34 research outputs found
Sviluppo e validazione di nuove misure di valutazione dello stato di malattia nella dermatomiosite giovanile
No full text[da inserire
Giulia Cabra, Il valore dell’altro. Intersoggettività, amore ed etica in Edmund Husserl, Mimesis, Sesto San Giovanni 2023
No full textAdvances in biomarkers for paediatric rheumatic diseases
No full textThe search for biomarkers in paediatric rheumatic diseases, particularly juvenile idiopathic arthritis (JIA), childhood lupus nephritis (LN), and juvenile idiopathic inflammatory myopathies (JIIMs) is attracting increased interest. In JIA, a number of biomarkers have shown potential for predicting clinical phenotype, disease activity and severity, clinical remission and relapse, response to treatment, and disease course over time. In systemic JIA, measurement of biomarkers that reflect the degree of activation and expansion of T cells and macrophages might be helpful for detecting subclinical macrophage activation syndrome. Urine biomarkers for childhood LN hold promise for facilitating early diagnosis and improving disease monitoring and assessment of response to therapy. Myositis-specific autoantibodies define distinct serological subgroups of JIIMs, albeit with similar clinical features, responses to therapy, and prognoses. Use of biomarkers may potentially help to avoid invasive procedures, such as renal biopsy in systemic lupus erythematosus and muscle biopsy in juvenile dermatomyositis. Incorporation of effective and reliable biomarkers into routine practice might facilitate adoption of a stratified approach to investigation and management, foster the implementation of research into the design of personalized and targeted therapies, and ultimately lead to more rational and effective clinical care
Il mondo ellenistico-romano e la dialettica in Hegel: dal linguaggio condiviso al metodo speculativo della soggettività
No full textPartendo dalle ricche e complesse discussioni hegeliane delle antiche civiltà
mediterranee, specialmente il Griechentum classico e la Romanitas, l’autore analizza
qui – specie in riferimento alle Lezioni sulla Storia della Filosofia, le Lezioni sulla Filo-
sofia della Storia, e la Fenomenologia dello Spirito – il modo in cui Hegel ricostruisce
la progressiva interiorizzazione (Innerlichmachung) del dialogo e della dialettica in
Grecia nella forma logica definitiva dello Spirito (Geist), sia categoria sia forza
storica. L’autore cerca così di gettare nuova luce sul significato epistemologico
dei principali episodi in questo processo: le morti di Socrate e di Cristo, l’Età
Alessandrina, lo Stoicismo e l’Epicureismo imperiali, e il dominio di Roma come
“scetticismo reale (real)” (e secoli più tardi, le Crociate, e poi la totale interioriz-
zazione nel Mondo Germanico).
Argomento che questo processo di interiorizzazione, così visto, contribuisce in
modo decisivo alla invenzione da parte di Hegel del metodo fenomenologico
del progresso da una Gestalt (figura) all’altra, poiché la struttura della soggettivi-
tà speculativa finale corrisponde a questa trasformazione dalla semplice comu-
nicazione intersoggettiva ad una soggettiva ed autocosciente ricomprensione,
quale è tipica delle forme autoriferite del Concetto (Begriff) e dello Spirito (Geist).
Su queste basi, l’autore avanza alla fine la tesi che l’ordinamento delle Gestalten,
come pure l’intera ricostruzione della storia della filosofia e del mondo, non
sono assolutamente un tentativo di deduzione a priori di eventi storici, ma si
configurano piuttosto come una razionalizzazione post festum sia di eventi sia di
concetti ermeneutici collegati.Taking his departure from Hegel’s rich and complex discussions of ancient Mediterranean civilizations, especially classical Griechentum and Romanitas, the author analyzes – mainly in reference to the Lessons on the History of Philosophy, the Lessons on the Philosophy of History, and the Phenomenology of Spirit – the way in which Hegel reconstructs the progressive interiorization (Innerlichmachung) of
dialogue and dialectic in Greece into the definitive logical form of Spirit (Geist), both category and historical force. The author tries to shed new light on the epistemological significance of the main episodes in this process: the deaths of Socrates and of Jesus Christ, Alexandria, Stoicism and Epicureanism in the Imperial Age, and the dominion of Rome as “real (real) skepticism” (and centuries later, the Crusades and afterwards total interiorization in the Germanic World).
The author argues that this process of interiorization, so seen, decisively contributes to Hegel’s invention of the phenomenological method of the progress from one Gestalt to another, since the structure of final speculative subjectivity matches this transformation from intersubjective communication simple to subjective and self-conscious re-comprehension, as is typical of the self-referring forms
of Concept (Begriff) and Spirit (Geist). On this basis, the author finally advances the thesis that the ordering of Gestalten, as well as the whole reconstruction of the history of philosophy and of the world, are in no way an attempt at a priori deduction of historical events but are rather a post festum rationalization of real events and hermeneutical concepts
Innovative Research Design to Meet the Challenges of Clinical Trials for Juvenile Dermatomyositis
No full textPurpose of Review: This paper aims to provide a summary of the recent therapeutic advances and the latest research on outcome measures for clinical trials in juvenile dermatomyositis (JDM). Recent Findings: Recent randomized controlled trials (RCTs) have demonstrated the superiority of the combination of prednisone with methotrexate over other conventional therapies and the potential effectiveness of rituximab in refractory cases. A multinational project has led to develop new criteria for the definition of minimal, moderate, and major improvement in future JDM clinical trials. This effort has been paralleled by the establishment of criteria for clinically inactive disease. The validation of the first composite disease activity score for JDM is in progress. Summary: The new outcome measures will increase the reliability of assessment of clinical response in JDM clinical trials and foster future multinational RCTs aimed to investigate novel treatment strategies for refractory forms of JDM
Experience with the use of mycophenolate mofetil in juvenile idiopathic inflammatory myopathies
No full textAbstract
Objective
The objective of this study was to evaluate the efficacy and safety of MMF in juvenile idiopathic inflammatory myopathies (JIIMs).
Methods
Patients diagnosed with JIIM and treated with MMF enrolled in the Juvenile Dermatomyositis Research Group (JDRG) in the UK or followed at the Giannina Gaslini Institute in Genoa, Italy, were included. The following information was collected retrospectively at MMF initiation, at 3, 6 and 12 months after treatment start, and at last follow-up visit: clinical manifestations, laboratory data, physicians’ subjective assessment of disease activity, standardized outcome measures of muscle strength/endurance, cutaneous disease activity, physical function, global disease activity, cumulative damage, and ongoing treatment.
Results
Of the 29 patients included, 23 had juvenile DM and 6 had overlap myositis. During administration of MMF, improvement in measures of muscle strength, skin disease activity, and overall disease activity was seen, with an increase in the frequency of normal scores for Manual Muscle Test-8 from 50.0% to 83.3%, Childhood Myositis Activity Score from 53.5% to 88.9%, muscle component of DAS from 55.2% to 84.2%, skin component of DAS from 31.0% to 42.1%, visual analogue scale for skin disease activity from 25.0% to 47.4%, and visual analogue scale for overall disease activity from 7.1% to 42.1%. The number of patients with inactive disease increased from 10.3% at baseline to 68.5% at last follow-up. CS dose was significantly reduced, from 0.3 to 0.1 mg/kg/day. No relevant side effects were reported.
Conclusion
Our experience suggests that MMF is a valuable therapeutic option for the management of JIIM.
</jats:sec
Exercise Capacity and Cardiorespiratory Fitness in Children with Congenital Heart Diseases: A Proposal for an Adapted NYHA Classification
No full textObjective: To propose and evaluate an adapted NYHA classification for children with congenital heart disease (CHD) as a feasible clinical tool for classifying patients’ fitness, cardiorespiratory efficiency and functional limitations during their ordinary daily activities, which are also characterized by vigorous and competitive physical exercise among peers. Methods: This cross-sectional investigation analyzed 332 patients (13.1 ± 3.01 y/o) who underwent surgical repair of CHD and performed Cardiopulmonary Exercise Testing (CPET). Patients were divided into NYHA class I, IIA and IIB by specific questioning regarding functional limitation and performance compared to peers and at strenuous intensity. Class IIA was characterized by slight exercise limitation only for strenuous/competitive activities, whereas IIB for already ordinary physical activities. These NYHA classes were compared with maximal CPET on treadmill. Results: Patients’ exercise capacity (exercise time, METs), aerobic capacity (VO(2)peak) and chronotropic response were found progressively impaired when NYHA class I was compared with IIA and IIB. Indeed, ventilatory-perfusion mismatch (PETCO(2), VE/VCO(2)) significantly worsened from NYHA class I to IIA, while no difference was found between IIA and IIB. Conclusion: This adapted NYHA-CHD classification could allow regular functional evaluations and accurate assessments by clinicians, leading to facilitated clinical management and timely medical interventions
Evaluation of 21-Numbered Circle and 10-Centimeter Horizontal Line Visual Analog Scales for Physician and Parent Subjective Ratings in Juvenile Idiopathic Arthritis
No full textBaricitinib treatment in children with COPA syndrome
No full textClinical Implications box: Baricitinib seems safe and effective in reducing the articular flares, controlling the systemic inflammation and lung disease progression of COPA patients. The drug dosage should be adjusted according to patient’s age and weight in order to prevent disease flares
Serial cardiopulmonary exercise testing in young patients after one-and-half ventricle repair and Fontan procedure: a comparative study
No full textAim The Fontan procedure is a palliative surgical treatment for different congenital heart diseases with a univentricular heart, but it has been associated with decreased exercise capacity, cardiovascular morbidity, and premature mortality. The one-and-half ventricle repair (1.5VR) was introduced as an alternative to the Fontan procedure, specifically for selected patients with borderline hypoplastic right ventricle (HRV), aiming for a more physiological circulation. Despite these efforts, the benefit of 1.5VR over Fontan circulation comparison on clinical and functional outcomes remains unclear. The aim of this study was to investigate and compare young patients with HRV after 1.5VR with those with functional single right or left ventricles (FSRV or FSLV) after Fontan palliation over a 10-year follow-up period.Methods and results In this retrospective observational study, serial cardiopulmonary exercise tests (CPETs) performed in patients with 1.5VR and Fontan circulation between September 2002 and March 2024 have been analysed. Only patients with at least 10 years of follow-up were considered. A total of 43 patients were included (age at baseline 8.6 +/- 2.6 years): 21 with FSLV, 12 with FSRV, and 10 with 1.5VR. No differences in cardiorespiratory fitness and efficiency were shown at the first CPET assessment among the three groups. At 10-year follow-up, 1.5VR had higher cardiorespiratory fitness and efficiency compared to FSLV and FSRV patients.Conclusion These findings suggest that the 1.5VR may provide superior long-term functional outcomes than the Fontan procedure in patients with borderline HRV. Further studies are needed to evaluate the impact on hard clinical endpoints.Graphical Abstrac
