Microstructure effect on localized corrosion susceptibility of 8090 Al-Li alloy in sea water

no abstract availabl

Thalassaemia is paradoxically associated with a reduced risk of in-hospital complications and mortality in COVID-19: Data from an international registry

Although numerous patient-specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalassaemic syndromes in COVID-19 patients remains poorly understood. We studied the outcomes of 137 COVID-19 patients with a history of transfusion-dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 +/- 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all-cause mortality. The presence of thalassaemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models

A prospective study of direct medical costs in a large cohort of consecutively enrolled patients with refractory epilepsy in Italy

Objective To evaluate direct medical costs and their predictors in patients with refractory epilepsy enrolled into the SOPHIE study (Study of Outcomes of PHarmacoresistance In Epilepsy) in Italy. Methods Adults and children with refractory epilepsy were enrolled consecutively at 11 tertiary referral centers and followed for 18 months. At entry, all subjects underwent a structured interview and a medical examination, and were asked to keep records of diagnostic examinations, laboratory tests, specialist consultations, treatments, hospital admissions, and day-hospital days during follow-up. Study visits included assessments every 6 months of seizure frequency, health-related quality of life (Quality of Life in Epilepsy Inventory 31), medication-related adverse events (Adverse Event Profile) and mood state (Beck Depression Inventory-II). Cost items were priced by applying Italian tariffs. Cost estimates were adjusted to 2013 values. Results Of 1,124 enrolled individuals, 1,040 completed follow-up. Average annual cost per patient was \ue2\u82\uac 4,677. The highest cost was for antiepileptic drug (AED) treatment (50%), followed by hospital admissions (29% of overall costs). AED polytherapy, seizure frequency during follow-up, grade III pharmacoresistance, medical and psychiatric comorbidities, and occurrence of status epilepticus during follow-up were identified as significant predictors of higher costs. Age between 6 and 11 years, and genetic (idiopathic) generalized epilepsies were associated with the lowest costs. Costs showed prominent variation across centers, largely due to differences in the clinical characteristics of cohorts enrolled at each center and the prescribing of second-generation AEDs. Individual outliers associated with high costs related to hospital admissions had a major influence on costs in many centers. Significance Refractory epilepsy is associated with high costs that affect individuals and society. Costs differ across centers in relation to the characteristics of patients and the extent of use of more expensive, second-generation AEDs. Epilepsy-specific costs cannot be easily differentiated from costs related to comorbidities

Recommendations for self-monitoring in pediatric diabetes: A consensus statement by the ISPED

none165noScaramuzza, Andrea; Cherubini, Valentino; Tumini, Stefano; Bonfanti, Riccardo; Buono, Pietro; Cardella, Francesca; D’Annunzio, Giuseppe; Frongia, Anna Paola; Lombardo, Fortunato; Monciotti, Anna Carla Maria; Rabbone, Ivana; Schiaffini, Riccardo; Toni, Sonia; Zucchini, Stefano; Frontino, Giulio; Iafusco, Dario; Arnaldi, Claudia; Banin, Patrizia; Barbetti, Fabrizio; Beccaria, Luciano; Benelli, Marzia; Berardi, Rosario; Biagioni, Martina; Bianchi, Giuliana; Bizzarri, Carla; Blasetti, Annalisa; Bobbio, Adriana; Boccato, Stefano; Bontempi, Franco; Bruzzese, Mariella; Cadario, Francesco; Calcaterra, Valeria; Cannatà, Alessandra; Cappa, Marco; Cardani, Roberta; Cardinale, Giuliana Marcella; Carloni, Ines; Castaldo, Vincenzo; Cauvin, Vittoria; Cerutti, Franco; Cester, Anna Maria; Chessa, Margherita; Chiarelli, Francesco; Chiari, Giovanni; Chiumello, Giuseppe; Cicchetti, Mario; Cirillo, Dante; Citriniti, Felice; Citro, Giuseppe; Coccioli, Maria Susanna; Cotellessa, Mario; Crinò, Antonino; De Berardinis, Fiorella; De Filippo, Gianpaolo; De Giorgi, Giovanni; De Luca, Filippo; De Marco, Rosaria; Delvecchio, Maurizio; Faleschini, Elena; Federico, Giovanni; Fifi, Anna Rita; Fontana, Franco; Franzese, Adriana; Frezza, Elda; Frongia, Annapaola; Gaiero, Alberto; Galderisi, Alfonso; Gallo, Francesco; Gargantini, Luigi; Ghione, Silvia; Giorgetti, Chiara; Gualtieri, Antonella; Guasti, Monica; Guerraggio, Lucia; Iannilli, Antonio; Ingletto, Dario; Iossa, Carmine; Iovene, Brunella; Iughetti, Lorenzo; Kaufmann, Peter; La Loggia, Alfonso; Lazzaro, Nicola; Lenzi, Lorenzo; Lera, Riccardo; Lia, Rosanna; Lo Presti, Donatella; Lorini, Renata; Lucchesi, Sonia; Luceri, Sergio; Madeo, Simona Filomena; Maffeis, Claudio; Mainetti, Benedetta; Mammi, Francesco; Manca Bitti, Maria Luisa; Marigliano, Marco; Marinari, Alessandra; Marinaro, Anna Maria; Meloni, Gianfranco; Marsciani, Alberto; Mastrangelo, Lisa; Mastrangelo, Costanzo; Meschi, Franco; Minasi, Domenico; Minenna, Adelaide; Minuto, Nicola; Monciotti, Carlamaria; Morganti, Gianfranco; Mozzillo, Enza; Nugnes, Rosa; Paradiso, Emanuela; Pardi, Daniela; Pasquino, Bruno; Patrizia Patera, Ippolita; Pennati, Cristina; Pepe, Rossella; Piccini, Barbara; Perrotta, Angelo; Piccinno, Elvira; Pinelli, Leonardo; Piredda, Gavina; Pocecco, Mauro; Ponzi, Giuseppe; Prandi, Elena; Predieri, Barbara; Prisco, Francesco; Quinci, Maria; Ricciardi, Maria Rossella; Rigamonti, Andrea; Ripoli, Carlo; Sabbion, Alberto; Salardi, Silvana; Salvatoni, Alessandro; Salvo, Caterina; Salzano, Giuseppina; Saporiti, Anna; Sardi, Rita; Schieven, Eleonardo; Scipione, Mirella; Soci, Cristina; Soro, Miriam; Spallino, Luisa; Stamati, Filomena; Suprani, Tosca; Savastio, Silvia; Taccardi, Rosa Anna; Tarchini, Luis; Tomaselli, Letizia; Tonini, Giorgio; Torelli, Cataldo; Tornese, Gianluca; Trada, Michela; Valerio, Giuliana; Vanelli, Maurizio; Vanini, Roberto; Vascotto, Marina; Vergerio, Amedeo; Viscardi, Matteo; Zaffani, Silvana; Zampolli, Maria; Zanatta, Manuela; Zanette, Giorgio; Zanfardino, Angela; Zecchino, Clara; Zedda, Maria Antonietta; Zuccotti, Gian VincenzoScaramuzza, Andrea; Cherubini, Valentino; Tumini, Stefano; Bonfanti, Riccardo; Buono, Pietro; Cardella, Francesca; D’Annunzio, Giuseppe; Frongia, Anna Paola; Lombardo, Fortunato; Monciotti, Anna Carla Maria; Rabbone, Ivana; Schiaffini, Riccardo; Toni, Sonia; Zucchini, Stefano; Frontino, Giulio; Iafusco, Dario; Arnaldi, Claudia; Banin, Patrizia; Barbetti, Fabrizio; Beccaria, Luciano; Benelli, Marzia; Berardi, Rossana; Biagioni, Martina; Bianchi, Giuliana; Bizzarri, Carla; Blasetti, Annalisa; Bobbio, Adriana; Boccato, Stefano; Bontempi, Franco; Bruzzese, Mariella; Cadario, Francesco; Calcaterra, Valeria; Cannatà, Alessandra; Cappa, Marco; Cardani, Roberta; Cardinale, Giuliana Marcella; Carloni, Ines; Castaldo, Vincenzo; Cauvin, Vittoria; Cerutti, Franco; Cester, Anna Maria; Chessa, Margherita; Chiarelli, Francesco; Chiari, Giovanni; Chiumello, Giuseppe; Cicchetti, Mario; Cirillo, Dante; Citriniti, Felice; Citro, Giuseppe; Coccioli, Maria Susanna; Cotellessa, Mario; Crinò, Antonino; De Berardinis, Fiorella; De Filippo, Gianpaolo; De Giorgi, Giovanni; De Luca, Filippo; De Marco, Rosaria; Delvecchio, Maurizio; Faleschini, Elena; Federico, Giovanni; Fifi, Anna Rita; Fontana, Franco; Franzese, Adriana; Frezza, Elda; Frongia, Annapaola; Gaiero, Alberto; Galderisi, Alfonso; Gallo, Francesco; Gargantini, Luigi; Ghione, Silvia; Giorgetti, Chiara; Gualtieri, Antonella; Guasti, Monica; Guerraggio, Lucia; Iannilli, Antonio; Ingletto, Dario; Iossa, Carmine; Iovene, Brunella; Iughetti, Lorenzo; Kaufmann, Peter; La Loggia, Alfonso; Lazzaro, Nicola; Lenzi, Lorenzo; Lera, Riccardo; Lia, Rosanna; Lo Presti, Donatella; Lorini, Renata; Lucchesi, Sonia; Luceri, Sergio; Madeo, Simona Filomena; Maffeis, Claudio; Mainetti, Benedetta; Mammi, Francesco; Manca Bitti, Maria Luisa; Marigliano, Marco; Marinari, Alessandra; Marinaro, Anna Maria; Meloni, Gianfranco; Marsciani, Alberto; Mastrangelo, Lisa; Mastrangelo, Costanzo; Meschi, Franco; Minasi, Domenico; Minenna, Adelaide; Minuto, Nicola; Monciotti, Carlamaria; Morganti, Gianfranco; Mozzillo, Enza; Nugnes, Rosa; Paradiso, Emanuela; Pardi, Daniela; Pasquino, Bruno; Patrizia Patera, Ippolita; Pennati, Cristina; Pepe, Rossella; Piccini, Barbara; Perrotta, Angelo; Piccinno, Elvira; Pinelli, Leonardo; Piredda, Gavina; Pocecco, Mauro; Ponzi, Giuseppe; Prandi, Elena; Predieri, Barbara; Prisco, Francesco; Quinci, Maria; Ricciardi, Maria Rossella; Rigamonti, Andrea; Ripoli, Carlo; Sabbion, Alberto; Salardi, Silvana; Salvatoni, Alessandro; Salvo, Caterina; Salzano, Giuseppina; Saporiti, Anna; Sardi, Rita; Schieven, Eleonardo; Scipione, Mirella; Soci, Cristina; Soro, Miriam; Spallino, Luisa; Stamati, Filomena; Suprani, Tosca; Savastio, Silvia; Taccardi, Rosa Anna; Tarchini, Luis; Tomaselli, Letizia; Tonini, Giorgio; Torelli, Cataldo; Tornese, Gianluca; Trada, Michela; Valerio, Giuliana; Vanelli, Maurizio; Vanini, Roberto; Vascotto, Marina; Vergerio, Amedeo; Viscardi, Matteo; Zaffani, Silvana; Zampolli, Maria; Zanatta, Manuela; Zanette, Giorgio; Zanfardino, Angela; Zecchino, Clara; Zedda, Maria Antonietta; Zuccotti, Gian Vincenz

An Italian consensus on the management of Lennox-Gastaut syndrome

Purpose: Although international guidelines exist, the clinical heterogeneity of Lennox-Gastaut syndrome (LGS) and the increasing availability of new and repurposed drugs (e.g., fenfluramine and cannabidiol) requires a practical guide to patient management in the clinical context. We report the results of a consensus survey among 42 Italian experts in the diagnosis and treatment of LGS. Methods: The consensus procedure followed a modified Delphi approach. Statements were formulated, based on the most recent published evidence and the clinicians' personal experience, then discussed, and agreed upon by the experts through a two-round voting procedure. Approval of a statement was reached with an average score >= 7. Results: Thirteen statements dealing with three main topics (i.e., clinical diagnosis and prognosis, impact on the Quality of Life (QoL), and treatment strategies) were generated. Six statements achieved a level of agreement sufficient for approval on the first voting round. Following the discussion and a few consequent amendments, most of the statements increased their level of agreement and all 13 were approved. Conclusions: Overall, the statements draw a slightly more benign picture of this rare and severe disease, highlighting the possibility of remission - albeit modest -, an apparent trend towards lower mortality, and the availability of several effective drugs, to which greater accessibility would be hoped for. Valproate remains a major therapeutic option in LGS patients although lamotrigine, rufinamide, topiramate, cannabidiol, and clobazam are popular therapeutic options in Italy, allowing for a tailor-made antiseizure therapy

The changing landscape of neonatal diabetes mellitus in Italy between 2003-2022

Context: In the last decade Sanger method of DNA sequencing has been replaced by next generation sequencing (NGS). NGS is valuable in conditions characterized by high genetic heterogeneity such as neonatal diabetes mellitus (NDM). Objective: To compare results of genetic analysis of patients with NDM and congenital severe insulin resistance (c.SIR) identified in Italy in 2003-2012 (Sanger) versus 2013-2022 (NGS). Methods: We reviewed clinical and genetic records of 104 cases with diabetes onset before 6 months of age (NDM+c.SIR) of the Italian dataset. Results: Fiftyfive patients (50 NDM + 5 c.SIR) were identified during 2003-2012 and 49 (46 NDM + 3 c.SIR) in 2013-2022. Twenty-year incidence was 1:103,340 (NDM) and 1:1,240,082 (c.SIR) live births. Frequent NDM/c.SIR genetic defects (KCNJ11, INS, ABCC8, 6q24, INSR) were detected in 41 and 34 probands during 2003-2012 and 2013-2022, respectively. We identified a pathogenic variant in rare genes in a single proband (GATA4) (1/42 or 2.4%) during 2003-2012 and in 8 infants (RFX6, PDX1, GATA6, HNF1B, FOXP3, IL2RA, LRBA, BSCL2) during 2013-2022 (8/42 or 19%, p= 0.034 vs 2003-2012). Notably, five among rare genes were recessive. Swift and accurate genetic diagnosis led to appropriate treatment: patients with autoimmune NDM (FOXP3, IL2RA, LRBA), were subjected to bone marrow transplant; patients with pancreas agenesis/hypoplasia (RFX6, PDX1) were supplemented with pancreatic enzymes and the individual with lipodystrophy caused by BSCL2 was started on metreleptin. Conclusions: NGS substantially improved diagnosis and precision therapy of monogenic forms of neonatal diabetes and congenital SIR in Italy