A Case of William’s Syndrome in a Ugandan Child: A Feasible Diagnosis Even in a Low-Resource Setting

Background: Williams–Beuren syndrome (WS) is a rare, complex, congenital developmental disorder including cardiovascular manifestations, intellectual disability and a peculiar cognitive and behavior profile. Supravalvular aortic stenosis (SVAS) is the most frequent cardiovascular abnormality in WS children. Data on WS patients in sub-Saharan Africa are scarce. A genetic study is usually required for a definite diagnosis, but genetic testing is often unavailable in developing countries and the combination of a typical clinical phenotype and echocardiographic profile helps to confirm the diagnosis. Case Report: We report the case of a 5-year-old Ugandan child admitted to a large no profit hospital after he was initially managed as a case of infective endocarditis. A physical examination revealed the typical features of WS. A cardiac echo showed severe SVAS (peak gradient 80 mmHg) with a normal anatomy and function of the aortic valve and mild valvular pulmonary stenosis. The child also had a moderate intellectual disability and a characteristic facies consistent with WS. Conclusion: We present the first reported case of WS in Uganda. Cardiac echo and a characteristic clinical picture could be enough to exclude more common causes of heart failure (i.e., rheumatic heart disease) and to make the diagnosis even when specific genetic tests are not available.

Inattentive Drowsiness Behavior Profile Detection with Heavy Eyed Interval Approach for Biomedical Applications in Health Monitoring System

The efficient tracking of vehicle drivers can be used to prevent collisions through visual human behaviour analysis. Many different methods have not been satisfactory enough such as iris-sklera research, driver’s approximation of gaze, and Hough transforming technological performance. Since these methods make it more difficult to spot drivers’ sleepiness and carelessness. This paper therefore suggested that it be careful to estimate the profile after finding the left eye, right eye, mouth and nose Absence of each of these traits marks a non-frontal approach. The Rectangular Face Classificatión control system monitors frontal faces by moving a rectangular filter on the image for testing the dullness of the face area. Once the facial regions are tracked, the Hybrid Balanced Networks separates the eye area from it depending on the greater axis and the smaller axis. Heavy Eyed Approach is often used to spot drowsiness and twitch of the brow. The intensity of the horizontal plot is measured and successive frames in the eye twitch are not counted as a closed eye for three seconds. The result of the proposed work therefore effectively improves accuracy efficiency.

A multi-layer approach for advanced persistent threat detection using machine learning based on network traffic

Advanced Persistent Threat (APT) is a dangerous network attack method that is widely used by attackers nowadays. During the APT attack process, attackers often use advanced techniques and tools, thus, causing many difficulties for information security systems. In fact, to detect the APT attacks, intrusion detection systems cannot rely on one technique or method but often combine multiple techniques and methods. In addition, the approach for APT attack detection using behavior analysis and evaluation techniques is facing many difficulties due to the lack of characteristic data of attack campaigns. For the above reasons, in this paper, we propose a method for APT attack detection based on a multi-layer analysis. The multi-layer analysis technique in our proposal computes and analyzes various events in Network Traffic to detect and synthesize abnormal signs and behaviors in order to make conclusions about the existence of APT in the system. Specifically, in our proposal, we will use serial 3 main layers for the APT attack detection process including i) Detecting APT attacks based on analyzing abnormal connection; ii) Detecting APT attacks based on analyzing and evaluating Suricata log; iii) Detecting APT attacks based on analyzing behavior profiles that are compiled from layers (i) and (ii). To achieve these goals, the multi-layer analysis technique for APT attack detection will perform 2 main tasks: i) Analyzing and evaluating components of Network Traffic based on abnormal signs and behaviors. ii) building and classifying behavior profile based on each component of network traffic. In the experimental section, we will compare and evaluate the effectiveness of the APT attack detection process of each layer in the multi-layer analysis model using machine learning. Experimental results have shown that the APT attack detection method based on analyzing behavior profile has yielded better results than individual detection methods on all metrics. The research results shown in the paper not only demonstrate the effectiveness of the multilayer analysis model for APT attack detection but also provide a novel approach for detecting several other cyber-attack techniques.

Central Alteration in Peripheral Neuropathy of Trembler-J Mice: Hippocampal pmp22 Expression and Behavioral Profile in Anxiety Tests

Charcot–Marie–Tooth (CMT) type 1 disease is the most common human hereditary demyelinating neuropathy. Mutations in pmp22 cause about 70% of all CMT1. Trembler-J (TrJ/+) mice are an animal model of CMT1E, having the same spontaneous pmp22 mutation that is found in humans. We compared the behavior profile of TrJ/+ and +/+ (wild-type) in open-field and elevated-plus-maze anxiety tests. In these tests, TrJ/+ showed an exclusive head shake movement, a lower frequency of rearing, but a greater frequency of grooming. In elevated-plus-maze, TrJ/+ defecate more frequently, performed fewer total entries, and have fewer entries to closed arms. These hippocampus-associated behaviors in TrJ/+ are consistent with increased anxiety levels. The expression of pmp22 and soluble PMP22 were evaluated in E17-hippocampal neurons and adult hippocampus by in situ hybridization and successive immunohistochemistry. Likewise, the expression of pmp22 was confirmed by RT-qPCR in the entire isolated hippocampi of both genotypes. Moreover, the presence of aggregated PMP22 was evidenced in unmasked granular hippocampal adult neurons and shows genotypic differences. We showed for the first time a behavior profile trait associated with anxiety and a differential expression of pmp22/PMP22 in hippocampal neurons of TrJ/+ and +/+ mice, demonstrating the involvement at the central level in an animal model of peripheral neuropathy (CMT1E).

The Nucleus Accumbens and Ventral Pallidum Exhibit Greater Dopaminergic Innervation in Humans Compared to Other Primates

Recent evidence suggests that increased dopaminergic signaling within the dorsal striatum played a central role in the evolution of the human brain. This increase has been linked to human pro-sociality and language in what has been described as a dopamine-dominated striatum personality style. Increased striatal dopamine is associated with an increase in ventral striatal activity and promotes externally-driven behaviors, including cooperation and social conformity. In contrast, decreased striatal dopamine is associated with increased dorsal striatal activity andfavors internally driven and goal-oriented behaviors. Previous comparative studies have focused on the dorsal striatum, measuring dopaminergic innervation in the dorsal and medial caudate nucleus and putamen. Here, we add to this knowledge by examining regions of the ventral striatum. We quantified the density of tyrosine hydroxylase-immunoreactive axons, as a measure of dopaminergic innervation, in the nucleus accumbens and ventral pallidum of humans, great apes, platyrrhine and cercopithecid monkeys. Our data show that humans have a significantly greater dopaminergic innervation in both structures, supporting the hypothesis that selection for a prosocial neurochemistry in the human basal ganglia may have contributed to the evolution of our uniquely social behavior profile.

Neuropsychological profiles in children and young adults with spina bifida

Purpose A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and completed a neuropsychological battery in order to evaluate their cognitive, personality, and behavior profile. Methods Enrolled children underwent cognitive assessment by means of the Weschler WISC-IV cognitive test and assessment of the attention sustained through the LEITER test. In addition, parents were asked, in order to obtain a personality and behavior profile of the children, to fill in a “CBCL 6-18 years” questionnaire and to fill in a Barthel Index questionnaire. Results Processing Speed Index of the WISC-IV QI scale was statistically significant (p = 0.027), with the highest value presented by autonomous patients (95.8 ± 12.8) and the lowest by patients using a wheelchair (75.5 ± 19). WISC-IV QI mean value is 98 (±15.7) for lipoma patients and 78.7 (±17.6) for LMMC and MMC patients (p = 0.001). In more detail, Perceptual Reasoning (p < 0.0005), Working Memory (p = 0.01), and Processing Speed Index (p = 0.001) highlighted a significant difference between the groups. The attention sustained subscale of the LEITER presented a mean of 6.9 (±3.1) for lipoma patients and a men value of 4.6 (±3.1) for LMMC and MMC patients (p = 0.024). Patients with hydrocephalus had statistically significant worse cognition and autonomy (Barthel Index) score (p < 0.001) compared with those without hydrocephalus, and normal scores regarding attention and depression scales. Conclusion These results can be useful in planning dedicated therapeutic protocols such as suitable rehabilitation treatments, speech therapy, psychomotor skills, and cognitive enhancement and to develop prevention protocols particularly tailored for children with hydrocephalus who appear to have the more deficient skills.

Risky Sexual Behavior in Adolescence: Studies Overview

Risky sexual behavior is the most complicated form of antisocial risky teenage behavior, both in its manifestations and in terms of availability for study and interventions. Sexual behavior and romantic relationships of teenagers are viewed, on the one hand, as important developmental stages, encouraging one's positive psychosocial adaptation, on the other hand, as an element of one's problem behavior profile with lots of unfavorable outcomes, e.g. sexually transmitted diseases, unwanted pregnancies etc, as well as a number of associated risks (substance use, offences etc). The article presents major research focuses dealing with the problem of risky teenage behavior, including the role of genetics, factors of social environment, and also the key results concerning the relationship between adolescents' risky sexual behavior and characteristics of their families, other teens of the same age around them, specifics of the area of their residence and of their local community, as well as adolescents' trade of sexual services.

More Similar, Better Belonging: Effect of Organizational Citizenship Behavior Profile Similarity on Ostracism

As positive nontask behavior, organizational citizenship behavior (OCB) is a well-known concept that has been investigated by numerous studies. However, weakness in the research stream is viewing this concept from the perspective of the actor. In this study, OCB is considered as a social activity that assists actors’ survival in their organizations, and how OCB affects ostracism that effectively reflects belonging in the organization is investigated. Moreover, to identify the relationship in detail, three different independent variables are used, including OCB, OCB aggregate gap, and OCB profile similarity, using social exchange theory and similarity attraction theory. The analysis is conducted using samples from 210 employees who work for Korean companies. The results indicate that OCB profile similarity has a stronger effect on reducing ostracism than the absolute level of OCB and the OCB aggregate gap.