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L’EMBOLIZZAZIONE BRONCHIALE NEI PAZIENTI CON EMOTTISI IN FIBROSI CISTICA HA UN IMPATTO NEGATIVO SUL DECORSO CLINICO? ANALISI COMPARATIVA IN DUE POPOLAZIONI.
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Analisi retrospettiva delle embolizzazioni bronchiali in Fibrosi cistica e follow up a lungo termine
No full textNEW GENETIC AND PHARMACOLOGICAL TREATMENTS FOR CYSTIC FIBROSIS
No full textCystic Fibrosis (CF) is a still life-treathening disease, although therapies have augmented the life span of CF individuals. Isolation of the CF gene, named CFTR (CF Transmembrane Conductance Regulator), led to the discovery that it encodes for a protein kinase A-regulated chloride channel, expressed by epithelial cells mainly in mucosal tissues.
Mutated or absent CFTR brings about altered muco-ciliary clearance, by a yet disputed mechanism (likely involving overfunction of the epithelial sodium channel [ENaC]), and ultimately to colonization and infection of the airways by a few opportunistic bacteria species, including Pseudomonas aeruginosa (P.a.). An exuberant and persistent acute inflammatory reaction is commonly observed in the lung from CF patients, representing a key pathogenetic event of lung damage and
respiratory insufficiency. Lung disease is the chief cause of morbidity and mortality in CF patients and current therapies are aimed at controlling the respiratory symptoms by antibiotic and anti-inflammatory treatments. Major improvements in the strategy to fight pulmonary P.a. infection are based on treatment of first colonization with P.a., of chronic infection and of multidrug resistant bacteria. Controlling inflammation is a challenging, however crucial, task in CF therapy. Since classical steroidal and non-steroidal anti-inflammatory drugs are endowed with serious side-effects, alternative antiinflammatory strategies are being developed, including drugs which modulate cytokine expression, nitric oxide production and the oxidation unbalance in the CF airways. Soon after gene identification, gene transfer vectors to replace the defective gene were developed and underwent a number of human trials which have not yet produced a viable clinical gene therapy strategy. Novel gene therapy vectors and strategies for delivering them more efficiently and safely to the
lung are being studied. In the last few years various in vitro studies have demonstrated the possibility of pharmacological intervention to correct the primary defect in CF. This may be obtained by directly addressing the CFTR protein (by means of so-called correctors and potentiators) or by modulating the activity of other types of ion transport in epithelial cells (such as with Moli1901 or denufosol). Novel interventions aimed to improve the mucociliary clearance, including
hypertonic saline, mannitol and ENaC inhibitors, will be finally discussed
[Anti-Pseudomonas aeruginosa antibodies and lung disease in cystic fibrosis]
Get PDFThe aim of our study was to diagnose and to control three aspects of the evolution of lung disease in CF: the absence of infection, the intermittent colonization and chronic infection by Pseudomonas aeruginosa. Therefore a study of anti-pseudomonas antibodies (Ab) (anti-protease, anti-elastin and antihexo-toxin A) for diagnosis and follow-up of CF patients was considered. Moreover, we related the presence of Ab to the sputum culture, to FEV1, to patient age and to genotype. Tbe Ab were dosed in 121 patients by quantitative ELISA method. Values < 1: 500 were considered negative, values> 1: 500 and < 1:1250 borderline, and > 1:1250 positive. 16.5% of patients did not have Ab, 17% had borderline values and 69.5% had positive values. All the patients with negative Ab had negative sputum culture; 47% of patients with borderline values had at least one positive culture while 53% were negative. 87% of patients with positive values had chronic colonization, 13% intermittent colonization. The increase in the Ab rate is statistically related to a more severe lung disease (p < 0.013). The presence of a severe mutation (?F 508) is related to positive values of Ab. Evaluation of anti-Pseudomonas aeruginosa is an important tool for diagnosis and follow-up of CF lung diseas
Qualità della vita, regolazione affettiva e capacità resilienza in pazienti adulti affetti da fibrosi cistica
No full textObiettivo. Noi riteniamo che in pazienti affetti da Fibrosi Cistica (FC) lo studio della qualità della vita (QdV), costrutto strettamente associato al recente miglioramento dei protocolli di cura e all’aumento dell’età media di sopravvivenza, dovrebbe prendere in considerazione anche il ruolo della regolazione affettiva. Con il presente lavoro si intende studiare 1) la relazione tra la qualità della vita, la regolazione affettiva e la capacità di resilienza, ed esplorare 2) la relazione tra i suddetti psicologici e le condizioni mediche dei pazienti affetti da FC.
Metodi. Quaranta pazienti maggiorenni afferenti al Centro Regionale FC di Palermo hanno compilato 3 questionari: Cystic Fibrosis Quality Of Life (CFQoL), Toronto Alexithymia Scale (TAS-20) e Resilience Scale (RS). Per ciascun paziente sono stati raccolti anche i dati medici e le informazioni socio-demografiche.
Risultati. È emersa una correlazione significativa tra la capacità di resilienza e la QdV, in particolare con alcune sottoscale del CFQoL riguardanti il funzionamento sociale ed emotivo, le relazioni interpersonali e le preoccupazioni per la carriera. Altre correlazioni significative sono emerse tra la capacità di resilienza e la regolazione affettiva, e tra la percezione della QdV e le condizioni mediche dei pazienti.
Conclusioni. La valutazione dell’assetto psicologico permette l’identificazione di funzionamenti psicologici a rischio per la salute psico-fisica dei pazienti affetti da FC, ciò potrebbe contribuire all’implementazione di modelli di cura olistici per i pazienti con FC
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Get PDF“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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