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The large current generated by a direct start of the super-large-capacity high-voltage induction motor would have a huge impact on the power grid as well as the motor itself. Traditional soft starters have the shortcomings of discontinuous adjustment, voltage sags, sudden torque mutation, secondary current impact and high cost. To resolve this issue, the authors propose a novel coupled autotransformer and magnetic-control (CATMC) soft-start method. The structure of the new CATMC soft starter combines the functions of the autotransformer and magnetic control reactor via an innovative electric and magnetic circuit design. In this study, the authors analyse the magnetic circuit structure and working principles of the CATMC soft starter. Then, to validate its principle and performance, the authors conduct a simulation study using ANSYS software and design and test an 18 MW/10 kV CATMC soft starter prototype. The simulation results demonstrate that the CATMC soft starter effectively avoids secondary current impact and constrains the motor starting current to

A 7-week-old girl with a normal birth history suddenly developed respiratory distress while feeding. Cardiopulmonary resuscitation was initiated at home after she had a cardiac arrest and was continued in the emergency room but all efforts at resuscitation proved unsuccessful and she died 2 h after presentation. Investigations performed in the emergency room revealed that she had a significantly high white blood cell count and severe anaemia. The cause of death was identified as KMT2A-rearranged infantile acute lymphoblastic leukaemia based on cytogenetic tests. She had no abnormalities at the 4-week check-up; however, she developed a skin nodule on her abdomen thereafter, and the family did not consult a doctor for fear of contracting COVID-19. Early detection and diagnosis could have changed the prognosis of the patient. The present case highlights the negative impact of the reduction of outpatient consultations during the COVID-19 pandemic.

Mutations in the mitochondrial tRNAMet gene have been reported in only five patients to date, all of whom presented with muscle weakness and exercise intolerance as signs of myopathy. We herein report the case of a 12-year-old girl with focal epilepsy since the age of eight years. At age 11, the patient developed sudden visual disturbances and headaches accompanied by recurrent, stroke-like episodes with lactic acidosis (pH 7.279, lactic acid 11.6 mmol/L). The patient frequently developed a delirious state, exhibited regression of intellectual ability. Brain magnetic resonance imaging revealed high-intensity signals on T2-weighted images of the left occipital lobe. Mitochondrial gene analysis revealed a heteroplasmic m.4450G > A mutation in the mitochondrial tRNAMet. The heteroplasmic rate of the m.4450G > A mutation in blood, skin, urinary sediment, hair, saliva, and nail samples were 20, 38, 59, 41, 27, and 35%, respectively. The patient’s fibroblast showed an approximately 53% reduction in the oxygen consumption rate, compared to a control, and decreased complex I and IV activities. Stroke-like episodes, lactic acidosis, encephalopathy with brain magnetic resonance imaging findings, and declined mitochondrial function were consistent with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. To our knowledge, the findings associated with this first patient with MELAS syndrome harboring the m.4450G > A mutation in mitochondrial tRNAMet expand the phenotypic spectrum of tRNAMet gene.

To reduce the iron loss of a three-phase reactor composed of grain-oriented (GO) silicon steel plates, we proposed two reactor models. One applies oblique joints between the legs and the yokes, and the other applies butt joints composed of non-oriented (NO) silicon steel plates at the L and T joint parts. The proposed models are aimed to relax the flux concentration inside the iron cores that occurs due to the anisotropic magnetic characteristics of GO silicon steel plates. The effectiveness of the proposed models are investigated through magnetic field and iron loss analyses considering the anisotropic magnetic characteristics of the iron cores. It is found that the flux concentration in the yoke can be relaxed in both proposed models. And iron loss of the proposed model with NO steel plates at the L- and T-shaped joints can be reduced about 12% compared with the ordinary one.

Local high iron loss may occur due to the deviation of magnetic flux from the rolling direction of electrical steel in the T-joint area of the transformer core. In this paper, to reduce the iron core loss in the T-joint region, a hybrid iron core model using nonoriented and grain-oriented magnetic materials is proposed. Three-dimensional magnetic field analysis considering the nonlinearity and laminated structure of the original and proposed iron core models is carried out by using the finite element analysis. The flux and iron loss distributions of the original and the hybrid cores are compared. The results show that approximately 2% iron loss reduction can be achieved by using the proposed hybrid structure.

BackgroundCongenital central hypoventilation syndrome (CCHS) is a rare disease characterized by sleep apnea. Anoxia often occurs soon after birth, and it is important to prevent anoxia-mediated central nervous system complications; however, data on the relationship between respiratory management and the prognosis for intellectual development of patients with CCHS is not well yet investigate.MethodsWe performed a retrospective chart review cohort study of patients with CCHS in Japan. We investigated the risk and prognostic factors for developmental outcomes and examined the disease in terms of its symptoms, diagnosis, complications, and treatment.ResultsOf the 123 patients with CCHS included in the survey, 88 patients were 6 years old and older. They were divided into two group based on their intelligence quotient. Those treated using positive-pressure ventilation via tracheostomy in the first three months of life had a better developmental prognosis than those managed via tracheostomy after three months of age and those treated by ventilation using mask (OR = 3.80; 95% CI: 1.00–14.37, OR = 4.65; 95% CI: 1.11–19.37). There was no significant difference in physical development (P = 0.64).ConclusionsThe best respiratory treatment for patients with CCHS is ventilation via tracheostomy, initiated ideally before the age of three months.

Background: Congenital central hypoventilation syndrome (CCHS) is a rare disease characterized by sleep apnea. Anoxia often occurs soon after birth, and it is important to prevent anoxia-mediated central nervous system complications; however, data on the relationship between respiratory management and the prognosis for intellectual development of patients with CCHS is scarce. Methods: We performed a retrospective chart review cohort study of patients with CCHS in Japan. We investigated the risk and prognostic factors for developmental outcomes and examined the disease in terms of its symptoms, diagnosis, complications, and treatment. Results: Of the 123 patients with CCHS included in the survey, 88 patients were 6 years old and older. They were divided into two group based on their IQ. Those treated using positive-pressure ventilation via tracheostomy in the first three months of life had a better developmental prognosis than those managed via tracheostomy after three months of age and those treated by ventilation using mask (OR = 3.80; 95% CI: 1.00–14.37, OR = 4.65; 95% CI: 1.11–19.37). There was no significant difference in physical development (P = 0.64). Conclusions: The best respiratory treatment for patients with CCHS is ventilation via tracheostomy, initiated ideally before the age of three months.

Ring chromosome 20 syndrome is an epileptic and neurodevelopmental encephalopathy that occurs in children, characterised by a triad of refractory frontal lobe seizures, recurrent non-convulsive status epilepticus and frontal lobe-dominant paroxysmal discharges. However, details of other clinical features associated with ring chromosome 20 syndrome remain unknown. Here, we report two patients with ring chromosome 20 syndrome who had praxis-induced reflex seizures. Case 1 was an 11-year-old girl who presented with seizures triggered by specific activities such as mental and written calculations, writing, decision-making, recall, sudden changes in routine or ambient temperature and bathing. During calculations, left frontal lobe-dominant, 3-Hz slow-wave bursts were observed on EEG. Lacosamide effectively suppressed her tonic seizures. Case 2 was a six-year-old boy who presented with seizures triggered by specific activities such as calculations, recall and bathing. During calculations, frontal lobe-dominant, 3-Hz spike and slow-wave bursts were observed on EEG. Although his epilepsy was refractory, gabapentin reduced the frequency of focal seizures. In both cases, the hyperexcitability in the frontal lobe may have spread to the motor cortex and precipitated praxis-induced seizures. Therefore, in addition to the known characteristic triad, praxis-induced reflex seizures may also be a feature of ring chromosome 20 syndrome.

To investigate the noise reduction of an electrical machine, the deformation and vibration of the cores are evaluated by using the coupled magnetic and mechanical analyses [1]. In these analyses, the local force calculated by using the flux distribution obtained from the magnetic field analysis is required in the mechanical analysis. In the local force calculation [2], the Maxwell stress tensor is usually used. The Maxwell stress tensors are often represented by the Minkowski and Chu models with linear and nonlinear energy expressions, respectively. In the nonlinear magnetic field analysis, the volume forces, which move the magnetic bodies, obtained from both models coincide with each other, whereas their local forces, which deform the magnetic bodies, are different. Therefore, more investigation is required to clarify the suitable expression of the Maxwell stress tensor. In this paper, to clarify the proper expression of the Maxwell stress tensor for the local force calculation of a nonlinear magnetic body, the Maxwell stress tensors are derived from the Fleming’s left hand rule because the volume current density can be used to determine the magnetization in the nonlinear magnetic body. As a result, a new Maxwell stress tensor is derived. The surface force obtained from the new Maxwell stress tensor is compared with those obtained from the ordinary Minkowski and Chu models, and the equivalent magnetizing current method [3] to show the effectiveness and validity of the proposed Maxwell stress tensor.

In this paper, an excess loss modeling method of the laminated iron core is proposed. The 1-D single sheet silicon steel plate analysis is combined with the 3-D solid-core analysis to model the laminated structure. The modeling is improved by including the domain wall bowing due to pinning effect, which is considered as the main cause of excess loss. The domain wall bowing due to pinning is considered in the 1-D steel plate analysis because the excess loss generated by the flux density in the parallel direction of the stack is dominant compared with that in the perpendicular direction. In the 1-D analysis, an initial bowing flux distribution along the thickness direction of one lamination is introduced. The results of iron losses show that the hysteresis and eddy current losses vary with the flux and eddy current distribution in the laminated iron cores. Finally, the 1-D–3-D combined method is applied to a single-phase reactor; the magnetic flux and eddy current distribution are analyzed in detail. The eddy currents generated by the parallel flux are increased, hence, the skin effect of flux in the parallel direction of laminations is enhanced; thus, the hysteresis and eddy current losses are increased and the iron loss is increased by 8%.

A novel direct current system fault current limiter (DCSFCL) based on the principle of nonlinear magnetization was proposed to limit fast rising fault current of the dc system. Different materials were also tested to find the iron core material. However, the existing material is not able to obtain both two necessary indexes, namely, a large saturation point and fast demagnetization speed. Hence, the performance is not satisfactory using a single material. In order to obtain the best performance for the DCSFCL, mixed materials were applied to combine the advantages of different soft magnetic materials. Furthermore, magnetic valves were also added to the original DCSFCL topology, which is an effective method to optimize the clipping performance and reduce the usage of materials. The 2-D models were built and finite-element analyses were carried out to verify these assumptions. Compared with a traditional smoothing reactor, the proposed new model not only limits the fault current by 55.4%, but also reduces the usage of both magnetic material and permanent magnets by over 20%.

Soft magnetic composite (SMC) is composed of ferromagnetic particles surrounded by electrical insulation. SMC has advantages such as isotropic magnetic characteristics, low eddy current loss, and easy to be manufactured into complex shapes compared with electrical steel sheets. In this paper, to establish the homogenization technique for the magnetic field analysis of an electrical machine using SMC, a method to evaluate the effective permeability and iron loss taking account of nonlinear magnetic characteristics and eddy currents is proposed by using a cell model composed of one particle of SMC. Then, the effects of the frequency and particle size on the effective permeability and iron loss are investigated numerically. Finally, the effective permeability and iron losses of an actual SMC are calculated, and they are compared with measured results to verify the proposed method. It is shown that theoretically proper results are obtained by using the proposed method. The calculated iron losses coincide well with the measured ones below 10 kHz, whereas the calculated effective permeability is smaller than the measured one due to different effective gap lengths.

Short-chain enoyl-CoA hydratase (ECHS1) is involved in amino acid and fatty acid catabolism in mitochondria and its deficiency causes Leigh syndrome or exercise-induced dystonia. More than 60 patients with this condition have been reported till date. The accumulation of intermediate metabolites of valine is assumed to be responsible for the cytotoxicity. Since protein restriction, including valine reportedly improves neurological symptoms, it is essential to consider the possible incidence of and diagnose ECHS1 syndrome in the earlier stages. This study reported the liquid chromatography with tandem mass spectrometry (LC-MS/MS) urine and plasma metabolite analysis in six cases, including four new cases with ECHS1 deficiency. The values of urine cysteine/cysteamine conjugates from valine metabolites, S-(2-carboxypropyl) cysteine/cysteamine from methacrylyl-CoA, and S-(2-carboxyethyl) cysteine/cysteamine from acryloyl-CoA were separated between six patients and six normal controls. The LC-MS/MS analysis revealed that these metabolites can be used for the early diagnosis and evaluation of diet therapy.

It is necessary for companies supplying reclaimed oil to analyze polychlorinated biphenyls (PCBs), because there is a possibility of the presence of contaminants due to trace-level PCBs in the reclaimed oil. However, common analysis methods of PCBs are time-consuming and complicated. Fast-GC triple stage quadrupole mass spectrometer with the 13-component quantitation method is an official method for analyzing PCBs in insulating oil in Japan. This method is extremely fast and simplified. The purpose of this study involves an investigation of the aforementioned fast and simple method for potential use in the analysis of reclaimed oil. Furthermore, it was attempted to combine the method with sample preparation involving only hexane dilution. The effect of sample dilutions corresponding to 100, 300, and 500 times was evaluated for reducing the matrix effect. The matrix effect was suppressed at a dilution ratio equal to or exceeding 300 times. Calibration curves of four points, namely 0.01, 0.05, 0.1, and 0.5 ng/mL, (ignored origin) by using an internal standard method were prepared for the 13 components. The square of regression coefficient (R2) values of all calibration curves exceeded 0.997. This method was adopted for the analysis of reclaimed oil containing 0.5 μg/mL PCBs, which corresponds to the judgment criteria, and accurate quantitation (accuracy value, 94.0–102%) and good repeatability (%RSD, 3.6%) were obtained. Furthermore, the required sensitivity was maintained even when 800 samples were analyzed without a cleaning ion source and an exchanging analysis column.

The saturated-core fault current limiter (SFCL) is an effective device for limiting fault current. This paper proposes and analyzes a novel bridge-type SFCL (BSFCL) that uses a permanent magnet (PM) to reduce dc bias while improving the stability of the PM. The bridge structure in the BSFCL consists of four coils and a fault-limiting reactor which is used to limit fault current. The ac and the dc share coils to help reduce energy loss. The iron core uses air-gap branches to offer paths for ac flux, which reduces eddy current in the PMs and enhances their stability. The operating principle and characteristics of the BSFCL were analyzed using the electromagnetic coupling method. To investigate the effects of parameters on the performance of the BSFCL, various finite-element analysis simulations were performed by Maxwell. Experimental results of a 220 V prototype verify the effectiveness of the BSFCL.

The permanent-magnet-biasing saturated core fault current limiter (PMFCL) is one of the most promising devices to limit the fault current. To improve the biasing ability of permanent-magnets (PMs) and reduce the cost of PMFCL, this paper proposes a novel compact permanent-magnet-biased fault current limiter (CPMFCL). The three-limb structure can reduce the size and cost; the optimal small-section structure can improve the biasing ability of PMs and reduce the usage amount of PMs. First, the principle, equivalent magnetic circuit, and small-section optimized structure are introduced. To validate the principle and performance of CPMFCL, various finite element analysis simulation and optimization study are performed in Maxwell-3D. A 220 V/10 A CPMFCL prototype is designed and tested. The simulation and experimental results demonstrate that, compared with traditional PMFCLs, the CPMFCL has the advantages of smaller size, better biasing capacity of PMs, lower possibility of demagnetization, and excellent fault clipping performance.

The application of fault current limiter is an effective method to limit fault current. The saturated-core fault current limiter (SFCL) is one of the promising devices. However, its commercialization is limited by the high demands for the ferromagnetic material and the dc source generating magneto motive force. This paper proposes a novel three-phase compact SFCL (CSFCL) in which a common core and a permanent magnet are used to solve the mentioned challenges. The common core can significantly reduce the amount of required ferromagnetic material. The basic operating principle of CSFCL is introduced based on the analysis of the CSFCL’s magnetic circuit and equivalent electrical circuit. Finite-element analysis (FEA) simulations were performed by ANSOFT to validate the effectiveness of the CSFCL’s fault clipping performance.

Herein we report the case of a 6-year-old girl with autism spectrum disorder (ASD) and weakness in the distal portion of the right upper limb. Although difficult to perform, nerve conduction studies indicated demyelinating neuropathy. Magnetic resonance imaging (MRI) showed swelling a nd high-intensity signals in the right brachial plexus and cervical spinal roots. The symptoms recovered after a single course of i.v. immunoglobulin. Electrophysiological indices and MRI findings also improved after treatment. This case demonstrates the utility of neuroimaging in addition to electrophysiological assessments for the diagnosis of demyelinating neuropathy, particularly in young patients with ASD.

When a magnetically shielded room (MSR) to shield leakage fluxes of electric machines is designed by using magnetic field analysis, it is difficult to represent the leakage magnetic field of an electric machine accurately. In this paper, a method of modeling it using a simple solid model with multiple magnetization vectors is proposed. In this method, the magnetization vectors are determined by an inverse technique to represent a real leakage flux distribution. First, the proposed method is explained and verified using a simple 2-D linear magnetostatic one-phase transformer model. Then, the method is applied to a 3-D model and the effect of selected measured surface on representation of leakage flux is investigated. Finally, an experimental verification is carried out. It is shown that the proposed method can represent the leakage magnetic field with small number of magnetizations. The leakage flux only in the region behind the measured surface can be represented, and it is verified by experiment. Moreover, the shielding performance of MSR can be evaluated by using the proposed solid model with suitable permeability.

The saturated core fault current limiter (SFCL) is one of the most effective methods for limiting fault current. A permanent magnet (PM)-biased SFCL (PFCL) can reduce dc bias current and additional energy consumption. However, the thermal stability of NdFeB PMs in PFCL poses a major challenge. This paper proposes a novel hybrid-type SFCL (HSFCL) topology that can reduce this risk. In the HSFCL, a hybrid PM/dc coil is designed to saturate the core, and a fault-limiting reactor is used to guarantee fault-clipping performance. The new structure provides air-gap branches for the time-varying magnetic field produced by alternating current, which reduces the eddy current in the PMs and enhances their stability. Electromagnetic coupling is used to analyze the operating principles and characteristics of HSFCL. Finite-element analysis simulations were performed using Maxwell and compared with verification experiments, proving that HSFCL is efficient in fault clipping and PM reliability enhancement.

Eddy-current loss of NdFeB permanent magnets (PMs) is widely analyzed in PM motors. This paper proposes a coupled 2-D transient finite-element analysis (FEA) of the saturated core fault current limiter (SCFCL) and 3-D analytical model of PMs to evaluate eddy-current loss in SCFCL. The comparison between the result from coupled method and that from 3-D FEA verifies the applicability of the coupled method in analyzing eddy current loss of PMs in SCFCL. The 3-D analytical model of PM is applied to analyze the effect of segmentation on the reduction of PM's eddy-current loss. And the results show that segmentation along width has a better performance, which is also verified by 3-D FEA.

Introduction Levetiracetam has a high tolerability and is effective against various seizure types and epilepsy syndromes. However, no study has specifically evaluated the efficacy of levetiracetam in children with refractory epilepsy based on magnetic resonance imaging (MRI) findings and the presence of intellectual disability (ID). Methods We retrospectively evaluated levetiracetam efficacy and safety in 49 pediatric patients who met the following inclusion criteria: (1) diagnosis of refractory epilepsy with first-line antiepileptic (AED) treatment ⩾2 years, (2) younger than 20 years old, and (3) received oral levetiracetam treatment for ⩾6 months. We assessed the relationships of these outcomes with MRI findings and ID status. Results Eighteen (37%) patients achieved a ⩾50% reduction in seizure frequency, and the majority (78%) had no remarkable side effects. Twenty-two (45%) patients had previously been treated with more than seven antiepileptic drugs prior to levetiracetam. Among 18 patients who achieved a ⩾50% reduction in seizure frequency, 13 and 5 had negative and positive MRI findings, and 9 and 9 had and did not have ID, respectively. Conclusions Our findings suggest that even for intractable pediatric cases with symptomatic etiology (i.e., MRI lesion and ID), levetiracetam has favorable efficacy for refractory epilepsy with tolerable adverse effects.

Introduction Neuronal ceroid lipofuscinoses (NCLs; CLN) are mainly autosomal recessive neurodegenerative disorders characterized by the accumulation of autofluorescent lipopigments in neuronal and other cells. Symptoms include visual disabilities, motor decline, and epilepsy. Causative genes are CLN1, CLN2, CLN3, CLN5, CLN6, CLN7, CLN8, CLN10, CLN11, CLN12, CLN13, and CLN14. We present the fourth Japanese case with a CLN6 mutation. Case presentation At 3 years of age, our patient became clumsy and fell down easily. He developed focal seizures with impaired consciousness and was started on carbamazepine. He showed ataxic walking and dysarthria with increased deep tendon reflexes. Interictal electroencephalogram revealed slow waves in the left temporal and occipital areas. Brain magnetic resonance imaging showed cerebellar atrophy and ventriculomegaly. In optical coherence tomography (OCT), the inner layer of the retina was thick and highly reflective. Exome sequencing revealed a known homozygous mutation, C.794_976del, p. (Ser265del) in CLN6. Discussion A total of 130 cases of NCL with CLN6 mutations have been reported globally, of which only four were from Japan including the current patient. The deletion of serine at position 265 has been reported in six cases. Ser265 is located in a region of short repeated sequences that is susceptible to mutation. Clinical trials of gene therapy using adeno-associated virus serotype 9 have started for NCL6, making early diagnosis crucial. OCT examination might be helpful in achieving a diagnosis.

Rescue robots, which are robots that have been designed for the purpose of rescuing people, are useful in natural and man-made disasters. In this paper, we develop a remote controlled rescue robot using ZigBee communication. The main body of the rescue robot is a caterpillar with two arm crawlers. Two arm crawlers are used for running over obstacles. Users can control the rescue robot forward/backward and right/left with a remote controller through ZigBee communication. Also it is possible to observe the situation of a disaster area by a Wi-Fi camera on PC. The Wi-Fi camera is rotated right/left with the remote controller. The effective distance between the rescue robot and the remote controller is 120 m outdoors.

In this paper, we present a dual lock system using radio-frequency identification (RFID) and fingerprint recognition. It is a secure and reliable locking system which can be implemented in home, offices, school and in any kind of organization. Even if the users lost the RFID card, they can unlatch the lock with their fingerprints. Only the registered users are able to access using their fingerprints and the RFID tags. If any unauthorized persons try to have an access, first of all short message service (SMS) will send a text message to an owner using global system for mobile communications (GSM), and at the same time a TTL camera will take a snap of that person and store it in a SD card for reference of the owner. The core of the system is the Arduino Mega 2560 which is interfaced with a fingerprint scanner module, a RFID card reader module, the TTL camera, an SD card module, a GSM shield, a liquid crystal display (LCD) and an electric door strike.

Design education is important at technical universities and colleges. In general, real product design requires collaborative work. In this chapter, the authors discuss collaborative design education. An A360 cloud platform on Autodesk's 3D computer-aided design “AutoCAD” is adopted to illustrate a collaborative design activity implemented in the Engineering Graphics class offered at the College of Science and Technology, Royal University of Bhutan. By using A360 cloud, students can share a 3D model with group members. Based on feedback received, students can modify the initial model, share it, print, and discuss the modified object with members. This collaborative work allows students to create enhanced 3D design objects while engaged in discussions and interactions. The authors also discuss some difficulties encountered during the collaborative process and offer recommendations and future research ideas.

Compared with grain-oriented SiFe sheets, the domain structure of nonoriented (NO) SiFe sheets is very different, and the magnetization processes of NO SiFe sheets are much less investigated. Thus, the excess loss of NO SiFe sheets is less well-known. In this paper, we propose a numerical method to estimate the excess loss in NO SiFe sheets, considering the pinning and skin effects. We apply an initial magnetic field distribution that reflects the pinning effect in our nonlinear eddy current finite element analysis model, which reflects the skin effect of the magnetic flux. Thus, the pinning effect can be coupled with the skin effect in the proposed numerical method. The hysteresis loss and eddy current loss calculated from the flux and eddy current distributions are directly obtained from the proposed analysis method. Thus, excess loss is included in both the hysteresis and the eddy current loss calculations. We first apply the proposed method to two different grades of NO SiFe sheets. The calculated iron losses in one sheet are compared at various frequencies with the measured losses from catalogue data, and less than 2% deviation can be achieved. Finally, we apply the proposed method to the loss calculation for the iron core of a reactor.

Joubert syndrome (JS) is rare recessive disorders characterized by the combination of hypoplasia/aplasia of the cerebellar vermis, thickened and elongated superior cerebellar peduncles, and a deep interpeduncular fossa which is defined by neuroimaging and is termed the 'molar tooth sign'. JS is genetically highly heterogeneous, with at least 29 disease genes being involved. To further understand the genetic causes of JS, we performed whole-exome sequencing in 24 newly recruited JS families. Together with six previously reported families, we identified causative mutations in 25 out of 30 (24 + 6) families (83.3%). We identified eight mutated genes in 27 (21 + 6) Japanese families, TMEM67 (7/27, 25.9%) and CEP290 (6/27, 22.2%) were the most commonly mutated. Interestingly, 9 of 12 CEP290 disease alleles were c.6012-12T>A (75.0%), an allele that has not been reported in non-Japanese populations. Therefore c.6012-12T>A is a common allele in the Japanese population. Importantly, one Japanese and one Omani families carried compound biallelic mutations in two distinct genes (TMEM67/RPGRIP1L and TMEM138/BBS1, respectively). BBS1 is the causative gene in Bardet-Biedl syndrome. These concomitant mutations led to severe and/or complex clinical features in the patients, suggesting combined effects of different mutant genes.

A method of 3-D finite element magnetomechanical analysis is developed to investigate the effect of magnetostriction (MS) and stress on the core loss and vibration of motors. Shrink-fit stress calculation is carried out using static structural analysis and equivalent thermal force calculated by a novel method using the thermal stress tensor. A method of core loss calculation is proposed to take account of the 3-D stress in the core loss calculation. The methods are applied to the core loss and the vibration analysis of a permanent magnet motor. Numerical results show that the stator core loss increases significantly due to the shrink-fit stress. However, the radial velocity of the housing surface slightly decreases due to the MS and further decreases due to the shrink-fit stress because the MS increases under compressive stress.

Mutations in the mitochondrial tRNA

With the rapid development of the power system, the fault current becomes a big problem. The current limiting measures become more and more important. The saturated-core fault current limiter(SFCL) is one of the promising methods [1]. The most serious fault in electric power system is three-phase short-circuit fault, and the fault current is more likely to exceed the circuit breaker's capacity. The use of three single-phase current limiters at the same time will need a lot of ferromagnetic material and space. A compact three-phase SFCL was proposed in [2], but it cannot limit three-phase fault current [2]. So we propose a novel structure of Economical Three-phase Saturated-core Fault Current limiter(ETSFCL). The novel structure has good clipping performance at three-phase fault conditions. Compared to three single-phase SFCLs proposed in [3], it saves a lot of ferromagnetic material and reduces the AC flux through the permanent magnet. The stability of the permanent magnet is greatly improved and the risk of demagnetization is reduced. In this paper, the operating principle of the proposed structure is explained by analyzing its equivalent magnetic circuit and electrical circuit. FEA models were established in ANSOFT to verify the its effectiveness.

In order to protect high voltage direct current(HVDC) system from destructive damage caused by large DC fault current, a HVDC system saturable core type fault current limiter(DCSFCL) is proposed previously. However, through principle investigation and simulation analysis of DCSFCL, coil inductance of DCSFCL do not have a large changing rate (CR) when a fault occurs, so clipping performance of it will not be quite satisfying. In order to increase CR and improve clipping performance of original DCSFCL, further improvements need to be carried out. In this digest, a modified three limb structure of DCSFCL(TSFCL) is proposed and equivalent magnetic and electric circuit are analyzed. Simulations carried out on ANSYS shows that clipping performance is improved by a large scale.

The start current can reach up to as high as 4–7 times of the motor's rated current when high voltage motors start directly. For super large capacity motors (voltage above 6 kV, power above 10000 kW), the direct start current could reach 5000 Ampere or more [1]. Such large start current will not only have a huge impact on the motor itself but also cause a sudden decline of the grid voltage [2], [3]. In order to resolve this issue, a novel coupled auto-transformer and magnetic control (CATMC) soft starter method was proposed in this paper. The new structure of CATMC soft starter combines the two functions of the auto-transformer and the magnetic control reactor (MCR) through ingenious electric and magnetic circuit design. Hence the start current is reduced in two ways and the structure becomes more compact and flexible.

For future high voltage direct current (HVDC) grids, the dc protection system is often considered as one of th e main challenges due to the difficulty in interrupting a large dc current and the stringent time constraint imposed by the fast rising fault current[']. Therefore, a novel DC system fault current limiter based on saturable core reactor theory (DCSFCL) was proposed previously. In order to obtain better performance of DCSFCL that is unable to be accomplished by single magnetic material, mixed magnetic materials were applied to take advantages of different magnetic materials. Furthermore, magnetic valves were also added to original DCSFCL topology, which is a good way to optimize clipping performance and reduce the usage of both magnetic materials and permanent magnets (PM). ANSYS models were built and finite element analysis were carried out to verify the feasibility of these deductions.

Grain-oriented (GO) materials are widely used in the iron cores of power transformers and reactors, as they provide a high permeable path within low iron losses. However, magnetic properties of GO materials exhibit strong anisotropy, and this intensifies the flux concentration inside the iron core. Furthermore, in a three-phase transformer, rotational magnetic field is generated at the T-joint part. Therefore, the magnetic flux path is not parallel to the rolling direction of the steel plate in the T-joint area, thus results in higher iron loss at the T-joint area. To overcome the foregoing problems, we proposed two kinds of hybrid iron core models applying different grades of silicon steel plates and their effectiveness is investigated by using magnetic field analysis and iron loss analysis with finite element method.

To reduce the iron loss of a three-phase reactor composed of Grain-oriented silicon steel plates, we proposed two reactor models: applying oblique joints between the legs and yokes, and applying isotropic brick iron cores at the L and T shaped joints. The effectiveness of the proposed models are investigated through magnetic field and iron loss analyses considering the anisotropic magnetic characteristics. It is found that the iron loss of the proposed model with isotropic butt joints can be about 12 % reduced compared with the ordinary one.

The rapid progress in and the expanding complexity of information and technology systems have made data analysis increasingly relevant. Data having a variety of elements are complex, and making very difficult to evaluate a state of a model from observed data generated probabilistically by the model. To evaluate these hidden states, we propose Spherical-Self Organizing Map (S-SOM) with a Hidden Markov Model (HMM) that infers such hidden states.