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Abstract Background Autosomal recessive polycystic kidney disease (ARPKD) is a genetically inherited pediatric disorder. It is caused by a mutation in the PKHD1 gene located on chromosome 6. The predominant phenotype is characterized by early-onset bilateral enlarged kidneys, as well as fibrocystic changes in the kidney and liver. Fetuses or infants usually present with Potter syndrome, and they are more likely to develop severe renal insufficiency. Generally, patients die perinatally or in infancy. Liver involvement has been reported in adults with ARPKD who have survived the neonatal period and childhood. However, renal involvement is rarely expected in adulthood. The case is being presented for its clinical rarity, in addition to emphasize the critical role of NGS approaches in diagnosis. Case presentation We hereby describe a 33-year-old female with adult-onset proteinuria and nephromegaly. She had a rare homozygous missense mutation of the PKHD1 gene with autosomal recessive inheritance. The proband has consanguineous heterozygote parents. The mutation was identified by whole-exome sequencing, and the results were confirmed by segregation analysis. Conclusion Here, we reported a thorough literature review of late-onset autosomal recessive polycystic kidney disease. Furthermore, we explored the importance of genetic work-up in families with genetic disorders and consanguineous marriages, particularly in underdeveloped countries.

Abstract Amidst the increasing complexity of microgrid optimization, characterized by numerous decision variables and intricate non-linear relationships, there is a pressing need for highly efficient algorithms. This study introduces a tailored Mixed Integer Nonlinear Programming (MINLP) model that optimizes the charging and discharging schedules of electric vehicles (EVs) and energy storage systems (ESS) while incorporating Distributed Flexible AC Transmission System (D-FACTS) devices. To address these challenges, a novel approach based on the Large-Scale Two-Population Algorithm (LSTPA) is proposed. The model's effectiveness was evaluated using a 33-node microgrid, where the proposed method achieved a total purchased energy of 1.2 MWh, a voltage deviation of 0.0357 p.u, and a CPU time of 551 s, outperforming traditional methods like NSGA-II, PSO, and JAYA. Additionally, in a 69-node microgrid, the approach resulted in a total purchased energy of 0.3 MWh and a voltage deviation of 0.0078 p.u. These results demonstrate the superior performance of the proposed method in terms of energy efficiency, voltage stability, and computational time, advancing the efficiency of microgrid management.

Abstract The integration of renewable energy sources into smart distribution grids poses substantial challenges in maintaining grid stability, efficiency, and reliability due to their inherent variability and intermittency. This study addresses these challenges by proposing a novel two-level optimization model aimed at enhancing operational efficiency and robustness in smart distribution grids. The model synergistically integrates renewable energy sources, energy storage systems, electric vehicles, and demand-side management through a dynamic reconfiguration approach. It employs a robust optimization framework combined with a two-stage second-order cone optimization model to manage real-time operations and strategic grid reconfiguration. Key findings from simulations on the IEEE 33 and 69-bus networks underscore the model’s effectiveness. In the 33-bus system, implementing the demand response program led to a significant reduction in power losses, from 0.64 MW to 0.52 MW, and improved voltage stability, with the minimum voltage increasing from 0.970 to 0.980 p.u. Similarly, in the 69-bus system, power losses decreased from 0.85 MW to 0.79 MW, and voltage stability improved, with the minimum voltage rising from 0.962 to 0.972 p.u. The model also demonstrated reduced energy procurement needs, showcasing its impact on enhancing grid efficiency and reliability. These results highlight the model’s potential for advancing smart grid management strategies, offering significant improvements in operational performance and stability under varying demand conditions.

This paper presents a model for the optimization of a multi-area distribution grid considering the high penetration levels of photovoltaic (PV) resources and electric vehicle charging station (EVCSs) based on distributed flexible AC transmission system (D-FACTS) devices including on-load tap changer (OLTC) transformers and step voltage regulator (SVR) tap control and shunt capacitor (SC) and shunt reactor (ShR) to reduce losses and voltage deviation. The overarching research aim was to avoid the overvoltage caused by power injection by PV sources and the voltage drop caused by charging EVs in distribution grids. The proposed model was designed based on distribution network load flow and was optimized using a modified particle swarm optimization (M-PSO) algorithm. Two different test networks were tested under different scenarios to analyze the proposed model, revealing the efficiency of the proposed model.

Abstract The objective of this study was to investigate the effects of corn processing index (CPI)—particularly at 70% and 85%—in starter feed in combination with the provision of forage, either alfalfa hay (AH) or wheat straw (WS), on feed intake, growth performance, rumen pH, and blood metabolites of dairy calves. Forty-eight male Holstein calves (43.0 ± 1.5 kg body weight) were randomly assigned (n = 12 calves per treatment) to one of four dietary treatments: (1) a textured starter diet containing 70% CPI and AH (70CPIAH), (2) a textured starter feed containing 70% CPI combined with WS (70CPIWS), (3) a textured starter feed containing 85% CPI and AH (85CPIAH), (4) a textured starter feed containing 85% CPI combined with WS (85CPIWS). Intake of starter feed (TMR) and milk was individually monitored and recorded daily, while body weight was measured weekly. On days 30 and 65, blood samples were collected from all calves 3 h after morning feeding. No interaction was detected between the CPI of starter feed diets and forage provision for starter intake, dry matter intake (DMI), metabolizable energy intake (MEI), feed efficiency (FE), average daily gain (ADG), and body weight (BW). The preweaning and overall DMI, preweaning, postweaning and overall FE and ADG, preweaning and overall starter intake, preweaning and overall ME intake, and postweaning and overall BW were greater for calves fed 85CPI than those fed 70CPI starter diets. Postweaning and overall ADG and postweaning FE were greater in calves fed WS than those fed AH. Body length and hip width were greater for calves offered 85CPI than in fed 70CPI. Wheat straw supplementation increased rumen pH at day 65 in calves fed 70CPI, but not in those fed 85CPI. No interaction was found between the CPI in the starter feed diet and the forage source for starter intake and DMI, MEI, FE, and BW. The results showed that including corn with 85% processing index in dairy calf starters improved their growth performance.

Introduction Prescribing long-term opioid therapy is a nuanced clinical decision requiring careful consideration of risks versus benefits. Our goal is to understand patient, provider and context factors that impact the decision to prescribe opioids in patients with cancer.Methods We conducted a secondary analysis of the raw semistructured interview data gathered from 42 prescribers who participated in one of two aligned concurrent qualitative studies in the USA and Australia. We conducted a two-part analysis of the interview: first identifying all factors influencing long-term prescribing and second open coding-related content for themes.Results Factors that influence long-term opioid prescribing for cancer-related pain clustered under three key domains (patient-related, provider-related and practice-related factors) each with several themes. Domain 1: Patient factors related to provider–patient continuity, patient personality, the patient’s social context and patient characteristics including racial/ethnic identity, housing and socioeconomic status. Domain 2: Provider-related factors centred around provider ‘personal experience and expertise’, training and time availability. Domain 3: Practice-related factors included healthcare interventions to promote safer opioid practices and accessibility of quality alternative pain therapies.Conclusion Despite the differences in the contexts of the two countries, providers consider similar patient, provider and practice-related factors when long-term prescribing opioids for patients with cancer. Some of these factors may be categorised as cognitive biases that may intersect in an already disadvantaged patient and exacerbate disparities in the treatment of their pain. A more systematic understanding of these factors and how they impact the quality of care can inform appropriate interventions.

BACKGROUND: Cancer pain is highly prevalent and often managed in primary care or by oncology providers in combination with primary care providers. OBJECTIVES: To understand interdisciplinary provider experiences coordinating opioid pain management for patients with chronic cancer-related pain in a large integrated healthcare system. DESIGN: Qualitative research. PARTICIPANTS: We conducted 20 semi-structured interviews with interdisciplinary providers in two large academically affiliated VA Medical Centers and their associated community-based outpatient clinics. Participants included primary care providers (PCPs) and oncology-based personnel (OBPs). APPROACH: We deductively identified 94 examples of care coordination for cancer pain in the 20 interviews. We secondarily used an inductive open coding approach and identified themes through constant comparison coming to research team consensus. RESULTS: Theme 1: PCPs and OBPs generally believed one provider should handle all opioid prescribing for a specific patient, but did not always agree on who that prescriber should be in the context of cancer pain. Theme 2: There are special circumstances where having multiple prescribers is appropriate (e.g., a pain crisis). Theme 3: A collaborative process to opioid cancer pain management would include real-time communication and negotiation between PCPs and oncology around who will handle opioid prescribing. Theme 4: Providers identified multiple barriers in coordinating cancer pain management across disciplines. CONCLUSIONS: Our findings highlight how real-time negotiation about roles in opioid pain management is needed between interdisciplinary clinicians. Lack of cross-disciplinary role agreement may result in delays in clinically appropriate cancer pain management.

Background and Aim: Enuresis is one of the common problems among children. The present pilot study was conducted to investigate the effect of Rosa damascene on nocturnal enuresis in children. Materials and Methods: A randomized pilot study was performed from April 2017 to March 2018. The study participants were 40 children aged between 5-12 years with nocturnal enuresis. They were randomly divided to intervention and control groups using block randomization. The intervention group received R. damascena petals under the brand name Gole-Ghand (Barij- essence, Iran) as 5 cc orally, every 8 hours for one month. The control group received desmopressin as nasal spray (Sina Darou, Iran) 1-2 puffs for one month (maximum 20 mg daily). The primary outcome was complete or partial improvement in enuresis. The secondary outcome was frequency of side effects in patients. Results: Twenty-two out of 39 patients 22 (56.4%) were male and 17 patients (43.6%) were female with the average age of 7.3±1.86 years. There was no noticeable distinction between the groups in age, sex, urbanity, history of treatment, and primary or secondary enuresis (p>0.05). Complete improvement was observed in 14 patients (70%) in the control group. Partial improvement was observed in four patients (20%) in the control group and in six patients (31.5%) in the intervention group (p:0.001). No side effect was reported during the study in the intervention group. Conclusion: Based on the results of this pilot study, the use of R. damascena may lead to partial improvement against enuresis in some children. Further studies are required to evaluate this hypothesis.

Introduction: The necessity of a dynamic education in the field of medicine with the aim of improving the quality of education in order to train more efficient doctors has always led to the provision of new educational methods. The purpose of this study is to investigate the effect of the implementation of the selected educational program of pediatric outpatient drugs on the level of knowledge of interns. Methods: This study is a two-group semi-experimental interventional study of pre-test-post-test type with a control group, which included 70 people (through random assignment, 35 people were considered as the control group and 35 people were considered as the intervention group) from the medical internship students of the year 2016-2018 academic year of Mashhad University of Medical Sciences who were introduced to Imam Reza, Qaim and Dr. Sheikh hospitals of Mashhad were selected by available sampling method. The data collection tool was a structured test titled measuring the level of students' knowledge and awareness of pediatric outpatient drugs, based on the variables studied. Data analysis was done using descriptive statistical methods (mean and frequency of standard deviation) and analysis of covariance test. Results: The mean and standard deviation of the interns' knowledge in the pre-test was 5.93±0.57 in the control group and 5.93±0.57 in the intervention group, and in the post-test the mean and standard deviation of the interns' knowledge in the control group (13.89±1.28) and intervention group (16.82±1.34). The average scores of the two control and intervention groups in the post-test were significant (p=0.001), which indicates the effect of the complementary educational program of pediatric outpatient medicine in the intervention group. The test statistic was (F=85.407). Conclusion: The present study confirmed the effect of the selected educational program of pediatric outpatient drugs on the promotion of regular education and as an effective supplementary education. Therefore, it seems that the implementation of this educational program in medical sciences universities across the country should be considered in the coming years.

Multisystem inflammatory syndrome in children (MIS-C) is a novel syndrome in children following the coronavirus disease 2019 (COVID-19) pandemic, which has similar symptoms to Kawasaki disease or toxic shock syndrome. The most prevalent symptoms in MIS-C patients are fever and gastrointestinal symptoms, with substantial cardiac complications. Cardiac involvement is frequently reported in MIS-C patients and includes arrhythmia, coronary artery aneurysm and dilation, conduction abnormalities, and ventricular dysfunction. Cardiogenic or vasodilatory shock may develop in patients with severe MIS-C, necessitating inotropic support, fluid resuscitation, mechanical ventilation, and extracorporeal membrane oxygenation. Empirical therapies have attempted to reverse the inflammatory response, and steroids or intravenous immunoglobulin have all been commonly used. Most children will survive with prompt diagnosis and appropriate treatment, but since the disease's outcomes are unclear, long-term follow-ups are necessary. This narrative review summarizes the available studies regarding cardiac involvement in MIS-C cases as well as clinical considerations for cardiac examination and follow-up.

This paper addresses the new type of backscatter communication based on motion-modulated chipless Radio Frequency IDentification (RFID) tags. To clearly explain the concept, the different methods of backscatter communication are classified from a system point of view based on the two fundamental properties of linearity and variation in time. The principle of classical chipless RFID technology, as a non-modulated backscatter method, and the motion-modulated chipless RFID are described with general mathematical demonstrations, while the performance of the two approaches is compared in terms of read range. Motion-modulated chipless RFID is presented as an effective backscatter communication method for identification and sensing of moving objects at large distances. Three different types of motion-induced modulation as phase (Doppler) modulation, polarization modulation, and directional modulation are addressed based on three specially designed moving resonant scatterers. The modulation process in each case is theoretically described, and the performance of the motion-modulated tag is experimentally verified in terms of identification capability and large read range.

Nowadays, quality management is an important field for organizations, including the banking system, which faces fierce competition. Organizational Excellence Models serve as guides to help organizations improve their quality. The main aim of this study was to investigate the dynamic relationships between the approaches and criteria of the EFQM organizational excellence model and optimize the simulation of these relationships. To do this, we first identified the common approaches in the organizational excellence model and clustered them using the fuzzy clustering method. Next, we used the systems dynamics method of causal rings to discover the dynamic relationships between these approaches. Finally, using data from the banking system in Iran, we simulated cases and optimized the growth rate of organizational excellence in the sample over 20 years (2018-2019).

Abstract The directional amplitude backscatter modulation with suppressed Doppler is demonstrated based on the scattering from a symmetrically rotating resonant loop. The concept is studied theoretically and experimentally with perfectly compatible results. The symmetrical rotation of the scatterer and the effect of radial resonance, as the two crucial points to realize the idea, are highlighted through the comparison between the symmetric and non-symmetric cases, and the results obtained for scatterers with and without radial resonance. The presented backscattering modulation technique provides an amplitude modulating waveform which is uniquely linked to the directional reradiation pattern of the rotating loop scatterer in a definite resonant mode. With the pure directional amplitude modulation (DAM) induced on the backscattered wave, the envelope waveform can be accurately retrieved form the received signal using the In-phase and Quadrature (IQ) representation. The contribution of the background in a real environment can be detected and removed to obtain the exact modulating waveform. This property of the proposed backscattering modulation method can be applied for sensing, localization, and identification purposes with high sensitivity, read range, and robustness.

The current study was conducted to evaluate the effects of a yeast cell wall product (YCWP) on the performance, the population of Lactobacillus and coliform bacteria and PGC-1α, TLR4, IL-10 and PPARγ genes expression in the intestine of broilers. Dietary treatments were as follows: basal diet (control), control diet + 0.3 g/kg colistin or 0.15, 0.3, 0.45 and 0.6 g/kg YCWP. Feed intake and feed conversion ratio were lower in the YCWP-fed birds compared to the control. Colistin and YCWP fed birds had higher Lactobacillus and lower coliform bacteria counts than control birds (p

Background: Pulmonary hypertension (PH) is a common finding in chronic kidney disease (CKD) and is associated with increased mortality and morbidity. The effect of kidney transplantation on PH is not yet well evaluated. This study aimed to compare pulmonary artery pressure (PAP) before and after kidney transplantation in CKD patients.Methods: This longitudinal study was conducted on 33 CKD patients who were candidates for kidney transplantation in a tertiary hospital in Mashhad, Iran. Pulmonary artery pressure and ejection fraction (EF) were assessed using trans-thoracic echocardiography (TTE). Demographic and clinical findings, including age, gender, and body mass index (BMI), as well as laboratory assessments, including hemoglobin, serum calcium and phosphorus level, and parathyroid hormone, were recorded before transplantation. TTE assessment was repeated one year after transplantation. Results: Mean age of the study patients (17 males and 16 females) was 30.42 ± 9.71 years. The majority of patients (85%) received hemodialysis before transplantation. Compared to before transplantation, PAP significantly decreased (from 33.67 ± 6.78 to 26.06 ± 5.78 mmHg, p

Background: Enuresis is a common problem that affects up to 15% of 5‐year‐old children and may have significant psychological, emotional, and social consequences for both the child and their family. Enuresis is the inability of toilet‐trained children to hold urine during the night. It can be classified as primary and secondary. Secondary enuresis occurs in children who have had a dry duration of more than six months; otherwise, it is called primary enuresis. Objectives: This narrative review aims to summarize the available data on the epidemiology, etiology, pathophysiology, evaluation, and treatment of children with enuresis. Methods: This study was conducted through a literature search with the keywords based on Medical Subject Headings (MESH) and were combined with other keywords, including enuresis, pediatric, incontinence, and treatment using PubMed, Embase, Web of Sciences, Scopus, and Cochrane databases. Results: The initial evaluation of enuresis needs a detailed medical history and a careful physical examination with no need for radiology and invasive procedures. The treatment’s mainstay is non‐pharmacological treatments, such as behavioral intervention followed by pharmacotherapy. The appropriate treatment chosen depends on the children's age, midnight voiding patterns, and family and child preferences. Conclusions: Enuresis is a common disorder that affects both the child and the family in many ways. Enuresis's etiology is complex, and it is still not well understood. The child and family must be included in the treatment process, and potential pathophysiological causes must be taken into account.

Introduction: Concerns over male infertility have grown, with over fifty percent of infertility cases linked to male causes. The impact of radiofrequency electromagnetic radiation (RF-EMR) of cellphones on the fragmentation of sperm has been investigated. This mini-review evaluates previous investigations on the relation between RF-EMR and sperm DNA fragmentation (SDF). Methods: A systematic review was carried out using Google Scholar and PubMed databases up to July 2020. MeSH terms related to DNA fragmentation, sperm, mobile phones, radiofrequency, and related synonyms identified relevant studies. Nine studies were selected, and their methodologies examined. Results: The studies reviewed presented diverse findings on the correlation between RF-EMR from cellphones and SDF. Out of nine investigations--five in vitro and four in vivo--all in vivo research works found significant DNA fragmentation in men who used their phones extensively, especially when carried in pants pockets. Three of the five in vitro tests showed a substantial effect, while the other two found no significant change between exposed and unexposed samples. Conclusion: Although more studies reported decreased sperm quality with prolonged and intense RF-EMR exposure, the evidence regarding DNA fragmentation remains inconclusive. Given how often cellphones are used, it is critical to further investigate their potential impact on male fertility and reproductive health. Existing evidence emphasizes the necessity of more studies in this field. [ABSTRACT FROM AUTHOR]

Complex spin textures in itinerant electron magnets hold promises for next-generation memory and information technology. The long-ranged and often frustrated electron-mediated spin interactions in these materials give rise to intriguing localized spin structures such as skyrmions. Yet, simulations of magnetization dynamics for such itinerant magnets are computationally difficult due to the need for repeated solutions to the electronic structure problems. We present a convolutional neural network (CNN) model to accurately and efficiently predict the electron-induced magnetic torques acting on local spins. Importantly, as the convolutional operations with a fixed kernel (receptive field) size naturally take advantage of the locality principle for many-electron systems, CNNs offer a scalable machine learning approach to spin dynamics. We apply our approach to enable large-scale dynamical simulations of skyrmion phases in itinerant spin systems. By incorporating the CNN model into Landau-Lifshitz-Gilbert dynamics, our simulations successfully reproduce the relaxation process of the skyrmion phase and stabilize a skyrmion lattice in larger systems. The CNN model also allows us to compute the effective receptive fields, thus providing a systematic and unbiased method for determining the locality of the original electron models.

Introduction[1] The provision of nutrients during the late gestation not only affects maternal status and reproductive performance, but also affects prenatal and postnatal litter growth and offspring’s health. Although trace elements are needed by the body in small amounts, they are essential nutrients for several metabolic functions such as growth, development, reproduction, and immunity. Copper (Cu) is often one of the most limiting trace elements for the fetus and neonate for normal development and copper play a major etiologic role in decrease of fetal growth and development. Deficiency of copper impairs fetal growth and causes serious consequences and can cause death. When intakes of Cu are deficient, maternal transfer of Cu to the fetus is insufficient for normal development, and abnormalities to the central nervous system, skeleton, and metabolism result. In ruminants, newborn animals are dependent on their dams for transferring nutrients via the placenta and mammary glands. To our knowledge, little information is available on the effects of maternal supplementation of copper via intra-ruminal administration of slow-release bolus at the late gestation on the copper status of ewes and their lambs. Because the slow-release ruminal bolus cannot be used in the newborn lambs until weaning, this study aimed to determine the effects of maternal supplementation of copper via intra-ruminal administration of slow-release boluses at the late gestation (8 weeks prepartum) on performance and some blood metabolites of ewes and their lambs until weaning.Materials and Methods To evaluate the performance and changes in some blood parameters of lambs born from ewes receiving slow-release boluses of copper, 80 Lori-Bakhtiari ewes (The fourth pregnancy with a body condition score 3 to 3.5) were divided into two groups of 40 heads each in a completely randomized design. Treatments were 1) control ewes and 2) ewes received slow-release boluses of copper, 60 days prepartum. After parturition, birth weight, sex, and the birth status of lambs (singlet or twin) were recorded. Lambs were weighed at birth and weaning at age 90 days. On the first day, days 10 and 60 postpartum, blood samples were taken from the ewes, while the blood samples of their lambs were taken at age 10 and 60 days. Serum samples were used to determine copper and ceruloplasmin concentrations in the ewes and their lambs, while iron, zinc, hematocrit, red blood and white cells, and hemoglobin were only determined in the born lambs.Results and Discussion The serum concentration of copper in the ewes were within the normal physiological range of 0.55 to 0.95 mg/l. Serum copper and ceruloplasmin concentrations of ewes at the first day of experiment were lower compared to days 10 and 60 postpartum (P0.05). Lambs born from ewes receiving slow-release bolus of copper had higher weaning weight, average daily weight gain, copper and ceruloplasmin concentrations, hematocrit percentage, red and white blood cell counts, and hemoglobin concentration compared to those born from control ewes (P

BackgroundSteroid resistant nephrotic syndrome (SRNS) represents a significant renal disease burden in childhood and adolescence. In contrast to steroid sensitive nephrotic syndrome (SSNS), renal outcomes are significantly poorer in SRNS. Over the past decade, extensive genetic heterogeneity has become evident while disease-causing variants are still only identified in 30% of cases in previously reported studies with proportion and type of variants identified differing depending on the age of onset and ethnical background of probands. A genetic diagnosis however can have implications regarding clinical management, including kidney transplantation, extrarenal disease manifestations, and, in some cases, even causal therapy. Genetic diagnostics therefore play an important role for the clinical care of SRNS affected individuals.Methodology and resultsHere, we performed NPHS2 Sanger sequencing and subsequent exome sequencing in 30 consanguineous Iranian families with a child affected by SRNS with a mean age of onset of 16 months. We identified disease-causing variants and one variant of uncertain significance in 22 families (73%), including variants in NPHS1 (30%), followed by NPHS2 (20%), WT1 (7%) as well as in NUP205, COQ6, ARHGDIA, SGPL1, and NPHP1 in single cases. Eight of these variants have not previously been reported as disease-causing, including four NPHS1 variants and one variant in NPHS2, ARHGDIA, SGPL1, and NPHP1 each.ConclusionIn line with previous studies in non-Iranian subjects, we most frequently identified disease-causing variants in NPHS1 and NPHS2. While Sanger sequencing of NPHS2 can be considered as first diagnostic step in non-congenital cases, the genetic heterogeneity underlying SRNS renders next-generation sequencing based diagnostics as the most efficient genetic screening method. In accordance with the mainly autosomal recessive inheritance pattern, diagnostic yield can be significantly higher in consanguineous than in outbred populations.