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The aim of the present study was to present a case of maxillary aspergillosis with unusual clinical presentation and imaging findings.The range of lesions and conditions that affect the maxillary sinus is wide and vast. This necessitates a dynamic multidisciplinary approach for proper diagnosis and effective management.We present an unusual case of maxillary aspergillosis that mimicked apical periodontitis and was most consistent with lymphoma from the diagnostic imaging perspective. Histopathology, however, established the correct diagnosis; appropriate management commenced promptly.It is imperative that maxillary aspergillosis be diagnosed and managed properly and promptly to improve prognosis. Advanced imaging is required, but it plays an adjunct role to histopathology. Long-term follow-up is also necessary to ensure complete resolution of the infection.Correlating clinical and imaging findings is a crucial step and any inconsistencies should be resolved promptly so as not to delay adequate management. Histopathology often serves to dispute any inconsistencies and allows the establishment of a proper diagnosis.

Burosumab, a fully human IgG1 monoclonal antibody to FGF23, is approved in Canada and Brazil to treat XLH in patients ≥1 year of age and in the US to treat XLH in patients ≥6 months of age. Burosumab has also received conditional marketing authorization in Europe to treat XLH with radiographic evidence of bone disease in children ≥1 year of age and in adolescents with growing skeletons. Burosumab significantly improved serum phosphorus, fracture/pseudofracture healing, stiffness, and physical functioning in a phase 3, double-blind, multicenter study (CL303, NCT02526160). In this trial, subjects were randomized 1:1 to receive burosumab or placebo subcutaneously every 4 weeks. At Week 24, subjects in the placebo group crossed over to receive burosumab (total duration ≥96 weeks). Here, we report final long-term safety results from this trial. Most (119/134, 89%) subjects completed 96 weeks and received 1 mg/kg burosumab; protocol-specified dose reductions were required for 11/134 (8.2%) subjects to effectively manage hyperphosphatemia (all mild [Grade 1]). Mean (±SE) baseline serum phosphorus was 1.98 (±0.03) mg/dL and was 2.97 (±0.05) mg/dL at Week 94 (midpoint of dose interval). Mean (±SE) iPTH level was 96 (±3.8) pg/mL at baseline and progressively declined to 79 (±3.3) pg/mL at Week 96. Nephrocalcinosis score at Week 96 changed by 0 in 101 subjects, -1 in 9 subjects, +1 in 10 subjects (14 subjects not available). There were no meaningful changes in ectopic mineralization. There were no neutralizing antibodies. No treatment-emergent adverse events led to study or treatment withdrawal. Serum phosphorus was maintained with long-term burosumab treatment, with no evidence of loss of effect in adults with XLH. Burosumab dose reductions effectively managed mild hyperphosphatemia. Frequency, severity, and types of AEs reported were consistent with previous burosumab trials.

Tumor-induced Osteomalacia (TIO) is a rare condition in which excess FGF23 produced by a tumor leads to renal phosphate wasting, impaired 1,25(OH)2D synthesis, osteomalacia, fractures, weakness, fatigue, and decreased mobility. In an ongoing open-label Phase 2 study (NCT02304367), 17 adults with TIO or cutaneous skeletal hypophosphatemia syndrome (CSHS) were enrolled and received burosumab, a fully human monoclonal antibody against FGF23. Key endpoints were changes in serum phosphorus and osteomalacia as assessed from trans-iliac crest bone biopsies. This report excludes 3/17 subjects who did not have TIO: 2 subjects diagnosed with X-linked hypophosphatemia post-enrollment and 1 subject with CSHS. Serum phosphorus increased from baseline (BL; 1.60 mg/dL) and was maintained after titration, from Week (W) 22 (2.85 mg/dL, dosing cycle midpoint) to W144 (2.56 mg/dL, dosing cycle endpoint, p

We present a case of successful cryoablation of the left extension of the atrioventricular (AV) node for treatment of a recurrent atrioventricular nodal reentry tachycardia without the use of fluoroscopy. Three-dimensional electroanatomic mapping system and intracardiac echocardiography were used to navigate catheters in the heart and position them according to anatomical landmarks. Due to the nature of cryoablation lesion formation, lesions were able to be applied safely in right atrium, as well as in left atrium, without damaging AV node or bundle of His.

After a century of relative stability of the electricity industry, extensive deployment of distributed energy resources and recent advances in computation and communication technologies have changed the nature of how we consume, trade, and apply energy. The power system is facing a transition from its traditional hierarchical structure to a more deregulated model by introducing new energy distribution models such as peer-to-peer sharing for connected communities. The proven effectiveness of P2P sharing in benefiting both prosumers and the grid has been demonstrated in many studies and pilot projects. However, there is still no extensive implementation of such sharing models in today's electricity markets. This paper aims to shed some light on this gap through a comprehensive overview of recent advances in the P2P energy system and an insightful discussion of the challenges that need to be addressed in order to establish P2P sharing as a viable energy management option in today's electricity market. To this end, in this article, we provide some background on different aspects of P2P sharing. Then, we discuss advances in P2P sharing through a systematic domain-based classification. We also review different pilot projects on P2P sharing across the globe. Finally, we identify and discuss a number of challenges that need to be addressed for scaling up P2P sharing in the electricity market followed by concluding remarks at the end of the paper., Comment: 33 pages, 6 figures, 4 tables

Background Transseptal puncture (TSP) for catheter ablation (CA) of left-sided tachycardias is traditionally performed with the aid of fluoroscopy. Compared to fluoroscopy, intracardiac echocardiography (ICE) can provide better orientation and imaging of soft tissue structures that are relevant to TSP. Objective Our aim was to evaluate feasibility and safety of ICE-guided TSP in CA treatment of left-sided tachyarrhythmias. Methods Consecutive fluoroless CA procedures with combined use of three-dimensional electroanatomic mapping system and ICE requiring TSP were evaluated in 357 patients (269 male, mean age 54 ± 18 years) referred to our hospital from July 2014 to November 2018. Among CA treated left-sided tachyarrhythmias 55 patients had accessory pathway, 16 had focal atrial tachycardia, 276 had atrial fibrillation or atypical atrial flutter and 10 had ventricular tachycardia. Success of transseptal access and complications related to TSP were analyzed. Adverse event requiring additional intervention was defined as major complication. Results Double TSPs were performed in 253 patients and single in 104 patients. Additional ablation procedures were needed in 26 patients and the rest had a single CA procedure. Altogether, 661 TSPs (274 double and 111 single) were attempted and 659 were successful (99.7%). Both TSPs failed due to severely thickened interatrial septum after previous cardiac surgery. In 14 patients (21 TSPs, 3.2%) a cardiac implantable electronic device (CEID) was present. ICE provided excellent lead visualisation and no lead dislodgements were recorded. Additional radiofrequency energy application to the transseptal needle was used for 3 challenging TSPs. Minor complications (pericardial effusions managed conservatively) occurred after 7 double TSP procedures (12/661, 1.8%). Major complication with pericardial tamponade (requiring pericardiocentesis) occurred during one double TSP (2/661, 0.3%). No TSP-related embolic complications were observed. Conclusion ICE-guided TSP in CA of left-sided tachyarrhythmias is safe with excellent success rates. In addition, ICE could provide additional reassurance in difficult cases and in patients with CEIDs.

Progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome is an unusual Mendelian phenotype of unidentified origin that causes profound intellectual disability, optic nerve/cerebellar atrophy, epileptic seizures, developmental progress, pedal edema, and early death. Uncharacteristic affected individuals are often classified as having PEHO-like syndrome, although they may be misdiagnosed as having epileptic encephalopathy, a potential result of early birth. In this study, we report a consanguineous Saudi family with a novel homozygous nonsense mutation of the CCDC88A gene causing PEHO-like syndrome. The children were suffering from developmental delay, epilepsy, mental disability, optic nerve/cerebellar atrophy, and pedal edema. Whole exome sequencing was conducted for the members of the family who have the disorder to study the novel mutation. Whole exome sequencing data analysis, confirmed by subsequent Sanger sequencing validation, identified a novel homozygous nonsense mutation c. 1292G > A, which was caused by p.Trp431* stop gain. This mutation was ruled out in 100 unrelated healthy controls. The nonsense homozygous mutation detected in this study has not yet been reported as pathogenic in the literature or various databases. In conclusion, a complete loss of protein function due to premature stop gain was caused by a mutation in exon 12 of CCDC88A. This loss may lead to PEHO phenotype. CCDC88A gene may therefore play an important and critical role for multiple aspects of normal human neurodevelopment.

A prospective study on 100 patients presented with a mesioangular impacted mandibular third molar in close proximity to the inferior alveolar nerve canal (IANC) was done to evaluate the efficacy of tooth sectioning in reducing the incidence of inferior alveolar nerve (IAN) injury during their surgical removal. The patients were divided into two groups : group A, where tooth sectioning was not performed, and group B , where tooth sectioning was performed . Patients were recalled 7 days, 15 days, 30 days , 3 months , and 6 months postoperatively for evaluation of nerve injury and its recovery. A total of 13 patients suffered from nerve damage out of which 10 patients belonged to group A (1050/ or 20%) and 3 patients belonged to group B (350/ or 6%). All patients showed complete recovery from nerve damage within 6 months except 1 patient . It was concluded from this study that tooth sectioning significantly reduces the incidence of nerve damage by 14% . Deviation of the IANC, increased depth of the impacted tooth , intraoperative IO hemorrhage within socket , nerve exposure , and increased duration of procedure were found to be significant risk factors associated with nerve injury.

Background: Dental extraction is a common procedure that is subject to complications and errors including extraction of the wrong tooth. This study aimed to determine the prevalence and identify the causes of wrong tooth extractions and explore the attitude of dentists after extraction of a wrong tooth. Methods: A questionnaire was adapted to fit the needs of this project and was distributed among all the dentists in four teaching dental clinics. The questionnaire was available in both the English and Arabic languages. Results: Of the 486 questionnaires, 186 questionnaires were returned (response rate of 37%) and used for the analysis. The prevalence of wrong tooth extraction was 21.1%. The three most common reasons for extracting a wrong tooth were miscommunication (31.6%), inadequate referral (28.9%), and exhaustion of an overworked dentist (28.9%). Surprisingly, only 50% informed the patient and documented the incident in the patient's chart. Few dentists apologized to their patients or offered any kind of solution or compensation. Conclusion: Wrong tooth extraction is a prevalent yet preventable problem. Most of the common causes of this problem appear to be more system rather than individual related. There is a pressing need to implement the universal protocol for the prevention of wrong site, wrong procedure, and wrong person surgery.

Objectives: To evaluate the treatment approach and compliance of pediatric neurologists with evidence-based guidelines across Kingdom of Saudi Arabia (KSA). These guidelines that clarify the optimal management of infantile spasms (IS) are not widely followed for various practical reasons. Methods: Physicians practicing in the field of pediatric neurology in KSA were contacted from the database of national societies. A cross-sectional study was conducted using a structured 20-item on-line survey designed to examine their clinical experience with IS and their treatment choices. Results: A total of 52 pediatric neurologists completed the survey (69% estimated capture rate). They received their formal training within KSA (40%), North America (33%), or Europe (14%). The majority practiced in 2 major cities, Riyadh (46%) or Jeddah (19%). Vigabatrin was favored over adrenocorticotropic hormone (ACTH) as first line drug for patients without tuberous sclerosis complex (48% vs. 21%). Several factors correlated with correctly selecting ACTH as first line including western training (33% vs. 5%, p=0.001), practicing in the city of Riyadh (25% vs. 14%, p=0.001), or having >10 years of clinical experience (25% vs. 5%, p=0.017). Reasons for not complying with the recommended treatment guidelines included lack of availability of ACTH (42%), side effect profile of steroids (29%), and personal preferences (14%). Only 4% admitted lack of awareness of the currently published management guidelines. Conclusion: Many pediatric neurologists in KSA are not following the published IS management guidelines. Using ACTH as first line correlated with their training, practice location, and years of experience. Lack of drug availability and side effect profile were common reasons for not complying with the management guidelines.

In X-linked hypophosphatemia (XLH), inherited loss-of-function mutations in the PHEX gene cause excess circulating levels of fibroblast growth factor 23 (FGF23), leading to lifelong renal phosphate wasting and hypophosphatemia. Adults with XLH present with chronic musculoskeletal pain and stiffness, short stature, lower limb deformities, fractures, and pseudofractures due to osteomalacia, accelerated osteoarthritis, dental abscesses, and enthesopathy. Burosumab, a fully human monoclonal antibody, binds and inhibits FGF23 to correct hypophosphatemia. This report summarizes results from a double-blind, placebo-controlled, phase 3 trial of burosumab in symptomatic adults with XLH. Participants with hypophosphatemia and pain were assigned 1:1 to burosumab 1 mg/kg (n = 68) or placebo (n = 66) subcutaneously every 4 weeks (Q4W) and were comparable at baseline. Across midpoints of dosing intervals, 94.1% of burosumab-treated participants attained mean serum phosphate concentration above the lower limit of normal compared with 7.6% of those receiving placebo (p < 0.001). Burosumab significantly reduced the Western Ontario and the McMaster Universities Osteoarthritis Index (WOMAC) stiffness subscale compared with placebo (least squares [LS] mean ± standard error [SE] difference, -8.1 ± 3.24; p = 0.012). Reductions in WOMAC physical function subscale (-4.9 ± 2.48; p = 0.048) and Brief Pain Inventory worst pain (-0.5 ± 0.28; p = 0.092) did not achieve statistical significance after Hochberg multiplicity adjustment. At week 24, 43.1% (burosumab) and 7.7% (placebo) of baseline active fractures were fully healed; the odds of healed fracture in the burosumab group was 16.8-fold greater than that in the placebo group (p < 0.001). Biochemical markers of bone formation and resorption increased significantly from baseline with burosumab treatment compared with placebo. The safety profile of burosumab was similar to placebo. There were no treatment-related serious adverse events or meaningful changes from baseline in serum or urine calcium, intact parathyroid hormone, or nephrocalcinosis. These data support the conclusion that burosumab is a novel therapeutic addressing an important medical need in adults with XLH.© 2018 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals, Inc.

Objectives: To investigate changes in the effective radiation dose ( E ) in relation to variations in voxel (VOX) size settings and the field-of-view (FOV) centralization. Methods : This cross-sectional dosimetry study used nanoDot OSLD dosimeters placed at 25 pre-determined sites in the head and neck slices of a RANDO male phantom to measure the absorbed radiation. The imaging scans took place at King Abdulaziz University Dental Hospital, Jeddah, Saudi Arabia between September 2016 and May 2017 using the i-CAT classic cone beam computed tomography (CBCT) unit. Three VOX size setting were examined: 0.2 mm, 0.3 mm and 0.4 mm. The FOV was alternatively eccentrically centered on the maxillary and mandibular arches while holding all other factors constant. Effective radiation doses were calculated for each VOX size and FOV centralization setting. Results : An inverse and indirect relation was found between E and the VOX size setting with smaller VOX yielding higher E . CBCT scans centered on the mandible resulted in higher E than scans centered on the maxilla. Conclusion : Effective radiation doses are significantly affected by changes in VOX size settings and FOV centralization options. This reflects on the potential radiation risk to patients and highlights the importance of choosing exposure parameters carefully. Saudi Med J 2018; Vol. 39 (5): 470-475 doi: 10.15537/smj.2018.5.21658 How to cite this article: Jadu FM, Alzahrani AA, Almutairi MA, Al-Amoudi SO, Jan AM, Khafaji MA. The effect of varying cone beam computed tomography image resolution and field-of-view centralization on effective radiation dose. Saudi Med J . 2018 May;39(5):470-475. doi: 10.15537/smj.2018.5.21658.

Paradoxically, elderly persons with type 2 diabetes mellitus (T2DM) fracture despite having higher bone density than nondiabetics. Systemic factors associated with aging and T2DM may have detrimental, local effects on the skeleton. One such factor could be by altering the microenvironment of the mesenchymal stem cells (MSCs), multipotent progenitors capable of differentiating into adipocytes or osteoblasts.Sera were obtained from four participant groups (n = 40 total, 10 per group): (1) young women with normal glucose tolerance (NGTY), (2) postmenopausal women with NGT), (3) postmenopausal women with impaired glucose tolerance (IGT), and (4) postmenopausal women with T2DM. Sera were incubated with human MSCs for 14 days. Cell proliferation and apoptosis were measured using EdU and TUNEL labeling assays, respectively. MSC differentiation for each group was determined using osteogenic and adipogenic gene expression markers quantified by qRT-PCR, as well as Alizarin Red and Oil Red O staining.Expression of adipogenic genes was greater than twofold higher (P 0.05) in MSCs cultured with T2DM sera compared to those incubated with NGTY, NGT, or IGT sera. The increase in adipogenic gene expression corresponded with increased Oil Red O staining. Despite the increased adipogenic differentiation of MSCs exposed to T2DM sera, cell proliferation and apoptosis rates as well as osteoblastic activity were not significantly different among the four conditions.Systemic, circulating factors in the serum of older women with T2DM may promote MSC differentiation into adipocytes versus osteoblasts. Increased differentiation of MSCs into adipocytes is one possible mechanism by which T2DM increases fracture risk.

Wypadki, awarie i straty operacyjne skłaniają do podkreślenia znaczenia odpowiednich systemów i mechanizmów kontroli w celu radzenia sobie z ryzykiem operacyjnym (OR). W artykule opisano metodologię przeprowadzania oceny ryzyka w zakresie ogólnej działalności włókienniczej, tj. opracowanie ram koncepcyjnej oceny ryzyka i przeprowadzenie metodycznej realizacji wybranego elementu ryzyka operacyjnego z wykorzystaniem proponowanego podejścia. Proponowany model oceny ryzyka obejmuje koncepcję modelowania strukturalnego probabilistycznej oceny ryzyka z wykorzystaniem podejścia bayesowskiego w swojej uogólnionej formie, która może być stosowana do określonych ustawień operacyjnych wyrobów włókienniczych z definicją wymiarów i skal dla określonego środowiska włókienniczego. Proponowany ogólny model może również znaleźć zastosowanie w różnych branżach tekstylnych, wprowadzając rzeczywiste dane do testowania i walidacji. Proponowany model prognozowania OR, oparty na interfejsie GUI, jest skalowalny, rozszerzalny i może być zastosowany w przypadku dowolnych operacji z niewielkimi modyfikacjami w węzłach nadrzędnych/końcowych w ramach określonego elementu ryzyka. Dane zaprezentowane w artykule mogą być przydatne w zakresie zarządzania ryzykiem włókienniczym, a także wnoszą wkład do zrównoważonego rozwoju działalności przemysłowej w przyszłości.

The ability to define the potential value that energy storage systems (ESSs) could generate through various applications in electric power systems, and an understanding of how these values change due to variations in ESS performance and parameters, market structure, utility structures, and valuation methodologies is highly important in advancing ESS deployment. This paper presents a taxonomy for assigning benefits to the use cases or services provided by ESSs, defines approaches for monetizing the value associated with these services, assigns values, or more precisely ranges of values, to major ESS applications by region based on a review of an extensive set of literature, and summarizes and evaluates the capabilities of several available tools currently used to estimate value for specific ESS deployments.

Objective: To assess the perceived availability of various services provided by Autism centers in our region. Methods: A cross sectional study was conducted from March to May 2015 involving health care workers at various governmental and private autism centers in Jeddah, Kingdom of Saudi Arabia. A structured 30-item questionnaire was designed to assess their demographics, training, experience, and the availability of various services at their centers. Results: Twelve autism centers were included and 136 employees participated in the study. Seventy-eight (57%) participants indicated that their center lacked important and essential services. These included programs for home care and outreach (59%), family recognition incentives and rewards (51%), integrative educational programs (39%), and occupational therapy (16%). Access for outside referral for these services was available in only 24% of cases. They cited several major obstacles in providing adequate service including; family involvement (24%), child’s behavioral problems (13%), increased number of students (9%), and work environment and space (5%). Conclusion: Significant deficiencies exist in the availability of autism services in our region. Access for referrals for important services is also limited. We identified several areas that can be targeted to help develop, promote, and improve the provided services for children with autism spectrum disorder.

Fibroblast growth factor 23 (FGF23) is an important regulator of phosphate and vitamin D metabolism and its excessive or insufficient production leads to a wide variety of skeletal disorders. This article reviews the FGF23-α-Klotho signaling pathway, highlighting the latest developments in FGF23 regulation and action, and describes the disorders associated with FGF23 excess or deficiency.

Background The availability of pediatric neurologists remains a global problem. Strategic planning and tackling issues related to optimizing the care of children with neurologic disorders depend on the workforce, which we aim to evaluate in this study. Method Physicians practicing in the field of pediatric neurology in Saudi Arabia were identified from the database of national societies. A structured 30-item online survey was designed to examine their clinical practice, experience, and availability of supporting services. Results A total of 56 pediatric neurologists completed the survey, mostly (48%) practicing in the capital city of Riyadh. Many of them worked alone (30%) or with only one other colleague (21%). Based on their responses, the estimated total number of pediatric neurologists across the country was 75 with a ratio of 1.06 pediatric neurologists per 100,000 children 15 patients per clinic and 79% reporting difficulties in finishing on time. The majority (87.5%) felt overwhelmed, which correlated with working alone (p = 0.002), having no assistant consultant (p = 0.039), and having no access to international sent-out laboratories (p = 0.02). The majority (94.5%) needed additional help in dealing with the volume and complexity of their patients, which correlated with feeling overwhelmed (p = 0.006). Conclusion Although the total number of pediatric neurologists across Saudi Arabia has improved significantly over the last decade, their distribution remains uneven leading to shortages in many regions. This adds to the excessive workload and affects the quality of the provided services.

To strengthen student interest in aerospace engineering including manufacturing and utilize the known benefits of active, project- based learning, a new undergraduate course on airfoil and nozzle design and manufacturing is under development at the University of California Davis. The course will demonstrate the air flow around airfoils at high speeds by using a water table experiment. The water table utilizes the hydraulic analogy and is a much simpler and cheaper setup than a wind tunnel. The students will learn about computational design of airfoils and nozzles, sustainable manufacturing, and experimental testing with self-made models. These models highlight capabilities of different manufacturing processes (in particular turning, milling, water jet cutting, and wire extrusion as additive manufacturing process) to produce specific shapes, dimensions and surface qualities. Special regard is given to process planning, costing, energy, post-processing and waste. The dimensional accuracy will impact the results in the water table experiments. From this, the students can understand that both analytical modeling and experimentation are necessary to solve engineering problems.

The American Society for Bone and Mineral Research and the United States National Osteoporosis Foundation (NOF) formed a working group to develop principles of goal-directed treatment and identify gaps that need to be filled to implement this approach. With goal-directed treatment, a treatment goal would first be established and choice of treatment determined by the probability of achieving that goal. Goals of treatment would be freedom from fracture, a T-score > –2.5, which is above the NOF threshold for initiating treatment, or achievement of an estimated risk level below the threshold for initiating treatment. Progress toward reaching the patient's goal would be periodically and systematically assessed by estimating the patient's compliance with treatment, reviewing fracture history, repeating vertebral imaging when indicated, and repeating measurement of bone mineral density (BMD). Using these data, a decision would be made to stop, continue, or change therapy. Some of these approaches can now be applied to clinical practice. However, the application of goal-directed treatment cannot be fully achieved until medications are available that provide greater increases in BMD and greater reduction in fracture risk than those that are currently approved; only then can patients with very high fracture risk and very low BMD achieve such goals. Furthermore, assessing future fracture risk in patients on treatment requires a new assessment tool that accurately captures the change in fracture risk associated with treatment and should also be sensitive to the importance of recent fractures as predictors of imminent fracture risk. Lastly, evidence is needed to confirm that selecting and switching treatments to achieve goals reduces fracture risk more effectively than current standard care. © 2016 American Society for Bone and Mineral Research.

PGAP2 (Post-GPI Attachment to Proteins 2) gene is involved in lipid remodeling steps of Glycosylphosphatidylinositol (GPI)-anchor maturation. At the surface of the cell this gene is required for proper expression of GPI-anchored proteins. Hyperphosphatasia with mental retardation syndrome-3 is an autosomal recessive disorder usually characterized by severe mental retardation. Mutations in the PGAP2 gene cause hyperphosphatasia mental retardation syndrome-3. We have identified a large consanguineous family from Saudi origin segregating developmental delay, intellectual disability, epilepsy and microcephaly. Whole exome sequencing with 100× coverage was performed on two affected siblings of the family. Data analysis in the patient revealed a novel missense mutation c.191C>T in PGAP2 gene resulting in Alanine to Valine substitution (Ala64Val). The mutation was reconfirmed and validated by subsequent Sanger sequencing method. The mutation was ruled out in 100 unrelated healthy controls. We suggest that this pathogenic mutation disrupts the proper function of the gene proteins resulting in the disease state.

Background Catheter ablation (CA) of supraventricular tachycardias (SVTs) is traditionally performed with the aid of fluoroscopy. However, ionizing radiation is associated with potentially harmful deterministic and stochastic effects to the patient and operator. Many methods have been suggested to reduce the radiation exposure, one of the methods, which completely eliminates ionizing radiation exposure, is zero-fluoroscopy catheter ablation. Purpose Our aim was to assess feasibility, success rate and safety of zero-fluoroscopy CA for treatment of various right and left-sided SVTs with the use of three-dimensional (3D) electroanatomical mapping system (EAM) and intracardiac echocardiography (ICE). Methods Consecutive 274 patients (140 male, mean age 33.5±21.8 years) with documented SVTs underwent CA in our center from April 2014 to May 2018. All procedures were performed with the 3D EAM without any use of fluoroscopic guidance. ICE was used as primary visual modality when left-sided approach was required. The procedural endpoint for atrioventricular nodal reentrant tachycardia (AVNRT) was nodal rhythm during radiofrequency (RF) energy delivery and non-inducibility. The procedural endpoint for focal atrial tachycardia (AT) was termination and non-inducibility. The procedural endpoint for accessory pathway (AP) mediated tachycardia was absence of bidirectional conduction over the AP. The procedural endpoint for typical atrial flutter (AFL) was bidirectional block over the cavo-tricuspid isthmus. Results One hundred thirty two patients had AVNRT, 79 had AP mediated tachycardia (47 left-sided, 25 septal and 7 right-sided), 31 patients had AT (8 left-sided), 32 patients had AFL. Cryo-ablation was used in 14 (14/132, 10.6%) patients with AVNRT and 5 patients with septal AP (5/25, 20%), RF was used in the rest. The procedural endpoint was achieved in all procedures (100%). During the mean follow-up of 343±253 days the recurrence rate for AVNRT, AP mediated tachycardia, focal AT, AFL was 7.5%, 16.4%, 32.3% and 9.4%, respectively. No procedural complications were observed. Conclusions Zero-fluoroscopy CA of right and left-sided SVTs with the use of the 3DEAM and ICE is feasible, safe and results in promising long-term success rates after single CA procedure. Acknowledgement/Funding None

We obtain a condition describing when the quasimodular forms given by the Bloch-Okounkov theorem as $q$-brackets of certain functions on partitions are actually modular. This condition involves the kernel of an operator {\Delta}. We describe an explicit basis for this kernel, which is very similar to the space of classical harmonic polynomials., Comment: 12 pages; corrected typos

The atrial irregular rhythm, often reflected in atrial fibrillation, undulation or flutter, is recognized as one of the major causes of brain stroke and entails an increased risk of thromboembolic events because it increases the likelihood of blood clots formation. Its early detection is becoming an increasingly important preventive measure. The paper presents a simple methodology for the detection of atrial irregular rhythm by ECG body gadget that can perform long-term measurements, e.g. several weeks or more. Multi-channel ECG, on the body surface, gives a more detailed insight into the atrial activity in comparison to standard 12-lead ECG. The information from MECG is compared with single-channel patch ECG. The obtained results suggest that the proposed methodology could be useful in treatments of atrial irregular rhythm. One can obtain a reliable information about the time and duration of fibrillation events, or determine arrhythmic focuses and conductive pathways in heart atria, or study the effects of antiarrhythmic drugs on existing arrhythmias and on an eventual development of new types of arrhythmias.

Tumor-induced Osteomalacia (TIO) and Epidermal Nevus Syndrome with osteomalacia (ENS) are rare conditions in which ectopic production of FGF23 by tumor (TIO) and bone (ENS) lead to renal phosphate wasting, impaired 1,25(OH)2D synthesis, osteomalacia, fractures, weakness, fatigue and decreased mobility. In an ongoing open-label Phase 2 study (NCT02304367), 17 adults were enrolled and treated with burosumab, a fully human monoclonal antibody against FGF23. Key endpoints were change in serum phosphorus and osteomalacia as assessed from trans-iliac crest bone biopsies. The per protocol (PP) analysis included 14/17 subjects who received 0.3-2.0 mg/kg burosumab every 4 weeks (W). Three subjects were excluded: 1 received subthreshold dosing (0.3 mg/kg at Day 0 and 0.15 mg/kg at W8, W32, and W72); 2 were diagnosed with X-linked hypophosphatemia post-enrollment. Ten subjects in the PP group had paired bone biopsies at baseline and W48. Mean ± SE histomorphometric values for the 8/10 subjects with osteomalacia at baseline were 20.4 ± 4.2 µm for osteoid thickness (OT), 23.0 ± 7.2% for osteoid volume/bone volume (OV/BV), and 66.1 ± 10.6% for osteoid surface/bone surface (OS/BS); baseline median (Q1, Q3) for mineralization lag time (MLT) was 1672 (1102, 2929) days. At W48, histomorphometric indices improved as shown by mean percentage changes in OT (37%), OV/BV (40%), OS/BS (-5%), and MLT (median percentage change -78%). Serum phosphorus, fatigue, and physical functioning are reported for the PP group. Mean (SD) serum phosphorus was 1.5 (0.3) mg/dL at baseline and 2.6 (0.8) mg/dL when averaged across the mid-point of the dose interval through W24. After W24, serum phosphorus, assessed only at the end of the dose interval, maintained this increase through W72. Mean (SD) Global Fatigue Score decreased from 5.3 (2.8) at baseline to 3.6 (2.9) at W48 (p=0.020) and to 3.3 (2.7) at W72 (p=0.004). The SF-36 mean (SD) physical component summary score increased from 34 (11) at baseline to 39 (10) at W48 (p=0.059) and to 42 (10) at W72 (p=0.003). Mean (SD) vitality score increased from 41 (14) to 47 (12) at W48 (p=0.075) and to 49 (12) at W72 (p=0.012). The mean (SD) number of sit-to-stand repetitions increased from 6.9 (4.0) at baseline to 8.6 (4.2) at W48 (n=10; p=0.004). By W72, all 17 subjects had ≥1 adverse event (AE). There were 13 serious AEs in 6 subjects, none were considered drug-related. Tumor progression occurred only in subjects with a history of tumor progression prior to enrollment. One subject discontinued treatment prior to W48 to treat tumor progression with chemotherapy. There was 1 death, considered unrelated to treatment. In adults with TIO Syndrome, burosumab was associated with improvements in serum phosphorus, osteomalacia, mobility, quality of life, and reductions in fatigue.

Burosumab, a fully human monoclonal antibody to FGF23, is the only approved treatment for X-linked hypophosphatemia (XLH), a rare genetic disorder characterized by renal phosphate wasting and substantial cumulative musculoskeletal morbidity. During an initial 24-week randomized, controlled trial, 134 adults with XLH received burosumab 1 mg/kg (n = 68) or placebo (n = 66) every 4 weeks. After 24 weeks, all subjects received open-label burosumab until week 48. This report describes the efficacy and safety of burosumab during the open-label treatment period. From weeks 24-48, serum phosphorus concentrations remained normal in 83.8% of participants who received burosumab throughout and were normalized in 89.4% who received burosumab after placebo. By week 48, 63.1% of baseline fractures/pseudofractures healed fully with burosumab, compared with 35.2% with burosumab after placebo. In both groups, burosumab was associated with clinically significant and sustained improvement from baseline to week 48 in scores for patient-reported outcomes of stiffness, pain, physical function, and total distance walked in 6 min. Rates of adverse events were similar for burosumab and placebo. There were no fatal adverse events or treatment-related serious adverse events. Nephrocalcinosis scores did not change from baseline by more than one grade at either week 24 or 48. These data demonstrate that in participants with XLH, continued treatment with burosumab is well tolerated and leads to sustained correction of serum phosphorus levels, continued healing of fractures and pseudofractures, and sustained improvement in key musculoskeletal impairments.

Reconstruction error is a prevalent score used to identify anomalous samples when data are modeled by generative models, such as (variational) auto-encoders or generative adversarial networks. This score relies on the assumption that normal samples are located on a manifold and all anomalous samples are located outside. Since the manifold can be learned only where the training data lie, there are no guarantees how the reconstruction error behaves elsewhere and the score, therefore, seems to be ill-defined. This work defines an anomaly score that is theoretically compatible with generative models, and very natural for (variational) auto-encoders as they seem to be prevalent. The new score can be also used to select hyper-parameters and models. Finally, we explain why reconstruction error delivers good experimental results despite weak theoretical justification., 9 pages, 3 figures, submitted to NeurIPS 2019

Background. To determine the agreement rate between histopathologic diagnosis and radiographic interpretation of jaw lesions. Methods. Cases with jaw pathologies that have diagnostically adequate histopathologic samples and radiographic examinations were reviewed retrospectively. Two board-certified oral and maxillofacial pathologists (OMFP) independently determined the histopathologic diagnosis, while two board-certified oral and maxillofacial radiologists (OMFR) determined the radiographic interpretations independently. Then the histopathologic diagnosis and the radiographic interpretation were compared for agreement. Results. A total of 104 cases (53% females) were included with a mean age of 31 years. The agreement rate between histopathologic diagnosis and radiographic interpretation was 49%. OMFP required OMFR consultations to reach a diagnosis in 16% of cases. The most commonly encountered lesions were by far odontogenic cysts of inflammatory origin and the agreement for this disease category was 49.1%. However, agreement rates were highest for the disease category of tumors (62.5%). Conclusion. The agreement rate between OMFP and OMFR was higher for tumors than cysts. Agreement rates between OMFP and OMFR improved with efficient consultation between the two disciplines.

Background: Burnout syndrome is a common professional problem causing mental fatigue, depersonalization, and diminished self-value. Burnout during pediatric residency can significantly influence the resident’s performance and the quality of their training. Objectives: To evaluate the burnout status of pediatric residents across Jeddah, KSA. Methods: A cross-sectional, descriptive study involving pediatric residents across Jeddah, Saudi Arabia was conducted from the 1 st of August to 1 st of December, 2012. The Maslach Burnout Inventory was utilized in addition to questions about their work environment and lifestyle. Results: Sixty pediatric residents (67% females) were included with ages ranging between 25 - 30 years (mean 26.5). They practiced in various institutions, mostly (41%) in ministry of health hospitals. Burnout scores were abnormal in 49 (82%) and in 19 (32%) the syndrome was severe. Males were more likely to reach a severe burnout category when compared to females (32% vs 19%, p = 0.01). Residents working in the university hospital (23%), were more likely to have severe burnout when compared to those working in other hospitals (p = 0.002). Junior residents (R1 and R2) were also more likely to have severe burnout when compared to senior residents (34% vs 21%, p = 0.013). Conclusions: Many pediatric residents are suffering from burnout syndrome. It is more common among males, junior residents, and those working in a university hospital setting. Specific strategies should be developed to prevent resident burnout.