Development of interactive algorithm for clinical management of acute events related to sickle cell disease in emergency department

Sickle cell disease (SCD ORPHA232; OMIM 603903) is a rare hereditary red cell disorder, which global distribution is changed in the last decade due to immigration-fluxes from endemic areas to Western-countries. One of the main clinical manifestations of SCD are the acute painful vaso-occlusive crisis, which cause frequent accesses of SCD patients to the emergency departments (EDs). This has generated the requirement of feasible tools for emergency givers. In the context of the scientific-Italian-Society for the study of Thalassemias and Hemoglobinopathies (SITE), we developed an algorithm with interactive windows to guide physicians in managing SCD patients in EDs

Deferiprone versus Deferoxamine in Sickle Cell Disease: Results from a 5-year long-term Italian multi-center randomized clinical trial.

Blood transfusion and iron chelation currently represent a supportive therapy to manage anemia, vasculopathy and vaso-occlusion crises in Sickle-Cell-Disease. Here we describe the first 5-year long-term randomized clinical trial comparing Deferiprone versus Deferoxamine in patients with Sickle-Cell-Disease. The results of this study show that Deferiprone has the same effectiveness as Deferoxamine in decreasing body iron burden, measured as repeated measurements of serum ferritin concentrations on the same patient over 5-years and analyzed according to the linear mixed-effects model (LMM) (p=0.822). Both chelators are able to decrease, significantly, serum ferritin concentrations, during 5-years, without any effect on safety (p=0.005). Moreover, although the basal serum ferritin levels were higher in transfused compared with non-transfused group (p=0.031), the changes over time in serum ferritin levels were not statistically significantly different between transfused and non-transfused cohort of patients (p=0.389). Kaplan-Meier curve, during 5-years of study, suggests that Deferiprone does not alter survival in comparison with Deferoxamine (p=0.38). In conclusion, long-term iron chelation therapy with Deferiprone was associated with efficacy and safety similar to that of Deferoxamine. Therefore, in patients with Sickle-Cell-Disease, Deferiprone may represent an effective long-term treatment option

The seismic design of steep reinforced earth.

The seismic design of reinforced earth should be arranged in the frame of both serviceability and ultimate limit states, in agreement with the suggestions outlined in the European and the Italian codes (EUROCODE 8 2005, AGI 2005, NTC 2008). The reason may be found in the fact that it is more realistic to accept different levels of damage in relation to a given return period of the design earthquake (perfor-mance-based design approach). However, even though much work has been done in this field to concrete and geotechnical structures, very little consideration has been devoted to composite and mixed works such as reinforced earth with geosynthetics. The paper deals with the different design methods for steep reinforced earth provided of geosynthetic reinforcements in relation to the possible limit states. Restrictions, capabilities and improvements of the design procedures are highlighted, with the aim of making easier the performance-based design of these structures

Elucidation of the Lipid Composition of Hemp (<i>Cannabis sativa</i> L.) Products by Means of Gas Chromatography and Ultra-High Performance Liquid Chromatography Coupled to Mass Spectrometry Detection

The growing demand in natural matrices that represent a source of dietary and nutraceutical molecules has led to an increasing interest in Cannabis sativa, considered to be a multipurpose, sustainable crop. Particularly, the considerable content in essential fatty acids (FAs) makes its derived-products useful food ingredients in the formulation of dietary supplements. In this research, the FA and triacylglycerol (TAG) composition of hempseed oils and flours were investigated using gas chromatography coupled to mass spectrometry and flame ionization detection as well as liquid chromatography coupled to mass spectrometry (LC-MS), respectively. Furthermore, a recently introduced linear retention index (LRI) approach in LC was successfully employed as a useful tool for the reliable identification of TAG species. A total of 30 FAs and 62 glycerolipids were positively identified in the investigated samples. Relative quantitative analyses confirmed linoleic acid as the most abundant component (50–55%). A favorable omega6/omega3 ratio was also measured in hemp-derived products, with the α-linolenic acid around 12–14%. Whereas, γ-linolenic acid was found to be higher than 1.70%. These results confirm the great value of Cannabis sativa as a source of valuable lipids, and the further improvement of the LRI system paves the way for the automatization of the identification process in LC

Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis

Background: The technology to recognize nucleated red blood cells (NRBC) automatically has only recently been developed. Modern hematology analyzers allow for rapid and accurate NRBC counts. The goal of our study was to evaluate NRBC counts and the concentrations of serum transferrin receptor (sTfR) in patients affected by different thalassemia syndromes and hereditary spherocytosis. We wished to gain a better understanding of the meaning of the presence of NRBC in peripheral blood and the relationship of the two parameters with effective and ineffective erythropoiesis in the different thalassemia syndromes. Methods: NRBC counts in peripheral blood were evaluated in a large group of patients with thalassemia (36 thalassemia major, 55 thalassemia intermedia and 61 Sβ-thalassemia patients) and compared with data from 29 patients with hereditary spherocytosis; in all the patients the concentration of sTfR was evaluated as an index of global erythropoiesis. Results: The NRBC count showed a good relationship with ineffective erythropoiesis: highest counts were observed in the thalassemia syndromes characterized by almost completely ineffective erythropoiesis. NRBCs were absent in patients affected by hereditary spherocitosis, a disease characterized by effective erythropoiesis. Conclusions: The NRBC count can be useful for better defining ineffective erythropoiesis in patients with thalassemia, and can help optimize transfusion therapy in severe thalassemia syndromes. Clin Chem Lab Med 2009;47:1539–42.Peer Reviewe

Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia

In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (&lt;15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU (“no HU” group). Among patients taking &lt;15 mg/kg/day HU on their first visit, half remained at the same dose, and the other half increased to ≥15 mg/kg/day. Among patients taking ≥15 mg/kg/day, 17% decreased to &lt;15 mg/kg/day, and 83% stayed at ≥15 mg/kg/day. The “no HU” group had fewer episodes of VOC and ACS. Both HU treatment groups had a reduction in both complications (p &lt; 0.0001). This improvement was observed in all SCD phenotypes. The white blood cell (WBC) counts were found to be lower, and HbF increased in both HU groups (p = 0.004, 0.001). The maximal HbF response to HU in HbS/β+-thalassemia was 20%, similar to those observed for HbSS (19%) and HbS/β0-thalassemia (22%). HbS/β+-thalassemia could have a similar disease severity as HbSS or HbS/β0-thalassemia. Patients with HbS/β0-thalassemia or HbS/β+-thalassemia phenotypes responded to HU

The use of hydroxyurea in the real life of MIOT network: an observational study

Background Hydroxyurea (HU) has been widely used in clinical practice to manage patients with non-transfusion dependent thalassemia (NTDT). Few data are available about the effects of its administration in Italian patients. We assessed hematological and non-hematological outcomes following short- and long-term exposure to HU. Research design and methods We considered 71 NTDT patients (30 females) enrolled in the Myocardial Iron Overload in Thalassemia Network and treated for >12 months with HU. Results The mean duration of HU treatment was 8.23 +/- 5.79 years, starting at a mean age of 37.02 +/- 12.06 years. A significant increase in hemoglobin and mean corpuscular volume values and a down-regulation of all erythropoietic and/or hemolysis indices were detected after at least 12 months of treatment. In 28 patients the hemoglobin increase was >= 1.0 g/dl, associated with a higher HU dose. The hematological response dropped in long-term treatment. A favorable impact of HU treatment in limiting the progression of several complications typical of NTDT syndrome was observed. Conclusion Our findings seemed to suggest that in several NTDT patients HU could be still a valid option to limit the advance in overall disease clinical burden without carrying significant adverse events and increase in mortality

Italian Guidelines in Patch Testing - adapted from the European Society of Contact Dermatitis (ESCD)

Patch testing is the standard procedure used to diagnose allergic contact dermatitis. It is an in-vivo test, which reproduces the reaction to a contact allergen. This in-vivo test aims to reproduce the elicitation phase of allergic contact dermatitis and is performed applying allergens under occlusion on the skin under standardized conditions. These guidelines for the best practice in performing patch test have been developed by an Italian group of experts taking in account the Italian legislation and local pharmacological governance. Guidelines are adapted from the original article under the guidance of the European Society of Contact Dermatitis (ESCD) and on the basis of the SIDAPA guidelines

Italian Guidelines in Patch Testing - adapted from the European Society of Contact Dermatitis (ESCD)

Patch testing is the standard procedure used to diagnose allergic contact dermatitis. It is an in-vivo test, which reproduces the reaction to a contact allergen. This in-vivo test aims to reproduce the elicitation phase of allergic contact dermatitis and is performed applying allergens under occlusion on the skin under standardized conditions. These guidelines for the best practice in performing patch test have been developed by an Italian group of experts taking in account the Italian legislation and local pharmacological governance. Guidelines are adapted from the original article under the guidance of the European Society of Contact Dermatitis (ESCD) and on the basis of the SIDAPA guidelines