Intermediate Segments Research Articles

Characterization and pathogenicity of a naturally reassortant and recombinant infectious bursal disease virus in China.

Infectious bursal disease virus (IBDV), an Avibirnavirus, is the pathogen of infectious bursal disease, which is a severely immunosuppressive disease in 3-15-week-old chickens. Different phenotypes of IBDV, including classical, variant, very virulent (vv) and attenuated IBDV, have been reported in many chicken-rearing countries worldwide. Here, we isolated and identified a naturally reassortant and recombinant IBDV (designated GXB02) from 20-day-old chickens with clinicopathological changes of infectious bursal disease (IBD) in Guangxi Province, China. Whole genomic sequencing showed that the strain GXB02 simultaneously has both reassortant and recombinant characteristics with segments A and B being derived from recombinant intermediate vaccine strain and classic strains of IBDV. Segment A of strain GXB02 was incorporated into the skeleton of an intermediate IBDV vaccine strain (W2512), where the breakpoints of two recombinant events located at nucleotide positions 1468 and 1648 were replaced by reassortant vvIBDV (PK2) and vvIBDV (D6948) of segment A, respectively. We used this GXB02 strain to inoculate 21-day-old specific-pathogen-free chickens to evaluate its pathogenicity. Strain GXB02 has clinicopathologic characteristics of IBD with severe bursal lesions, as evidenced by necrosis, depletion of lymphocytes, and follicle atrophy, indicating that reassortment with classical strains in segment B or/and recombination with very virulent strains increased pathogenicity of the strain GXB02 in chickens. These findings provide important insights into the genetic exchange between classic and attenuated strains of IBDV with two recombinant events occurring at the intermediate derivative segment A with vvIBDV strains, thereby increasing the difficulty of prevention and control of IBD due to novel reassortant-recombinant strains.

122. Does vertebral body tethering cause disc and facet joint degeneration? An MRI study with minimum two years of follow-up

<h3>BACKGROUND CONTEXT</h3> Disc health after various growth modulation techniques have been assessed in animal models, and tethering was claimed to prevent degeneration due to its less rigid nature compared to other growth-friendly techniques. Yet, the results of animal studies wherein tethering is applied on healthy spines to create a deformity cannot be extrapolated to humans, in which tethering is used for deformity correction. As biomechanical properties are different, effects of vertebral body tethering (VBT) surgery on discs and facet joints in clinical series are not precisely acknowledged. There are concerns that VBT surgery may cause degeneration at both intermediate and adjacent levels. <h3>PURPOSE</h3> Our aim was to investigate the integrity of discs and facet joints that are subject to compressive forces and relative stabilization during growth modulation. <h3>STUDY DESIGN/SETTING</h3> Retrospective analysis of a prospectively collected data. <h3>PATIENT SAMPLE</h3> This study included 25 (23F, 2M) consecutive adolescent idiopathic scoliosis (AIS) patients with a mean follow-up of 29 (24-62) months who underwent thoracoscopic VBT surgery between 2014 to 2017 were included. <h3>OUTCOME MEASURES</h3> Disc and facet joint degeneration scores on MRI. <h3>Methods</h3> Demographic, perioperative and radiographic data were collected. Overcorrection, mechanical and pulmonary complications, and reoperations were recorded. MRIs taken before surgery and at a minimum of 2 years of follow-up were evaluated for degeneration at the intermediate and adjacent segment intervertebral discs and facet joints by a blinded senior radiologist and compared. Discs were assessed using Pfirrmann grade. Facet joint degeneration was graded on a scale of 0-3. Changes from preop to follow-up MRIs were analyzed using McNemar's Test and Related Samples Marginal Homogeneity Test. <h3>Results</h3> The mean age at surgery was 12.2 (10–14) years and median Sanders stage was 3 (1-7). A mean of 7.7±1.1 (6-11) levels were tethered. Preoperative mean height of 155.3 (130-178) cm was increased to 163.4 (149-187) cm at latest follow-up. The mean preoperative MT curve magnitude of 46°±7.7° was corrected to 23.3°±5.9° at first erect, which was modulated to 12°±11.5° during follow-up. There was 1 (4%) broken tether, 5 (20%) overcorrected curves, 3 (12%) implant-related complications and 1 (4%) reoperation for a thoracoscopic tether release before the follow-up MRI was obtained. A total of 217 levels of discs and bilateral facet joints, were evaluated in the preoperative and follow-up MRI images. Analyses of disc and facet joint scores revealed no significant differences between patients. One case (4%) with an increase in disc scores already had multilevel moderate degeneration preoperatively, while another case (4%) displayed a single-level new-onset grade-2 bilateral facet degeneration. <h3>Conclusions</h3> This study reports MRI findings of 25 rapid and steady growing adolescents (Sanders 2-7) who had undergone thoracoscopic VBT surgery. At an average of 29 months (24-62) after growth modulation with VBT surgery, radiographic degenerative findings were not present in 97.7% of the intermediate and adjacent discs and 99.3% of the facet joints. Studies with longer follow-up are warranted to further analyze the effects of relative stabilization and altered biomechanical loads. <h3>FDA DEVICE/DRUG STATUS</h3> This abstract does not discuss or include any applicable devices or drugs.

Relationship Between Myocardial Perfusion and Myocardial Function in Dilated Cardiomyopathy by Shown Ultrasonography.

Myocardial contrast echocardiography (MCE) and two-dimensional speckle tracking echocardiography (2D-STE) were used to detect left ventricular myocardial microcirculation perfusion and myocardial systolic function in dilated cardiomyopathy (DCM) and to explore the relationship between the two.Conventional ultrasound, MCE, and 2D-STE examinations were performed on 30 patients and 30 controls. Left ventricular microcirculation perfusion, left ventricular longitudinal strain (GLS), and circumferential strain (GCS) were analyzed to further compare the correlation between left ventricular perfusion and myocardial strain parameters.Regional myocardial perfusion was reduced in patients with DCM, manifesting as a decrease in the rising slope (A) of the mid-segment of the posterior septum, the peak intensity (PI) of the mid-segment of the anterior septum and the posterior septum, the apical segment of the lateral wall, the area under the curve (AUC) of the posterior septum, the basal segment of the posterior wall, the anterior septum, posterior septum, posterior wall, mid-segment of the lateral wall, and apical segment of the lateral wall and the overall average PI and AUC of the mid-segment, compared with that in the controls (P < 0.05). The left ventricular systolic function and the strain parameters GLS and GCS of DCM patients were lower than those of the controls (P < 0.001). Correlation analysis revealed a positive correlation between the A of the mitral valve and GCS (r = 0.372, P = 0.043), and MV-E/e' had a positive correlation with the AUC of the basal and intermediate segments (r = 0.379, P = 0.039; r = 0.404, P = 0.027).In patients with DCM, regional myocardial microcirculation perfusion is reduced, and myocardial strain is impaired. Myocardial perfusion has a good positive correlation with myocardial mechanics.

Does vertebral body tethering cause disc and facet joint degeneration? A preliminary MRI study with minimum two years follow-up

BACKGROUND CONTEXTVertebral body tethering (VBT), a flexible compression-based growth modulation technique, was claimed to prevent disc degeneration due to its less rigid nature compared to other growth-friendly techniques. Yet, the consequences of VBT surgery on discs and facet joints have not been precisely acknowledged. PURPOSEThe purpose of this study was to determine the changes in the intermediate and adjacent levels at least 2 years after surgery. STUDY DESIGN/SETTINGProspectively-followed consecutive patient cohort PATIENT SAMPLEAdolescent idiopathic scoliosis patients who underwent thoracoscopic VBT between 2014 and 2017 were included. OUTCOME MEASURESDegeneration of the intervertebral discs using the Pfirrmann classification; Degeneration of facet joints using a scale of 0 to 3. METHODSDemographic, perioperative, clinical, radiographic data were collected. Skeletal maturity and height gain were assessed in every follow-up. Overcorrection, tether breakage, mechanical and pulmonary complications as well as readmission and reoperations were recorded. MRIs taken before surgery and at a minimum of 2 years follow-up were evaluated for degeneration at the intermediate and adjacent segment intervertebral discs and facet joints by a blinded senior radiologist and compared. RESULTSTwenty-five patients with a mean of 38.6±10.6 months (24–62) of follow-up were included. The mean age at surgery was 12.2 (10–14), and the median Sanders stage was 3 (1–7). A mean of 7.7±1.1 (6–11) levels were tethered. The mean preoperative main thoracic curve magnitude of 46°±7.7° was corrected to 23.3°±5.9° postoperatively, which was subsequently modulated to 12° ±11.5° during the follow-up. At the time of the MRI (mean 29±9.5 (24–62) months), the median Sanders stages was 7 (5–8). A total of 217 levels of discs and bilateral facet joints were evaluated in the preoperative and follow-up MRI images. Analyses of disc and facet scores revealed no significant differences between patients. Deterioration of previously degenerated discs was noted in one patient (from grade 2 to 3), while previously healthy lower adjacent facet joints were degenerated (grade 2) in another patient. CONCLUSIONSIntermediate discs and facet joints were preserved after growth modulation with VBT surgery at a mean of 29 months of follow-up. Studies in larger cohorts with longer follow-up are warranted to have more in-depth analyses of the effects of relative stabilization and altered biomechanical loads.

Hnf1b haploinsufficiency differentially affects developmental target genes in a new renal cysts and diabetes mouse model.

ABSTRACTHeterozygous mutations in HNF1B cause the complex syndrome renal cysts and diabetes (RCAD), characterized by developmental abnormalities of the kidneys, genital tracts and pancreas, and a variety of renal, pancreas and liver dysfunctions. The pathogenesis underlying this syndrome remains unclear as mice with heterozygous null mutations have no phenotype, while constitutive/conditional Hnf1b ablation leads to more severe phenotypes. We generated a novel mouse model carrying an identified human mutation at the intron-2 splice donor site. Unlike heterozygous mice previously characterized, mice heterozygous for the splicing mutation exhibited decreased HNF1B protein levels and bilateral renal cysts from embryonic day 15, originated from glomeruli, early proximal tubules (PTs) and intermediate nephron segments, concurrently with delayed PT differentiation, hydronephrosis and rare genital tract anomalies. Consistently, mRNA sequencing showed that most downregulated genes in embryonic kidneys were primarily expressed in early PTs and the loop of Henle and involved in ion/drug transport, organic acid and lipid metabolic processes, while the expression of previously identified targets upon Hnf1b ablation, including cystic disease genes, was weakly or not affected. Postnatal analyses revealed renal abnormalities, ranging from glomerular cysts to hydronephrosis and, rarely, multicystic dysplasia. Urinary proteomics uncovered a particular profile predictive of progressive decline in kidney function and fibrosis, and displayed common features with a recently reported urine proteome in an RCAD pediatric cohort. Altogether, our results show that reduced HNF1B levels lead to developmental disease phenotypes associated with the deregulation of a subset of HNF1B targets. They further suggest that this model represents a unique clinical/pathological viable model of the RCAD disease.

Asymmetrical pythagorean-hodograph (PH) spline-based C3 continuous corner smoothing algorithm for five-axis tool paths with short segments

• A real-time C 3 continuous corner smoothing algorithm is proposed for five-axis short linear segments. • The proposed method can achieve lower curvature based on asymmetrical PH splines. • Smoothing errors around the corners are bounded under given constraints. • The arc-length can be analytically calculated to achieve real-time interpolation. • Experiments verify that much lower curvature and higher machining efficiency can be realized. Local corner smoothing is widely used for the G01 motion commands to achieve the motion continuity of transition corners. Most splines constructed by the existing local corner smoothing methods oriented for five-axis machining mainly must be connected by linear segments. However, with respect to the five axis machining of the corners with short line segments, these kinds of methods need that both ends of the constructed splines usually must be shrunk with the same proportion to avoid the overlapping of adjacent splines. This will lead to relatively large curvatures of the obtained trajectories together with relatively low machining efficiency. This article proposes a new real-time corner smoothing algorithm for five-axis machine tools by constructing the C 3 continuous asymmetrical PH splines. The typical characteristic lies in that the control points related to the two ends of the PH spline can be independently adjusted through reasonably introducing two more adjustable variables, which are realized by analytically calculating and then adding two additional control points into the corresponding control polygon. As a result, the PH spline poses the property of the asymmetry about the angular bisector. Based on this, the short linear commands of tool tip and tool orientation can be smoothed only through inserting different lengths of asymmetrical PH splines that can be connected directly. The motion variance of the smoothed tool orientation related to the tool tip displacement is analytically proved to be C 3 continuous, and thus, the synchronization between tool tip and tool orientation is guaranteed. The outstanding advantage of the algorithm for short line segments is twofold. The first is that the performance of the drives can be more fully utilized to reduce cycle time since the curvature around corners are reduced by adopting the asymmetrical profile, and the second is that the synchronization procedure is greatly simplified since the algorithm eliminates the requirement to convert the intermediate line segments into a spline between the adjacent connected PH splines. Both simulation and the experimental results demonstrate that the proposed algorithm can realize a higher machining efficiency.

Lateral Modified Brandt-Daroff Exercises: A Novel Home Treatment Technique for Horizontal Canal BPPV.

Brandt-Daroff exercises (BDEs) are commonly used as an at-home treatment for posterior canalithiasis, but their efficacy in the treatment of benign paroxysmal positional vertigo (BPPV) of the horizontal canal (HC-BPPV) has not been previously studied. Using biomechanical model simulation, we investigated modifications that may optimize BDE use for HC-BPPV treatment. The BPPV Viewer, a three-dimensional model of the human labyrinth, was used to analyze BDE for HC-BPPV treatment. While moving the model through sequential BDE positions, the expected position of otoliths was demonstrated. Treatment steps were adjusted to maximize otolith movement around the canal circumference without compromising otolith repositioning into the semicircular duct's anterior arm. All adjustments were integrated into lateral modified BDEs (LMBDEs) presented here. By implementing several modifications, BDE can effectively treat HC-BPPV. Model simulation indicates tilting the head 20° upward in the lateral position, instead of 45° specified by the original technique, which significantly increases displacement of otoliths originating from the horizontal duct's anterior and intermediate segments. LMBDE can be performed as a direct two-step sequence without pausing in the upright position before switching sides. If the affected ear is known, positioning the head 45° below horizontal on the unaffected side as a third treatment step can promote actual canal evacuation. These treatment enhancements increase circumferential otolith movement around the canal and may promote horizontal canal evacuation. LMBDEs are a modification of BDE that may increase their effectiveness for use in patients with HC-BPPV. This safe treatment adjunct between office visits may promote long-term symptom reduction.

Optical Data Analysis: Decoding Optical Data with Machine Learning (Laser Photonics Rev. 15(2)/2021)

In article number 2000422, Yuebing Zheng and co-workers review the advances of machine learning (ML) in decoding complex optical data, enabling rapid and accurate analysis of optical spectra and images. ML-assisted optical data decoding can shorten the data analysis time by skipping the intermediate segments and provide an insight into the unknown physics. Recent progress has been discussed with a focus on applications in disease diagnosis, noninvasive biological study, information technology, fundamental studies in optics, and materials science and engineering.

Ways to diversify enterprises in the space sphere: Challenges, problems, and solutions

Subject. The article addresses the efficiency of commercialization through enterprise diversification in the rocket and space sphere. Objectives. The study aims to assess the condition of the space market, find ways of diversification, identify opportunities for commercialization and diversification of business in this area to generate profit, and highlight urgent problems in the space market. Methods. We employ econometric methods, like correlation and regression analysis, and methods of mathematical statistics. Results. We propose a large-scale structure of the space market, enabling to examine and explore the relationship between its segments. The paper provides an assessment of the space market and opportunities for commercialization and diversification of business in this area for generating profit. It includes a profit-making model based on intermediate segments and post-sale service, which can serve as a basis for business diversification in this sphere. It also presents recommendations for further investigation. Conclusions. The lack of communication between the space market segments makes it difficult to develop the production of launchers, and therefore, in the long run, will lead to a reduction in the number of launches and degradation of the industry.

Bilateral Biplanar Distraction Osteogenesis in Facial Deformity Secondary to Temporomandibular Ankylosis.

The purpose of this single-centre prospective clinical study was to evaluate the correction of severe facial deformity and Obstructive sleep apnoea hypopnoea syndrome (OSAHS) in 22 patients aged from 9 to 42years (mean 20.09) of bilateralTemporomandibular joint ankylosis. Patients underwent multisegment distraction using external bilateral bidirectional distractors. Facial deformity was evaluated clinically in terms of mouth opening and neck chin angle and cephalometrically in terms of corpus length (gonion-pogonion), ramal height (articulare-gonion), chin deficiency (N perpendicular to Pog and SNB). OSAHS was evaluated through Epworth sleepiness scale, Berlin's questionnaire, pharyngeal airway space, apnoeic hypoapnoeic index, oxygen desaturation index, average arterial oxygen saturation and minimal fall in O2 saturation. Patients were followed up for a period of 13-74months (mean 28.68). Statistically highly significant results were obtained in all parameters. Complications encountered were pin infection (13.63%), loosening of pins (4.5%), wound dehiscence (9.09%), tooth fracture (4.5%) and parotid fistula (9.09%). Intermediate segment necrosis and vector loss were not observed. This study is a large case series using two corticotomy cuts for external bilateral bidirectional distracters with concomitant neo callous moulding in patients both pre- and post-ankylosis release. It is extremely efficient in correcting mandibular corpal and ramal deficiency as well as improving OSAHS.

On the track of bruxism: quantitative, qualitative and intraindividual analyses of the BruxChecker in daily clinical routine

Introduction Bruxism is a relevant topic in daily dental routine. Bruxism has to be confirmed by instrumental procedures. The BruxChecker (BC) is an inexpensive instrument that does not affect the stomatognathic system while used and is suitable for routine use in diagnostics and follow-ups. A novel digital approach for analyzing BC is described, based on first standard values. Material and Method Within this pilot study, 30 participants (15 males, 15 females) used an upper BC for one night and a lower BC during another night. A standardized digitalization process and a unique software application measured all Tooth Contact Areas (TCAs) on the BC: number and size of each TCAs for each occlusal segment. Results The mean number of TCAs on upper BC is 28.17 (sd +/-7.84), for lower BC 27.70 (sd +/-7.41). The mean size (mm2) of TCAs on upper BC is 71.81 (sd +/-51.27), for lower BC 68.11 (sd +/-42.64). There are only minor, not significant, gender differences regarding the number and size of TCAs. The transversal right-left TCAs distribution is almost symmetrical; a slightly increased difference can be observed for the size of TCAs right and left. The sagittal distribution of the TCAs shows the dominance of the posterior contacts, while the intermediate segments are least involved. Conclusion Within the limits of this pilot study and based on the digital analyses of TCAs on BC, the paper presents first standard values and a two-step systematic individual BC analysis.

Early Sharp Wave Synchronization along the Septo-Temporal Axis of the Neonatal Rat Hippocampus

In the neonatal rat hippocampus, the first and predominant pattern of synchronized neuronal network activity is early sharp waves (eSPWs) occurring at a frequency of ~2–4 events per minute. However, how eSPWs are organized longitudinally along the septo-temporal hippocampal axis remains unknown. Using silicone probe recordings from the septal and intermediate segments of the CA1 hippocampus in neonatal rats in vivo we found that eSPWs are highly synchronized longitudinally. The amplitudes of eSPWs in the septal and intermediate segments of the hippocampus were also highly correlated. eSPWs also supported longitudinal synchronization of CA1 multiple unit activity. Spatial-temporal analysis revealed a septal-temporal gradient with more frequent initiation of eSPWs in the septal regions. The speed of eSPW longitudinal propagation attained ~ 250 mm/s. We suggest that longitudinal correlated activity supported by synchronized eSPWs emerges early during postnatal development and may participate in the formation of intrahippocampal connections in the developing hippocampus.

Intermediate segment OCT

Intermediate segment OCT

Sociodemographic inequalities in lifestyle patterns of the population of a large Brazilian city

Abstract Knowledge of avoidable exposures to noncommunicable diseases from the analysis of health behaviors or practices, and of attitudes and care related to lifestyle is an important strategy in identifying issues emerging to health and opportunities for the prevention of these problems. It was intended to analyze the combination of behaviors related to health, food and lifestyle, identify behavioral patterns and verify the inequalities present in its association with sociodemographic characteristics of the population of Campinas, São Paulo. This is a cross-sectional study conducted in 2014/15 that evaluated 1,205 people aged 20 years or older. From the behaviors regular consumption of raw and cooked vegetables, fruit and beans, consumption of low-fat milk, concern with salt intake, habit of reading food label, concern with the use of pesticides, consumption of organic foods, practice of leisure-time physical activity, consumption of meat with fat, frequent consumption of sausages and soft drinks, current smoking, alcohol abuse and short sleep duration were extracted through analysis factorial by main components. The association of these patterns with the variables gender, age and income were tested. Three lifestyle patterns were identified and nomined. The 'Healthy Advanced' lifestyle pattern was associated with females, the elderly and the best income groups can be identified; the 'Healthy Prudent' was associated with older adults, with the elderly and with the intermediate income segment. The 'Unhealthy' pattern was associated with males, with young people and did not have an association with income. It can be concluded that healthy lifestyle patterns were more present in female groups, with older age and better income level. The less healthy pattern was found more frequently among men and young people. It is noteworthy that this last pattern did not differ between income strata indicating that unhealthy practices may be equally disseminated in these groups. Key messages The better lifestyle patterns were more present in female groups, with older age and better income level. The less healthy pattern was found more frequently among men and young people, and did not differ between income strata.

Importance of segmental hair analysis in a suspected case of attempted homicide by flocoumafen and difenacoum

The 4-hydroxycoumarin derivatives are the most used rodenticides and act as classical anticoagulants, interfering with the production of clotting factors in liver by antagonizing the action of vitamin K reductase, thereby inhibiting recycling of vitamin K1, involved in activation of blood clotting factors, resulting in massive bleeding. In this paper, we present the case of a 72-year old man providing abnormal coagulation parameters (PT-INR between 16.1 and 19.1) after hospitalization. Blood samples tested positive for flocoumafen and difenacoum, two superwarfarin rodenticides. Patient’s hair specimens, sampled 19 days after his hospitalization, showed that traces of both difenacoum and flocoumafen were detected in the first 1 cm; in the intermediate segments (1–2 and 2–3 cm), both difenacoum and flocoumafen were absent, while in the distal segment (3–4.5 cm), only difenacoum was found, but in significant amounts (140 pg/mg).Exposure to difenacoum in the past months, at least 4–5 before hospitalization, was confirmed by the presence of the rodenticide in the distal segment. Moreover, among the seized material, two specimens resulted compatible with the two rodenticides.

Modelling of the reference STARs procedures in the context of RPAS integration in non-segregated airspace

PurposeThis work presents the part of the research in the integration of the remotely piloted aircraft systems (RPAS) in non-segregated airspace. The purpose of this study is to elaborate the reference shape of the Standard Instrument Arrivals (STARs) procedures of controlled airports. The STARs parameters are unique for the aerodromes and depend on navigational aids (NAVAIDs), manoeuvres and aircraft categories. Therefore, the elaboration of reference shapes was advisable in the context of RPAS integration research.Design/methodology/approachThe models were based on the procedure design guidelines by International Civil Aviation Organization. The statistics of existing STARs were prepared using Aeronautical Information Publications to determine the representative procedural parameters. Construction of procedural shapes required to define the nominal flight path and tolerance areas.FindingsIn statistics, the standard deviation of distances was below the determined reference mean values, thus the models were convergent with existing procedures.Research limitations/implicationsThe modelling was limited to initial, intermediate, final and missed approach segments. Arrival segment was not modelled. NAVAIDs include Instrument Landing System Category 1 (in final and missed approach) and very high-frequency omni-directional ranging or global navigation satellite systems (in initial and intermediate approach segments).Practical implicationsPrepared models may be used in research in the integration of the new types of aerial vehicles in existing air traffic management systems.Originality/valueThe reference STARs possess commonly used procedural manoeuvres (straight-in, turn, racetrack and base turn) and different NAVAIDs. The parameters of approach segments were determined as representative of the existing procedures. Moreover, the models are suitable to place at arbitrary origin and runway axis bearing.

Knowing Real Cardiac Anatomy Enhances Understanding of Cardiac Development

New ideas about cardiac chamber formation, interatrial septum formation and development of the outflow region can be better understood by knowing real cardiac anatomy. The definitive chambers of the heart can be regionalized into components that provide a basis for understanding their formation during prenatal embryonic development. Each atrium has a venous inlet, a body, an appendage and a vestibule, while each ventricle consists of an inlet, an apical trabeculated portion and an outlet. During development several precursors combine to form the components of definitive chambers. Recent studies have challenged the classical version of interatrial septum formation, particularly the presence of a secondary atrial septum (septum secundum), and the closure of the primary atrial foramen (ostium primum). These studies suggest that the primary atrial septum (septum primum) merges with a fold of myocardium from the roof of the right atrium rather than with a second septum. This fold can be demonstrated in the dissection laboratory. Closure of the primary atrial foramen is accomplished by fusion of the ingrowing vestibular spine fusing with the mesenchymal cap of the primary septum and the merged AV endocardial cushions. This forms the anterior inferior atrial buttress that might be considered a secondary atrial septum. Finally, formation of the outflow region can be better understood using a tripartite explanation rather that the traditional bipartite version. During cardiac looping, the outflow region elongates and develops a bend in the middle. This flexure can define the outflow region into proximal, intermediate and distal segments. From these segments the outflow regions of the ventricles, the aortic valves and the intrapericardial roots of the great vessels are formed. The septum dividing the proximal segment is formed exclusively from endocardial cushion tissue, while the septum that partitions the intermediate and distal outflow regions is derived from cushion tissue and neural crest ectomesenchyme. Traditionally the aorticopulmonary septum was described as partitioning the entire outflow region. More recent examination of outflow tract development suggests that the aorticopulmonary septum only partitions the distal portion of the outflow region and the aortic sac. The intrapericardial and extrapericardial roots of the great vessels form from the division of the distal outflow region and the aortic sac.

ACOX3 Dysfunction as a Potential Cause of Recurrent Spontaneous Vasospasm of Internal Carotid Artery

Recurrent spontaneous vasospasm of the extracranial internal carotid artery (RSV-eICA) is a rarely recognized cause of ischemic stroke in young adults. However, its pathophysiology remains largely unknown. Through whole-exome sequencing of the ACOX3 gene of two dizygotic Korean twin brothers affected by RSV-eICA, we identified two compound heterozygous missense variants c.235T > G (p.F79V) and c.665G > A (p.G222E). In silico analysis indicated that both variants were classified as pathogenic. In vitro ACOX3 enzyme assay indicated practically no enzyme activity in both F79V and G222E mutants. To determine the effect of the mutants on vasospasm, we used a collagen contraction assay on human aortic smooth muscle cells (HASMC). Carbachol, a cholinergic agonist, induces contraction of HASMC. Knockdown of ACOX3 in HASMC, using siRNA, significantly repressed HASMC contraction triggered by carbachol. The carbachol-induced HASMC contraction was restored by transfection with plasmids encoding siRNA-resistant wild-type ACOX3, but not by transfection with ACOX3 G222E or by co-transfection with ACOX3 F79V and ACOX3 G222E, indicating that the two ACOX3 mutants suppress carbachol-induced HASMC contraction. We propose that an ACOX3 dysfunction elicits a prolonged loss of the basal aortic myogenic tone. As a result, smooth muscles of the ICA's intermediate segment, in which the sympathetic innervation is especially rich, becomes hypersensitive to sympathomimetic stimuli (e.g., heavy exercise) leading to a recurrent vasospasm. Therefore, ACOX3 dysfunction would be a causal mechanism of RSV-eICA. For the first time, we report the possible involvement of ACOX3 in maintaining the basal myogenic tone of human arterial smooth muscle.

Electrodiagnostic Patterns of Demyelination and Hughes Functional Grading in Typical Chronic Inflammatory Demyelinating Polyneuropathy

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by progressive or relapsing motor or sensory symptoms, with variants differing in the relative distribution of these symptoms and electrophysiologic findings. We aimed to correlate the electrodiagnostic patterns of demyelination using Hughes Functional Grading Scale in patients with CIDP. Methods: A case–control study was conducted at the neurophysiology department of Al-Imamian Al-Kadhymian Medical city and Nursing Home Hospital, Medical City, Baghdad from December 2017 to June 2018. Fifteen patients with CIDP aged 30–60 years with disease duration between 6 months and 2 years and 20 age-matched healthy subjects (control group) were included in the study. The participants were submitted to medical history, clinical neurological examination, and electrophysiologic tests. Results: Patients with CIDP demonstrated prolonged distal sensory and motor latencies, decreased sensory nerve action potential amplitude, slowing of sensory and motor nerve conduction velocity, and prolonged mean F-wave latency. The majority showed absent sural sensory responses. Significant relationships were demonstrated between the Hughes Functional Grading Scale and different neurophysiologic parameters, and no correlation was found with the terminal latency index. Conclusions: Patients with high Hughes functional scoring also have severe abnormalities in motor parameters, usually in the range of demyelination. The involvement of nerve segments was multifocal affecting mostly the proximal and intermediate nerve segments; the terminal segments were involved to a lesser extent.

Comparison of Lewis-Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.

Whether the Lewis-Sumner syndrome (L-SS) is a distinct entity from other types of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP-ot) remains controversial. The clinical/electrophysiological characteristics and long-term outcomes of 45 L-SS and 35 CIDP-ot patients were retrospectively compared. The CIDP-ot group was composed of 11 patients with a typical CIDP, 17 with a pure sensory form, four with a distal form and three with a pure motor form. In the L-SS group, asymmetric (P<0.001) and monomelic involvement (P=0.04) of the upper limbs (P<0.001) was significantly more frequent; paucisymptomatic forms (Overall Neuropathy Limitations Scale≤1) were less frequent (P<0.001); electroneuromyography showed that conduction block in intermediate nerve segments was the main demyelinating feature, with frequent F-wave abnormalities on nerves without conduction block (44%). Long-term prognosis was globally poorer in the L-SS group with more frequent aggravation during treatment (P=0.02), less frequent treatment withdrawal (P=0.03) and longer time to achieve successful withdrawal (39 vs. 15months). Our study suggests that L-SS patients have a less favourable therapeutic response rate and long-term outcomes. Rapid differentiation of L-SS from other forms of CIDP is important in order to anticipate a more complicated disease course management, with from one side the inefficacy or even harmfulness of corticosteroids and from the other side a difficult weaning procedure. A prospective study is necessary to confirm these results.