Risk and sustainability analysis of complex hydrogen infrastructures

Building a network of hydrogen refuelling stations is essential to develop the hydrogen economy within transport. Additional, hydrogen is regarded a likely key component to store and convert back excess electrical power to secure future energy supply and to improve the quality of biomass-based fuels. Therefore, future hydrogen supply and distribution chains will have to address several objectives. Such a complexity is a challenge for risk assessment and risk management of these chains because of the increasing interactions. Improved methods are needed to assess the supply chain as a whole. The method of "Functional modelling" is discussed in this paper. It will be shown how it could be a basis for other decision support methods for comprehensive risk and sustainability assessments

AIF-1 gene does not confer susceptibility to Behçet's disease: Analysis of extended haplotypes in Sardinian population

Background BehcEet's disease (BD) is a polygenic immune-mediated disorder characterized by a close association with the HLA-B∗51 allele. The HLA region has a strong linkage disequilibrium (LD) and carries several genetic variants (e.g. MIC-A, TNF-α genes) identified as associated to BD because of their LD with HLA-B∗51. In fact, the HLA-B∗51 is inherited as part of extended HLA haplotypes which are well preserved in patients with BD. Sardinian population is highly differentiated from other Mediterranean populations because of a distinctive genetic structure with very highly preserved HLA haplotypes. Patients and methods In order to identify other genes of susceptibility to BD within the HLA region we investigated the distribution of human Allograft Inflammatory Factor-1 (AIF-1) gene variants among BD patients and healthy controls from Sardinia. Six (rs2736182; rs2259571; rs2269475; rs2857597; rs13195276; rs4711274) AIF-1 single nucleotide polymorphisms (SNPs) and related extended haplotypes have been investigated as well as their LD within the HLA region and with HLA-B∗51. Overall, 64 BD patients, 43 HLA-B∗51 positive healthy controls (HC) and 70 random HC were enrolled in the study. Results HLA-B∗51 was the only allele with significantly higher frequency (pc = 0.0021) in BD patients (40.6%) than in HC (9.8%). The rs2259571TAIF-1 variant had a significantly reduced phenotypic, but not allelic frequency in BD patients (72.1%; pc = 0.014) compared to healthy population (91.3%). That was likely due to the LD between HLA-B∗51 and rs2259571G(pc= 9E-5), even though the rs2259571Gdistribution did not significantly differ between BD patients and HC. Conclusion No significant difference in distribution of AIF-1 SNPs haplotypes was observed between BD patients and HC and between HLA-B∗51 positive BD patients and HLA-B∗51 positive HC. Taken together, these results suggest that AIF-1 gene is not associated with susceptibility to BD in Sardinia

Association of the HLA-A2, Cw2, B27, S31, DR2 haplotype with Ankylosing Spondylitis. A possible role of non-B27 factors in the disease

With the aim of searching for HLA haplotypes and non-B27 allele frequency variations in Sardinian AS patients, HLA-A, B, Cw, DR, DQ and Bf, C4A and C4B typing and haplotype assignment was carried out in the families of 25 AS patients and in 44 healthy individuals, all B27 heterozygotes. In the AS patients a significant increase of the A2, Cw2, B27, DR2, DQ1 haplotype was found. This depends only partially on the linkage disequilibrium existing in the Sardinian population between B27 and the other alleles of this haplotype, and rather seems to be due to a primary association of Cw2 and DR2 alleles with AS. Preliminary data seem to show that this haplotype bears the S3l complotype and the ORB1 * 1601 allele both in the AS patients and in the healthy controls. The pathogenetic implications of these findings are discussed

Distribution of killer cell immunoglobulin-like receptors genes in the Italian Caucasian population

BACKGROUND: Killer cell immunoglobulin-like receptors (KIRs) are a family of inhibitory and activatory receptors that are expressed by most natural killer (NK) cells. The KIR gene family is polymorphic: genomic diversity is achieved through differences in gene content and allelic polymorphism. The number of KIR loci has been reported to vary among individuals, resulting in different KIR haplotypes. In this study we report the genotypic structure of KIRs in 217 unrelated healthy Italian individuals from 22 immunogenetics laboratories, located in the northern, central and southern regions of Italy. METHODS: Two hundred and seventeen DNA samples were studied by a low resolution PCR-SSP kit designed to identify all KIR genes. RESULTS: All 17 KIR genes were observed in the population with different frequencies than other Caucasian and non-Caucasian populations; framework genes KIR3DL3, KIR3DP1, KIR2DL4 and KIR3DL2 were present in all individuals. Sixty-five different profiles were found in this Italian population study. Haplotype A remains the most prevalent and genotype 1, with a frequency of 28.5%, is the most commonly observed in the Italian population. CONCLUSION: The Italian Caucasian population shows polymorphism of the KIR gene family like other Caucasian and non-Caucasian populations. Although 64 genotypes have been observed, genotype 1 remains the most frequent as already observed in other populations. Such knowledge of the KIR gene distribution in populations is very useful in the study of associations with diseases and in selection of donors for haploidentical bone marrow transplantation

Retrospective screening of solid organ donors in Italy, 2009, reveals unpredicted circulation of West Nile virus

Since the occurrence of West Nile virus (WNV) infection in humans in 2008 in Italy, concerns have been raised about the potential risks associated with solid organ transplantation (SOT). A nationwide retrospective survey showed that 1.2% of SOT donors in 2009 were WNV-seropositive and demonstrated that human WNV infection is distributed throughout several Italian regions. Transmission of WNV or other arboviruses through SOT is a possibility and risk assessment should be carried out before SOT to avoid infection through transplantatation

Unrelated bone marrow transplantation in Thalassemia. The experience of the Italian Bone Marrow transplant Group (GITMO)

BACKGROUND AND OBJECTIVES: Allogeneic bone marrow transplantation (BMT) is a widely accepted therapeutic approach in homozygous beta-thalassemia. However, the majority of patients do not have a genotypically identical donor within the family. This prompted us to conduct a pilot study to investigate the feasibility of matched unrelated bone marrow transplantation in thalassemia. The major drawback was the high risk of immunologic and transplant-related complications, mainly graft-versus-host disease (GvHD) and graft failure. DESIGN AND METHODS: Our aim was to reduce this risk through careful selection of donor/recipient pairs. HLA haplotypes that show a high linkage disequilibrium among their class I, class II and class III alleles are considered extended or ancestral haplotypes. RESULTS: These haplotypes are conserved and can be shared by apparently unrelated individuals. Our study shows that matching for these haplotypes significantly improves the outcome of unrelated bone marrow transplantation in thalassemia. In fact, results were comparable to those obtained in transplants using HLA-identifical family donors. INTERPRETATION AND CONCLUSIONS: Better results were obtained in patients with lesser iron overload and when the donor shared an identity for the DPB1 alleles

HLA class II DNA typing in a large series of European patients with systemic lupus erythematosus: correlations with clinical and autoantibody subsets

We conducted this study to determine the HLA class II allele associations in a large cohort of patients of homogeneous ethnic derivation with systemic lupus erythematosus (SLE). The large sample size allowed us to stratify patients according to their clinical and serologic characteristics. We studied 577 European Caucasian patients with SLE. Antinuclear antibodies (Hep-2 cells), anti-dsDNA antibodies (Crithidia luciliae), and antibodies to extractable nuclear antigens Ro (SS-A), La (SS-B), U1-RNP, Sm, Jo1, SCL70, and PCNA, were detected in all patients. Molecular typing of HLA-DRB1, DRB3, DQA1, and DQB1 loci was performed by the polymerase chain reaction-sequence specific oligonucleotide probes (PCR-SSOP) method. We found a significantly increased frequency of DRB1*03, DRB1*15, DRB1*16, DQA1*0102, DQB1*0502, DQB1*0602, DQB1*0201, DQB1*0303, and DQB1*0304 in lupus patients as compared with healthy controls. In addition, DRB1*03 was associated with anti-Ro, anti-La, pleuritis, and involvement of lung, kidney, and central nervous system. DRB1*15 and DQB1*0602 were associated with anti-dsDNA antibodies; DQB1*0201 with anti-Ro and anti-La, leukopenia, digital skin vasculitis, and pleuritis; and DQB1*0502 was associated with anti-Ro, renal involvement, discoid lupus, and livedo reticularis. In conclusion, our study shows some new HLA clinical and serologic associations in SLE and further confirms that the role of MHC genes is mainly to predispose to particular serologic and clinical manifestations of this disease

Considerazioni tecniche in ausilio alla valutazione del rischio inerente la riduzione della distanza di sicurezza tra il dispenser di erogazione GNL ed il relativo serbatoio di stoccaggio

Il presente articolo riassume i risultati di uno studio eseguito allo scopo di fornire elementi utili per valutare il grado di sicurezza risultante da una riduzione delle distanze tra l’apparecchio di distribuzione ed il serbatoio GNL nel caso di rifornimento di metano in forma liquida agli autoarticolati. In particolare vengono analizzati gli effetti dell'incendio di un autoarticolato in rifornimento sul serbatoio GNL, la simulazione di detto scenario è stato effettuata utilizzando il codice FDS e le informazioni raccolte riguardano le temperature raggiunte dalla superficie esterna del serbatoio attraverso le quali sono stati stimati gli effetti sul prodotto all’interno dello stesso

Caratteristiche antropometriche e composizione corporea di atleti praticanti il bodybuilding e la kickboxing

In this paper athletes practising bodybuilding and kickboxing were compared, as in these two disciplines weight and body composition have a great importance. The research was carried out on a sample from Sardinia (Italy) constituted by 22 subjects practising bodybuilding and 24 practising kickboxing. The following anthropometric measurements were taken: height, weight, circumferences and skinfold thickness, from which different anthropometric indices were calculated: Body Mass Index (BMI), Waist Hip Ratio (WHR), Arm Muscle Area (AMA), Arm Fat Area (AFA). The percentage of fat mass (FAT%%) was evaluated through skinfold techniques, using Jackson and Pollock (3 skinfolds) and Durnin and Womersley (4 skinfolds) equations. The average value of total height of athletes under scrutiny (172.94±6.45 cm for kickboxers and 172.06±8.98 cm for bodybuilders) was slightly higher than the average value of Sardinians (173.3 cm). Mean BMI for both groups fell within the range of normal weighted, but with a value significantly higher for (24.14±1.71 vs 22.76±1.71, p < 0.05). The two groups of athletes showed a different distribution of muscle mass, located prevalently in the upper limbs for the bodybuilders, in fact they presented higher values of arm circumference and AMA and lower values of biceps and triceps skinfold thickness. Kickboxers presented a lower distribution of subcutaneous fat in the trunk. Moreover, low percentage of fat mass characterize both kickboxers (3 skinfolds=7.70±2.32%; 4 skinfolds=11.53±2.99%) and bodybuilders (3 skinfolds=8.35±3.35%; 4 skinfolds=13.14±3.15%), and it is probably due to the intensive physical activity practised: 7.80±0.79 hours a week for kickboxers and 6.50±1.77 hours a week for bodybuilders.In questo lavoro si sono voluti mettere a confronto sportivi praticanti bodybuilding e la kickboxing, attività per le quali il peso e la composizione corporea assumono una valenza significativa. La ricerca è stata condotta su un campione, di origine sarda, di 22 atleti di bodybuilding e 24 atleti di kickboxing. Sono state rilevate le seguenti misure antropometrici: peso, statura, circonferenze e pliche, con i quali sono stati calcolati: l¿indice di massa corporea (BMI, Body Mass Index), il rapporto vita/fianchi (WHR, Waist Hip Ratio), l¿area muscolare e adiposa del braccio (AMA, Arm Muscle Area e AFA, Arm Fat Area). Il grasso percentuale (FAT%) è stata stimato tramite plicometria, utilizzando le formule Jackson e Pollock (3 pliche) e Durnin e Womersley (4 pliche). Il valore medio della statura totale degli atleti esaminati (172,94±6,45 cm nei kickboxer e 172,06±8,98 cm nei bodybuilder) è di poco superiore alla media regionale sarda di 171,3 cm. Il BMI medio rientra, per entrambi i gruppi, nel range di normopeso, ma con un valore significativamente superiore nei bodybuilder (24,14±1,71 vs 22,76±1,71, p < 0,05). Tra i due gruppi di atleti si è evidenziata una differente distribuzione della muscolarità, localizzata per i praticanti bodybuilding maggiormente nell¿arto superiore, essi registrano infatti valori più alti della circonferenza del braccio e dell¿indice di muscolarità AMA e più bassi valori delle pliche bicipitale e tricipitale, i kickboxer presentano una minor distribuzione di grasso sottocutaneo a livello del tronco. Si è riscontrato inoltre una ridotta massa grassa sia nei kickboxer (3 pliche=7,70±2,32%; 4 pliche=11,53±2,99%) che nei bodybuilder (3 pliche=8,35±3,35%; 4 pliche= 13,14±3,15%), dovuta sicuramente alla rilevante attività fisica praticata: 7,80±0,79 ore a settimana per i kickboxer e 6,50±1,77 ore a settimana per i bodybuilder