Search

Your search keyword '"Pinto, Valeria Maria"' showing total 5 results
Start Over Author "Pinto, Valeria Maria" Topic sickle cell disease
5 results on '"Pinto, Valeria Maria"'

4. Development of Algorithm for Clinical Management of Sickle Cell Bone Disease: Evidence for a Role of Vertebral Fractures in Patient Follow-up.

Author

De Franceschi, Lucia, Gabbiani, Daniele, Giusti, Andrea, Forni, Gianluca, Stefanoni, Filippo, Pinto, Valeria Maria, Sartori, Giulia, Balocco, Manuela, Dal Zotto, Chiara, Valenti, Maria Teresa, and Dalle Carbonare, Luca

Subjects
SICKLE cell anemia, MEDICAL protocols, BONE density, VERTEBRAL fractures, BONE diseases, HEMOLYTIC anemia
Abstract

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T-score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD. [ABSTRACT FROM AUTHOR]

Published
2020
Full Text
View/download PDF

5. Transfusional Approach in Multi-Ethnic Sickle Cell Patients: Real-World Practice Data From a Multicenter Survey in Italy

Author

Giovanna Graziadei, Lucia De Franceschi, Laura Sainati, Donatella Venturelli, Nicoletta Masera, Piero Bonomo, Aurora Vassanelli, Maddalena Casale, Gianluca Lodi, Vincenzo Voi, Paolo Rigano, Valeria Maria Pinto, Alessandra Quota, Lucia D. Notarangelo, Giovanna Russo, Massimo Allò, Rosamaria Rosso, Domenico D'Ascola, Elena Facchini, Silvia Macchi, Francesco Arcioni, Federico Bonetti, Enza Rossi, Antonella Sau, Saveria Campisi, Gloria Colarusso, Fiorina Giona, Roberto Lisi, Paola Giordano, Gianluca Boscarol, Aldo Filosa, Sarah Marktel, Paola Maroni, Mauro Murgia, Raffaella Origa, Filomena Longo, Marta Bortolotti, Raffaella Colombatti, Rosario Di Maggio, Raffaella Mariani, Alberto Piperno, Paola Corti, Carmelo Fidone, Giovanni Palazzi, Luca Badalamenti, Barbara Gianesin, Frédéric B. Piel, Gian Luca Forni, Graziadei, Giovanna, De Franceschi, Lucia, Sainati, Laura, Venturelli, Donatella, Masera, Nicoletta, Bonomo, Piero, Vassanelli, Aurora, Casale, Maddalena, Lodi, Gianluca, Voi, Vincenzo, Rigano, Paolo, Pinto, Valeria Maria, Quota, Alessandra, Notarangelo, Lucia D, Russo, Giovanna, Allò, Massimo, Rosso, Rosamaria, D'Ascola, Domenico, Facchini, Elena, Macchi, Silvia, Arcioni, Francesco, Bonetti, Federico, Rossi, Enza, Sau, Antonella, Campisi, Saveria, Colarusso, Gloria, Giona, Fiorina, Lisi, Roberto, Giordano, Paola, Boscarol, Gianluca, Filosa, Aldo, Marktel, Sarah, Maroni, Paola, Murgia, Mauro, Origa, Raffaella, Longo, Filomena, Bortolotti, Marta, Colombatti, Raffaella, Di Maggio, Rosario, Mariani, Raffaella, Piperno, Alberto, Corti, Paola, Fidone, Carmelo, Palazzi, Giovanni, Badalamenti, Luca, Gianesin, Barbara, Piel, Frédéric B, and Forni, Gian Luca

Subjects
multi-ethnicity, transfusion therapy, alloimmunization, hydroxycarbamide, sickle cell disease, General Medicine
Abstract

Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Gold standard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC) transfusion regimens to limit disease progression. Here, we report a retrospective study on 1,579 SCD patients (median age 23 years; 802 males/777 females), referring to 34 comprehensive Italian centers for hemoglobinopathies. Although we observed a similar proportion of Caucasian (47.9%) and African (48.7%) patients, Italian SCD patients clustered into two distinct overall groups: children of African descent and adults of Caucasian descent. We found a subset of SCD patients requiring more intensive therapy with a combination of HC plus chronic transfusion regimen, due to partial failure of HC treatment alone in preventing or reducing sickle cell-related acute manifestations. Notably, we observed a higher use of acute transfusion approaches for SCD patients of African descent when compared to Caucasian subjects. This might be related to (i) age of starting HC treatment; (ii) patients' low social status; (iii) patients' limited access to family practitioners; or (iv) discrimination. In our cohort, alloimmunization was documented in 135 patients (8.5%) and was more common in Caucasians (10.3%) than in Africans (6.6%). Alloimmunization was similar in male and female and more frequent in adults than in children. Our study reinforces the importance of donor-recipient exact matching for ABO, Rhesus, and Kell antigen systems for RBC compatibility as a winning strategy to avoid or limit alloimmunization events that negatively impact the clinical management of SCD-related severe complications.Clinical Trial RegistrationClinicalTrials.gov, identifier: NCT03397017.

Published
2022
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results