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Background: Aicardi-Goutières Syndrome is a monogenic type 1 interferonopathy with infantile onset, characterized by a variable degree of neurological damage. Approximately 7% of Aicardi-Goutières Syndrome cases are caused by pathogenic variants in the ADAR gene and are classified as Aicardi-Goutières Syndrome type 6. Here, we present a new homozygous pathogenic variant in the ADAR gene. Currently, Janus Kinase inhibitors have been proposed to treat selected interferonopathies such as Aicardi-Goutières Syndrome, although limited information is available on its use and results in the neonatal presentation of this disease. Case presentation: We present two siblings, a male neonate with congenital petechial rash, severe thrombopenia and generalized hypotonia and his deceased sister who had normal development until 5 months of age, when she suffered acute encephalopathy. We describe the clinical course, complementary examinations and follow-up with early treatment of the newborn with ruxolitinib. The homozygous variant c.2908G > A (p.Ala970Thr) in the ADAR gene was found in both siblings, parents were heterozygous carriers. Conclusions: The homozygous variant c.2908G > A (p.Ala970Thr) in the ADAR gene causes Aicardi-Goutières Syndrome type 6. Intrafamilial phenotypic spectrum of the disease varies among individuals with the same pathogenic variant. Early initiation of ruxolitinib improved systemic signs but did not prevent the progression of neurological disease. [ABSTRACT FROM AUTHOR]

Background: Cariprazine (CAR), an antipsychotic with partial agonism at the D3 receptor and higher affinity than dopamine, has shown significant procognitive effects in preclinical animal studies. This study systematically reviews CAR's effects on cognitive measures in patients with schizophrenia and bipolar disorder. Methods: Two independent reviewers systematically searched PubMed, Web of Science, Scopus, and the Cochrane Library up to May 2024, following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Additional studies were found by hand searching the references of included studies. Eligible studies were randomized controlled trials (RCTs) in English that assessed CAR's effects on cognition in patients with schizophrenia or bipolar disorder. Quality was assessed using the Jadad scale. Results: Out of 139 reports, 5 studies (involving 6,104 patients with schizophrenia or bipolar disorder) were included. In schizophrenia, CAR showed better cognitive outcomes (mainly indirect measures) than placebo (PBO) in both early and late stages. It also outperformed risperidone and aripiprazole in attention-related cognitive tests. In bipolar disorder, CAR improved cognition compared to PBO (also using indirect measures). Most studies found the greatest cognitive benefits with low doses of CAR (1.5-3 mg/d). Conclusions: CAR improved cognitive measures compared to PBO and other D2 antagonists or partial agonists in RCTs, especially in patients with greater baseline impairment. Thus, CAR may be a promising option forenhancing cognition in schizophrenic and bipolar patients; though, more trials using specific cognitive assessment tools are needed. [ABSTRACT FROM AUTHOR]

There might be differential characteristics between those who have attempted suicide once in their lifetime (single attempters) and those who have attempted suicide two or more times (multiple attempters). We aimed to identify the factors that differentiate single and multiple attempters in child and adolescents. This study was conducted following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, and the review protocol was registered in PROSPERO. We conducted a systematic literature search in three databases to identify original studies exploring the characteristics of single attempters vs. multiple attempters among adolescents. We considered a wide range for the definition of adolescent, following most recent recommendations: 10–24 years. We carried out a meta-analysis. Fourteen studies were included in the systematic review and 13 in the meta-analysis with a total sample of with a total of 4286 participants. The factors statistically significantly associated with being a multiple attempter in the meta-analysis were: anxiety disorders, depression severity, alcohol abuse, substance abuse, aggressiveness, and hopelessness. Multiple attempters have a more severe clinical profile, with greater severity of symptoms. Knowledge of the risk factors associated with being a multiple attempter could help us to predict which patients are more likely to reattempt suicide and need further monitoring and a tailored treatment. Prevention programs tailored for the adolescent population, along with identification of early risk factors, could help to prevent suicidal behavior among this vulnerable population. [ABSTRACT FROM AUTHOR]

Suicidal behavior and Non-Suicidal Self-Injuries (NSSIs) are a major health problem in the adolescent population. New technologies can contribute to the development of innovative interventions in suicide prevention. Here, we present the SmartCrisis-Teen study protocol. The study consists of a randomized clinical trial which aims to evaluate the effectiveness of a digital safety plan to prevent suicidal behavior and NSSIs in adolescents. This is a multicentric study which will be conducted among the adolescent population, both in clinical and student settings, with a target sample of 1080 participants. The intervention group will receive an Ecological Momentary Intervention (EMI) consisting of a digital safety plan on their mobile phone. All participants will receive their Treatment As Usual (TAU). Participants will be followed for six months, with weekly and monthly telephone visits and face-to-face visits at three and six months. Participants will be assessed using traditional questionnaires as well as Ecological Momentary Assessment (EMA) and Implicit Association Tests (IATs). With this intervention, we expect a reduction in NSSIs through the acquisition of coping strategies and a decrease in suicidal behavior over the course of follow-up. This study provides a novel, scalable digital intervention for preventing suicidal behavior and NSSIs in adolescents, which could contribute to improving adolescent mental health outcomes globally. [ABSTRACT FROM AUTHOR]

Background: Osteogenesis imperfecta (OI) is a rare disease characterized by low bone mass and bone fragility, associated with an increased risk of fractures, and skeletal and extra-skeletal symptoms that results in an impairment of health-related quality of life of OI patients. Since published studies on OI in Spain are limited, this study aimed to determine the epidemiology, assessed the disease burden, management and unmet needs of OI patients in Spain. Thirty-four experts in the management of patients with osteogenesis imperfecta completed two rounds of online consultation and reported real-life experience and data from Spanish hospitals. Delphi study questionnaires were based on literature review. A working group of nationally recognized clinical experts supported the development of the study questionnaires and the final validation of results. Results: The estimated prevalence of patients diagnosed with OI in Spain is 0.56:10,000 inhabitants (95%CI: 0.54–0.59), which represents that, approximately, 2,669 OI patients are currently managed in Spanish hospitals. It is estimated that approximately 269 new patients would be diagnosed with OI each year in Spain, representing an estimated incidence of 0.06 (95%CI: 0.05–0.06) per 10,000 inhabitants per year. Clinical management of OI in Spain is performed by a range of medical specialists; however, multidisciplinary care is not fully implemented. The absence of an approved curative treatment or a treatment to reduce the clinical features of the disease remains the main unmet need. Conclusions: This study provides a snapshot of the current situation of patients with OI in Spain reported by clinical experts. The results provide an estimation of the epidemiology of the disease, and complement the available evidence on disease burden, clinical management, and unmet needs of these patients in Spain. [ABSTRACT FROM AUTHOR]

The implementation of a system to assess the risk of Amyloodinium ocellatum occurrence in rearing ponds in fish farms located in southern Spain is a fundamental aspect to ensure the economic viability of these facilities. For this purpose, a computer program (called Amy) for Windows PCs and an application for mobile devices (AmyAPP), based on the Android operating system, were developed integrating transformation functions and weightings associated with environmental parameters and fish behavioural factors from which it is possible to estimate the level of risk of occurrence of A. ocellatum. The weights for each of the environmental parameters and behavioural factors were estimated from the responses of a panel of experts (the fish farmers) using a Delphi methodology. The results indicate that, under operational validation, Amy/AmyAPP responses were statistically sensitive to the occurrence of A. ocellatum outbreaks in sea bream (Sparus aurata) and sea bass (Dicentrarchus labrax) rearing ponds. [ABSTRACT FROM AUTHOR]

Mental disorders in children and adolescents may follow different trajectories, such as remission, change of diagnosis, or addition of two or more comorbid diagnoses, showing a heterotypic pattern. This study aims to describe the main diagnostic trajectories across a broad range of mental disorder diagnostic categories, from childhood to adolescence and from adolescence to young adulthood in a clinical population. A prospective study was conducted among a clinical sample of children and adolescents who were aged 3–17 years at the face-to-face baseline interview. Electronic health records of these participants were reviewed 10 years later. The diagnostic stability over time was examined using the kappa coefficient, and factors associated with stability were explored using simple logistic regression. The study included a sample of 691 participants. The kappa coefficient for diagnostic stability across all diagnoses was 0.574 for the transition from childhood to adulthood, 0.614 from childhood to adolescence, and 0.733 from adolescence to adulthood. Neurodevelopmental diagnoses had the highest stability. Factors associated with higher diagnostic stability included family history of mental disorders, receiving psychopharmacological treatment, and symptom severity at baseline. We found a variable diagnostic stability across different diagnoses and age categories. The different life transitions represent complex periods that should not be overlooked from a clinical standpoint. An appropriate transition from child and adolescent mental health services to adult mental health services may have a positive impact on children and adolescents with mental disorders. [ABSTRACT FROM AUTHOR]

Chronic hydrocarbon spills are a threat to coastal ecosystems, and their impact on the water quality along watercourses should be characterised to medium long term in order to assess the risk and define cost-effective and sustainable remediation strategies. For this purpose, a methodology for the measurement of total petroleum hydrocarbons (TPH) on a spatio-temporal scale was designed, implemented and validated in the Punta Carnero estuary, in the Santa Elena province, western Ecuador, which is affected by hydrocarbon pollution. The selection of monitoring stations was made according to the different inflows along the water system corresponding to the different anthropogenic activities in the area, in order to assess whether the effluents involve critical levels of pollution. Measurements of hydrodynamic, environmental and vegetation cover parameters were also carried out to assess their possible variations as a consequence of the impact of the spatio-temporal TPH concentrations. An increase in hydrocarbon concentrations was observed from 2013 to 2021. Mean annual TPH levels were above the maximum allowable limit for the protection of aquatic and other wildlife in estuarine waters according to the Ecuadorian regulation for environmental quality. An increase in mangrove tree cover could contribute to an exponential decrease in contamination levels. The methodology used in this study may support the quantifying of TPH content in future applications, which is a priority task for the restoration of degraded ecosystems. [ABSTRACT FROM AUTHOR]

Attention-Deficit/Hyperactivity Disorder (ADHD) is a neurodevelopmental disorder known for its significant heterogeneity and varied symptom presentation. Describing the different subtypes as predominantly inattentive (ADHD–I), combined (ADHD–C), and hyperactive–impulsive (ADHD–H) relies primarily on clinical observations, which can be subjective. To address the need for more objective diagnostic methods, this pilot study implemented a Microsoft Kinect-based Stroop Color–Word Test (KSWCT) with the objective of investigating the potential differences in executive function and motor control between different subtypes in a group of children and adolescents with ADHD. A series of linear mixture modeling were used to encompass the performance accuracy, reaction times, and extraneous movements during the tests. Our findings suggested that age plays a critical role, and older subjects showed improvements in KSWCT performance; however, no significant divergence in activity level between the subtypes (ADHD–I and ADHD–H/C) was established. Patients with ADHD–H/C showed tendencies toward deficits in motor planning and executive control, exhibited by shorter reaction times for incorrect responses and more difficulty suppressing erroneous responses. This study provides preliminary evidence of unique executive characteristics among ADHD subtypes, advances our understanding of the heterogeneity of the disorder, and lays the foundation for the development of refined and objective diagnostic tools for ADHD. [ABSTRACT FROM AUTHOR]

This article introduces a control architecture designed for the development of Hybrid Remotely Operated Underwater Vehicles. The term "Hybrid" characterizes Remotely Operated systems capable of autonomously executing specific operations. The presented architecture maintains teleoperation capabilities while enabling two fully autonomous applications. The approach emphasizes the implementation of reactive navigation by exclusively utilizing data from a Mechanical Scanned Imaging Sonar for control decisions. This mandates the control system to solely react to data derived from the vehicle's environment, without considering other positioning information or state estimation. The study involves transforming a small-scale commercial Remotely Operated Underwater Vehicle into a hybrid system without structural modifications, and details the development of an intermediate Operational Control Layer responsible for sensor data processing and task execution control. Two practical applications, inspired by tasks common in natural or open-water aquaculture farms, are explored: one for conducting transects, facilitating monitoring and maintenance operations, and another for navigating toward an object for inspection purposes. Experimental results validate the feasibility and effectiveness of the authors' hypotheses. This approach expands the potential applications of underwater vehicles and facilitates the development of Hybrid Remotely Operated Underwater Vehicles, enabling the execution of autonomous reactive tasks. [ABSTRACT FROM AUTHOR]

Purpose of Review: We aimed to examine the factors that differentiate single and multiple suicide attempters in adult population. We followed the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines to conduct this review and meta-analysis. The review protocol was registered in PROSPERO. We carried out a systematic literature search in three databases to identify original studies that explored the differences between single and multiple suicide attempters among adult population. Recent Findings: There might be meaningful differences between those individuals that attempt suicide once in their lifespan and those who make multiple attempts in terms of sociodemographic and clinical characteristics. There are no previous meta-analysis addressing this topic in the adult population. Summary: A total of 75 studies were included in the review and 69 were included in the meta-analysis. Multiple attempters were more likely to present certain disorders such as mood and psychotic disorders, as well as personality or substance use disorders. Higher suicide ideation and suicide intent scores also characterized this group. Childhood trauma experiences, stressful life events, and higher rates of hopelessness were statistically significant in multiple attempters. Identifying the factors predicting multiple suicide attempts helps to delineate a high-risk suicidal profile that should be taken into account in the clinical and suicide prevention scenario. [ABSTRACT FROM AUTHOR]

Background: Attention deficit hyperactivity disorder (ADHD) symptoms may persist into adulthood and are likely to cause great problems in young adults. To date, few studies have explored the characteristics of patients diagnosed with ADHD that might influence the utilization of adult mental health services (AMHS). We aimed to examine and identify predictive symptoms of AMHS. Methods: We analysed data from 114 participants diagnosed with ADHD from a cohort of adolescents recruited at the age of 12–17 years, who, at the time of data analysis, were over 18 years old. Results: Among AMHS users, hyperactivity/impulsivity measures were significantly more severe (t = 2.668, df = 112, p <.001), ADHD combined subtype diagnosis (χ2 = 4.66, df = 1, p =.031) was more frequent and dysregulation profile in the SDQ‐P was also significantly higher (t = −2.497, df = 109, p =.014). However, the dysregulation profile did not remain statistically significant after controlling for type of AMHS contact. Conclusions: Our findings suggest that adolescents with ADHD are more likely continue their care under AMHS if they present more severe symptoms of hyperactivity/impulsivity and emotional dysregulation. The better characterization of the patient profile will help clinicians to early identify groups at‐risk and to tailor interventions and prevention strategies. [ABSTRACT FROM AUTHOR]

Copyright of Reumatología Clínica is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Tumor-necrosis-factor-α inhibitors (anti-TNF-α) are associated with an increased risk of tuberculosis (TB) disease, primarily due to reactivation of latent TB infection (LTBI). We assessed the performance of parallel LTBI screening with tuberculin skin test (TST) and QuantiFERON-TB Gold In-Tube assays (QFT-GIT) before anti-TNF-α treatment in children with immune-mediated inflammatory disorders in a low TB-burden setting. We conducted a multicenter cohort study involving 17 pediatric tertiary centers in Spain. LTBI was defined as the presence of a positive TST and/or QFT-GIT result without clinical or radiological signs of TB disease. A total of 270 patients (median age:11.0 years) were included, mainly with rheumatological (55.9%) or inflammatory bowel disease (34.8%). Twelve patients (4.4%) were diagnosed with TB infection at screening (LTBI, n = 11; TB disease, n = 1). Concordance between TST and QFT-GIT results was moderate (TST+/QFT-GIT+, n = 4; TST−/QFT-GIT+, n = 3; TST+/QFT-GIT-, n = 5; kappa coefficient: 0.48, 95% CI: 0.36–0.60). Indeterminate QFT-GIT results occurred in 10 patients (3.7%) and were associated with young age and elevated C-reactive protein concentrations. Eleven of 12 patients with TB infection uneventfully completed standard LTBI or TB treatment. During a median follow-up period of 6.4 years, only 2 patients developed TB disease (incidence density: 130 (95% CI: 20–440) per 100,000 person-years), both probable de novo infections. Conclusion: A substantial number of patients were diagnosed with LTBI during screening. The dual strategy identified more cases than either of the tests alone, and test agreement was only moderate. Our data show that in children in a low TB prevalence setting, a dual screening strategy with TST and IGRA before anti-TNF-α treatment is effective. What is Known: • The optimal screening strategy for latent tuberculosis in children with immune-mediated inflammatory disorders remains uncertain. • Children receiving anti-TNF-α drugs are at increased risk of developing severe tuberculosis disease. What is New: • A dual screening strategy, using TST and an IGRA assay, identified more children with latent tuberculosis than either of the tests alone. • Identification and treatment of latent tuberculosis before initiation of anti-TNF-α therapy averted incident tuberculosis cases. [ABSTRACT FROM AUTHOR]

Symptoms of Attention Deficit Hyperactivity Disorder (ADHD) include excessive activity, difficulty sustaining attention, and inability to act in a reflective manner. Early diagnosis and treatment of ADHD is key but may be influenced by the observation and communication skills of caregivers, and the experience of the medical professional. Attempts to obtain additional measures to support the medical diagnosis, such as reaction time when performing a task, can be found in the literature. We propose an information recording system that allows to study in detail the behavior shown by children already diagnosed with ADHD during a car driving video game. We continuously record the participants' activity throughout the task and calculate the error committed. Studying the trajectory graphs, some children showed uniform patterns, others lost attention from one point onwards, and others alternated attention/inattention intervals. Results show a dependence between the age of the children and their performance. Moreover, by analyzing the positions by age over time using clustering, we show that it is possible to classify children according to their performance. Future studies will examine whether this detailed information about each child's performance pattern can be used to fine-tune treatment. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency during the open label extension (weeks 41–113) of the randomized controlled CLUSTER trial. Methods During a 72-week period, patients received open-label canakinumab 150 or 300 mg, every 4 or 8 weeks. The disease activity was evaluated every 8 weeks using physician global assessment and counting the number of flares. Concentrations of CRP and serum amyloid A protein were measured. The safety was studied by determination and classification of observed adverse events. The safety and efficacy were analysed separately in three subgroups of patients receiving a cumulative dose of less than <35 mg/kg, ≥35 to <70 mg/kg or ≥70 mg/kg. Results Of the 74 patients who started the CLUSTER study, 66 entered Epoch 4 and 65 completed it. During the 72-week period, 42 (64%) patients experienced no flares, while 13 (20%) had one flare, as compared with a median of 12 flares per year reported at baseline. Low physician global assessment scores were seen at the end of the study for all groups with >90% reporting minimal disease activity or none at all. Median CRP concentrations were consistently equal or lower than 10 mg/l, while median serum amyloid A concentrations remained only slightly above the normal range of 10 mg/l. The study showed no new or unexpected adverse events. Conclusion Canakinumab proved effective to control disease activity and prevent flares in mevalonate kinase deficiency during the 72-week study period. No new safety concerns were reported. Trial registration NCT02059291. https://clinicaltrials.gov. [ABSTRACT FROM AUTHOR]

Background: This study reviews the evidence on ecological momentary interventions (EMIs) for managing and preventing suicidal thoughts and behaviors. Methods : This review follows the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Its protocol was registered in the PROSPERO database. We conducted a systematic literature search of five databases: PubMed, EMBASE, Web of Science, PsycInfo, and Cochrane library. The most recent search date was 10 September 2021. Results : After screening and full-text review, 27 studies were included, totaling 19 different interventions. Many of the available interventions have not yet been clinically tested. Those that have undergone effectiveness evaluation (10 interventions) showed good rates of effectiveness and feasibility, with some exceptions. The most widely used intervention model is the safety plan, which allows the user to implement coping and distracting strategies in case of suicidal ideation. Conclusions : Ecological momentary interventions provide certain advantages, such as their wide availability, versatility, and potential for customization. These interventions can be useful complements to traditional care, especially in situations in which face-to-face care is not possible. Evidence on the effectiveness of such interventions is still scarce. Furthermore, barriers limiting implementation in clinical practice remain. The constant advance of technology means that these interventions have great potential for improvement in the coming years. [ABSTRACT FROM AUTHOR]

Attention-deficit/hyperactivity disorder (ADHD) is a prevalent and serious disorder among children. Video games have shown potential for aiding in child healthcare. Video games could contribute to the assessment and management of ADHD, but there are no previous reviews on this topic. Here, we systematically review the evidence about video game-based assessment tools and interventions for children diagnosed with ADHD. This review followed the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. The review protocol was registered in PROSPERO database. We searched four databases—PubMed, PsycInfo, Embase and clinicaltrials.gov—to identify original studies exploring either video game-based interventions or video game-based assessment tools in children with ADHD. After initial screening, full text revision and study selection, 22 articles were finally included in the review. Most studies used PC as platform, with a minority using a video console, pad, or 3D device. Video game-based assessment tools were generally effective in discriminating ADHD cases from controls, and in discriminating between ADHD subtypes. Video game-based therapeutic interventions were well accepted and generally effective in improving cognitive areas and decreasing ADHD symptoms. Gamification and cognitive training could be the main mechanisms underlying the usefulness and effectiveness of video game-based assessment tools and interventions. Software optimization and greater collaboration between developers and healthcare professionals are some of the priorities for future research in this area. [ABSTRACT FROM AUTHOR]

Video games are a promising area of intervention for children diagnosed with Autism Spectrum Disorders (ASD). However, reviews on this topic are scarce. This review on studies exploring video games for the treatment of ASD followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and its protocol was registered in PROSPERO. PubMed, PsycInfo, Embase, WebOfScience and clinicaltrials.gov databases were searched. Twenty-four articles were included in the review. Video game-based interventions were effective for alleviating ASD symptoms, albeit with small effect sizes. High rates of acceptability and adherence to treatment were obtained. Conclusion: Video games are a promising area for improving the treatment of children with ASD. Exploring commercial video games is one of the lines for future research. [ABSTRACT FROM AUTHOR]

The deep lasso algorithm (dlasso) is introduced as a neural version of the statistical linear lasso algorithm that holds benefits from both methodologies: feature selection and automatic optimization of the parameters (including the regularization parameter). This last property makes dlasso particularly attractive for feature selection on small samples. In the two first conducted experiments, it was observed that dlasso is capable of obtaining better performance than its non-neuronal version (traditional lasso), in terms of predictive error and correct variable selection. Once that dlasso performance has been assessed, it is used to determine whether it is possible to predict the severity of symptoms in children with ADHD from four scales that measure family burden, family functioning, parental satisfaction, and parental mental health. Results show that dlasso is able to predict parents' assessment of the severity of their children's inattention from only seven items from the previous scales. These items are related to parents' satisfaction and degree of parental burden. [ABSTRACT FROM AUTHOR]

This paper aims to evaluate the possibility of using non-utilized hydraulic energy in urban water distribution systems. For this purpose, the viability and possible alternatives of installing conventional turbines or pumps working as turbines (PATs = Pumps as Turbines) at the head of pressure hydraulic networks in two towns in the province of Huelva have been studied: Cañaveral de León and Aracena (Andalucía, southwest Spain). Sensitivity analyses are carried out for: (a) To study the feasibility and efficiency of installing turbines or PATs in the hydraulic networks; (b) To determine the required hydraulic capacity of the turbines or PATs; (c) To analyse the installation points in the pipeline network; and (d) To evaluate the possible generation of electrical energy. The evaluation of the recoverable amount of hydraulic energy in both water distribution systems requires the application of the energy conservation equation, taking into account the calculation of load losses, the Darcy-Weisbach equation. A diagram energy height-flow rate with different working ranges of commercial turbines/PATs was used for the selection of the appropriate hydraulic machine. The results obtained of the Aracena hydraulic network show that the best solution would be the installation of a 2 kW BANKI that could generate an approximate annual energy of 17,52 MWh/year. In the Cañaveral de León water supply system a 0,75 kW BANKI turbine with an approximate annual energy generation of 6,57 MWh/year is proposed as the best solution. [ABSTRACT FROM AUTHOR]

Background: There has been an extensive debate about a potential association between intelligence and social cognition. In this study, we aimed to assess the association between social cognition as measured with the Reading the Mind in the Eyes test (RMET) and intelligence as measured with the fourth edition of the Wechsler Intelligence Scale for Children (WISC-IV) in children and adolescents diagnosed with Asperger Syndrome (AS). Methods: We conducted a cross-sectional study among 84 children diagnosed with AS aged 6–16 years (mean = 11.64; standard deviation = 2.75; 92.9% males). We analyzed the association between RMET performance and WISC-IV total score as well as the association between RMET performance and each of the four WISC-IV indexes (processing speed index, PSI; working memory index, WMI; perceptual reasoning index, PRI, and verbal comprehension index, VCI). Results: We found a positive correlation between RMET performance and full-scale intelligence quotient (r = 0.340; p < 0.01), VCI (r = 0.310; p < 0.01), PRI (r = 0.401; p < 0.01), and WMI (r = 0.292; p < 0.01). In the linear regression model, age was a significant predictor of RMET score (β = 0.409; p < 0.001) as was PRI (β = 0.309; p = 0.019). Conclusion: Our results suggest that intelligence quotient positively influences RMET performance, indicating that intelligence increases social cognition in individuals diagnosed with AS. However, weak-to-moderate size effects were found. This study contributes to understanding the mechanisms underlying the disturbance of social cognition in children and adolescents diagnosed with AS. [ABSTRACT FROM AUTHOR]

Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the fibrillin‐1 (FBN1) gene, and cardiovascular involvement is the leading cause of mortality. We sought to examine the genotype/phenotype realtionship in 61 consecutive patients with a phenotype and genotype compatible with MFS. The FBN1 gene was analyzed by massive sequencing using a hybridization capture‐based target enrichment custom panel. Forty‐three different variants of FBN1 were identified, of which 17 have not been previously reported. The causal variants of MFS were grouped into mutations resulting in haploinsufficiency (HI group; 23 patients) and mutations producing a dominant‐negative effect (DN group; 38 patients). Patient information was collected from electronic medical records and clinical evaluation. While no significant differences were found between the two groups, the HI group included more cases with aortic dissection and occurring at a younger age that the DN group (34.7% vs. 15.8%; p = 0.160). Irrespective of the mutation group, males presented with a higher probability of aortic involvement (4‐fold higher risk than females) and aortic dissections events occurred at younger ages. Patients with DN variants carrying a cysteine substitution had a higher incidence of ectopia lentis. [ABSTRACT FROM AUTHOR]

Copyright of Maintenance & Reliability / Eksploatacja i Niezawodność is the property of Polish Scientific & Technical Society Consumables, Polish Maintenance Society and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We conducted a meta-analysis of 18 studies to establish whether a relation exists between Reading the Mind in the Eye Test (RMET) performance and intelligence quotient (IQ) in individuals diagnosed with autism-spectrum disorders (ASD) and controls, taking into account relevant characteristics such as age, gender, and autism quotient. Our findings indicate that RMET performance was better in controls compared with those diagnosed with ASD. We found that RMET performance is dependent on full and verbal IQ and age in controls. However, RMET performance is negatively correlated with performance IQ in individuals diagnosed with ASD. These results suggest that the methodology applied by ASD when taking the RMET is different from control individuals and might depend less on verbal abilities. [ABSTRACT FROM AUTHOR]

Ecological momentary assessment is an excellent tool for the measurement of different day-to-day domains in patients and capturing real-world and real-time data. The purpose of this review is to evaluate feasibility in current ecological momentary assessment studies on emotional and behavioral functioning, functional impairments, and quality of life patients with an attention-deficit/hyperactivity disorder diagnosis. This systematic review follows the recommendation of Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines selecting articles published from January 1, 1990, up to the latest access on May 2018, identifying a pool of 23 eligible studies. Twenty-three studies demonstrate the validity of ecological momentary assessment methodology in evaluating different aspects of patients with attention-deficit/hyperactivity disorder. Fifteen studies focus on the child’s or adolescent’s daily behavior, while eight studies only focus on adults. The studies presented in this review monitored patients and their families over a maximum period of 28 days. We can conclude that ecological momentary assessment can be successfully implemented with attention-deficit/hyperactivity disorder patients to evaluate diverse backgrounds. However, more studies are needed with a longer monitoring period, especially in adolescents, to determine the effectiveness of ecological momentary assessment on patients with attention-deficit/hyperactivity disorder. [ABSTRACT FROM AUTHOR]

Objective To evaluate the efficacy and safety of canakinumab treatment in active hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Methods This was a 3-part open-label study with an initial 6-month treatment period in which patients with HIDS (n = 9) received canakinumab subcutaneously at a dose of 300 mg (or 4 mg/kg for those weighing ≤40 kg) every 6 weeks (period 1 [P1]), followed by a 6-month withdrawal period (period 2 [P2]), and then a 24-month extension treatment period with canakinumab at the same dose (period 3 [P3]). The primary end point was reduction in the frequency of attacks during treatment periods as compared to the historical period (HP; defined as the period in which patients did not receive drugs other than nonsteroidal antiinflammatory drugs and/or steroids). Results All 9 patients completed P1 and P2, whereas only 8 patients completed P3. All patients achieved a complete response during P1, and only 2 required dose adjustments. The number of attacks per patient decreased from a median of 5 (range 3-12) during the HP to a median of 0 (range 0-2) during P1. During P2, 7 of 9 patients experienced a disease flare within a median of 110 days (range 62-196) after the last canakinumab dose. Laboratory findings were normalized by day 15 of treatment and remained at normal levels throughout the study. Analysis of blood transcriptome profiles, assessed during P1, showed up-regulated levels of interferon and myeloid-related inflammatory responses in untreated patients compared to healthy controls, and these rapidly decreased following canakinumab injection, reaching levels comparable to those of healthy controls. At least 1 adverse event (AE) was detected in all 9 patients. Most of the AEs were mild in intensity, with infections being the most frequent AE. Serious AEs were reported in 4 patients. Conclusion The results of this study demonstrate the efficacy and safety of canakinumab treatment to control active HIDS and to suppress inflammation-related transcriptional responses. [ABSTRACT FROM AUTHOR]

We assessed the potential for simulation and modelling of the blackspot seabream ( Pagellus bogaraveo) population in the Strait of Gibraltar to discriminate the environmental effects of fishery impacts. A discrete biomass-abundance dynamic model was implemented to obtain a simulated monthly time series of blackspot seabream biomass. On this simulated time series, autoregressive integrated moving average ( ARIMA) models were fitted. The best ARIMA fit provided a significant correlation of 0.76 and persistence index higher than 0.85. The proportion of variance non-explained by the ARIMA models was correlated with a time series of sea surface temperature ( SST) and North Atlantic Oscillation ( NAO). The analysis of global, annual and winter correlation between the proportion of variance not explained by the ARIMA models and environmental variables showed that significant associations were not detected over the full time series. Our analysis therefore suggests that overexploitation is the main factor responsible for the commercial depletion of blackspot seabream in the Strait of Gibraltar. [ABSTRACT FROM AUTHOR]

Objective To assess the efficacy of tocilizumab (TCZ) for the treatment of juvenile idiopathic arthritis (JIA)-associated uveitis. Methods We conducted a multicenter study of patients with JIA-associated uveitis that was refractory to conventional immunosuppressive drugs and anti-tumor necrosis factor (anti-TNF) agents. Results We assessed 25 patients (21 female; 47 affected eyes) with a mean ± SD age of 18.5 ± 8.3 years. Uveitis was bilateral in 22 patients. Cystoid macular edema was present in 9 patients. Ocular sequelae found at initiation of TCZ included cataracts (n = 13), glaucoma (n = 7), synechiae (n = 10), band keratopathy (n = 12), maculopathy (n = 9), and amblyopia (n = 5). Before TCZ, patients had received corticosteroids, conventional immunosuppressive drugs, and biologic agents (median 2 [range 1-5]), including adalimumab (n = 24), etanercept (n = 8), infliximab (n = 7), abatacept (n = 6), rituximab (n = 2), anakinra (n = 1), and golimumab (n = 1). Patients received 8 mg/kg TCZ intravenously every 4 weeks in most cases. TCZ yielded rapid and maintained improvement in all ocular parameters. After 6 months of therapy, 79.2% of patients showed improvement in anterior chamber cell numbers, and 88.2% showed improvement after 1 year. Central macular thickness measured by optical coherence tomography in patients with cystoid macular edema decreased from a mean ± SD of 401.7 ± 86.8 μm to 259.1 ± 39.5 μm after 6 months of TCZ ( P = 0.012). The best-corrected visual acuity increased from 0.56 ± 0.35 to 0.64 ± 0.32 ( P < 0.01). After a median follow-up of 12 months, visual improvement persisted, and complete remission of uveitis was observed in 19 of 25 patients. Significant reduction in the prednisone dosage was also achieved. The main adverse effects were severe autoimmune thrombocytopenia in 1 patient, pneumonia and then autoimmune anemia and thrombocytopenia in 1 patient, and viral conjunctivitis and bullous impetigo in 1 patient. Conclusion TCZ appears to be a useful therapy for severe refractory JIA-associated uveitis. [ABSTRACT FROM AUTHOR]

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To develop recommendations on the transition from pediatric care to adult care in patients with chronic inflammatory rheumatic diseases with childhood onset based. Recommendations were generated following nominal group methodology and Delphi technique. A panel of 16 experts was established. A systematic literature review (on transitional care) and a narrative review were performed and presented to the panel in the first panel meeting to be discussed. A first draft of recommendations was generated and circulated. Focal groups with adolescents, young adults and parents were organized. In a second meeting, the focus group results along with the input from invited psychologist were used to establish definitive recommendations. Then, a Delphi process (two rounds) was carried out. A group of 72 pediatric and adult rheumatologists took part. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70 % voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford center for evidence-based medicine levels of evidence. Transition care was defined as a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic inflammatory rheumatic diseases with childhood onset as they move from child-centered to adult-oriented healthcare systems. The consensus covers: transition needs, barriers and facilitators, transitional issues (objectives, participants, content, phases, timing, plans, documentation and responsibilities), physicians' and other health professionals' knowledge and skill requirements, models/programs, and strategies and guideline for implementation. Preliminary recommendations and agreement grade are shown in the Table (first Delphi round). These recommendations are intended to provide health professionals, patients, families and other stakeholders with a consensus on the transition process from pediatric to adult care. [ABSTRACT FROM AUTHOR]

Severe variants of fibrodysplasia ossificans progressiva (FOP) affect <2% of all FOP patients worldwide, but provide an unprecedented opportunity to probe the phenotype-genotype relationships that propel the pathology of this disabling disease. We evaluated two unrelated children who had severe reduction deficits of the hands and feet with absence of nails, progressive heterotopic ossification, hypoplasia of the brain stem, motor and cognitive developmental delays, facial dysmorphology, small malformed teeth, and abnormal hair development. One child had sensorineural hearing loss, microcytic anemia, and a tethered spinal cord and the other had a patent ductus arteriosus and gonadal dysgenesis with sex reversal (karyotype 46, XY female). Both children had an identical mutation in ACVR1 c.772A>G; p.Arg258Gly (R258G), not previously described in FOP. Although many, if not most, FOP mutations directly perturb the structure of the GS regulatory subdomain and presumably the adjacent αC helix, substitution with glycine at R258 may directly alter the position of the helix in the kinase domain, eliminating a key aspect of the autoinhibitory mechanism intrinsic to the wild-type ACVR1 kinase. The high fidelity phenotype-genotype relationship in these unrelated children with the most severe FOP phenotype reported to date suggests that the shared features are due to the dysregulated activity of the mutant kinase during development and postnatally, and provides vital insight into the structural biology and function of ACVR1 as well as the design of small molecule inhibitors. © 2015 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective To evaluate the interleukin-6 receptor inhibitor tocilizumab for the treatment of patients with polyarticular-course juvenile idiopathic arthritis (pcJIA). Methods This three-part, randomised, placebocontrolled, double-blind withdrawal study (NCT00988221) included patients who had active pcJIA for ≥6 months and inadequate responses to methotrexate. During part 1, patients received open-label tocilizumab every 4 weeks (8 or 10 mg/kg for body weight (BW) <30 kg; 8 mg/kg for BW ≥30 kg). At week 16, patients with ≥JIA-American College of Rheumatology (ACR) 30 improvement entered the 24- week, double-blind part 2 after randomisation 1:1 to placebo or tocilizumab (stratified by methotrexate and steroid background therapy) for evaluation of the primary end point: JIA flare, compared with week 16. Patients flaring or completing part 2 received open-label tocilizumab. Results In part 1, 188 patients received tocilizumab (<30 kg: 10 mg/kg (n=35) or 8 mg/kg (n=34); ≥30 kg: n=119). In part 2, 163 patients received tocilizumab (n=82) or placebo (n=81). JIA flare occurred in 48.1% of patients on placebo versus 25.6% continuing tocilizumab (difference in means adjusted for stratification: -0.21; 95% CI -0.35 to -0.08; p=0.0024). At the end of part 2, 64.6% and 45.1% of patients receiving tocilizumab had JIA-ACR70 and JIAACR90 responses, respectively. Rates/100 patient-years (PY) of adverse events (AEs) and serious AEs (SAEs) were 480 and 12.5, respectively; infections were the most common SAE (4.9/100 PY). Conclusions Tocilizumab treatment results in significant improvement, maintained over time, of pcJIA signs and symptoms and has a safety profile consistent with that for adults with rheumatoid arthritis. Trial registration number: NCT00988221. [ABSTRACT FROM AUTHOR]

Uveitis associated with juvenile idiopathic arthritis (JIA) typically involves the anterior chamber segment, follows an indolent chronic course, and presents a high rate of uveitic complications and a worse outcome as compared to other aetiologies of uveitis. Disease assessment, treatment, and outcome measures have not been standardized. Collaboration between pediatric rheumatologists and ophthalmologists is critical for effective management and prevention of morbidity, impaired vision, and irreparable visual loss. Although the Standardization of Uveitis Nomenclature Working Group recommendations have been a great advance to help clinicians to improve consistency in grading and reporting data, difficulties arise at the time of deciding the best treatment approach in the individual patient in routine daily practice. For this reason, recommendations for a systematized control and treatment strategies according to clinical characteristics and disease severity in children with JIA-related uveitis were developed by a panel of experts with special interest in uveitis associated with JIA. A clinical management algorithm organized in a stepwise regimen is here presented. [ABSTRACT FROM AUTHOR]

Familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome are dominantly inherited autoinflammatory diseases associated to gain-of-function NLRP3 mutations and included in the cryopyrin-associated periodic syndromes (CAPS). A variable degree of somatic NLRP3 mosaicism has been detected in ≈35% of patients with CINCA. However, no data are currently available regarding the relevance of this mechanism in other CAPS phenotypes. Objective To evaluate somatic NLRP3 mosaicism as the disease-causing mechanism in patients with clinical CAPS phenotypes other than CINCA and NLRP3 mutation-negative. Methods NLRP3 analyses were performed by Sanger sequencing and by massively parallel sequencing. Apoptosis-associated Speck-like protein containing a CARD (ASC)-dependent nuclear factor kappa-light chain-enhancer of activated B cells (NF-κB) activation and transfection-induced THP-1 cell death assays determined the functional consequences of the detected variants. Results A variable degree (5.5-34.9%) of somatic NLRP3 mosaicism was detected in 12.5% of enrolled patients, all of them with a MWS phenotype. Six different missense variants, three novel (p.D303A, p.K355T and p.L411F), were identified. Bioinformatics and functional analyses confirmed that they were disease-causing, gain-of-function NLRP3 mutations. All patients treated with anti-interleukin1 drugs showed long-lasting positive responses. Conclusions We herein show somatic NLRP3 mosaicism underlying MWS, probably representing a shared genetic mechanism in CAPS not restricted to CINCA syndrome. The data here described allowed definitive diagnoses of these patients, which had serious implications for gaining access to anti-interleukin 1 treatments under legal indication and for genetic counselling. The detection of somatic mosaicism is difficult when using conventional methods. Potential candidates should benefit from the use of modern genetic tools. [ABSTRACT FROM AUTHOR]

The aim of this paper is to test the ability of neural network approaches to hindcast the spring standardized precipitation index on a 6-month time scale (SPI6) in Portugal, based on winter large-scale climatic indices. For this purpose, the linkage of the spring SPI time series with the winter North Atlantic Oscillation (NAO) and the sea surface temperature (SST) was investigated by means of maps of the correlation coefficient for the period from October 1910 to September 2004. The results indicate that the winter NAO is a good predictor for the SPI6 of the spring (SPI6 finishing in April, May and June, SPI6April, SPI6May and SPI6June, respectively) for the northern, central and southern regions of Portugal. The winter SST1 (area of the Mediterranean Sea) must only be considered for the northern region, and the winter SST3 (area of the North Atlantic between Iberia and North America) only for the southern region. Spatial maps of predictive SPI6 for April, May and June were created and validated. The neural models explained more than 81% of the total variance for the SPI6April and SPI6May and more than 64% of the total variance for the SPI6June. Probability maps were also developed considering the values predicted by the neural methods for the spring months and all drought categories (moderate, severe and extreme). These maps indicating the probability of droughts can provide valuable support for the integrated planning and management of water resources throughout Portugal. Copyright © 2012 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objective. To propose a reduced joint power Doppler US (PDUS) assessment and provide preliminary evidence of its validity, feasibility, reliability and sensitivity to change compared with a comprehensive (i.e. 44 joints) PDUS assessment in evaluating synovitis in JIA.Methods. This multicentre study included 42 children with active JIA with &geq;4 clinically involved joints requiring modified therapy. At each visit, clinical and PDUS assessments were performed blinded. Each joint was scored for greyscale (GS) synovitis and power Doppler signal according to a 4-point semiquantitative scale with calculation of US composite indices and US composite joint counts. A process of data reduction based on the frequency of US joint involvement was performed to obtain a reduced PDUS assessment. The relationship between the comprehensive and the reduced PDUS assessments was investigated by Spearman’s coefficient at all visits, as well as the relationship between changes in the two PDUS assessments during follow-up. In addition, the metric properties of the comprehensive and the reduced PDUS assessments were tested.Results. The 10-joint PDUS assessment, including bilateral knee, ankle, wrist, elbow and the second MCP joints, detected 100% of children with GS synovitis and power Doppler signal. The two PDUS assessments were highly correlated at all visits. The reduced model had a higher responsiveness than the comprehensive model. Intraobserver and interobserver agreement was good for both US findings.Conclusion. The 10-joint PDUS assessment is valid and feasible for assessment of synovitis in JIA in clinical practice. [ABSTRACT FROM PUBLISHER]

For monitoring hydrological events characterized by high spatial and temporal variability, the number and location of recording stations must be carefully selected to ensure that the necessary information is collected. Depending on the characteristics of each natural process, certain stations may be spurious or redundant, whereas others may provide most of the relevant data. With the objective of reducing the costs of the monitoring system and, at the same time, improving its operational effectiveness, three procedures were applied to identify the minimum network of rain gauge stations able to capture the characteristics of droughts in mainland Portugal. Drought severity is characterized by the standardized precipitation index applied to the timescales of 1, 3, 6 and 12 consecutive months. The three techniques used to reduce the dimensionality of the network of rain gauges were as follows: (i) artificial neural networks with sensitivity analysis, (ii) application of the mutual information criterion and (iii) K-means cluster analysis using Euclidean distances. The results demonstrated that the best dimensionality reduction method was case dependent in the three regions of Portugal (northern, central and southern) previously identified by cluster analysis. All the reduction techniques lead to the selection of a subset of rain gauges capable of reproducing the original temporal patterns of drought. For specific severe drought events in Portugal in the past, the comparison between drought spatial patterns obtained with the original stations and the selected subset indicated that the subset produced statistically satisfactory results (correlation coefficients higher than 0.6 and efficiency coefficients higher than 0.5). Copyright © 2012 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objectives To develop data-driven criteria for clinically inactive disease on and off therapy for juvenile dermatomyositis (JDM). Methods The Paediatric Rheumatology International Trials Organisation (PRINTO) database contains 275 patients with active JDM evaluated prospectively up to 24 months. Thirty-eight patients off therapy at 24 months were defined as clinically inactive and included in the reference group. These were compared with a random sample of 76 patients who had active disease at study baseline. Individual measures of muscle strength/ endurance, muscle enzymes, physician's and parent's global disease activity/damage evaluations, inactive disease criteria derived from the literature and other ad hoc criteria were evaluated for sensitivity, specificity and Cohen's ? agreement. Results The individual measures that best characterised inactive disease (sensitivity and specificity >0.8 and Cohen's ? >0.8) were manual muscle testing (MMT) ⩾78, physician global assessment of muscle activity=0, physician global assessment of overall disease activity (PhyGloVAS) ⩽0.2, Childhood Myositis Assessment Scale (CMAS) ⩾48, Disease Activity Score ⩽3 and Myositis Disease Activity Assessment Visual Analogue Scale ⩽0.2. The best combination of variables to classify a patient as being in a state of inactive disease on or off therapy is at least three of four of the following criteria: creatine kinase ⩽150, CMAS ⩾48, MMT ⩾78 and PhyGloVAS ⩽0.2. After 24 months, 30/31 patients (96.8%) were inactive off therapy and 69/145 (47.6%) were inactive on therapy. Conclusion: PRINTO established data-driven criteria with clearly evidence-based cut-off values to identify JDM patients with clinically inactive disease. These criteria can be used in clinical trials, in research and in clinical practice. [ABSTRACT FROM AUTHOR]

A model comprising blocks of artificial neural networks (ANNs) combined in sequence was used to simulate the inflow and outflow in a water resources system under a shortage of water. We assessed the selection of appropriate input data using linear and non-linear cross-correlation functions and sensitivity analysis. The potential model inputs were flow, precipitation and temperature data from various gauging stations throughout the upper watershed of the 'Guadiana Menor' River (southern Spain), and the model considered various input time lags. The ANNs based on the selected inputs were effective relative to those with no relevant inputs, and produced more parsimonious models. We also investigated conceptual analogies inherent in the ANN models by analyzing the response profiles of the modelled variables (inflow and outflow) in relation to each of the selected input data. The results demonstrate that the neural approach approximated the behaviour of various components of the water resources system in terms of various hydrologic cycle processes and management rules. Our findings suggest that in dry periods a mean temperature increase of 1°C in low altitude locations of the region will result in a mean decrease of approximately 2% in the inflow to the water resources system, and a mean increase of approximately 12% in the outflow requirements for irrigation purposes. [ABSTRACT FROM AUTHOR]

This study investigated the frequency of droughts for the period September 1910 to October 2004 in mainland Portugal, based on monthly precipitation data from 144 rain gauges distributed across the country. The drought events were characterized using the standardized precipitation index (SPI) applied to time scales of 1, 3, 6 and 12 consecutive months. Based on the SPI time scale series a regional frequency analysis of drought magnitudes was undertaken using two approaches: annual maximum series (AMS) and partial duration series (PDS). Three spatially defined regions (north, central and south) were identified by cluster analysis and analyzed for homogeneity. Maps of drought magnitude were developed using a kriging technique for several return periods. Similar uniform spatial patterns were found throughout the country using the AMS and PDS approaches. For several SPI time scales a comparison of the observed and estimated maximum magnitude (269-year empirical return period) showed that the AMS combined with the selected probability distribution models (Pearson type III, general Pareto and Kappa) provided better results than the PDS approach combined with the same models. A general and simplified characterization of drought duration revealed a relatively uniform pattern of droughts events across the country. [ABSTRACT FROM AUTHOR]

An analysis of droughts in mainland Portugal based on monthly precipitation data, from September 1910 to October 2004, in 144 rain gages distributed uniformly over the country is presented. The drought events were characterized by means of the Standardized Precipitation Index (SPI) applied to different time scales (1, 6, and 12 consecutive months and 6 months from April to September and 12 months from October to September). To assess spatial and temporal patterns of droughts, a principal component analysis (PCA) and K-means clustering (KMC) were applied to the SPI series. In this way, three different and spatially well-defined regions with different temporal evolution of droughts were identified (north, central, and south regions of Portugal). A spectral analysis of the SPI patterns obtained with principal component analysis and clusters analysis, using the fast Fourier transform algorithm (FFT), showed that there is a manifest 3.6-year cycle in the SPI pattern in the south of Portugal and evident 2.4-year and 13.4-year cycles in the north of Portugal. The observation of the drought periods supports the occurrence of more frequent cycles of dry events in the south (droughts from moderate to extreme approximately every 3.6 years) than in the north (droughts from severe to extreme approximately every 13.4 years). These results suggest a much stronger immediate influence of the NAO in the south than in the north of Portugal, although these relations remain a challenging task. [ABSTRACT FROM AUTHOR]

In this paper, we propose an alternative method to predict the Gonadosomatic Index (GSI), based on a technique known as computational neural networks (CNNs), with two main objectives: (1) to develop a quick and reliable method for the prediction of the fish reproductive period under variable environmental conditions, and thus (2) to reduce the field sampling and laboratory efforts. Three different neural architectures (5-6-6-1, 5-8-8-1 and 5-10-10-1), whose 'training' was carried out controlling three threshold determinism coefficients (Rt 2: 0.7, 0.8 and 0.9), were trained to estimate the GSI of an introduced pumpkinseed ( Lepomis gibbosus) population inhabiting a highly fluctuating Mediterranean stream in southern Spain. This GSI estimate was made using several easily measured fish and environmental variables. The correlation (R) between the GSI observed (GSIr) and the GSI predicted by the CNN (GSIe) was very high (>0.8 in all cases). The optimal CNN structure was the 5-6-6-1 with $ R^{2}_{t} = 0.8 $ because it produced the best generalization of the confidence limits of GSIe with respect to GSIr. To compare with traditional multiple regression analysis, we submitted the data to the same process as with CNN. The validation of the regression model produced a much lower correlation (R) than the CNN models. As an example of the predictive capacities of the CNN models, we predict the hypothetical pumpkinseed reproductive cycle of our population but under the environmental conditions found in the Camargue marshes (South France). [ABSTRACT FROM AUTHOR]

The derivation of the mean velocity profile for a given vorticity distribution over the pipe cross-section is presented in this paper1. The velocity profile and the vorticity distribution are axisymmetric, which means that the radius is the only variable. The importance of the vortex field for the flow analysis is discussed in the paper. The polynomial function with four free parameters is chosen for the vorticity distribution. Free parameters of this function are determined using boundary conditions. There are also two free exponents in the polynomial. These exponents are determined based on the comparison of this analytical formula with experimental data. Experimental data are taken from the Princeton superpipe data which consist of 26 velocity profiles for a wide range of Reynolds numbers (Re). This analytical formula for the mean velocity profile is more precise than the previous one and it is possible to use it for the wide range of Reynolds number <31,577; 35,259,000>. This formula is easy to use, to integrate, or to derivate. The empirical formulas for the profile parameters as a function of Re are also included in this paper. All information for the mean velocity profile prediction in the mentioned Re range are in the paper. It means that it is necessary to know the average velocity v(av), the pipe radius R, and Re to be able to predict the turbulent mean velocity profile in a pipe. [ABSTRACT FROM AUTHOR]

The main purpose of the presented research is to raise the efficiency of pumping stations' operational work by developing a model for reducing energy costs in urban water supply systems. Pumping systems are responsible for a significant portion of the total electrical energy use. Significant opportunities exist to reduce the pumping energy through smart design, retrofitting, and operating practices. Today, considering the increase in pumping energy prices in water conveyance systems, the problem of optimal operation of pumping stations is very actual. The optimal operation of pumping stations was determined using a Genetic Algorithm Optimization (GAO) to achieve the minimum energy cost. The paper presents a novel management model for the optimal design and operation of water pumping systems on a real case study for the town of Gonabad in Iran. To achieve this goal, three days in a year were selected randomly. The results indicate that the proposed mode in conjunction with a GAO is a versatile management model for the design and operation of the real pumping station. Modeling results show that optimization with a GAO reduces power consumption by about 15–20%. [ABSTRACT FROM AUTHOR]

One of the unit operations involved in the production of olive oil is the separation of liquid–liquid systems (and other multiphase flows) in their fundamental phases. The use of helical separators could be an alternative to be considered for that task in order to reduce energy consumption and improve the quality of products in olive oil mills ('almazaras'). In this work, four models of helical separators have been built and tested in order to manage olive oil and water two-phase flows (with olive oil as the majority phase). Separation yields were analyzed from a dimensional analysis perspective, considering variables such as density and viscosity, flow rate, head losses, or the water concentration in the flows studied. The best separation yields (of the order of 80% to 100%) were obtained for olive oil–water two-phase flows in which the water concentrations could be higher, in some cases, than 5–10% for Reynolds numbers of below 60. [ABSTRACT FROM AUTHOR]

Leakages in water distribution systems have great economic and environmental impacts and are a major issue for water utilities. In this work, the water balance and the Minimum Night Flow (MNF) method for evaluating the amount of water loss, as well as the power and Fixed and Variable Area Discharge (FAVAD) equations for analyzing the relationship between leakage and pressure, were applied to a fully monitored District Metered Area (DMA) located in Gorino Ferrarese (FE, Italy). Time series of (a) the water consumption of each user, (b) the DMA inflow, and (c) the pressure at the DMA inlet point were monitored with a 5 min time step. The results of an analysis carried out by exploiting the collected time series highlighted that: (a) The application of the MNF method based on literature values can lead to significant inaccuracies in the presence of users with irregular consumption, and (b) the estimation of the parameters of the power and FAVAD equations is highly affected by the amounts and types of observed data used. [ABSTRACT FROM AUTHOR]

This paper shows the results of a field appliance study of the hydraulic well method to prevent embankment piping, which is proposed by the Japanese Matsuyama River National Highway Office. The large-scale embankment experiment and seepage analysis were conducted to examine the hydraulic well. The experimental procedure is focused on the pore water pressure. The water levels of the hydraulic well were compared with pore water pressure data, which were used to look over the seepage variations. Two different types of large-scale experiments were conducted according to the installation points of hydraulic wells. The seepage velocity results by the experiment were almost similar to those of the analyses. Further, the pore water pressure oriented from the water level variations in the hydraulic well showed similar patterns between the experiment and numerical analysis; however, deeper from the surface, the larger pore water pressure of the numerical analysis was calculated compared to the experimental values. In addition, the piping effect according to the water level and location of the hydraulic well was quantitatively examined for an embankment having a piping guide part. As a result of applying the hydraulic well to the point where piping occurred, the hydraulic well with a 1.0 m water level reduced the seepage velocity by up to 86%. This is because the difference in the water level between the riverside and the protected side is reduced, and it resulted in reducing the seepage pressure. As a result of the theoretical and numerical hydraulic gradient analysis according to the change in the water level of the hydraulic well, the hydraulic gradient decreased linearly according to the water level of the hydraulic well. From the results according to the location of the hydraulic well, installation of it at the point where piping occurred was found to be the most effective. A hydraulic well is a good device for preventing the piping of an embankment if it is installed at the piping point and the proper water level of the hydraulic well is applied. [ABSTRACT FROM AUTHOR]