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Journal articles on the topic 'Nina Siciliana'
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1
Berkovitz, B. K. B., C. Grigson, and M. C. Dean. "Caroline Crachami, the Sicilian dwarf (1815-1824): Was she really nine years old at death?" American Journal of Medical Genetics 76, no. 4 (April 1, 1998): 343–48. http://dx.doi.org/10.1002/(sici)1096-8628(19980401)76:4<343::aid-ajmg10>3.0.co;2-o.
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Fornari, Thais A., Carolina Lanaro, Dulcinéia M. Albuquerque, Regiane Ferreira, and Fernando F. Costa. "Featured Article: Modulation of fetal hemoglobin in hereditary persistence of fetal hemoglobin deletion type-2, compared to Sicilian δβ-thalassemia, by BCL11A and SOX6-targeting microRNAs." Experimental Biology and Medicine 242, no. 3 (October 4, 2016): 267–74. http://dx.doi.org/10.1177/1535370216668052.
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Hereditary persistence of fetal hemoglobin deletion type-2 (HPFH-2) and Sicilian-δβ-thalassemia are conditions described as large deletions of the human β-like globin cluster, with absent β-globin chains and a compensatory variable increase in γ-globin. HPFH, in general, may be distinguished from DB-Thalassemia by higher fetal hemoglobin (HbF) levels, absence of anemia and hypochromic and microcytic erythrocytes. MicroRNAs (miRNAs) regulate a range of cellular processes including erythropoiesis and regulation of transcription factors such as the BCL11A and SOX6 genes, which are related to the regulation of γ-globin expression. In this report, a possible association among the overexpression of miRNAs and the expression of the γ-globin gene was analyzed in these two conditions. Forty-nine differentially expressed miRNAs were identified by microarrays in CD34+-derived erythroid cells of two subjects heterozygous for Sicilian-δβ-thalassemia, 2 for HPFH-2 and 3 for controls after 13 days of culture. Some of these miRNAs may participate in γ-globin gene regulation and red blood cell function. The BCL11A gene was found to be potentially targeted by 12 miRNAs that were up-regulated in HPFH-2 or in DB-Thal. A down-regulation of BCL11A gene expression in HPFH-2 was verified by quantitative polymerase chain reaction. These data suggest an important action for miRNA that may partially explain the phenotypic differences between HPFH-2 and Sicilian δβ-thalassemia and the increased expression of γ-globin in these conditions.
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Maggio, Antonella, Monica Rosa Loizzo, Luana Riccobono, Maurizio Bruno, Maria Concetta Tenuta, Mariarosaria Leporini, Tiziana Falco, et al. "Comparative chemical composition and bioactivity of leaves essential oils from nine Sicilian accessions of Myrtus communis L." Journal of Essential Oil Research 31, no. 6 (May 3, 2019): 546–55. http://dx.doi.org/10.1080/10412905.2019.1610089.
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Malicka, Paulina. ""A ccanciu ri Maria" e "l’Antologia" di Nino De Vita. Il caso di una scrittura “latitante” allo scoperto." Annales Universitatis Paedagogicae Cracoviensis | Studia de Cultura 9, no. 3 (July 5, 2018): 256–65. http://dx.doi.org/10.24917/20837275.9.3.24.
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Lo scopo dell’articolo è di segnalare l’importanza dell’ultima produzione in versi del poeta siciliano Nino De Vita considerato una delle voci liriche e narrative più originali nell’ambito della poesia dialettale e della letteratura in lingua italiana. La riflessione verrà articolata attorno al poemetto devitiano A ccanciu ri Maria pubblicato nel 2015 che coincide con la pubblicazione dell’Antologia bilingue del poeta percepita nei termini di un vero e proprio evento editoriale. Un evento che copre oltre trent’anni dell’attività poetica e narrativa segnata da un continuo intrecciarsi dell’esperienza di vita e di scrittura “in latitanza”."A ccanciu ri Maria" i "Antologia" Nina De Vity. Przypadek ujawnionego pisania „w ukryciu”Celem artykułu jest zwrócenie uwagi na ostatnie dokonania sycylijskiego poety Nina De Vity, uważanego za jeden z najbardziej oryginalnych głosów poetyckich – zarówno w pisarstwie dialektalnym, jak i w literaturze włoskiej. Refleksja dotyczy jego ostatniego opowiadania, pisanego wierszem A ccanciu ri Maria (W zamian za Marię), które ukazało się w 2015 roku. Publikacja ta zbiega się w czasie z innym ważnym wydarzeniem edytorskim, a mianowicie z ukazaniem się dwujęzycznej antologii obejmującej 30 lat twórczości poetyckiej i narracyjnej De Vity, naznaczonej nieustannym przenikaniem się doświadczeń życia i pisania „w ukryciu”.
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Copani, V., G. Testa, A. Lombardo, and S. L. Cosentino. "Evaluation of populations of Dactylis glomerata L. native to Mediterranean environments." Crop and Pasture Science 63, no. 12 (2012): 1124. http://dx.doi.org/10.1071/cp12276.
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Several morphological and agronomic traits and the genetic diversity of nine Dactylis glomerata L. populations collected throughout Sicily (semi-arid Mediterranean environment) were evaluated for two successive years. Significant differences were recorded for morphological traits (plant height, leaf length, leaf width). In relation to the measurement of summer dormancy, the results suggest the expression of different levels of dormancy (completely dormant, semi-dormant, and non-dormant). For biomass yield, some Sicilian populations (SD63 and SD56) characterised by low levels of summer dormancy show production levels similar to the summer-active control varieties (Medly and Porto). However, SD46, with a much higher level of dormancy, gave biomass yield higher than the summer-dormant control variety (Kasbah). The genetic diversity evaluated by fAFLP analysis confirms the observed morphological and agronomic variability.
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Craig, JE, RA Barnetson, J. Prior, JL Raven, and SL Thein. "Rapid detection of deletions causing delta beta thalassemia and hereditary persistence of fetal hemoglobin by enzymatic amplification." Blood 83, no. 6 (March 15, 1994): 1673–82. http://dx.doi.org/10.1182/blood.v83.6.1673.1673.
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Abstract A considerable number of deletions of variable size and position that involve the beta-globin gene complex on chromosome 11 are associated with the clinical entities of hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia. Specific deletions appear to be associated with consistent phenotypes and some are known to be recurrent. To facilitate the molecular diagnosis of uncharacterized patients with HPFH and delta beta thalassemia, oligonucleotide primers have been designed to enzymatically amplify deletion-specific products for nine known deletions, which include those responsible for HPFH-1, HPFH-2, HPFH-3, Spanish (delta beta)zero thalassemia, hemoglobin (Hb) Lepore, Sicilian (delta beta)zero thalassemia, Chinese G gamma(A gamma delta beta)zero thalassemia, Asian-Indian inversion-deletion G gamma(A gamma delta beta)zero thalassemia, and Turkish inversion-deletion (delta beta)zero thalassemia. Using this approach, we have successfully characterized the molecular basis for delta beta thalassemia in 23 individuals from 16 families of diverse ethnic origins. Thirteen individuals from this group were shown to be heterozygous for the 13.4- kb Sicilian deletion, two were heterozygous for the 100-kb Chinese G gamma(A gamma delta beta)zero deletion, four were heterozygous for the Turkish form of inversion-deletion delta beta thalassemia, and three were heterozygous for the Asian-Indian form of inversion-deletion G gamma(A gamma delta beta)zero thalassemia. One Vietnamese subject was heterozygous for a 12.6-kb deletion, which we have fully characterized at the molecular level. Sequence analysis of the breakpoint regions of the Chinese deletion and the Turkish rearrangement indicates that, in each case, the mutation is likely to have arisen from a single origin. This hypothesis is supported by the evident geographical clustering of the various deletions described here.
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Craig, JE, RA Barnetson, J. Prior, JL Raven, and SL Thein. "Rapid detection of deletions causing delta beta thalassemia and hereditary persistence of fetal hemoglobin by enzymatic amplification." Blood 83, no. 6 (March 15, 1994): 1673–82. http://dx.doi.org/10.1182/blood.v83.6.1673.bloodjournal8361673.
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A considerable number of deletions of variable size and position that involve the beta-globin gene complex on chromosome 11 are associated with the clinical entities of hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia. Specific deletions appear to be associated with consistent phenotypes and some are known to be recurrent. To facilitate the molecular diagnosis of uncharacterized patients with HPFH and delta beta thalassemia, oligonucleotide primers have been designed to enzymatically amplify deletion-specific products for nine known deletions, which include those responsible for HPFH-1, HPFH-2, HPFH-3, Spanish (delta beta)zero thalassemia, hemoglobin (Hb) Lepore, Sicilian (delta beta)zero thalassemia, Chinese G gamma(A gamma delta beta)zero thalassemia, Asian-Indian inversion-deletion G gamma(A gamma delta beta)zero thalassemia, and Turkish inversion-deletion (delta beta)zero thalassemia. Using this approach, we have successfully characterized the molecular basis for delta beta thalassemia in 23 individuals from 16 families of diverse ethnic origins. Thirteen individuals from this group were shown to be heterozygous for the 13.4- kb Sicilian deletion, two were heterozygous for the 100-kb Chinese G gamma(A gamma delta beta)zero deletion, four were heterozygous for the Turkish form of inversion-deletion delta beta thalassemia, and three were heterozygous for the Asian-Indian form of inversion-deletion G gamma(A gamma delta beta)zero thalassemia. One Vietnamese subject was heterozygous for a 12.6-kb deletion, which we have fully characterized at the molecular level. Sequence analysis of the breakpoint regions of the Chinese deletion and the Turkish rearrangement indicates that, in each case, the mutation is likely to have arisen from a single origin. This hypothesis is supported by the evident geographical clustering of the various deletions described here.
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Salvinelli, Fabrizio, Manuele Casale, Luca D’Ascanio, Luca Firrisi, Fabio Greco, and Alfonso Baldi. "Hearing loss associated with 35delG mutation in Connexin-26 (GJB2) gene: audiogram analysis." Journal of Laryngology & Otology 118, no. 1 (January 2004): 8–11. http://dx.doi.org/10.1258/002221504322731547.
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35delG is the most common mutation in the Connexin-26 gene, representing a major cause of autosomal recessive hearing loss. The aim of this study was to evaluate the relationship between the audiological phenotype and the 35delG mutation in 64 Sicilians with non-syndromic deafness. Pure-tone audiometry and a screening for 35delG mutation were performed. Audiograms were evaluated according to the classification of Liu and Xu. Thirteen homozygotes and nine heterozygotes for the investigated mutation were found. Symmetrical hearing loss was significantly (p=0.008) more common in homozygous subjects than in those without the Connexin-26 mutation. Profound-severe hypoacusia was found in 92.3 per cent of 35delG homozygous, 22.3 per cent of heterozygous and 58.7 per cent of 35delG absent patients. Residual shape audiograms were more frequent in homozygotes. A molecular analysis for the 35delG mutation should be performed in cases of symmetric, severe-profound congenital hearing loss, as a genetic cause is probable in such cases.
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Cassar, Silvana, and Salvo Creaco. "Towards Tourism Sustainability: General Aspects and Empirical Evidence of the Italian Experience at Decentralized Level, with Specific Reference to Sicily." Baltic Journal of European Studies 6, no. 1 (February 1, 2016): 95–138. http://dx.doi.org/10.1515/bjes-2016-0005.
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AbstractEven though the concepts and observations underlying sustainability are not a novelty, sustainable development was given impetus and made popular in 1987 by the Brundtland Report. This report introduced development policies and strategies that acknowledged the importance of resource and environmental constraints and the limitations imposed on growth patterns. Although sustainable development proved difficult to define and make operative, the concept was progressively applied to those economic sectors which had major detrimental effects on income, employment and wealth. Sustainable tourism was effectively one part of the effort to take full account of the current and future economic, social and environmental impacts of the sector, addressing the needs of visitors, industry, environment and host communities. After reviewing the main questions referring to the environmental, economic and social aspects of tourism development, this paper tries to identify a balanced indicator showing the impact of tourist accommodation facilities and related infrastructure in Sicily in terms of the three fundamental pillars of sustainability. Based on this indicator, the ranking of the nine Sicilian provinces is provided. In a wider perspective, the proposed approach is applied to make a comparison of tourism sustainability in Sicily and in other Italian regions.
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Tommonaro, Giuseppina, Concetta Piscitelli, Margherita Lavorgna, Marina Isidori, Chiara Russo, Rocco De Prisco, Gennaro Roberto Abbamondi, and Carmine Iodice. "ANTIOXIDANT AND ANTIPROLIFERATIVE ACTIVITIES OF DIFFERENT CULTIVARS OF TOMATOES (LYCOPERSICON ESCULENTUM) ON TUMORAL CELL LINES." JOURNAL OF ADVANCES IN BIOLOGY 10, no. 2 (August 7, 2017): 2061–72. http://dx.doi.org/10.24297/jab.v10i2.6187.
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To increase the knowledge about the biological properties of tomatoes, the antioxidant and antiproliferative activities of extracts of nine cultivars of Lycopersicon esculentum, as well as their chemical composition, were studied. The highest antioxidant capacity has been revealed in San Marzano Cirio 3 and Pomodoro Giallo cultivars, both in hydrophilic and lipophilic fractions, while San Marzano, Corbarino di Corbara, and Pomodoro Giallo exhibited the best radical scavenger activity in methanolic fraction. As regards the antiproliferative activity, the median inhibition concentrations of the lipophilic extracts ranged from 272.3 (Pomodoro Giallo) to 364.4 (Corbarino di Corbara) mg dried extract L-1 on Hep-G2 and from 324.6 (San Marzano Cirio 3) to 455.4 (Nero di Sicilia) on Caco-2. The methanolic extracts were more active on Caco-2 than Hep-G2, while the hydrophilic extracts were not active. These biological properties could be ascribed to the identified carotenoids and phenolic acids as well as to a pull of minor compounds exerting their synergistic effect.
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De Cola, Maria C., Viviana Lo Buono, Agata Mento, Mariella Foti, Silvia Marino, Placido Bramanti, Alfredo Manuli, and Rocco S. Calabrò. "Unmet Needs for Family Caregivers of Elderly People With Dementia Living in Italy: What Do We Know So Far and What Should We Do Next?" INQUIRY: The Journal of Health Care Organization, Provision, and Financing 54 (January 1, 2017): 004695801771370. http://dx.doi.org/10.1177/0046958017713708.
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Care of the elderly with dementia represents one of the major challenges for the modern society worldwide. The burden of dementia care often falls on the family members, entailing heavy psychosocial and economic consequences. The aim of this study was to evaluate the caregiver’s perspective concerning the support for disease management on behalf of the physicians and the local Sicilian administrations (Italy), and the burden of care and effects on their lifestyle, to propose new prevention strategies and service for managing dementia and caregiver’s burden. Fifty-nine caregivers of Italian elderly people with dementia (mean age, 73; age range: 63-83) were interviewed, and 55 of them completed an ad hoc self-report questionnaire composed of 54 multiple-choice questions. Our findings suggest that caregivers need more information on the disease’s management, as well as on how to deal with the stress due to the disease burden. Moreover, a negative perception about the services offered from the local administration emerged. Assistive technology (AT) could be useful in promoting interaction between general practitioners and specialized centers for diagnosis, pharmacological and psychosocial treatments, and in saving costs. Moreover, case manager could follow patients and support family members within the care pathway, besides collecting and sharing information among the different health professionals involved. Further studies should be aimed at investigating whether AT and/or the use of specific educational strategies could be the right approach for meeting the needs of families living with dementia.
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Fasciana, Teresa, Chiara Mascarella, Salvatore Antonino Distefano, Cinzia Calà, Giuseppina Capra, Angela Rampulla, Paola Di Carlo, Mario Palermo, and Anna Giammanco. "Cluster of Legionnaires’ Disease in an Italian Prison." International Journal of Environmental Research and Public Health 16, no. 11 (June 11, 2019): 2062. http://dx.doi.org/10.3390/ijerph16112062.
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Background: Legionella pneumophila (Lp) is the most common etiologic agent causing Legionnaires’ Disease (LD). Water systems offer the best growth conditions for Lp and support its spread by producing aerosols. From 2015 to 2017, the Regional Reference Laboratory of Clinical and Environmental Surveillance of Legionellosis of Palermo monitored the presence of Lp in nine prisons in Western Sicily. During this investigation, we compared Lp isolates from environmental samples in a prison located in Palermo with isolates from two prisoners in the same prison. Methods: We collected 93 water samples from nine Sicilian prisons and the bronchoalveolar lavages (BALs) of two prisoners considered cases of LD. These samples were processed following the procedures described in the Italian Guidelines for the Prevention and Control of Legionellosis of 2015. Then, genotyping was performed on 19 Lp colonies (17 from water samples and 2 from clinical samples) using the Sequence-Based Typing (SBT) method, according to European Study Group for Legionella Infections (ESGLI) protocols. Results: Lp serogroup (sg) 6 was the most prevalent serogroup isolated from the prisons analyzed (40%), followed by Lp sg 1 (16%). Most of all, in four penitentiary institutions, we detected a high concentration of Lp >104 Colony Forming Unit/Liter (CFU/L). The environmental molecular investigation found the following Sequence Types (STs) in Lp sg 6: ST 93, ST 292, ST 461, ST 728, ST 1317 and ST 1362, while most of the isolates in sg 1 belonged to ST 1. We also found a new ST that has since been assigned the number 2451 in the ESGLI-SBT database. From the several Lp sg 1 colonies isolated from the two BALs, we identified ST 2451. Conclusions: In this article, we described the results obtained from environmental and epidemiological investigations of Lp isolated from prisons in Western Sicily. Furthermore, we reported the first cluster of Legionnaires’ in an Italian prison and the molecular typing of Lp sg 1 from one prison’s water system and two BALs, identified the source of the contamination, and discovered a new ST.
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Baiamonte, Giorgio, Pietro Di Dio, and Mustafa Elfahl. "Verification of IRRILAB Software Application for the Hydraulic Design of a Micro-Irrigation System by Using IRRIPRO for an Apple Farm in Sicily." Water 13, no. 5 (March 4, 2021): 694. http://dx.doi.org/10.3390/w13050694.
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In recent years, many studies have been performed to develop simple and accurate methods to design micro-irrigation systems. However, most of these studies are based on numerical solutions that require a high number of iterations and attempts, without ensuring to maximize water use efficiency and energy-saving. Recently, the IRRILAB software, which is based on an analytical approach to optimally design rectangular micro-irrigation units, has been developed, providing the solution corresponding to the maximum energy-saving condition, for any slope of the laterals and of the manifold. One IRRILAB limitation is that, according to its theoretical basis, the rectangular planform geometry and uniform slope of the laterals and of the manifold are required. On the contrary, IRRIPRO software, which is based on the traditional numerical solution, does not have the aforementioned limitations, but requires an important number of attempts, especially when common emitters are used. In this study, the results of a joint use of IRRILAB and IRRIPRO software applications are illustrated, towards the aim to verify the IRRILAB performance in a large number of micro-irrigation sectors belonging to a Sicilian apple farm, which is characterized by a high irregular topography, thus it is suitable for the purpose of this study. First, only five irrigation sectors, for the actual subdivisions of the farm, were considered, showing limited reasonable IRRILAB results. Dividing the farm into a higher number of sectors so as to provide a better uniformity in planform geometry and slope revealed that IRRILAB results improved in terms of emission uniformity and energy consumption, as verified by IRRIPRO applications. The energy-saving provided by IRRILAB (in one step) with respect to that by IRRIPRO (by attempts) resulted higher for common emitters (CEs) (−15% for five sectors and −9% for nine sectors) than for pressure compensating emitters (PCEs) (−7% for five sectors and −6% for nine sectors). In absolute terms, the energy is greater for five-sector subdivision than for nine-sector subdivision. For both software, the use of PCEs always required less energy than CEs, because of the higher range of pressure compensating of PCEs than CEs. However, PCEs are characterized by less durability and by a higher manufacturing variation coefficient, thus they should not be the first choice. In conclusion, IRRILAB software could be recommended because it is easy to use, making it possible to save energy, especially when sectors are almost rectangular and uniform in slopes.
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Rizza, S., F. Conti, G. Pasquini, and M. Tessitori. "First Report of Plum pox virus Strain M Isolates in Apricot in Sicily, Italy." Plant Disease 98, no. 11 (November 2014): 1591. http://dx.doi.org/10.1094/pdis-05-14-0458-pdn.
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Sharka or plum pox disease is one of the most economically important virus diseases of stone fruits. Plum pox virus (PPV), the causal agent, is a member of the genus Potyvirus of the family Potyviridae transmitted by aphids in a non-persistent manner and by grafting. To date, nine PPV strains have been described on the basis of their biological, serological, and molecular properties: M and D are the most widespread and economically important strains, PPV-Rec and PPV-C have been reported mainly in Europe, PPV-EA confined to Egypt, PPV-T to Turkey, PPV-W from Canada, Ukraine, Latvia, and Russia, PPV-CR detected in Russia, and finally a putative PPV strain infecting plum in Albania described as the ancestor of the M. PPV-M is responsible for major epidemics in many Italian regions and despite phytosanitary measures, the infection rate increases each year. The D and Rec isolates are sporadically reported while PPV-C, once signaled in Apulia, has been successfully eradicated. Except for a report from the 1980s, which is no longer traceable, Sicily was considered free from the virus (2). In 2012, two new foci of sharka in a coastal area of Catania in Sicily were first reported by the national plant protection service to the European Commission (DG-SANCO). In spring 2013, plants of different varieties of apricot (Prunus armeniaca) and peach (P. persica) showing typical symptoms of flower color break, yellowing and leaf deformation, chlorotic spots or rings, and malformation on fruits were tested positive to PPV by DAS-ELISA using polyclonal antibodies. In order to characterize two isolates from apricot varieties (Carmen Top and Ninfa), total RNAs, extracted using the RNeasy Plant Mini Kit (Qiagen) from ELISA-positive samples, were analyzed by RT-PCR with primers P1/P2, targeting the 3′-terminal region of the coat protein (CP) gene (5) followed by RFLP analysis after digestion with Rsa1. Subsequently total RNAs were analyzed with the type-specific primers P1/PM and P1/PD (3), P3M/P4b and P3D/P4b amplifying the N-terminal region of the CP gene (1) and, finally, with primers mD5/mD3, mM5/mM3, and mD5/mM3, amplifying the region 3′NIb-5′CP, including the recombination site of Rec isolates (4). Only primer pairs P1/P2, P1/PM, P3M/P4b, and mM5/mM3 produced amplicons of the expected size (243, 198, 466, and 459 bp, respectively). The RFLP assay confirmed both isolates belonging to the M strain. Moreover, no reaction was obtained with primer pair mD5/mM3, excluding isolates belonging to Rec-type. Isolate characterization was completed by direct sequencing in both directions of the of P1/P2 and P3M/P4b amplicons obtained from apricot samples L9-1 (Carmen Top isolate) and 9-335 (Ninfa isolate). The P1/P2 sequences (KJ994235, KJ994237) showed 98% similarity with PPV-M or PPV-Rec isolates. The P3M/P4b sequences (KJ994236, KJ994238) confirmed that Sicilian isolates belong to the PPV-M strain showing 99% similarity with those already present in GenBank, thus ruling out the possibility of an infection with a PPV-Rec isolate. This outbreak of the Marcus strain of PPV in Sicily represents a high risk for the expanding production of stone fruit in southern Italy. An eradication plan was quickly activated by the regional phytosanitary service. References: (1) T. Candresse et al. Phytopathology 101:611, 2011. (2) EPPO. PQR-EPPO database on quarantine pests (available online). http://www.eppo.int , 2014. (3) A. Olmos et al. J. Virol. Methods 68:127, 1997. (4) Z. Subr et al. Acta Virol. 48:173, 2004. (5) T. Wetzel et al. J. Virol. Methods 33:355, 1991.
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Maggio, Aurelio, Paolo Rigano, and Disma Renda. "Treatment with Hydroxyurea in Sickle Cell/ ß Thalassemia: A Long-Term Experience." Blood 104, no. 11 (November 16, 2004): 1678. http://dx.doi.org/10.1182/blood.v104.11.1678.1678.
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Abstract Sickle cell/ß-Thalassemia is a common disease in areas where ß-Thal and ßS genes are endemic, like in Sicily. In the current study we evaluated clinical and hematological data of Sicilian patients with Sickle cell/ß-Thalassemia treated with Hydroxyurea (HU). The endpoint of the study was to evaluate the efficacy of HU in terms of reduction of sickle cell crises after 2 years of treatment in comparison with the 2 years before. Moreover, we evaluated the outcome after long-term treatment. Fortytwo patients (18 males, mean age 36, range 18–53) were treated with HU (mean dosage 15 mg/kg, range 10–30) for an average 6.6 years follow-up (range 3–9 years). Twentytwo were ß0/ßS and 20 ß+/ßS genotype. All had 3 or more crises in the year before starting HU. We observed a significant reduction in sickle cell crises (7.8 ± 6.9 crises per year versus 0.9 ± 1.8 per year, P < 0.0001), hospitalizations (2.5 ± 2.9 per year versus 0.3 ± 1.5, P < 0.0001), and days in hospital (22.4 ± 21.9 per year versus 1.2 ± 2.3, P < 0.0001). Altogether, there was a 86% reduction in vasoocclusive events in comparison with the 2 years before (P < 0.001). Moreover, there was a significant increase of MCV (71.4 versus 97.5fl, P < 0.0001), HbF (7.5 versus 25.2 %, P < 0.0001), and decrease of WBC (11.4 versus 9.2 109/L P < 0.01) and reticulocytes (14.1 versus 10.2%, P< 0.01). Finally, redution of hyperdense cells and increase of erithropoyetin were seen. After a mean follow-up of 6.6 years, 39 patients are alive. Three died (2 end stage HCV related liver disesases, 1 bleeding after ERCP). Nine of the 40 alive patients developed complications: 1 acute chest syndrome, 2 strokes, 2 myocardial infarctions, 4 bone necrosis. Brain MRI of 15 patients after and during a mean of 6.9 years of HU treatment showed 2 new onset strokes, 1 of which in a patient with a previous stroke. Moreover, 4 patients developed new onset asymptomatic ischemic brain lesions. In every case there had been a significant reduction of sickle cell crises. There were two cases of cancer, occurring in two patients who were brother and sister: lung cancer in the former, a proeviously heavy smoker, breast cancer in the latter. No further serious adverse events were seen. Five patients with iron overload were treated with Deferiprone: no drug interaction with HU was noticed. Our study confirms that HU is effective in reducing clinical relevant crises of patients with Sickle cell/ß-Thalassemia. However, our preliminary data suggest that chronic organ damages are not prevented by HU. Safety has to be assessed by more prolonged studies.
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Fornari, Thais Arouca, Carolina Lanaro, Regiane Ferreira, Paula Barbim Donate, Geraldo Passos, Nicola Conran, Sara T. O. Saad, and Fernando Ferreira Costa. "HPFH and Delta-Beta Thalassemia Have Different Profiles Of Micrornas and Transcription Factors." Blood 122, no. 21 (November 15, 2013): 186. http://dx.doi.org/10.1182/blood.v122.21.186.186.
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Abstract Introduction Hereditary Persistence of Fetal Hemoglobin (HPFH) and δβ-thalassemia are genetic disorders characterized by elevated levels of fetal hemoglobin (HbF) in adulthood. Deletions of variable sizes, and at different positions, that involve the β-globin gene complex on chromosome 11 are associated with these disorders. The distinction between these conditions is made based on clinical and hematological data. Deletional HPFH includes a wide range of conditions, but typically it is characterized in heterozygotes by levels of HbF of 15% to 30% with normal red blood cell indices, while heterozygotes for δβ-thalassemia tend to have elevated levels of HbF that are lower (5% to 20%) and accompanied by mild anemia with hypochromic, microcytic red blood cell indices. MicroRNAs (miRNAs) have been described to have a possible role in globin switching and can modulate transcriptional erythroid-specific regulators. To the best of our knowledge miRNAs have not been analyzed in HPFH and δβ-thalassemia. The aim of this study was to investigate the miRNAs expression profile and possible post-transcriptional role of these molecules in relation to the lack of normal suppression of γ genes in these genetic disorders. Methods CD34(+)-derived erythroid cells from two HPFH-2 individuals and two δβ-thalassaemia Sicilian type patients (DB) and healthy controls (CTRL) were cultured for 13 days to examine the expression profile of miRNAs. The miRNAs were hybridized using an Agilent miRNA microarray platform and the profiles were obtained through bioinformatics data analysis using GeneSpring software. qPCR analysis was used to validate the miRNA expression (TaqMan® miRNAs assays) and to quantify gene expression of 19 transcription factors. Different databases, such as miRBase, TargetScan, microRNA.org and BioGPS were used to determine the predicted targets of miRNAs data found. Results Six miRNAs were up-regulated in HPFH and in DB compared to CTRL: miR-146b-5p, miR-181a, miR-342-3p, miR-362-3p, miR-362-5p and miR-365. Five miRNAs were up-regulated in HPFH, compared to DB: miR-223, miR-630, miR-638, miR-1246 and miR-1290. Nine miRNAs were up-regulated in DB compared to HPFH: miR-10a, miR-21*, miR-33b*, miR-96, miR-128, miR-194, miR-210, miR-424 and miR-1275. The ALK4 and the GATA2 mRNAs were significantly up-regulated in HPFH, compared to DB. The BCL11A and the SH3BGRL2 mRNAs were significantly down-regulated in HPFH compared to DB. In silico analysis and the literature show that several miRNAs have targets related to HbF production or erythropoiesis. These include miR-10a, miR-33b*, miR-96, miR-128, miR-210, miR-223, miR-342-3p, miR-362-3p, miR-424 and miR-630. Conclusion The comparison of miRNA and transcription factor profiles suggests differences in the expressions of several miRNAs that may influence γ-globin gene expression. These data may contribute to understanding the phenotypic differences found between deletional HPFH and δβ-thalassemia. Financial support by FAPESP and CNPq/INCTS. Disclosures: No relevant conflicts of interest to declare.
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Giammanco, G., S. Ciriminna, I. Barberi, L. Titone, M. Lo Giudice, and L. R. Biasio. "Universal varicella vaccination in the Sicilian paediatric population: rapid uptake of the vaccination programme and morbidity trends over five years." Eurosurveillance 14, no. 35 (September 3, 2009). http://dx.doi.org/10.2807/ese.14.35.19321-en.
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Following the licensure of the Oka/Merck varicella vaccine in Italy in January 2003, the Sicilian health authorities launched a universal vaccination programme in all nine Local Health Units. A two-cohort vaccination strategy was adopted to minimise the shift of the mean age of varicella occurrence to older age groups, with the goal of vaccinating with one dose at least 80% of children in their second year of life and 50% of susceptible adolescents in their 12th year of life. Two studies were implemented in parallel to closely monitor vaccination coverage as well as varicella incidence. Overall, the programme achieved its target, with 87.5% vaccine coverage for the birth cohort 2005 and 90.2% for adolescents born in 1995 and 1996. Varicella surveillance data obtained from a total of 28,188 children (0-14 years-old) monitored by family paediatricians showed a decline in incidence rates from 95.7 (95% confidence interval (CI): 72.2-126.8) for 1,000 person-years (PY) in 2004 to 9.0 (95% CI: 6.4-12.6) for 1,000 PY in 2007. In Europe, the only similar experience is the routine childhood varicella vaccination programme in Germany that started in 2004 with a single dose at the age of 11-14 months. The two-cohort universal vaccination programme implemented in Sicily, as well as the network for the surveillance study, can offer a model to other European countries that are considering introducing universal childhood varicella vaccination.
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"Oeuvrecatalogus." Oud Holland - Quarterly for Dutch Art History 109, no. 1-3 (1995): 52–62. http://dx.doi.org/10.1163/187501795x00340.
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AbstractThe seventeenth-century, probably Flemish, artist Abraham Casembroot (Bruges? before or in Ι593 - Messina Ι658) spent the latter half of his life in Sicily. His entire extant oeuvre was produced there, which is probably why he is so little known in the Netherlands. The painter lived in the east-coast port of Messina, where for the last nine years of his life he held the post of consul for the Republic of the United Netherlands. Some of the bulletins he sent to the States General in his consular capacity are kept in the Rijksarchief at The Hague. So are the documents which confirm his appointment in Ι649 and that of his successor and thus establish Ι658 as the year of Casembroot's death. Information about his life can be found in biographies of Messina artists. The most detailed account is by Francesco Susinno in his Vite de' pittori messinesi of Ι724, although later vite of Messina artists also devote a comparatively large amount of space to Casembroot. As a painter he was evidently held in considerable esteem in Messina, where he had five local pupils. Casembroot specialized in harbour and marine views and tempests; according to the vite his paintings were much in demand in both Sicily and the Netherlands. The well-known seventeenth-century collection amassed by Don Antonio Ruffo of Messina boasted no fewer than ten works by Casembroot, one of which was very likely the large canvas The Swordfish Catch, now in the Museo di San Martino in Naples. Incidentally, a hitherto unpublished document reveals that one of Casembroot's consular duties was to deal with the ship that in all probability had on board Rembrandt's Aristotle, commissioned by Ruffo. It is remarkable that currently only four authenticated paintings by Casembroot are known, plus a fifth which is attributed to him on convincing grounds. His imaginary harbours resemble those by less well-known Netherlandish painters in Italy such as Cornelis de Wael and notably Adriaen van der Cabel. For a long time the latter was thought to have been responsible for four large series of sketchbook drawings with Sicilian studies which were in the Kupferstichkabinett in Berlin until the I9305 and subsequently scattered. In I973 Hans Mielke observed numerous stylistic correspondences between sheets from this former Berlin series and a preliminary drawing by Casembroot for an etching. There is also a stylistic resemblance to other traceable sheets from the former Berlin series (a considerable number of which are now in the Fitzwilliam Museum in Cambridge), some of which have been identified as preliminary studies for etchings by Casembroot. Consequently, three of the four Berlin series of sketchbooks previously attributed to Van der Cabel must now be established as Casembroot's work. Compared with Casembroot's paintings, his etchings and drawings exhibit more obvious personal stylistic characteristics, which Mielke had already observed in the nervous lines and the distinctive manner in which the small human figures are depicted; this enables reliable attributions to be made now. Mielke also demonstrated that Van der Cabel made use of drawings by Casembroot for his own etchings, and he is quite likely to have owned drawings by Casembroot. Casembroot's best-known work is a series of etchings of views of Messina and the surrounding countryside which he dedicated to the collector Lucas van Uffelen. Preliminary drawings for this series and for an authentic painting have survived. Casembroot utilized the drawings he did from nature in his sketchbooks as preliminary studies for etchings and paintings. Surviving sheets show small figures engaged in various activities, topographical sketches and accurate studies of local ships. The artistic quality of Casembroot's oeuvre is not particularly high. Its current significance is largely due to the topographical depictions of Messina and its environs, which look completely different today as the result of natural disasters. Despite his lengthy sojourn in Sicily, Casembroot remained a recognizably northern painter. His subject-matter displayed typically Dutch features which were uncommon in Messina, where local painters generally confined themselves to history pieces. With his harbour and sea views Casembroot seems to have discovered a gap in the market, which may account for his success in Messina.
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Raimondo, Maria Luisa, Francesco Lops, and Antonia Carlucci. "First report of Phaeoacremonium oleae and P. viticola associated with olive trunk diseases in Italy." Plant Disease, July 8, 2021. http://dx.doi.org/10.1094/pdis-06-21-1198-pdn.
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Over 300 trunk, branch and stem samples with vascular discolouration, necrotic wood and shoot death were collected from olive (Olea europaea) orchards in Lecce, Brindisi, Bari and Foggia provinces (Apulia region, Italy) from October to May from 2013 to 2019. Small chips of symptomatic wood samples were surface sterilised (5% NaOCl, 3 min; 70% ethanol, 30 s), rinsed (sterile distilled water, ×3), and placed onto potato dextrose agar (PDA) plates amended with 500 ppm streptomycin sulphate. After 14 days at 25 °C in the dark, hyphal tips of growing fungi, including different taxa, for instance Phaeoacremonium and Botryosphaeriaceae spp., were transferred to new PDA plates and incubated until sporulation. Monoclonal colonies resembling Phaeoacremonium-like genus (Mostert et al. 2006) were selected for further study, and genomic DNA of 59 representative isolates was extracted (Carlucci et al. 2013). Partial actin and β-tubulin genes were amplified with primers ACT-513F/ACT-783R (Carbone & Kohn 1999), and T1(O’Donnell & Cigelnik 1997) and Bt2b (Glass & Donaldson 1995), respectively. The sequenced amplicons were compared by BLAST algorithms with reference strains of Phaeoacremonium spp. retrieved from GenBank. Forty-four isolates showed 99% to 100% similarity with reference strains P. italicum, P. minimum, P. parasiticum, P. scolyti and P. sicilianum (Carlucci et al. 2015), nine with P. oleae, and six with P. viticola. Actin and β-tubulin sequences of P. oleae (Pm14) and P. viticola (Pm34) were submitted to GenBank (MW714561, MW714563; MZ318697, MZ318696). Microscopy of P. oleae isolates showed: conidiophores branched and unbranched, (18.7–)21.9–57.1(–67.8) × (2.9–)3.3–4.7(–5.2) (mean, 38.9×4.1) μm (n=30); conidia oblong-ellipsoidal to obovoid or subcylindrical 3.4 to 5.5 μm long, and 1.5 to 2.4 (mean, 4.6 × 2.2) μm wide (n=30). Microscopy of P. viticola isolates showed: conidiophores subcylindrical, branched at base (6.7–)8.9–27.2(–29.3) × (2.0–)2.6–3.3(–3.7) (mean, 21.4 × 3.2) μm (n=30); conidia oblong-ellipsoidal to obovoid or subcylindrical 3.3 to 6.8 μm long, and 1.1 to 2.2 (mean, 4.2 × 1.6) μm wide (n=30). In spring 2020, artificial inoculations were carried out with P. oleae (Pm14, Pm46) and P. viticola (Pm34, Pm43) strains on 10 healthy, 2-year-old olive seedlings cultivar ‘Coratina’. Agar plugs (diameter, 0.3–0.5 cm) from 10-day-old cultures grown on water agar at 23 (±2) °C were inserted under the bark of small wounds in the stems (length, 0.4–1.0 cm) made with a sterile scalpel. After inoculation, the wounds were wrapped with wet sterile cotton wool and sealed with Parafilm. Ten control olive seedlings were inoculated with sterile agar plugs. The experiment was replicated three times. All inoculated young olive plants were grown in pots in a greenhouse without temperature control. After 120 days, inoculated plants showed decline symptoms, and when cut longitudinally, brown streaks were observed in the wood. For P. oleae these streaks measured 3.0-5.5 cm long (standard deviation [SD], 0.9 cm, and for P. viticola they were 1.8-3.5 cm (SD, 0.62). Both fungal species were re-isolated from the symptomatic wood from 85% and 80%, respectively, of these inoculated olive seedlings, fulfilling Koch’s postulates. No symptoms were observed from olive seedlings used as control. P. oleae was first described as a fungal pathogen of wild olive (Olea europaea subsp. cuspidate) in South Africa by Spies et al. (2018), and P. viticola as a fungal pathogen of grapevine in France by Dupont et al. (2000). To the best of our knowledge, this is the first report of P. oleae associated with olive trunk disease in Italy, and the first report of P. viticola associated with olive trunk disease worldwide. References: Carbone I. & Kohn L.M. 1999. Mycologia 91:553. Carlucci A. et al. 2015. Eur. J. Plant Pathol. 141:717. Carlucci A. et al. 2013. Phytopathol. Mediterr. 52:517. Dupont et al. 2000. Mycologia 92:499-504. Glass N. L. & Donaldson G. C. 1995. J. Cl. Microbiol. 41: 1332. Mostert L. et al. 2006. Stud. Mycol. 54:1. O’Donnell K. & Cigelnik E. 1997. Mol. Phylogenetics Evol 7:103. Spies et al. 2018. Persoonia 40:26.