Emotional impact in β-thalassaemia major children following cognitive-behavioural family therapy and quality of life of caregiving mothers

Cognitive-Behavioural Family Therapy (CBFT) can be an effective psychological approach for children with beta-thalassaemia major, increasing compliance to treatment, lessening the emotional burden of disease, and improving the quality of life of caregivers

Compliant gluten-free children with celiac disease: an evaluation of psychological distress

<p>Abstract</p> <p>Background</p> <p>Children with chronic illnesses are known to have increased risks for emotional and behavioral problems. In the present study, children and adolescent suffering from celiac disease (CD) were compared with healthy controls to assess differences in the psychological profile.</p> <p>Methods</p> <p>A total of 100 well-treated and compliant CD patients (65 females/35 males; age mean ± SD: 10.38 ± 2.71) were compared to 100 normal controls (58 females/42 males; age mean ± SD: 11.47 ± 2.61). Emotional and behavioral problems were assessed by the Child Behavior Checklist (CBCL), the Children's Depression Inventory (CDI) and the Multidimensional Anxiety Scale for Children (MASC).</p> <p>Results</p> <p>Subjects with CD self-reported an increased rate of anxiety and depression symptoms and showed higher scores in "harm avoidance" and "somatic complaints", in the CBCL parent-report questionnaire, as compared to healthy control subjects. Furthermore, gender differences could be observed in the group of CD patients, with males displaying significantly higher CBCL externalizing scores, in social, thought and attention problems, as compared to female, who in turns showed more prominent internalizing symptoms such as depression.</p> <p>Conclusions</p> <p>The increased rate of emotional and behavioral problems in children and adolescent with CD emphasizes the importance of an early detection of mental health problems in these children.</p

Systems engineering approach for pre-conceptual design of DEMO divertor cassette

Abstract This paper presents the pre-conceptual design activities conducted for the European DEMO divertor, focusing on cassette design and Plasma Facing Components (PFC) integration. Following the systems engineering principles, a systematic design method, the Iterative and Participative Axiomatic Design Process (IPADeP), has been adopted. Basing on Axiomatic Design, IPADeP supports the early conceptual design of complex systems. The work moved from the geometrical and interface constraints imposed by the 2015 DEMO configuration model. Then, since different materials will be used for cassette and PFCs, the divertor geometry has been developed taking into account the cooling parameters of the cassette Eurofer steel and the integration of PFCs cooling system. Accordingly, the design process led to a double wall cassette structure with internal reinforcing ribs to withstand cassette coolant pressure and three different kinds of piping schemes for PFCs with dual circuits. These three solutions differs in the feeding pipes layouts and target manifold protection and they have been proposed and evaluated considering heat flux issues, shielding problems, interface requirements with blanket and vacuum vessel and remote maintenance needs. A cassette parametric shell model has been used to perform first structural analyses of the cassette body against coolant pressure. Taking advantages of the parametric surface modelling and its linkage with Finite Element (FE) code, the cassette ribs layout and thickness has been evaluated and optimized, considering at the same time the structural strength needed to withstand the coolant parameters and the maximum stiffness required for cassette preloading and locking needs

Sleep disturbances and behavioral symptoms in pediatric Sotos syndrome

Background: Sotos syndrome (SoS) is a rare overgrowth genetic disease caused by intragenic mutations or microdeletions of the NSD1 gene located on chromosome 5q35. SoS population might present cognitive impairment and a spectrum of behavioral characteristics, with a worse profile in patients with microdeletion. Although patients with SoS are known to have impaired sleep habits, very little data are available. The present study aimed to assess the prevalence of sleep disorders (SDs) in a pediatric cohort of patients with SoS and their correlation with neuropsychiatric profiles. Methods: We included patients with a SoS diagnosis and age &lt; 18 years; all patients underwent a comprehensive neuropsychological assessment, including evaluation of cognition, adaptive functions through the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and behavioral problems using the Achenbach Child Behavior Checklist (CBCL) and Conners’ Parent Rating Scale-Revised (CPRS-R:L) questionnaire. To investigate the presence of SD parents, the Sleep Disturbance Scale for Children (SDSC) was completed. Results: Thirty-eight patients (M 61%, F 39%, mean age 11.1 ± 4.65 years) were included in the study. Although only two had a prior SD diagnosis, 71.1% (N = 27) exhibited pathological scores on SDSC. No statistically significant associations were found between positive SDSC results and genetic microdeletion, intellectual disability (ID), or other medical conditions/treatments. However, a positive correlation emerged between SDSC scores and Conners’ Global Index (p = 0.048) and Restless/Impulsive (p = 0.01) scores, CBCL externalizing (p = 0.02), internalizing (p = 0.01), and total scores (p = 0.05). Conversely, a negative linear relationship was observed between the SDSC score and the ABAS GAC and ABAS CAD scores (p = 0.025). Conclusion: We detected an SD in 71.1% of our sample, with a positive relation between SD and internalizing and externalizing symptom levels, especially hyperactivity and impulsivity. Our study demonstrated a high prevalence of SD in pediatric patients with SoS, highlighting that all patients should be screened for this problem, which has a great impact on the quality of life of patients and their families.</p

The Role of Quantic Molecular Resonance (QMR) in the Treatment of Inferior Turbinate Hypertrophy (ITH): Our Experience With Long-Term Follow-Up in Allergic and Nonallergic Rhinitis Refractory to Medical Therapy. Preliminary Results.

Objective: The aim of this study was to assess the long-term effectiveness of quantic molecular resonance (QMR) in the treatment of inferior turbinate hypertrophy (ITH) in allergic and nonallergic rhinitis refractory to medical therapy. Methods: This study enrolled 281 patients, 160 males (56.9%) and 121 females (43.1%), mean age 37.8 ± 4.1 years, range 18 to 71. Fifty-four patients have been lost to follow up and have been therefore excluded from the final analysis. Based on skin prick test results, 69 patients were considered allergic (group A) and 158 nonallergic (group B). All subjects underwent before surgery (T0) and 3 (T1), 12 (T2), 24 (T3), and 36 months (T4) after QMR treatment to: 4-phase rhinomanometric examination, nasal endoscopy evaluation, and visual analogue scale to quantify the subjective feelings about nasal obstruction. Results: Subjective and objective parameters showed statistically significant improvement in both groups. Group B parameters not changed during follow-up, while group A showed significant worsening between T1 and subsequent assessments. T4 outcome indicates a better result in nonallergic patients. Conclusions: In accordance with the literature, our preliminary data validate QMR treatment as a successful therapeutic option for nasal obstruction due to ITH. Nonallergic patients had a very good T4 outcome. Allergic patients showed a worsening trend after 1 year probably due to other causes

The influence of physical activity performed at 20-40 years of age on cardiovascular outcomes in medical patients aged 65-75

Summary Introduction Several studies show that physical activity can reduce the risk of cardiovascular disease, but the vast majority of these focus on the short- to intermediate-term benefits or refer to very specific populations. This observational study was conducted to determine whether physical activity performed during the third or fourth decade of life influences the occurrence of cardiovascular events in patients aged 65-75 years. Materials and methods We studied a cohort of 2191 unselected patients admitted to Internal Medicine Departments. Data were collected on the patients' medical history and their physical activity level when they were 20 to 40 years old. For the latter purpose, we used a specific questionnaire to assess the levels of physical activity related to the patients' job, daily life, leisure time, and sports. Results Almost half (44.2%) of the patients we evaluated reported moderate-intense physical activity when they were 20-40 years old. Around one third (35.8%) of the patients had experienced at least one major cardiovascular event, and there was a slight trend towards fewer cardiovascular events in patients with histories of physical activity (mean risk reduction: 4%, multivariate analysis). More evident benefits were observed in the subgroup of patients with diabetes, where cardiovascular outcomes were much better in patients who had been physically active than in those with sedentary life-styles (mean risk reduction: 24%). Conclusions Given its design, our study may have underestimated the cardiovascular benefits of physical activity. Nonetheless, our results suggest that moderate-intense exercise during young adulthood may have limited beneficial effects on cardiovascular disease in old age, except in specific high-risk populations (diabetic patients). More evident benefits are probably associated with regular physical activity throughout life

Sleep disturbances and behavioral symptoms in pediatric Sotos syndrome

BackgroundSotos syndrome (SoS) is a rare overgrowth genetic disease caused by intragenic mutations or microdeletions of the NSD1 gene located on chromosome 5q35. SoS population might present cognitive impairment and a spectrum of behavioral characteristics, with a worse profile in patients with microdeletion. Although patients with SoS are known to have impaired sleep habits, very little data are available. The present study aimed to assess the prevalence of sleep disorders (SDs) in a pediatric cohort of patients with SoS and their correlation with neuropsychiatric profiles.MethodsWe included patients with a SoS diagnosis and age &lt; 18 years; all patients underwent a comprehensive neuropsychological assessment, including evaluation of cognition, adaptive functions through the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and behavioral problems using the Achenbach Child Behavior Checklist (CBCL) and Conners’ Parent Rating Scale-Revised (CPRS-R:L) questionnaire. To investigate the presence of SD parents, the Sleep Disturbance Scale for Children (SDSC) was completed.ResultsThirty-eight patients (M 61%, F 39%, mean age 11.1 ± 4.65 years) were included in the study. Although only two had a prior SD diagnosis, 71.1% (N = 27) exhibited pathological scores on SDSC. No statistically significant associations were found between positive SDSC results and genetic microdeletion, intellectual disability (ID), or other medical conditions/treatments. However, a positive correlation emerged between SDSC scores and Conners’ Global Index (p = 0.048) and Restless/Impulsive (p = 0.01) scores, CBCL externalizing (p = 0.02), internalizing (p = 0.01), and total scores (p = 0.05). Conversely, a negative linear relationship was observed between the SDSC score and the ABAS GAC and ABAS CAD scores (p = 0.025).ConclusionWe detected an SD in 71.1% of our sample, with a positive relation between SD and internalizing and externalizing symptom levels, especially hyperactivity and impulsivity. Our study demonstrated a high prevalence of SD in pediatric patients with SoS, highlighting that all patients should be screened for this problem, which has a great impact on the quality of life of patients and their families

Progress in the pre-conceptual CAD engineering of European DEMO divertor cassette

This paper presents the recent progress in the pre-conceptual design activities for the EU-DEMO divertor Cassette Body, performed in the framework of the work package \u201cDivertor\u201d of the EUROfusion Power Plant Physics & Technology (PPPT) program. According to Systems Engineering Principles, the divertor CAD model is reviewed, considering the updates in the DEMO configuration model presented by the Programme Management Unit (PMU) in 2017. The design parameters affected by these changes and their impact on the divertor design and on the interfaced systems are analysed. Then, the paper focuses on the integration on the new cassette geometry of the divertor sub-systems. This includes the design of a \u201cshielding liner\u201d for cassette body and Vacuum Vessel protection, as well as the development of the cassette body-to-Vacuum Vessel fixation system. The design activities related to these main sub-systems are discussed in detail, in terms of CAD model and thermo-mechanical calculations. \ua9 201