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Long-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor on Nocturnal Cardiorespiratory Polygraphy Parameters in Patients with Cystic Fibrosis: A Prospective Study
No full textCystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene, leading to multi-system impairment. Sleep respiratory disorders (SRDs) are frequent in individuals with CF—even in those with normal or mildly impaired lung function—and may adversely affect overall health. The triple combination of elexacaftor, tezacaftor, and ivacaftor (ETI) has markedly improved clinical outcomes in CF; however, its long-term impact on SRDs remains unclear. This study aimed to assess the effects of ETI on nocturnal cardiorespiratory parameters in individuals with CF over a two-year period. Thirty-five clinically stable patients aged ≥13 years, eligible for ETI therapy, were enrolled. Nocturnal cardiorespiratory polygraphy and spirometry were performed at baseline (T0), one year (T1), and two years (T2) after ETI initiation. After one year, significant improvements were observed in mean oxygen saturation (mSpO2), time with SpO2 ≤ 90% (t ≤ 90%), and respiratory rate. Spirometric indices (FEV1, FVC, FEF) also significantly increased (p < 0.05). Correlation analysis revealed positive associations between mSpO2 and FEV1 (ρ = 0.515, p = 0.002) and between FEV1 and FVC (ρ = 0.894, p < 0.001), while t ≤ 90% negatively correlated with FEV1 (ρ = −0.404, p = 0.016). No additional significant changes were found at T2. ETI therapy resulted in sustained improvements in nocturnal oxygenation and lung function, supporting the importance of nocturnal respiratory monitoring during follow-up
Morphological and immunohistochemical study of ventral photophores of ichthyococcus ovatus (Cocco, 1838) (Fam: Stomiidae)
No full textPhotophores are light-producing organs found in many fish species living in the mesopelagic, bathypelagic, and abyssal layers of the ocean. They function to attract prey, confuse predators, and communicate with other individuals of the same species. Understanding the structure and function of photophores is crucial to exploring bioluminescence and the ecological adaptations of marine life in deep-sea environments. The present study is the first to investigate the photophore anatomy of the mesopelagic fish Ichthyococcus ovatus (Cocco, 1838), using specimens naturally stranded along the coast of the Strait of Messina. The morphology of the ventral photophores of I. ovatus includes four functional parts: a tank containing photogenic cells, a lens filter, a reflector surrounding the entire organ, and a pigmented layer. An immunohistochemical assay was conducted using anti-nNOS and anti-S100p antibodies. The presence of nNOS/NOS type I immunolabeling the pigmented layer surrounding the photophores and the nerve fibers reaching the lens suggests a potential role of neuronal nitric oxide signaling in modulating light shielding by the pigment sheath, controlling light exposure, and adjusting light focusing though the lens-associated nerves. S100p immunostaining was observed in the nerve fibers reaching the photophores, highlighting its potential involvement in regulating neuronal calcium levels and, consequently, influencing signal transmission to control bioluminescence output. A sensory feedback pathway from the photophore to the CNS is suggested. Within the lens and in the irregularly shaped cells located in the photophore’s lens, S100p immunolabeling could indicate active signaling and differentiation processes. These findings expand our understanding of light-emitting systems in mesopelagic fishes and offer a valuable foundation for future studies on the functional and evolutionary significance of photophores
Mechanisms and Efficacy of Contrast Therapy for Musculoskeletal Painful Disease: A Scoping Review
No full textOptical properties of carbon dots generated in liquid by pulsed IR laser at 1064 nm
No full textBiocompatible carbon dots (CDs) have been synthesized by the laser ablation of graphite and vegetable carbon (charcoal) placed in a phosphate-buffered saline (PBS) solution. The carbon nanoparticles are produced by a pulsed IR laser beam, operating at 1064 nm, 100 mJ energy, and 3 ns pulse duration, interacting with the carbon matrix placed in the liquid at a 0.2 Hz repetition rate. The ablation rate, in terms of removed carbon mass per laser shot, was evaluated to be about 75.8 ng/pulse and 758 ng/pulse for graphite and charcoal, respectively. Optical and electronic microscopy, UV-Vis spectroscopy, and luminescence measurements were employed to evince the CDs dispersion through its photoluminescence emission. The UV excitation at a 365 nm wavelength induces CDs luminescence in the visible region, as a result of electron transition, at a wavelength band depending on the laser spot size, i.e., of the laser fluence. CDs luminescence emission obtained from charcoal is independent of the laser fluence, showing a major emission peak around 475 nm and a secondary emission peak at 515 nm. CDs luminescence emission obtained from graphite depends on the laser fluence, showing a minor wavelength band, around 474 nm, at high laser fluences and a major wavelength band, around 670 nm, at low laser fluence. The CDs luminescence of the produced biocompatible solution finds many applications in the bio-medical field, as will be presented and discussed
Natural history of Becker muscular dystrophy: DMD gene mutations predict clinical severity
No full textBecker muscular dystrophy (BMD) is an X-linked neuromuscular disease attributable to mutations in DMD, leading to a deficient and less functional dystrophin, mainly in skeletal and cardiac muscle. Understanding the natural history of BMD is crucial for optimizing patient care and developing targeted treatments. Retrospective data were collected from 943 patients diagnosed with BMD based on a combination of clinical, biochemical and genetic criteria followed by 17 Italian neuromuscular centres. Patients’ demographics, main signs and symptoms at BMD onset, neuropsychiatric comorbidities, age at loss of ambulation, cardiac left ventricular ejection fraction, pulmonary forced vital capacity and DMD mutations were collected. Disease milestones were analysed in specific DMD mutational groups. The median age at the last assessment was 26.0 (16.6–41.9) years, with a median age at diagnosis of 7.5 (4.0–14.0) years. In 55% of patients, the diagnosis was prompted by the incidental finding of hyperCKaemia. At the last assessment, 13.5% of patients had lost the ability to walk at a median age estimated by Kaplan–Meier analysis of 69 years. Thirty per cent of patients exhibited left ventricular impairment and 2.7% respiratory involvement. Ten per cent of patients carried out-of-frame mutations, 4% nonsense mutations and 86% in-frame deletions/duplications. The subset of in-frame deletions was classified further based on the specific mutations. Patients carrying del45–49 compared with del45–47 were associated with an earlier loss of ambulation (P = 1 × 10−4), whereas patients with del45–55 (P = 0.005), del48 (P = 0.02) and del48–49 (P = 0.02) were correlated with a later loss of ambulation compared with del45–47. Both del45–55 (P = 0.002) and del48 (P = 0.003) were significantly associated with decreased odds of developing a pathological left ventricular ejection fraction compared with del45–47. Our results contribute to a better understanding of the natural history of BMD and capture precious data in the era of emerging therapies. The knowledge of the specific DMD mutation might help to define a prognosis in a subset of BMD patients and will serve as a model for the design of future therapies
LA GENITORIALITÀ SOCIALE NEL PRISMA DEL DIRITTO COMPARATO
No full textL'articolo esamina gli sviluppi in tema di genitorialità sociale come ultima frontiera dell'evoluzione in materia di famiglia, con specifico riferimento alla posizione dei soggetti che si prendono cura dei figli al di là della posizione formale di genitore legittimo
Cogeneration processes based on giant reed gasification combined with ORC and district heating for heat recovery: Comparative energy and exergy analysis
No full textThis research work develops a comprehensive exergy and energy assessment of a cogeneration process using Giant Reed as feedstock, which consists of a biomass dryer, a fluidized bed gasifier, an internal combustion engine operating in a cogeneration mode (CHP), and two different users for the cogenerated heat. One process layout uses cogenerated heat in a district heating network (CHP + DH layout). In the second process layout, the cogenerated heat produces additional electricity through an Organic Rankine Cycle (CHP + ORC layout). In addition to the performance of reed gasification (cold gas efficiency about 0.6), the results showed that the highest rational efficiency was reached in the cogeneration unit, while the highest relative irreversibilities were found in the gasifier and the dryer. The overall energy efficiencies are 0.46 and 0.22 for the CHP + DH and CHP + ORC layouts, respectively, while the overall exergy efficiencies are 0.21 and 0.20. The difference in the sustainability index is just 2 %. The results and methods of this research work can be used to properly design Giant Reed (or similar biomass) gasification plants and bioenergy systems for combined heat and power production, and developing case-studies considering the sustainable use of this feedstock according to a thermodynamic approach based on the second principle
Sistemi insediativi e topografia delle valli del Noce e del Mercure-Lao
No full textIl contributo esamina i sistemi insediativi e la topografia degli insediamenti delle valli di Noce e Lao tra VI e III sec. a.C
