) patients without heart damage using a multiparametric MRI. STUDY TYPE: Retrospective/cohort study. POPULATION: In all, 251 ß-TM patients with no known risk factors or cardiac disease, normal electrocardiogram, no macroscopic myocardial fibrosis, and all cardiac segments with T2 * ≥20 msec, and 246 healthy subjects. FIELD STRENGTH/SEQUENCE: 1.5T/cine steady-state free precession (SSFP), gradient-echo T2 *, late gadolinium enhancement (LGE) images. ASSESSMENT: Biventricular end-diastolic volume, end-systolic volume, stroke volume, and LV mass were normalized to BSA (EDVI, ESVI, SVI). STATISTICAL TESTS: Comparisons between the two groups was performed with two-samples t-test or Wilcoxon's signed rank test. For more than two groups, one-way analysis of variance (ANOVA) or a Kruskal-Wallis test were applied. RESULTS: Compared to controls, males with ß-TM showed significantlt higher LVEDVI in all the age groups, while for the other volumes the difference was significant only within one or more age groups. In females the volumes were comparable between ß-TM patients and healthy subjects in all the age groups. In the male ß-TM population we found a significant effect of age on LVEDVI (P = 0.017), LVESVI (P = 0.001), RVESVI (P = 0.029), and RVEF (P = 0.031), while for females none of the biventricular parameters were significantly different among the age groups (LVEDVI: P = 0.614; LVESVI: P = 0.449; LVSVI: P = 0.186; LV mass index: P = 0.071; LVEF: P = 0.059; RVEDVI: P = 0.374; RVESVI: P = 0.180; RVSVI: P = 0.206; RVEF: P = 0.057). In ß-TM patients all biventricular volume indexes as well as the LV mass index were significantly larger in males than in females (P < 0.0001 in all cases). The LV and the RV EF were comparable between the sexes (P = 0.568 and P = 0.268, respectively). DATA CONCLUSION: Appropriate "normal" reference ranges normalized to BSA, sex, and age are recommended to avoid misdiagnosis of cardiomyopathy in ß-TM patients. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY STAGE: 2.


Assuntos
Traumatismos Cardíacos , Talassemia beta , Superfície Corporal , Estudos de Coortes , Meios de Contraste , Feminino , Gadolínio , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Valores de Referência , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda , Talassemia beta/diagnóstico por imagem
8.
Ann Hematol ; 97(10): 1925-1932, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29926157

RESUMO

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. Biventricular function parameters were quantified by cine sequences. No patient treated with DFO had cardiac iron. At baseline, only one non-chelated patient showed a pathological heart T2* value (< 20 ms) and he recovered at the follow-up. The percentage of patients who maintained a normal heart T2* value was 100% in both groups. A significant increase in the right ventricular ejection fraction was detected in DFO patients (3.48 ± 7.22%; P = 0.024). The changes in cardiac T2* values and in the biventricular function were comparable between the two groups. In patients with hepatic iron at baseline (MRI liver iron concentration (LIC) ≥ 3 mg/g/dw), the reduction in MRI LIC values was significant only in the DFO group (- 2.20 ± 4.84 mg/g/dw; P = 0.050). The decrease in MRI LIC was comparable between the groups. In conclusion, in NTDT patients, DFO therapy showed no advantage in terms of cardiac iron but its administration allowed an improvement in right ventricular function. Moreover, DFO reduced hepatic iron in patients with significant iron burden at baseline.


Assuntos
Terapia por Quelação , Desferroxamina/uso terapêutico , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/prevenção & controle , Talassemia/tratamento farmacológico , Adolescente , Adulto , Idoso , Terapia por Quelação/métodos , Criança , Feminino , Seguimentos , Coração/diagnóstico por imagem , Coração/efeitos dos fármacos , Humanos , Ferro/metabolismo , Masculino , Pessoa de Meia-Idade , Miocárdio/metabolismo , Adulto Jovem
9.
Br J Haematol ; 180(6): 879-888, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29504129

RESUMO

We assessed whether male gender was associated with a higher risk of cardiac iron accumulation and fibrosis, heart dysfunction and complications in a large, multicentre cohort of thalassaemia major (TM) patients, in order to optimize the timing in cardiac follow-up. We considered 1711 TM patients (899 females, 31·09 ± 9·08 years), enrolled in the Myocardial Iron Overload in Thalassaemia Network. Clinical/instrumental data are recorded from birth to the first Cardiovascular Magnetic Resonance Imaging scan. Although having a similar risk of accumulating iron, males showed a significantly higher risk of developing cardiac dysfunction, heart failure, arrhythmias and cardiac complications overall, when compared to females (P < 0·0001). Up to 20-30 years of follow-up, the Kaplan-Meier curves for the outcomes for which the male sex was a significant prognosticator almost overlapped, whereas they clearly diverged after this period. In patients with follow-up longer than 20 years, males exhibited a significantly higher risk of ventricular dysfunction, heart failure, arrhythmias, and cardiac complications. Female patients may have an intrinsically better tolerance for iron toxicity. International guidelines suggest annual cardiac evaluation for thalassaemia patients. It is possible that female patients can be evaluated at longer intervals, thus reducing health costs.


Assuntos
Arritmias Cardíacas , Insuficiência Cardíaca , Imageamento por Ressonância Magnética , Caracteres Sexuais , Disfunção Ventricular , Adulto , Arritmias Cardíacas/diagnóstico por imagem , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/metabolismo , Arritmias Cardíacas/mortalidade , Intervalo Livre de Doença , Feminino , Seguimentos , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/metabolismo , Insuficiência Cardíaca/mortalidade , Humanos , Ferro/metabolismo , Masculino , Taxa de Sobrevida , Disfunção Ventricular/diagnóstico por imagem , Disfunção Ventricular/etiologia , Disfunção Ventricular/metabolismo , Disfunção Ventricular/mortalidade , Talassemia beta/diagnóstico por imagem , Talassemia beta/metabolismo , Talassemia beta/mortalidade , Talassemia beta/terapia
10.
Biomark Med ; 12(4): 341-348, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29569468

RESUMO

AIM: to evaluate the relationship between uric acid (UA), hepatic and cardiac iron overload (T2*-MRI), ferritin, endocrinological diseases and cardiac complications in a large thalassemia major (TM) cohort. METHODS: A total of 369 TM patients (187 men; 33 ± 6 years) were retrospectively studied, from the myocardial iron overload in thalassemia (MIOT) electronic databank. RESULTS: Multiple regression model identified male sex (p < 0.001), BMI (p < 0.001) and T2* (p ≤ 0.001) as UA independent correlates. Moreover, UA and derivatives of reactive oxygen species (an oxidative index; r = -0.3; p ≤ 0.05) are inversely correlated. Conversely, the multivariate logistic analysis identified low UA (NANHES-III criteria) as one independent predictor for low global heart T2* (p < 0.5) together with liver iron concentrations (>3 mg/g/dw), heart failure, endocrinopathies, ferritin (>2000 ng/l), alanine transaminase (>40 UI/l) and/or aspartate transaminase (>35 UI/l) and/or glutamyl transferase (>64 UI/l). DISCUSSION: UA appears directly associated to T2* and inversely with derivatives of reactive oxygen species, and as such reduced according to increased oxidative stress and cardiac iron overload in TM patients.


Assuntos
Miocárdio/metabolismo , Ácido Úrico/sangue , Talassemia beta/sangue , Adulto , Estudos de Coortes , Feminino , Ferritinas/sangue , Humanos , Ferro/metabolismo , Fígado/metabolismo , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Talassemia beta/diagnóstico por imagem , Talassemia beta/metabolismo
11.
Eur Heart J Cardiovasc Imaging ; 19(3): 299-309, 2018 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-28200076

RESUMO

Aims: Cardiovascular magnetic resonance (CMR) has dramatically changed the clinical practice in thalassemia major (TM), lowering cardiac complications. We prospectively reassessed the predictive value of CMR parameters for heart failure (HF) and arrhythmias in TM. Methods and results: We considered 481 white TM patients (29.48 ± 8.93 years, 263 females) enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network. Myocardial and liver iron overload were measured by T2* multiecho technique. Atrial dimensions and biventricular function were quantified by cine images. Late gadolinium enhancement images were acquired to detect myocardial fibrosis. Mean follow-up was 57.91 ± 18.23 months. After the first CMR scan 69.6% of the patients changed chelation regimen. We recorded 18 episodes of HF. In the multivariate analysis the independent predictive factors were myocardial fibrosis (HR = 10.94, 95% CI = 3.28-36.43, P < 0.0001), homogeneous MIO (compared with no MIO) (HR = 5.56, 95% CI = 1.37-22.51, P = 0.016), ventricular dysfunction (HR = 4.33, 95% CI = 1.39-13.43, P = 0.011). Arrhythmias occurred in 16 patients. Among the CMR parameters only the atrial dilation was identified as univariate prognosticator (HR = 4.26 95% CI=1.54-11.75, P = 0.005). Conclusions: CMR guided the change of chelation therapy in nearly 70% of patients, leading to a lower risk of iron-mediated HF and of arrhythmias than previously reported. Homogeneous MIO remained a risk factor for HF but also myocardial fibrosis and ventricular dysfunction identified patients at high risk. Arrhythmias were independent of MIO but increased with atrial dilatation. CMR by a multi-parametric approach dramatically improves cardiac outcomes and provides prognostic information beyond cardiac iron estimation.


Assuntos
Arritmias Cardíacas/etiologia , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/etiologia , Imagem Cinética por Ressonância Magnética/métodos , Talassemia beta/complicações , Talassemia beta/diagnóstico , Adulto , Arritmias Cardíacas/fisiopatologia , Terapia por Quelação/métodos , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Adulto Jovem , Talassemia beta/terapia
12.
Am J Hematol ; 90(11): 1008-12, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26228763

RESUMO

The aim of this study was to evaluate, in a large cohort of chronically transfused patients, whether the presence of extramedullary hematopoiesis (EMH) accounts for the typical patterns of cardiac iron distribution and/or cardiac function parameters. We retrospectively selected 1,266 thalassemia major patients who had undergone regular transfusions (611 men and 655 women; mean age: 31.3 ± 8.9 years, range: 4.2-66.6 years) and were consecutively enrolled within the Myocardial Iron Overload in Thalassemia network. The presence of EMH was evaluated based on steady-state free precession sequences; cardiac and liver iron overloads were quantified using a multiecho T2* approach; cardiac function parameters and pulmonary diameter were quantified using the steady-state free precession sequences; and myocardial fibrosis was evaluated using the late gadolinium enhancement technique. EMH was detected in 167 (13.2%) patients. The EMH+ patients had significantly lower cardiac iron overload than that of the EMH- patients (P = 0.003). The patterns of cardiac iron distribution were significantly different in the EMH+ and EMH- patients (P < 0.0001), with a higher prevalence of patients with no myocardial iron overload and heterogeneous myocardial iron overload and no significant global heart iron in the EMH+ group EMH+ patients had a significantly higher left ventricle mass index (P = 0.001) and a significantly higher pulmonary artery diameter (P = 0.002). In conclusion, in regularly transfused thalassemia patients, EMH was common and was associated with a thalassemia intermedia-like pattern of cardiac iron deposition despite regular transfusion therapy.


Assuntos
Hematopoese Extramedular , Sobrecarga de Ferro/metabolismo , Ferro/metabolismo , Miocárdio/metabolismo , Reação Transfusional , Talassemia beta/metabolismo , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Estudos Transversais , Feminino , Ventrículos do Coração/metabolismo , Ventrículos do Coração/patologia , Humanos , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/patologia , Fígado/metabolismo , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Estudos Retrospectivos , Talassemia beta/patologia , Talassemia beta/terapia
14.
J Cardiovasc Magn Reson ; 15: 1, 2013 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-23324167

RESUMO

BACKGROUND: Due to the limited data available in literature, the aim of this multi-centre study was to prospectively compare in thalassemia major (TM) patients the efficacy of combined deferiprone (DFP) and deferoxamine (DFO) regimen versus either DFP and DFO in monotherapy by cardiovascular magnetic resonance (CMR) over a follow up of 18 months. METHODS: Among the first 1135 TM patients in the MIOT (Myocardial Iron Overload in Thalassemia) network, we evaluated those who had received either combined regimen (DFO + DFP, N=51) or DFP (N=39) and DFO (N=74) monotherapies between the two CMR scans. Iron overload was measured by T2* multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. RESULTS: The percentage of patients that maintained a normal global heart T2* value was comparable between DFP+DFO versus both monotherapy groups. Among the patients with myocardial iron overload at baseline, the changes in the global heart T2* and in biventricular function were not significantly different in DFP+DFO compared with the DFP group. The improvement in the global heart T2* was significantly higher in the DFP+DFO than the DFO group, without a difference in biventricular function. Among the patients with hepatic iron at baseline, the decrease in liver iron concentration values was significantly higher with combination therapy than with either monotherapy group. CONCLUSIONS: In TM patients at the dosages used in the real world, the combined DFP+DFO regimen was more effective in removing cardiac iron than DFO, and was superior in clearing hepatic iron than either DFO or DFP monotherapy. Combined therapy did not show an additional effect on heart function over DFP.


Assuntos
Cardiomiopatias/tratamento farmacológico , Desferroxamina/uso terapêutico , Quelantes de Ferro/uso terapêutico , Fígado/efeitos dos fármacos , Miocárdio/metabolismo , Piridonas/uso terapêutico , Volume Sistólico/efeitos dos fármacos , Função Ventricular Esquerda/efeitos dos fármacos , Função Ventricular Direita/efeitos dos fármacos , Talassemia beta/tratamento farmacológico , Adulto , Análise de Variância , Cardiomiopatias/etiologia , Cardiomiopatias/patologia , Cardiomiopatias/fisiopatologia , Distribuição de Qui-Quadrado , Deferiprona , Quimioterapia Combinada , Feminino , Humanos , Itália , Fígado/metabolismo , Fígado/patologia , Imagem Cinética por Ressonância Magnética , Masculino , Miocárdio/patologia , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem , Talassemia beta/complicações , Talassemia beta/diagnóstico
15.
Magn Reson Med ; 65(3): 764-9, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21337408

RESUMO

Multiecho gradient-echo T*2 magnetic resonance imaging is a well-established technique for iron overload assessment but there are few reports concerning the pancreas. The aim of this work was to assess the feasibility and reproducibility of the magnetic resonance imaging for measuring pancreatic regional and global T*2 values, to establish the lower limit of normal in a large cohort of healthy subjects and to correlate the measured values with age and gender. One hundred and twenty healthy subjects (61 males, 51±17 years) underwent magnetic resonance imaging (1.5T) using a multiecho gradient-echo T*2 sequence. T*2 measurements were performed in pancreatic head, body, and tail. The global value was calculated as the mean. Measurement of pancreatic T*2 values was feasible in all subjects. For the T*2 global value the coefficient of variation for intraoperator and interoperator reproducibility were 7.7% and 13%, respectively. The global T*2 values ranged from 24 to 52 ms with the lower limit of normal of 26 ms. There were no significant differences among the regional pancreatic T*2 values. No significant correlation was found between T*2 and patient age or gender. In conclusion, pancreatic T*2 measurements appear to be feasible, reproducible, nontime-consuming and reliable. Gender- and age-related differences concerning pancreatic T*2 were not found.


Assuntos
Sobrecarga de Ferro/epidemiologia , Sobrecarga de Ferro/patologia , Imageamento por Ressonância Magnética/estatística & dados numéricos , Pâncreas/patologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Valores de Referência , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Distribuição por Sexo , Estatística como Assunto , Adulto Jovem
16.
Haematologica ; 96(1): 41-7, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20884710

RESUMO

BACKGROUND: Oral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and function has not yet been established. Our study aimed to compare the effectiveness of deferasirox, deferiprone and desferrioxamine on myocardial and liver iron concentrations and bi-ventricular function in thalassemia major patients by means of quantitative magnetic resonance imaging. DESIGN AND METHODS: From the first 550 thalassemia subjects enrolled in the Myocardial Iron Overload in Thalassemia network, we retrospectively selected thalassemia major patients who had been receiving one chelator alone for longer than one year. We identified three groups of patients: 24 treated with deferasirox, 42 treated with deferiprone and 89 treated with desferrioxamine. Myocardial iron concentrations were measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Liver iron concentrations were measured by T2* multiecho technique. RESULTS: The global heart T2* value was significantly higher in the deferiprone (34 ± 11 ms) than in the deferasirox (21 ± 12 ms) and the desferrioxamine groups (27 ± 11 ms) (P = 0.0001). We found higher left ventricular ejection fractions in the deferiprone and the desferrioxamine versus the deferasirox group (P = 0.010). Liver iron concentration, measured as T2* signal, was significantly lower in the desferrioxamine versus the deferiprone and the deferasirox group (P = 0.004). CONCLUSIONS: The cohort of patients treated with oral deferiprone showed less myocardial iron burden and better global systolic ventricular function compared to the patients treated with oral deferasirox or subcutaneous desferrioxamine.


Assuntos
Benzoatos/uso terapêutico , Desferroxamina/uso terapêutico , Ferro/metabolismo , Imageamento por Ressonância Magnética , Piridonas/uso terapêutico , Triazóis/uso terapêutico , Função Ventricular/efeitos dos fármacos , Talassemia beta/tratamento farmacológico , Adolescente , Adulto , Criança , Deferasirox , Deferiprona , Quimioterapia Combinada , Feminino , Humanos , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/induzido quimicamente , Sobrecarga de Ferro/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sideróforos/uso terapêutico , Adulto Jovem
17.
Rays ; 30(3): 269-72, 2005.
Artigo em Inglês | MEDLINE | ID: mdl-16512075

RESUMO

The case of a 69-year-old female patient come to our observation with the diagnosis of non-Hodgkin's lymphoma on histology and cystoscopy is discussed. Symptoms were characterized by dysuria, vaginal spotting and abdominal pain. CT and NMR for staging and control CT during chemotherapy were performed. They documented marked reduction of the neoformation protruding into the bladder and concentric urethral thickening associated with volumetric stability of intercavoaortic lymph node involvement and stable condition of the other localizations.


Assuntos
Linfoma de Células B/diagnóstico , Neoplasias Uretrais/diagnóstico , Idoso , Feminino , Humanos , Linfoma de Células B/diagnóstico por imagem , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Neoplasias Uretrais/diagnóstico por imagem
18.
Rays ; 29(2): 157-66, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-15587867

RESUMO

The case of a patient with predominant short-term memory and CT pattern suggestive of of normal pressure hydrocephalus, is discussed. Clinical and CT findings did not allow to predict the clinical response to a possible ventriculoperitoneal shunt. On MRI more accurate morphological evaluation with cine-MR functional assessment was obtained. Calculated CSF flow indexes evidenced hyperdynamia usually associated with a good outcome after ventriculoperitoneal shunting.


Assuntos
Hidrocefalia de Pressão Normal/diagnóstico , Imagem Cinética por Ressonância Magnética/métodos , Humanos
19.
Rays ; 28(1): 13-9, 2003.
Artigo em Inglês | MEDLINE | ID: mdl-14509176

RESUMO

The introduction of spiral multislice or multidetector CT (MSCT) has led to significant results in coronary diagnostic imaging. In fact with MSCT, isotropic (cubic voxel) three-dimensional imaging of large volumes (e.g. the entire cardiac volume) was possible in a single breath-hold. Moreover, with dedicated reconstruction algorithms, temporal resolution and scannable volume could be optimized, limiting the artifacts associated with the spiral technique. The quantification of calcium deposits in the coronary walls and, in particular, the morphologic study of these vessels represent an important challenge to this technique. Multislice CT with retrospective gating is now a relevant diagnostic instrument in coronary heart disease; however only most recent CT devices with 16 rows of detectors enable a real solution of problems of spatial (isotropic, submillimetric imaging) and temporal (< 0.5 s rotation time) required for correct identification of stenosis and plaque characterization, which are the two main goals of noninvasive coronary imaging.


Assuntos
Angiografia Coronária/instrumentação , Angiografia Coronária/métodos , Doença da Artéria Coronariana/diagnóstico por imagem , Tomografia Computadorizada por Raios X/instrumentação , Tomografia Computadorizada por Raios X/métodos , Algoritmos , Desenho de Equipamento , Humanos , Imageamento Tridimensional
20.
Rays ; 28(2): 167-74, 2003.
Artigo em Inglês | MEDLINE | ID: mdl-14509191

RESUMO

A case of a 37-year-old, 8 week pregnant woman come to the emergency service with the diagnosis of cervical pregnancy an metrorrhagia, is reported. Uterine artery embolization was performed to arrest the bleeding. BCF monitoring documented its disappearance after approximately 15 days with progressive decrease in serum beta-hCG levels. Once the death of the fetus was ascertained, placental detachment was facilitated with the administration of methotrexate therapy. In view of the curettage of the uterine cavity a second uterine artery embolization was performed. Twenty days after the diagnosis of abortion, curettage was performed and the patient could be discharged. The combined action of embolization, methotrexate therapy and curettage allowed to preserve the potential fertility of the woman.


Assuntos
Metrorragia/diagnóstico , Metrorragia/terapia , Gravidez Ectópica/diagnóstico , Gravidez Ectópica/terapia , Abortivos não Esteroides/uso terapêutico , Adulto , Terapia Combinada , Curetagem , Embolização Terapêutica , Feminino , Humanos , Metotrexato/uso terapêutico , Metrorragia/etiologia , Gravidez , Gravidez Ectópica/complicações
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