A5006216784- Hemoglobinopathies and Related Disorders
- Iron Metabolism and Disorders
- Blood groups and transfusion
- Prenatal Screening and Diagnostics
- Potassium and Related Disorders
- Pharmacological Effects and Toxicity Studies
- Erythrocyte Function and Pathophysiology
- Liver Disease Diagnosis and Treatment
- Hepatitis C virus research
- Cardiovascular Function and Risk Factors
- COVID-19 Clinical Research Studies
- Folate and B Vitamins Research
- Epigenetics and DNA Methylation
- Advanced MRI Techniques and Applications
- Trace Elements in Health
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Neonatal Health and Biochemistry
- Tuberculosis Research and Epidemiology
- Parvovirus B19 Infection Studies
- Hepatitis B Virus Studies
- Cardiac Imaging and Diagnostics
- Erythropoietin and Anemia Treatment
- Bone health and osteoporosis research
- Complement system in diseases
- Parathyroid Disorders and Treatments
University of Ferrara
1991-2025
Arcispedale Sant'Anna
2014-2023
Ospedale Sant'Anna
1998-2023
Fondazione Toscana Gabriele Monasterio
2015
Ospedali Riuniti di Ancona
2015
Azienda ospedaliera "Bianchi-Melacrino-Morelli"
2015
Ospedale Garibaldi
2015
Azienda Ospedaliero-Universitaria di Modena
2015
Ospedale Vincenzo Cervello
2015
Ospedale A. Perrino
2015
Not available.
A bstract : We studied survival and disease complications in 1,146 patients with thalassemia major, born from January 1, 1960 to December 31, 1987. At last follow‐up, March 1997, probability of age 20 years was 89% 25 82% for the 1970‐1974. Patients who died had a serum ferritin level, measured year before death, significantly higher than those survived. Diabetes present 5.4% patients; heart failure 6.4%; arrhythmias 5.0%, thrombosis 1.1%, hypothyroidism 11.6%, HIV infection 1.8%....
Abstract We report data on survival and complications for a longitudinal cohort of 709 transfusion‐dependent β ‐thalassemia major patients (51.1% males) born between 1970 1997 followed through 2020 at seven centers in Italy. Overall probability 30 years was 83.6% (95%CI: 78.5–89.1) the oldest birth (1970–1974) compared with 93.3% 88.6–98.3) youngest (1985–1997) ( p = 0.073). Females showed better than males 0.022). There were total 93 deaths median age 23.2 most frequent disease‐related...
In transfusion-dependent thalassemia patients who started regular transfusions in early childhood, we prospectively and longitudinally evaluated the efficacy on pancreatic iron of a combined deferiprone (DFP) + desferrioxamine (DFO) regimen versus either oral chelator as monotherapy over follow-up 18 months.
Summary The risk of developing hepatocellular carcinoma ( HCC ) in patients with thalassaemia is increased by transfusion‐transmitted infections and haemosiderosis. All Italian Thalassaemia Centres use an ad hoc form to report all diagnoses the Registry. Since our last report, 2002, up December 2012, 62 new cases were identified, 52% whom affected major TM 45% intermedia TI ). Two had sickle‐thalassaemia ST incidence tumour increasing, possibly because longer survival consequent exposure...
Background— Cardiovascular magnetic resonance (CMR) plays a key role in the management of thalassemia major patients, but few data are available pediatric population. This study aims at retrospective multiparametric CMR assessment myocardial iron overload, function, and fibrosis cohort patients. Methods Results— We studied 107 patients (61 boys, median age 14.4 years). Myocardial liver overload were measured by T2* multiecho technique. Atrial dimensions biventricular function quantified cine...
We systematically explored the link of pancreatic iron with glucose metabolism and cardiac complications in a cohort 1,079 patients thalassemia major (TM) enrolled Extension-Myocardial Iron Overload Thalassemia (E-MIOT) project.MRI was used to quantify overload (T2* technique) function (cine images) detect macroscopic myocardial fibrosis (late gadolinium enhancement technique). Glucose assessed by oral tolerance test (OGTT).Patients normal showed significantly higher global pancreas T2*...
Abstract Aims A tailored chelation therapy guided by magnetic resonance imaging (MRI) is a strategy to improve the prognosis in iron-loaded patients, many cases still hampered limited MRI availability. In order address this issue, Myocardial Iron Overload Thalassemia (MIOT) network was established Italy and we aimed describe impact of 10-year activity on cardiac burden thalassemia major (TM). Methods results Within MIOT network, 1746 TM patients (911 females; mean age 31.2 ± 9.1 years) were...
Summary The relationship between diabetes mellitus ( DM ) and cardiac complications has never been systematically studied in thalassaemia major TM ). We evaluated a large retrospective historical cohort of to determine whether is associated with higher risk heart complications. compared 86 patients affected by 709 without consecutively included the Myocardial Iron Overload Thalassaemia database where clinical/instrumental data are recorded from birth first cardiovascular magnetic resonance...
Introduction: β-thalassemia is caused by autosomal mutations in the β-globin gene, which induce absence or low-level synthesis of erythroid cells. It widely accepted that a high production fetal hemoglobin (HbF) beneficial for patients with β-thalassemia. Sirolimus, also known as rapamycin, lipophilic macrolide isolated from strain Streptomyces hygroscopicus serves strong HbF inducer vitro and vivo. In this study, we report biochemical, molecular, clinical results sirolimus-based NCT03877809...
We assessed the prognostic value of multiparametric cardiovascular magnetic resonance (CMR) in predicting death from heart failure (HF) thalassemia major (TM). considered 1398 white TM patients (30.8 ± 8.9 years, 725 women) without a history HF at baseline CMR, which was performed within Myocardial Iron Overload Thalassemia (MIOT) network. overload quantified by using T2* technique, and biventricular function determined with cine images. Late gadolinium enhancement (LGE) images were acquired...
Background: Cellular biobanks are of great interest for performing studies finalized in the development personalized approaches genetic diseases, including β-thalassemia and sickle cell disease (SCD), important diseases affecting hematopoietic system. These inherited characterized by a global distribution need intensive health care. The aim this report is to present an update on composition cellular Thal-Biobank, describe its utilization since 2016, data application fetal hemoglobin...
Background Cardiac MRI plays a critical role in the management of thalassemic patients. No accurate biventricular reference values are available. Purpose To establish ranges for normal left ventricular (LV) and right (RV) volumes ejection fraction (EF) LV mass normalized to body surface area (BSA), age, gender large cohort well‐treated beta‐thalassemia major (β‐TM) patients without heart damage using multiparametric MRI. Study Type Retrospective/cohort study. Population In all, 251 β‐TM with...
Summary We prospectively assessed the efficacy of deferasirox versus deferiprone or desferrioxamine as monotherapy in thalassaemia major (TM) patients by magnetic resonance imaging (MRI). selected enrolled Myocardial Iron Overload Thalassaemia network who received only one chelator between two MRIs (deferasirox = 235, 142, 162). overload was measured T2* technique and biventricular function cine images. Among with baseline myocardial iron, all three groups there a significant improvement...
A bstract : Osteoporosis and osteopenia are frequent complications of thalassemia major (TM) intermedia (TI). was found in 23/25 patients with TI 115/239 TM. In TM, no association specific polymorphisms candidate genes (vitamin D receptor, estrogen calcitonin collagen type 1 alpha 1). female TM strongly associated primary amenorrhea ( P < .0001 ), while male hypogonadism not significantly related to bone mineral density (BMD) = ). Low BMD also cardiomiopathy .01 diabetes mellitus chronic...
Cellular biobanking is a key resource for collaborative networks planning to use same cells in studies aimed at solving variety of biological and biomedical issues. This approach great importance on β-thalassemia, since the recruitment patients collection specimens can represent crucial often limiting factor experimental planning. Erythroid precursor were obtained from 72 patients, mostly β-thalassemic, expanded cryopreserved. Expression globin genes was analyzed by real time RT-qPCR....
Osteoporosis represents a relevant cause of morbidity in adult Thalassemia Major (TM) population. Antiresorptive drugs such as bisphosphonates were demonstrated effective preventing bone loss. Teriparatide (TP) is an anabolic agent approved for osteoporosis management the general population, but its use has been very limited TM patients so far. We evaluated TP efficacy and safety TM-associated real-life clinical practice. Retrospective evaluation 11 (6 males, 5 females; mean age = 45 ± 4.38...