Cardiovascular abnormalities and impaired exercise performance in adolescents with congenital adrenal hyperplasia

Context: Patients with classic Congenital Adrenal Hyperplasia (CAH) are treated with lifelong glucocorticoids (GCS). Cardiovascular (CV) and metabolic effects of such therapy in adolescents have never been quantified. Objective: To investigate left ventricular (LV) morphology, function and exercise performance in adolescents with CAH. Design and Setting: cross-sectional and controlled study conducted at a tertiary referral centre. Patients: Twenty patients with classic CAH (10 females) aged 13.6±2.5 years and 20 healthy controls comparable for sex and pubertal status were enrolled in the study and compared to a group of 18 patients without CAH receiving a similar dose of GCS for Juvenile Idiopathic Arthritis (JIA). Main Outcomes Measures: Echocardiographic assessment and symptom-limited exercise testing were performed. Anthropometric, hormonal and biochemical parameters were also measured. Results: Compared to healthy controls, patients with CAH exhibited an increased BMI (p<0.001), waist-to-height ratio (p<0.001), percentage of body fat (p<0.001) as well as higher insulin concentrations and HOMA index even after adjustment for BMI (p=0.03 and p=0.05, respectively). Moreover, CAH patients exhibited an impaired exercise capacity as shown by reduced peak workload (99±27 vs 126±27 W, p<0.01) and higher systolic blood pressure response at peak (156±18 vs 132±11 mmHg, p<0.01; Δ=45±24 vs 22±10 mmHg, p=0.05) with respect to healthy controls. CAH males displayed mild LV diastolic dysfunction as documented by significant prolongation of both isovolumic relaxation time (IRT) (118±18 vs 98±11ms, p<0.05) and mitral deceleration time (MDT) (138±25 vs 111±15 ms, p<0.01). No significant differences in CV function were found between CAH and JIA patients. Conclusion: Adolescents with CAH exhibit impaired exercise performance and enhanced systolic blood pressure response during exercise. In our population, such abnormalities appear related to GCS therapy rather than CAH per se. CAH males, but no females, present mild LV diastolic dysfunction that correlates with testosterone concentrations suggesting a sex hormone related difference

Self-tuning model predictive control for wake flows

This study presents a noise-robust closed-loop control strategy for wake flows employing model predictive control. The proposed control framework involves the autonomous offline selection of hyperparameters, eliminating the need for user interaction. To this purpose, Bayesian optimization maximizes the control performance, adapting to external disturbances, plant model inaccuracies, and actuation constraints. The noise robustness of the control is achieved through sensor data smoothing based on local polynomial regression. The plant model can be identified through either theoretical formulation or using existing data-driven techniques. In this work, we leverage the latter approach, which requires minimal user intervention. The self-tuned control strategy is applied to the control of the wake of the fluidic pinball, with the plant model based solely on aerodynamic force measurements. The closed-loop actuation results in two distinct control mechanisms: boat tailing for drag reduction and stagnation point control for lift stabilization. The control strategy proves to be highly effective even in realistic noise scenarios, despite relying on a plant model based on a reduced number of sensors

Heterogeneous design and mechanical analysis of HELIAS 5-B helium-cooled pebble bed breeding blanket concept

One of the most challenging objectives of the European research concerning nuclear fusion technology, promoted by the EUROfusion consortium, is to bring stellarator-type nuclear fusion devices to maturity. To this purpose, studies on a large HELIcal-axis advanced stellarator (HELIAS), extrapolated from Wendelstein 7-X and based on a 5-fold symmetry (HELIAS 5-B), are currently ongoing. The HELIAS 5-B stellarator reactor will be endowed with a breeding blanket (BB) system to allow for the self-sustainability of the nuclear fusion reaction and make it suitable for electricity generation. In this paper, we present the first ever heterogeneous mechanical design and the preliminary structural assessment of a bean-shaped ring of a HELIAS 5-B BB sector. The proposed mechanical design, which is based on the helium-cooled pebble bed (HCPB) BB concept and developed according to the “sandwich” architecture, foresees an actively cooled segment box connected to a back-supporting structure equipped with manifolds. The internal region (breeding zone) is reinforced by actively cooled steel plates. The proposed heterogeneous design was checked against nominal loads and an in-box loss of coolant accidental scenario, which is a typical design driver for BBs. The assessment has been performed according to the RCC-MRx structural design code. Our results are herewith presented and critically discussed, focusing on the potential follow-up of the HELIAS 5-B HCPB BB design

Acerrima Germanorum libertas. Il ruolo della Germania di Tacito nella formazione del liberalismo europeo

EnBefore the growth of Enlightened constitutional theory, that based the discourse of mixed government and individual rights on rational and universalistic premises, the struggles of European constitutionalism were mostly grounded on the authority of antiquity. In particular, between 15th and 17th centuries, while the Mediterranean carved the modern myth of an ancient republican liberty, northern Europe framed its resistance against the centralization of the monarchies on a 'Gothic Renaissance'. While absolute sovereigns started carving the 'divine right of kings' doctrine by rearranging the late-Roman lex regia de imperio to fit a national dimension, a heterogeneous front of monarchomaques opposed them the myth of an unwritten 'Gothic' constitution that for immemorial custom protected their ancient liberties. This constitutional quarrel was grounded on the age-old opposition between Romanitas and Germanitas. Where Rome embodied the symbol of a monistic and centralistic constitution, based on the absolute will of a legibus solutus sovereign, the ancient Germanic constitution was identified with a mixed constitution based on a weak idea of sovereignty, that limited the king's prerogative by means of a complex balance between intermediate powers. A system already described by Tacitus, and that Montesquieu's Esprit des Lois would celebrate as the barbaric root of modern liberty.FrAvant que le Siècle des Lumières fondât le discours des droits et des constitutions sur des bases rationnelles et universelles, les luttes du constitutionnalisme européen étaient en grande partie fondées sur l'autorité de l'antiquité. En particulier, entre le 15ème et le 17ème siècle, alors que le méditerranéen découpait le mythe moderne d'une ancienne liberté républicaine, l'Europe du nord encadrait sa résistance contre la centralisation des monarchies sur une 'Renaissance gothique'. Tandis que les souverains absolus commençaient à découper la doctrine de la 'monarchie de droit divin' en réadaptant la lex regia de imperio tardo-romaine au contexte de l'État national, un front hétérogène de monarchomaques opposait le mythe d'une constitution 'gothique' non écrite qui, selon une coutume immémoriale, avait toujours protégé leurs libertés fondamentales. Cette querelle était fondée sur l'opposition entre Romanitas et Germanitas. Là où Rome incarnait le symbole d'une constitution moniste et centraliste, basé sur la volonté d'un souverain legibus solutus, la constitution germanique était fondée sur une idée faible de souveraineté, qui limitait la prérogative du roi au moyen d'une complexe balance des pouvoirs. Un système déjà décrit par Tacite, et que l'Esprit des Lois de Montesquieu célébrerait comme la racine barbare de la liberté des modernes

Electromagnetic and electromechanical applications of graphene-based materials

This volume contains the extended abstracts of the contributions presented at the workshop Nanoscale Excitations in Emergent Materials (NEEM 2015) held in Rome from 12 to 14 October 2015, an event organized and supported in the framework of the Bilateral Cooperation Agreement between Italy and India within the project of major relevance "Investigating local structure and magnetism of cobalt nano-structures", funded by the Italian Ministry of Foreign Affairs and the Department of Science and Technology in India

Performance of MRI for standardized lymph nodes assessment in breast cancer: are we ready for Node-RADS?

Objectives: The Node-RADS score was recently introduced to offer a standardized assessment of lymph node invasion (LNI). We tested its diagnostic performance in accurately predicting LNI in breast cancer (BC) patients with magnetic resonance imaging. The study also explores the consistency of the score across three readers. Materials and methods: A retrospective study was conducted on BC patients who underwent preoperative breast contrast-enhanced magnetic resonance imaging and lymph node dissection between January 2020 and January 2023. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value were calculated for different Node-RADS cut-off values. Pathologic results were considered the gold standard. The overall diagnostic performance was evaluated using receiver operating characteristic curves and the area under the curve (AUC). A logistic regression analysis was performed. Cohen's Kappa analysis was used for inter-reader agreement. Results: The final population includes 192 patients and a total of 1134 lymph nodes analyzed (372 metastatic and 762 benign). Increasing the Node-RADS cut-off values, specificity and PPV rose from 71.4% to 100% and 76.7% to 100%, respectively, for Reader 1, 69.4% to 100% and 74.6% to 100% for Reader 2, and from 64.3% to 100% and 72% to 100% for Reader 3. Node-RADS &gt; 2 could be considered the best cut-off value due to its balanced performance. Node-RADS exhibited a similar AUC for the three readers (0.97, 0.93, and 0.93). An excellent inter-reader agreement was found (Kappa values between 0.71 and 0.83). Conclusions: The Node-RADS score demonstrated moderate-to-high overall accuracy in identifying LNI in patients with BC, suggesting that the scoring system can aid in the identification of suspicious lymph nodes and facilitate appropriate treatment decisions. Clinical relevance statement: Node-RADS &gt; 2 can be considered the best cut-off for discriminating malignant nodes, suggesting that the scoring system can effectively help identify suspicious lymph nodes by staging the disease and providing a global standardized language for clear communication. Key points: Axillary lymphadenopathies in breast cancer are crucial for determining the disease stage. Node-RADS was introduced to provide a standardized evaluation of breast cancer lymph nodes. RADS &gt; 2 can be considered the best cut-off for discriminating malignant nodes

Anesthetic management in a patient with Arnold‐Chiari malformation type 1,5: A case report

: A 42-year-old male patient with Arnold-Chiari malformation type 1,5 (ACM-1,5) came to implant a hip prosthesis. He underwent a previous general anesthesia, with difficult airway management and complication in awakening. In this second surgery, an extradural approach was preferred to keep intracranial pressure and hemodynamics stable

The exploitation of limpets in a Mediterranean Marine Protected Area: assessing the effectiveness of protection in the intertidal zone

Limpets are intertidal keystone grazers and their overexploitation could have significant consequences for intertidal communities. Limpets are harvested around the Sinis Peninsula (Sardinia, Italy) but harvesting is prohibited within the “Penisola del Sinis - Isola di Mal di Ventre” Marine Protected Area (MPA). This work assesses the effects of human harvesting on the population dynamics of three common Mediterranean species of Patella, namely, P. rustica, P. caerulea and P. ulyssiponensis, testing the effectiveness of the MPA and the role of site accessibility in limiting the intensity of such harvesting pressure. In the period between June 2015 and August 2016, limpet abundance and size were recorded on a monthly basis by means of photographic frames within linear transects at ten sites spread out along the coastline of the Sinis Peninsula to assess growth and temporal patterns. Limpets older than two years are extremely rare in the study area. Limpets are more abundant within the MPA in comparison with non-protected areas and within less accessible sites in comparison with areas where the intertidal zone is easily accessible from land. Despite this, overall human-induced mortality in the area is high, indeed the pool of limpets observed with a mean density of 104.3 ± 9.7 limpets/m2 during the first survey was reduced by 99.2%in less than one year. This work demonstrates that human harvesting strongly affects the population dynamics of Patella species in the area and that within the MPA this stressor is not efficiently reduced