Piera Samperi
University of Catania
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Featured researches published by Piera Samperi.
Hemoglobin | 1992
Gino Schilirò; Piera Samperi; C. Consalvo; S. Gangarossa; R. Testa; Vito Miraglia; L Lo Nigro
We report the clinical, hematological, and molecular findings observed in 32 Sicilian patients with sickle cell disease. None of our patients received regular blood transfusions and careful infectious disease prophylaxis was carried out for all. Haplotyping of beta S chromosomes was performed in all patients; all were homozygous for haplotype #19 (Benin). Gene mapping excluded the presence of an alpha-thalassemia in 13 of our patients; none of the relatives showed any evidence of the presence of alpha-thalassemia. Hb F levels were 11.8 +/- 5.9% with G gamma representing 39.6 +/- 3.6% of total gamma chain. Hb F levels were higher in females than in males (12.5 +/- 5.9% versus 9.7 +/- 6.5%) but the difference was not statistically significant. All patients, regardless of age and sex, were anemic with normal mean corpuscular hemoglobin concentration, high mean corpuscular volume and mean corpuscular hemoglobin, and mild reticulocytosis. Analysis of clinical manifestations suggests that our patients have a disease of moderate severity.
American Journal of Hematology | 1997
Taha Merghoub; Bruno Perichon; Micheline Maier-Redelsperger; S. P. Dibenedetto; Piera Samperi; Rolande Ducrocq; Nicole Feingold; Jacques Elion; Gino Schilirò; Dominique Labie; Rajagopal Krishnamoorthy
Expression of fetal hemoglobin (Hb F) is under polygenic control involving determinants both linked and unlinked to the β‐globin gene cluster on chromosome 11. Variations in the DNase I‐hypersensitive site 2 of the locus control region (LCR‐HS2) and a C → T change at position −158 from the Gγ‐gene (detected as an XmnI polymorphism) correlate with the high level of Hb F expression in patients with sickle‐cell anemia and β‐thalassemia. Interpretation of data under these conditions of anemic stress is difficult because the preferential survival of Hb F‐containing erythrocytes (F‐cells) may not reflect the true status of Hb F expression. We investigated the relationship between these markers and Hb F expression in terms of F‐cell levels in 48 unrelated non‐anemic AS heterozygotes from Sicily. The βS‐chromosome of all these individuals was of the Benin haplotype and they differed only by their βA chromosomes. We demonstrate that F‐cell expression is more strongly associated with LCR‐HS2 polymorphism than with XmnI polymorphism. The observed association between XmnI polymorphism and Hb F expression is very likely to be due to linkage disequilibrium with LCR‐HS2 sequences. Am. J. Hematol. 56:239–243, 1997.
European Journal of Haematology | 2000
Elena Mirabile; Piera Samperi; A. Di Cataldo; Amelia Poli; M. La Spina; Gino Schilirò
Abstract: We studied 15 Sicilian subjects with Hb H disease correlating clinical examinations with hematological and molecular data. Seven different α‐thal mutations were identified: four deletion types (‐‐MED, ‐‐CAL, ‐α3.7, ‐α4.2) and three nondeletion types (αNcolα, αHphα, αCSα). All the patients had a zero‐gene chromosome (‐‐MED or ‐‐CAL), while the third α gene was deleted (‐α3.7, ‐α4.2) or inactive (αNcolα, αHphα, αCSα). In patients with the nondeletion genotype the analysis of hematological values revealed lower levels of RBC and Hb A2 and significantly higher levels of Hb H. The clinical variability was remarkable, ranging from totally asymptomatic conditions, casually diagnosed, to severe thalassemia intermedia with marked hemolytic crises, liver and spleen enlargement and the necessity for frequent transfusions. The genotype did not justify the gravity of the phenotype in every case, and the differences in clinical manifestations, also notable, are not easily explainable in subjects who apparently have the same genotype.
Acta Haematologica | 1990
Piera Samperi; R. Testa; M. Mancuso; Gino Schilirò
Hb A2 was determined in 477 subjects: 77 were affected by iron deficiency anemia, 172 were carriers of beta-thalassemia trait and 228 were normal controls. Hb A2 was determined by both DE-52 microchromatography and high-performance liquid chromatography (HPLC). The analysis of the data by linear regression demonstrated that the methods furnish overlapping results. Our findings show that HPLC is a rapid and easily reproduced method which allows a quantitative and qualitative discrimination of the various Hb fractions, making it a valid tool in screening programs for hemoglobinopathies.
Journal of Pediatric Hematology Oncology | 1992
Manuela Caruso-Nicoletti; Mancuso M; Spadaro G; Piera Samperi; Consalvo C; Schilirò G
We have evaluated height, weight, bone age, somatome-din-C levels, and pubertal development in 114 Sicilian patients affected by sickle cell diseases (SCDs). Thirty-one had homozygous sickle hemoglobin (SS), 55 S-β0 thalassemia, and 28 S-β+ thalassemia. In both children and adults, the mean height and weight were 1 SD below the normal mean for age. The height was below the normal range only in a few subjects (8 children and 4 adult women). Somatomedin-C levels were within the normal range in most of the patients (37/44 children and 17/22 adults). Bone age revealed a slight delay in skeletal maturation (mean chronological age and bone age were 7.7 ± 3 and 7.11 ± 2.9 respectively; p < 0.05). Mean age at menarche was increased compared to normal subjects. Our findings show that Sicilian patients with SCD exhibit a moderate delay of growth and adolescence but attain a final height within the normal range.
Pharmacological Research | 1991
A. Vanella; Agata Campisi; Carmela Castorina; Valeria Sorrenti; Giuseppa Attaguile; Piera Samperi; Nunzia Azzia; Claudia Di Giacomo; Gino Schilirò
In the present study we have assayed the effect of divicine in G6PD-deficient red blood cells in the presence of deferoxamine (iron-chelating drug) and NaN3 (inhibitor of catalase). The effect of divicine has been compared to oxidative stress by H2O2; haemolysis is regarded as an index of cellular toxicity. In addition, we have tested antioxidant enzymatic systems. No significant change in antioxidant enzymatic systems was found in RBCs from subjects with G6PD deficiency when compared to the control group, either in oxidative haemolysis by divicine or by H2O2; a significant decrease in oxidative haemolysis by H2O2 was observed in the presence of deferoxamine, whereas no change was found in oxidative haemolysis by divicine. The replacement of incubation medium by homologous plasma or the supplementation with bovine serum albumin resulted in a marked decrease of percentage of haemolysis by divicine.
Hemoglobin | 1990
D. Corso; B. Cognata; C. Ciaccio; T. Piazza; S. P. Dibenedetto; Piera Samperi; G. Russo Mancuso; Gino Schilirò
The propositus, a 40-year-old married woman with two children, came to our attention because of mild chronic anemia and biliary colic due to the presence of gallstones. Her case history did not reveal any important previous illnesses
Vaccine | 2011
Raffaella Colombatti; Silverio Perrotta; Nicoletta Masera; Giovanni Palazzi; Lucia Dora Notarangelo; Anna Pusiol; Elisa Bonetto; Lucia De Zen; Agostino Nocerino; Piera Samperi; Giovanna Russo-Mancuso; Laura Sainati
During the recent H1N1 pandemic, children with Sickle Cell Disease (SCD) experienced more hospitalizations and more complications than the general pediatric population. We performed a retrospective multicenter survey at 9 Pediatric Haematology-Oncology Units across Italy. H1N1 admission rate was 5.2%, with all admissions occurring before vaccine availability. Length Of Stay (LOS) was 6.06 days (7.85 for Acute Chest Syndrome), longer than in other countries. Vaccination coverage was not homogeneous, ranging from 0 to 99%; several family-related and health-system related barriers in accessing vaccinations were identified that should be ameliorated to improve coverage in this high risk group of children.
European Journal of Haematology | 2009
Giovanna Russo-Mancuso; Piera Samperi; Simone Gangarossa; Carmela Consalvo; Gino Schilirò
To the editor: Sickle cell disease (SCD) patients have been considered to be at risk of having iron overload due to increased intestinal iron absorption and blood transfusion (1, 2). This hypothesis is based mainly on the finding of high serum ferritin values, which may be elevated during crises (3) and not be a real estimate of iron stores, unless they are measured at a temporal distance from crises. More recent studies, which evaluated patients during their steady state, do not confirm high serum ferritin levels, except for those patients who have been previously transfused (3,4); on the contrary, there is evidence that some patients are iron-deficient ( 5 , 6). However, literature data concern black patients affected by homozygous sickle cell anemia (SCA) (psps), while there are no data regarding white patients with compound heterozygosis pspth. In the present study we evaluated iron status in Sicilian patients with SCD, either psps or pSpth, during their steady state. We studied 31 pediatric patients (range 2-14 years) and 19 adults (range 1545 yr). psps disease was diagnosed in 16 patients, pspoth in 2 1 and f3”p + th in 13 as previously described (7). The patients were not transfused at all or had received not more than one transfusion a year and totally less than 5. Patients who had been previously polytransfused were excluded. None of the patients received any transfusions in the 3 months preceding the study, nor had any clinical manifestation of the disease in the 6-week period prior to blood collection. The following parameters were evaluated for each patient: hematological indexes using a Coulter Counter, percentage of the various Hb fractions by means of electrophoresis on cellulose acetate, HPLC, michrochromatography for HbA2 and Betke’s method for HbF (8), serum iron and transferrin saturation, serum ferritin, FEP and urinary iron secretion. Iron indexes are reported in Table 1 where the patients have been divided into three groups according to their genotype. The differences among the three groups are not significant, except for transferrin saturation which is higher in pspoth patients, for serum ferritin which is more elevated in psps patients, intermediate in pspoth and lower in psp+ th , and for FEP which is higher in psps patients. Urinary iron excretion was (mean 2 l S.D.) 1.39 1.05 mg/24 h compared to 0.22 & 0.19 mg/ 24 h in 24 normal controls ageand sex-matched (p < 0.005). Our patients exhibit iron indexes which fall within or nearly within the normal distribution, except for FEP which is elevated, probably due to reticulocytosis (9). Therefore our results suggest that Sicilian SCD patients who have not been regularly transfused do not show susceptibility to iron overload. As a matter of fact, another 20 patients who have been regularly transfused in the past for at least 2 yr and therefore have not been evaluated in this study did not show serum ferritin values that were excessively elevated (mean 324.4, range 39-700 pg/l). There are
Pediatric Blood & Cancer | 2018
Raffaella Colombatti; Giovanni Palazzi; Nicoletta Masera; Lucia Dora Notarangelo; Elisa Bonetti; Piera Samperi; Angelica Barone; Silverio Perrotta; Elena Facchini; Maurizio Miano; Giovanni Carlo Del Vecchio; Maria Elena Guerzoni; Paola Corti; Federica Menzato; Simone Cesaro; Maddalena Casale; Paolo Rigano; Gian Luca Forni; Giovanna Russo; Laura Sainati
The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population‐based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe.