Introduction Most currently available pacing and defibrillation leads utilize a stylet-based design that facilitates implantation. This has advantages, but also increases the lead diameter and adds the potential for metal fatigued-based conductor failure. Methods A systematic literature search was conducted, and the authors add their twenty-year experience with this lead design. Results The global experience with lumenless leads was reviewed both for “standard” positioning and with conduction system pacing. Methods for both placement and system modification are reviewed. Conclusions Lumenless leads have the potential to improve the durability of endocardial pacing and facilitate conduction system pacing.

Since hyperplastic aponeurosis and tendons cause limited muscle extension, patients with masticatory muscle tendon-aponeurosis hyperplasia (MMTAH) experience a limited mouth opening ability due to restrictions in muscle extension. Therefore, this article discusses five patients of MMTAH who underwent bilateral aponeurotomy combined with coronoidectomy in our department.

Objectives: Psychosocial assessment is a crucial step in delivering evidence-based care to both caregivers and children with cancer. The need for tailoring psychosocial interventions for each member of families that struggle with pediatric cancer, increasingly points to the fact that systematic and comprehensive psychosocial screening is very important. The Psychosocial Assessment Tool (PAT 3.1) is a brief parent report screener which is based on Pediatric Preventive Psychosocial Health Model theoretical framework (PPPHM; Clinical, Targeted, and Universal). The present study aimed to validate the PAT for an Iranian sample. Methods: Primary caregivers of 151 children diagnosed with cancer took part in this study and filled in the batteries of questionnaires at Mahak Hospital. Validation scales were used to examine the congruent validity. Results: Among the 8 subscales of the PAT, Social Support (α=0.82), Child problems (α=0.79), Sibling problems (α=0.90), Family problems (α=0.71), and the total score subscale (α=0.88) had strong internal consistency. However, Family beliefs (α=0.64), and Stress reaction (α=0.67) subscales had moderate internal consistency. The lowest internal consistency was related to Family structure/resource subscale (α=0.48). Nearly half of the families tired in Targeted level of psychosocial risks, 29.8 % were tired in Clinical level and 21.2 % were tired in Universal level of psychosocial risks. Conclusions: The results provide support for validity of the Farsi version of the PAT. Farsi PAT is a valid instrument for systematic screening of the psychosocial risks for children with cancer and their family members and delivering psychosocial care consistent with risk.

A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O), so called TAFRO syndrome, was first reported in 2010.1 Considered a variant of idiopathic multicentric Castleman’s disease, the recent discovery and rarity of this syndrome poses significant challenges to diagnosis and management. In this case report, we review the youngest patient to be reported in the literature with TAFRO syndrome. We highlight the patient’s diagnosis, management, and follow-up in the context of current recommendations for treatment and management of TAFRO syndrome.

Methotrexate is a chemotherapeutic and anti-inflammatory agent used in cancers, hematologic and rheumatologic diseases, binding to Dihydrofolate Reductase (DHFR) as a competitive inhibitor, modulating inflammation by inhibition of transmethylation reactions.1,2 Life threatening hypersensitivity reactions (HSRs) have been reported with methotrexate. 3,4,5 Alternative treatments result in suboptimal outcomes/ death due to cancer progression. Drug desensitization enables patients to continue treatment, preventing anaphylaxis by inducing a temporary tolerance.3 We report a case of a child with Acute B-cell Lymphoblastic Leukemia who successfully tolerated methotrexate desensitization, and subsequently weaned to a weekly oral challenge. He is now taking weekly methotrexate without any reactions.

Edge wave detection is a key technology in steel plate quality inspection, which is beneficial to improve the production quality of steel products and the efficiency of enterprises. In this letter, a novel algorithm based on visual projection and pixel statistics is proposed to detect the edge wave using 3D camera. The proposed algorithm firstly uses a series of image preprocessing to effectively remove the influence of irrelevant interference, and transforms the colour image into the binary image. Subsequently, three key positions of the edge wave in range image are precisely determined based on visual projection and pixel statistics. Finally, two 3D cameras are used to acquire the range images to evaluate the proposed algorithm's performance. The results show that the proposed algorithm can accurately detect the edge wave instead of human eye detection.

A Novel MECOM Variant Associated with Congenital Amegakaryocytic Thrombocytopenia and Radioulnar Synostosis Hanan Al-Abboh1, Akmal Zahra1 and Adekunle Adekile1,2Pediatric Hematology Unit, Mubarak Hospital1 and Department of Pediatrics, Faculty of Medicine, Kuwait University2, Kuwait Address Correspondence to: Professor Adekunle Adekile Department of Pediatrics Faculty of Medicine Kuwait University PO Box 24923 Safat 13110 Kuwait Email: adekunle.adekile@ku.edu.kw Tel: +96525319486To the EditorCongenital radioulnar synostosis (RUS) is a rare developmental anomaly of proximal fusion of the radius and ulna, resulting in limited pronation and supination of the forearm. It may accompany other abnormalities in the skeleton, kidney, heart and aneuploidy syndromes1,2. A subset of patients with RUS present with bone marrow failure (BMF) syndromes, characterized by amegakaryocytic thrombocytopenia (RUSAT), progressing to myelodysplasia and pancytopenia2,3. The hematological manifestations are quite variable, with some presenting with severe BMF in childhood, while others are mild and may not present until adulthood.Heterozygous germline variants in the homeobox A11 (HOXA11) gene were the first to be associated with RUS and designated RUSAT14, but lately, several families have been described with variants in the MDS1 and EVI1 complex (MECOM) locus, and referred to as RUSAT22,5,6. Many of these variants appear de novo , while others follow an autosomal dominant inheritance. We, hereby, report the case of a Kuwaiti patient who presented with congenital amegakaryocytic thrombocytopenia (CAMT) in the neonatal period and later noticed to have RUS. Whole exome sequencing revealed a novel MECOM variant.A.A. is a male Kuwaiti, the first child of consanguineous parents and was first seen at the age of 36 days, following antenatal ultrasound diagnosis of bilateral hydronephrosis and right renal cyst. He was a product of induced vaginal delivery with a birth weight of 2.3 kg. After delivery, he was kept under observation in the neonatal intensive care unit. His CBC showed isolated thrombocytopenia (Plt 34 x109/L). He received several platelet transfusions, as well as IVIG twice. Postnatal abdominal ultrasound showed multicystic right kidney, in addition to bilateral hydronephrosis. The mother had no history of thrombocytopenia during pregnancy and there was no other pertinent family history.Physical examination at presentation showed 2 café-au-lait spots, one on the back, measuring 1x2 cm and another over the left leg, that was less than 0.6 cm. There were no obvious dysmorphic features and other systems were unremarkable. CBC showed WBC 11.2 x109/L Hb 10.4 g/dL, MCV 83fl, Plt 49 x109/L, ANC 1.8 x109/L. Renal function tests were normal. Blood film showed no abnormal cells; there was true thrombocytopenia with giant forms. Antiplatelet antibody was negative. Abdominal ultrasound at age 1 month showed complete replacement of the right kidney by cystic changes with left moderate hydronephrosis. Skeletal survey was reportedly normal.Bone marrow biopsy showed normal distribution of granulocytic and erythroid precursors, with severe suppression of megakaryocytosis, consistent with a bone marrow failure syndrome. Chromosomal breakage study was normal. The patient was diagnosed with right undescended testis, as well as right inguinal hernia that were operated at age 1 year and 10 months. At the age 2 and a half years, A.A. was noticed to have limited bilateral arm movement supination and pronation. The mother volunteered that she has a similar defect. X-rays confirmed that the child had bilateral radioulnar synostosis. Whole exome sequencing showed that the patient is heterozygous for a previously-unreported MECOM gene, c.2282A>G mutation. Unfortunately, the parents have not been screened for these mutations.The patient has been under follow up for 4 years, his platelet count has been stable, ranging between 40-50 x109/L, with no bleeding tendency. In spite of his limited arm rotation, he currently functions normally in his daily activities, however, his hand writing skills and ability to engage in sports are yet to be observed since he is still pre-school age. Platelet transfusion is reserved only for severe bleeding, which he has not had. Bone marrow transplant may be considered in future if his bone marrow failure worsens and/or his marrow shows dysplastic changes.Dokal et al3 were the first to report an association between RUS and late-onset BMF, while Thompson et al described its association with CAMT and linked it to the c.872delA ,p.Asn291Thrfs3 variant of the HOXA11gene4,7. More recently, several germline mutations in the MECOM locus have been reported and appear to be the more common cause of RUSAT. Indeed, no other cases of HOXA11 mutations linked to RUSAT have been described since the initial report. Niihori et al8 reported the first 3 heterozygous MECOMmutations in 3 sporadic patients. These variants and those subsequently reported by Walne et al2 are in a highly conserved cluster within 10 amino acids (aa750-760) and impact on either the highly conserved Cys2His2 zinc finger motif (zinc finger 8, aa733-755) or the adjacent linker motif (aa756-760). It has been shown that removal of the 8th zinc finger causes granulopoiesis arrest while mutations and deletions in other parts of the complex, outside the 8th and 9th fingers, are associated with hematological disorders without RUS9.MECOM codes for a zinc finger transcription factor with important roles in normal development and oncogenesis and is involved in the regulation of embryonic development and hematopoietic stem-cell renewal. Hence the phenotype in individuals with these mutations is very variable ranging from BMF to different skeletal, cardiac, renal malformations, B cell deficiency and sensorineural deafness.Our patient showed a previously unreported variant in the region of the 8th zinc finger of the MECOM locus. This c.2282A>G missense variant results in the tyrosine to cysteine substitution at codon 761 (p.Tyr761Cys). The amino acid is in the Zinc finger, C2H2 and Zinc finger, C2H2-like protein domains and is highly evolutionarily conserved. Unfortunately, the parents were not screened for the mutation, however, the mother shows RUS, with normal blood counts. This is consistent with the marked variability in the clinical phenotype. The father is also physically and hematologically normal.Apart from thrombocytopenia, our patient also had renal abnormalities – hydronephrosis and multicystic kidney disease. The natural history of his condition is that he may develop pancytopenia and/or myelodysplasia in the future. He is under close follow up and will be considered for bone marrow transplantation if his condition worsens. In the meantime, he remains hypomegakaryocytic with a platelet count at 30 – 50 x 109/l while other blood cellular elements are normal. His renal function and hearing are being monitored, but still remain normal.AcknowledgementsWe thank the patient’s family for allowing us to report this case. The whole exome sequencing was done at the Laboratory of Genetics and Genomics, Cincinnati Children’s Hospital, Cincinnati, Ohio.References1. Rizzo R, Pavone V, Corsello G, Sorge G, Neri G, Opitz JM. Autosomal dominant and sporadic radio-ulnar synostosis. Am J Med Genet.1997;68(2):127-134.2. Walne A, Tummala H, Ellison A, et al. Expanding the phenotypic and genetic spectrum of radioulnar synostosis associated hematological disease. Haematologica. 2018;103(7):e284-e287.3. Dokal I, Ganly P, Riebero I, et al. Late onset bone marrow failure associated with proximal fusion of radius and ulna: a new syndrome.Br J Haematol. 1989;71(2):277-280.4. Thompson AA, Nguyen LT. Amegakaryocytic thrombocytopenia and radio-ulnar synostosis are associated with HOXA11 mutation. Nat Genet. 2000;26(4):397-398.5. Germeshausen M, Ancliff P, Estrada J, et al. MECOM-associated syndrome: a heterogeneous inherited bone marrow failure syndrome with amegakaryocytic thrombocytopenia. Blood Adv. 2018;2(6):586-596.6. Ripperger T, Hofmann W, Koch JC, et al. MDS1 and EVI1 complex locus (MECOM): a novel candidate gene for hereditary hematological malignancies. Haematologica. 2018;103(2):e55-e58.7. Thompson AA, Woodruff K, Feig SA, Nguyen LT, Schanen NC. Congenital thrombocytopenia and radio-ulnar synostosis: a new familial syndrome.Br J Haematol. 2001;113(4):866-870.8. Niihori T, Ouchi-Uchiyama M, Sasahara Y, et al. Mutations in MECOM, Encoding Oncoprotein EVI1, Cause Radioulnar Synostosis with Amegakaryocytic Thrombocytopenia. Am J Hum Genet.2015;97(6):848-854.9. Nielsen M, Vermont CL, Aten E, et al. Deletion of the 3q26 region including the EVI1 and MDS1 genes in a neonate with congenital thrombocytopenia and subsequent aplastic anaemia. J Med Genet.2012;49(9):598-600.

In this study, a novel water-soluble exopolysaccharide—dextran, biosynthesized by Leuconostoc Mesenteroides CICC-21725 was separated, purified, and comprehensively characterized. The yield of purified dextran was 30.50 g/L and, the weight-average molecular weight (Mw) was 8.39 × 107 Da. The structure of the purified dextran determined by FT-IR, and 1H,13C NMR spectroscopy demonstrated that the dextran was of α dextran type, mainly containing α-(1→6) glycosidic bonds. The morphological observation by SEM and AFM elucidated that dextran was a cluster of micro-nano size irregular spherical particles with a rough surface. The purified dextran obtained has good thermal stability. The water absorption rate of the dextran product was 22.61%, and the viscosity was 10.17 mPas.

Breast cancer has posed great global burden around the world, while cardiac myxoma is the most common primary benign cardiac neoplasm. Herein we reported a case of concurrent breast cancer and cardiac myxoma, emphasizing the importance of preoperative transthoracic echocardiography.

Light field (LF) enables high-dimensional image data representation since it can capture spatial and angular information of light rays simultaneously. The low spatial resolution caused by the limited imaging ability of the capturing equipment and the trade-off between spatial and angular resolution greatly affects the quality and application of LF images. In this letter, we propose an end-to-end LF super-resolution (SR) method via geometric feature interaction. Firstly, the low-resolution LF images are stacked in the horizontal and vertical epipolar plane image (EPI) directions and form 3D VI stacks. Then, these stacks are put into a dual-branch network, and we alternately perform 3D convolution on the viewpoint images (VIs) and EPIs by reshaping features for better feature extraction and interaction. The proposed method can fully explore the texture information and geometric consistency of the LF, and super-resolve all VIs at the same time. Experimental results on both real-world and synthetic LF datasets show that the proposed method has higher performance than other state-of-the-art methods.

A document by Luise Erpenbeck. Click on the document to view its contents.

Here, we present a rare finding of CNS type II in a 14-year old boy with celiac disease as well as the management and outcome of CNS among the paediatrics population. Although CNS has been associated with other conditions, there have been no documented association with celiac disease.

Next-generation phenotyping (NGP) is the application of advanced methods of computer vision on medical imaging data such as portrait photos of individuals with rare disorders. NGP on portraits results in gestalt scores that can be used for the selection of appropriate genetic tests, and for the interpretation of the molecular data. Here, we report on an exceptional case of a young girl that was presented at the age of eight and fifteen and enrolled in NGP diagnostics at the latter occasion. The girl had clinical features associated with Koolen-de Vries syndrome and a suggestive facial gestalt. However, chromosomal micro array (CMA), Sanger sequencing, multiplex ligation-dependent probe analysis (MLPA), and trio exome sequencing remained inconclusive. Based on the highly indicative gestalt score for Koolen-de Vries, the decision was made to perform genome sequencing to also evaluate non-coding variants. This analysis revealed a 4.7 kb deletion at the end of intron 6 of the KANSL1 gene, which is the smallest reported structural variant to date for this phenotype. The case illustrates how NGP can be integrated into the iterative diagnostic process of test selection and interpretation of sequencing results.

A 24-year-old man had vascular purpuric infiltrated, associated elbows and knees’s polyarthralgia, and abdominal pain. The proteinuria was 0.73 g/24 h. Skin histology showed a lymphocytic vasculitis lesion and perivascular infiltrates, without fibrinoid necrosis. The diagnosis IgA vasculitis was made according to the criteria of the ACR and EULAR/PRINTO/PRES.

In this paper, a metapopulation model has been developed and analysed to describe the transmission dynamics of cholera between two communities linked by migration, in the presence of imperfect vaccine and a varying media awareness impact. Stability analysis show that the disease free equilibrium is both locally and globally asymptotically stable when the vaccine reproduction number is less than unity. The endemic equilibria have also been shown to be locally asymptotically stable when the vaccine reproduction number is greater than unity. The simulation results show that with an imperfect vaccine and efficient media awareness, cholera transmission is reduced. The transmission rates have also been shown to be nonidentical in the two communities. It is therefore advisable, that health practitioners embrace the use of both vaccination and media awareness when designing and implementing community specific cholera intervention strategies.

In this paper, resonance and bifurcation of a nonlinear damped fractional-order Duffing system are studied. The amplitude and phase of the steady-state response of system are obtained by means of average method, and then the amplitude-frequency characteristic curves of the system under different parameters are drawn based on the implicit function equation of amplitude. Grunwald-Letnikov fractional derivative is used to discretize the system numerically, and the response curve and phase trajectory of the system under different parameters are obtained, and the dynamic behavior is analyzed. The forked bifurcation behavior and saddle bifurcation behavior of the system under different parameters are investigated by numerical simulation.

Let $\psi _{E}\left( z\right) =\sec h\left( z\right) =\underset{n=0}{\overset% {\infty }{\sum }}\frac{E_{n}}{n!}z^{n},$ where the constants $E_{n}$ are Euler numbers. The class $\mathcal{S}_{E}^{\ast }$ denote the class of normalized analytic functions $f$ satisfying $zf^{\prime }(z)/f(z)\prec \psi _{E}\left( z\right) $. For this class, we obtain structural formula, inclusion results, and some sharp radii problems such as radius of convexity, radius for the class of Janowski starlike functions and radius for some other subclasses of starlike functions. We also find sharp coefficient results and sharp Hankel determinants for functions in the class $\mathcal{S}_{E}^{\ast }.$

Noise is always inevitably appeared in images, which directly affects the performance of machine vision applications. Currently, the commonly used denoising methods can be divided into three strategies: the filtering-based, model-based, and deep learning-based methods. However, they are always difficult to get the considerable accuracy and efficiency simultaneously. In this study, a novel denoising method based on autocorrelation function is investigated, which improve the image quality by utilizing the independence of useful periodic information and noise. Simulations and experiments compared with the current denoising methods confirm that the investigated method has a good comprehensive effect on noise reduction and efficiency improvement.

Background: A James Lind Alliance Priority Setting Partnership (JLAPSP) was established by Fifth Sense together with UEA to identify the top 10 research questions in the field of smell and taste disorders in the United Kingdom. Methods: After steering group was established, an electronic survey was disseminated to all stakeholders (patients, healthcare professionals, family, carers, researchers) to determine the list of questions. After removing out-of-scope responses, the remainder were consolidated to create summary questions. A literature search was conducted to remove already answered questions. A second survey was used to determine the top questions that formed the subject of final debate at a workshop attended by clinicians and patients to determine the top 10 priorities. Results: The 665 respondents to the initial survey provided 1698 research questions. Thirteen were out-of-scope and removed; remaining 1685 were then consolidated to form 147 summary questions. Following literature search and discussion with the steering group, 37 questions remained for the second survey, which 235 people responded. The top ten priorities agreed in the workshop covered themes of improved understanding of pathophysiologlogy, improving health services, and managing long-term effects of smell/taste disorders. The most important research question agreed was “How can we further our understanding of the mechanism of disease in the nerve pathways that affect smell and taste disorders, including where parosmia and phantosmia exist.” Conclusions: We report the top 10 research priorities in smell and taste disorders. These priorities will now empower researchers to secure research funding and provide the basis of the Fifth Sense research hub.

Introduction Surgical trainees have to achieve a set level of operative competence under the timeframe set by the intercollegiate surgical curriculum. The operating theatre as the primary learning environment needs to be conductive to achieve successful training. In this project, we developed and validated a 27-item questionnaire which aims to evaluate the training experience of higher surgical trainees in the operating theatre. Methods Initial questionnaire was developed by reviewing existing instruments. The questionnaire was then modified in a focus group to explore themes perceived to be important to training. The modified questionnaire was sent out to obtain the content validity index (CVI) of each item. Lastly, the final version was disseminated and Cronbach’s alpha was derived to measure the internal consistency of the questionnaire. Results The Initial version of 33 questions was modified in focus group into a 29-item 4-point Likert scale questionnaire covering three areas. 14 responses were received from higher surgical trainee and consultants to determine the CVI. 27 out of 29 items reached the threshold index of 0.87. Finally, the 27-item questionnaire achieved a Cronbach’s alpha of 0.88 from 17 responses. Discussion The Cronbach’s coefficient of 0.89 in the resultant questionnaire is comparable to other existing medical education evaluation tools, which indicates a high internal consistency. Our validated questionnaire can be utilized to identify areas that needs to be improved as perceived by higher surgical trainees and to improve their training experience in theatre.

Key points instead of abstract as per author guidelines: • Chyle leaks (CLs) following neck dissection for metastatic head and neck malignancy are infrequent but represent a serious complication, with an incidence of 1-2.5%. • CL is associated with significant morbidity including metabolic imbalance, immunosuppression, dehydration, poor wound healing and prolonged length of hospital stay. • A protocolised approach to the management of CL post neck dissections is highlighted for expedient patient management. • Thoracic duct (TD) ligation using video-assisted thoracoscopic surgery (VATS) is an effective method of treating moderate (500-1000ml/24 hours) to high-volume CL (>1000ml/24 hours) not responding to medical therapy. • VATS TD ligation is a safe procedure to treat CL. It has a low morbidity compared to other surgical techniques (neck re-exploration or open thoracotomy).

A new global navigation satellite system (GNSS) spoofing signal power control algorithm under the power constraints of receiver in acquisition phase and the subsequent control is proposed in this letter. In spoofing algorithm, constraints of successful spoofing are analyzed, objective function is constructed, the power allocation optimization algorithm based on sequential quadratic programming (SQP) is proposed, and the best scheme of spoofing is given. Experimental platform is designed to prove that compared with conventional spoofing signal high power control algorithm, the new spoofing signal power control algorithm shortens Doppler frequency fluctuation time by 72.2% and reduces the range by 75.9%. The carrier-to-noise ratio of received signal is less than the threshold of receiver, and the range of three-dimensional coordinates of ECEF is significantly reduced during spoofing signal taking over receiver, This shows that the new design of spoofing signal power control algorithm can make spoofing behavior more covert, and it will make it more difficult for target receiver to detect spoofing behavior. The designed algorithm can take over receiver stealthily with the help of jamming and then pull the positioning results, which has good feasibility and effectiveness.

In recent years, many applications based on the Neural Network, Neuro-Fuzzy, and optimization algorithms have been more common for solving regression and classification problems. In the Adaptive neuro-fuzzy inference system(ANFIS), many researchers used the adaption of metaheuristic algorithms with ANFIS to propose the best estimation model. However, many researchers only focused on the experiment without the demonstration mathematical or indicating which characteristic of optimization algorithm, during the run, affect and settable in coordination with ANFIS. The paper provides an adaption of metaheuristic algorithms with ANFIS which has been performed by considering accuracy parameters in layer 1 and layer 4 for the estimation problem. It is integrated six well-known metaheuristic algorithms and extracting the characteristic of them. In the experiment, the metaheuristic algorithms based on the evolutionary computation have been demonstrated more stable than swarm intelligence methods in tuning parameters of ANFIS.

Small cell neuroendocrine carcinoma (SCNEC) of the ureter is a rare malignant tumor originating from the metaplasia of urothelial cells. This report presents a case of ureteral SCNEC that was preliminarily disclosed by transabdominal ultrasonography; thereafter, transrectal ultrasonography and magnetic resonance urography were performed to characterize the focus.