We report the case of a teenager with a neurofibromatosis Type 2 (NF2) presenting a locked-in syndrome due to a brainstem ischemic syndrome. The presence of sudden or rapidly worsening onset of neurological deficits in NF2 patients, should evoke this underknown entity and not only tumors as predisposed by NF2.

Study of antimicrobial properties of the studied heptylalkanoyldisulfides revealed effective bactericidal and fungicidal activities in suppression of fungi and bacteria allowing them to be used as biocides in storage and transportation of oil products.

It is widely admitted that the use of renewable energy is experiencing significant growth throughout the entire world. With the increasing demand for electric power, mainly for the needs of remote, desert and mountainous regions, the photovoltaic energy systems started finding great applications, particularly in telecommunication and water pumping systems.

Objectives— Early screening and dynamic monitoring of pulmonary vascular disease (PVD) in bronchopulmonary dysplasia (BPD) high-risk infants is of great clinical significance. Pulmonary artery acceleration time (PAAT) is a reliable and non-invasive method for assessing PVD in children over 1 year, but to date, few studies have used PAAT to assess pulmonary hemodynamics of preterm infants, especially those with BPD. Through dynamic monitoring the main hemodynamic indicators reflected PVD after birth, this study aimed to assess the value of PAAT in evaluating early PVD in BPD infants. Methods— 81 preterm infants at risk of BPD were divided into BPD and non-BPD groups according to whether BPD occurred. Clinical characteristics, PAAT, right ventricular ejection time (RVET) and other main hemodynamic indicators at 4 different time points after birth were studied and compared. Results— PAAT and PAAT/RVET increased gradually within 72 hours after birth in the BPD group ( P < 0.05), but the curve tended to be flat over time after 72 hours( P > 0.05). At PMA32 and 36 weeks, the PAAT (49.7±4.8 vs.54.8±5.7, P=0.001; 50.0±5.3 vs.57.0±5.3, P=0.001) and PAAT/RVET (0.33 ± 0.04 vs. 0.35 ± 0.03, P=0.001; 0.34 ± 0.03 vs. 0.37 ± 0.04, P = 0.001) in BPD group were significantly lower than those in the non-BPD group. Conclusions— PAAT and PAAT/RVET in the BPD group infants showed different change patterns compared to non-BPD group infants. PAAT can be used as a noninvasive and reliable screening method for screening and dynamic monitoring of PVD in BPD high-risk infants.

Leishmaniasis is a common cause of pancytopenia and hepatosplenomegaly in tropical and sub-tropical regions. A high index of suspicion is required to diagnose and manage patients with leishmaniasis. History of travel should always be elicited in patients with suggestive clinical presentation.

In this paper, the conformable Laguerre and associated Laguerre differential equations are solved using the Laplace transform. The solution is found to be in exact agreement with that obtained using the power series method. In addition some properties and some recursion relations of the Laguerre and associated Laguerre functions are discussed and proved. Then, the conformable Rodriguez's formula and generating function are propose

The hemispherical aortic annuloplasty reconstructive technology (HAART) is an internal geometric annuloplasty ring designed to restore a natural elliptical shape to the aortic annulus as part of aortic valve repair. We present 4D flow hemodynamic analysis before and after implementation of the HAART ring in patients undergoing ascending aortic replacement. HAART patients displayed similar or improved flow profiles when compared to a patient undergoing ascending aortic replacement alone.

Background Combined ONCAB and SAVR is the treatment of choice for concomitant severe aortic stenosis and coronary artery disease not amenable to PCI intervention. Extensive aortic calcification and atheromatous disease may prohibit cardiopulmonary bypass and aortic cross clamping. In these cases Anaortic OPCAB is a Class I (EACTS 2018) and Class IIA (AHA 2021) indication for surgical coronary revascularization. TAVR has similar benefits when compared to SAVR for this population (Partner 2 & 3). Herewith we describe a case series of concomitant Anaortic OPCAB and TAVR via the transfemoral approach for patients with coronary artery and valve disease considered too high risk for traditional CABG and SAVR due to severe aortic disease. Methods/Results Eight patients underwent anaortic OPCAB and transfemoral TAVR during the same anesthetic in a hybrid operating room. Seven patients with multi-vessel disease had anaortic OPCAB via a sternotomy using composite grafts, one patient with LAD disease had anaortic OPCAB using a Da Vinci assisted MIDCAB approach. All patients then had an Edwards Sapien 3 TAVR placed percutaneously via the common femoral artery. There was no thirty-day mortality or CVA in the series and all patients were discharged to home or a rehabilitation facility on day 4-13. Conclusions Combined anaortic OPCAB and transfemoral TAVR is a safe and feasible approach to treating concomitant extensive coronary artery disease and severe aortic stenosis. The aortic no-touch technique provides benefits in the elderly high-risk patients by reducing the risk of post-operative myocardial infarction and cerebrovascular stroke.

Outcomes of operations for total anomalous pulmonary venous connection (TAPVC) have improved.However, postoperative pulmonary venous obstruction (PVO) remains the most significant complication, with high morbidity and mortality. We introduce a window anastomosis technique for repair of supracardiac TAPVC in infants. The mainstay of the surgical technique is to resect the anterior wall of the pulmonary vein confluence(PVC) and part of the posterior wall of the left atrium to form a large and undistorted “window to window” anastomosis.

Neuroblastoma is the most common extracranial solid tumor in children. Approximately half of the patients with Stage MS disease have tumor regression, but 10-15% will have progression of disease. BRAF mutations are common in some cancers, such as melanoma and pediatric astrocytoma, but are rare in pediatric extracranial solid tumors, including neuroblastoma. Here we report the case of an infant with Stage MS neuroblastoma with a rare BRAF V600E mutation and subsequent progression to Stage M disease.

Title:Tranexamic acid-associated fatal status epilepticus in a paediatric patientAuthor:Dr Santosh Patel MD, FRCA, PG Dip (Med Edu)Consultant AnaesthetistDepartment of anaesthesiaTawam hospitalAl AinUnited Arab EmiratesCorrespondence address: as above.Email: skpatel@seha.aeFunding: No source of funding to declareConflicts of interest: Nothing to declareDear Editor,I have read with interest a case report and literature review published in the British journal of pharmacology on tranexamic acid (TXA) associated SE in a 4-year child who underwent tonsillectomy.1 I would like to congratulate the authors for reporting the case despite the fatal outcome. Considering that the use of TXA is expanding to minimise blood loss in a wide range of surgeries, an evidence-based therapeutic approach for its associated seizures is of paramount importance.The authors described in their report (also mentioned in Table 2) and concluded that general anaesthetics, propofol and halogenated inhaled anaesthetics are considered the first line of management of TXA-associated seizures due to their direct activity at glycine receptors. In support of their statement, they have quoted three references (references 35,45,46 in their report). However, in their articles, the authors did not recommend the use of general anaesthetics (propofol and halogenated inhalational anaesthetics) as the first line of treatment for TXA-related seizures. It is valuable to point out to the readers that their conclusion is not valid and needs clarification and correction.TXA-associated hyperexcitability of neural networks is because it is a competitive antagonist of glycine and GABAA receptors.2 Following IV TXA administration, not all seizures progress to status epilepticus. Although TXA-related seizures commonly manifest as generalised tonic-clonic activity, focal seizures have been reported; which are not an indication for the use of general anaesthetics. Refractory status epileptics (RSE) and super RSE are uncommon following IV TXA although this is a common feature following intrathecal TXA.3Propofol’s anticonvulsant, hypnotic, sedative and anaesthetic effects are mediated via multiple complex molecular mechanisms, including modulation of GABAA and glycine receptors. GABAA receptor modulation by propofol has distinct dose-dependent effects likely involving multiple sites of action; clinical concentrations of propofol potentiate GABA-activated currents, increase open channel frequency, and reduce the rate of desensitization, while intermediate concentrations directly activate GABAA channels, and even higher concentrations inhibit receptor function.4Propofol can cause neuroexcitatory effects, including tonic-clonic seizures, particularly during the start or weaning from propofol infusion.5 Among the various mechanisms that have been proposed for these neuroexcitatory symptoms are antagonism of glycine and dopamine receptors, hyposensitization of GABAergic pathways and dysregulated inhibition of NMDA glutamate receptors.6Its use is associated with side effects, including hypotension (and the associated use of vasopressors) and respiratory depression. With prolonged infusion, propofol infusion syndrome (PIS) may occur, which may contribute to morbidity and mortality of RSE. Children are more susceptible to developing this complication. Propofol infusion therapy is not recommended as the first line of treatment for TXA-associated seizures, and its use is reserved for severe cases in children.Inhalational anaesthetics are beneficial for the control of seizure activity via inhibition of NMDA excitotoxicity and potentiation of inhibitory functions of GABAA and glycine receptors. However, it is essential to highlight that there are several limitations to the use of inhalational anaesthetic agents. First, the only clinical evidence of their use is from the minimal number of case reports. Second, TXA-related seizures often manifest in the postoperative period in the recovery room or in ICU, where delivery and scavenging of inhalational agents via ventilator may not be feasible. Third effective end-tidal concentration and optimal therapeutic duration are not known. Finally, in higher concentrations, they cause cardiac depression and cerebral vasodilation. Therefore, their use is limited as salvage therapy for the management of TXA-associated RSE and super RSE.In summary, the authors’ conclusion is incorrect, and clinicians should follow currently available evidence-based professional guidelines to manage TXA-associated status epilepticus.8,9References:1. Aboul-Fotouh S, Habib MZ, Magdy SM, Hassan BEE. Tranexamic acid-associated fatal status epilepticus in a paediatric non-cardiac surgery: A case report and literature review. Bri J Clin Pharmacol 2022;1-6. doi:10.1111/bcp.152962. Lecker I, Wang D, Whissell P, et al. Tranexamic acid-associated seizures: causes and treatment. Ann of Neurol 2015;79(1):18-26. doi:10.1002/ana.245583. Patel S, Robertson B, McConachie I. (2019). Catastrophic drug errors involving tranexamic acid administered during spinal anaesthesia.Anaesthesia ,74(7),904-14 https://doi.org/10.1111/anae.146624. Platholi J, Hemmings H. (2022). Effects of general anesthetica on synaptic transmission and spasticity. Currt Neuropharmacol2022;20(1):27-54. doi: 10.2174/1570159X19666210803105232.5. Walder B, Tramèr MR, Seeck M. (2002). Seizure-like phenomena and propofol: a systematic review. Neurology 2002; 58(9):1327-32. 10.1212/wnl.58.9.13276. Pantelakis L, Alvarez V, Gex G, Godio M. Severe neuroexcitatory reaction: A rare and underrecognized life-threatening complication of propofol-induced anesthesia. The Neurohospitalist2021;11(1):49-53. doi: 10.1177/1941874420929536.7. Godec S, Gradisek MJ, Mirkovic T etal. Ventriculolumbar perfusion and inhalational anesthesia with sevoflurane in an accidental intrathecal injection of tranexamic acid: unreported treatment options. Reg Anesthe Pain Med 2022;47(1):65-68 10.1136/rapm-2021-1024988. Nelson SE, Varelas PN. Status epilepticus, refractory status epilepticus, and super-refractory status epilepticus. Continuum (Minneap Minn). 2018;24(6):1683-1707.9. Vossler DG, Bainbridge JL, Boggs JG etal.. Treatment of refractory convulsive status epilepticus: a comprehensive review by the American Epilepsy Society Treatments Committee. Epilepsy Curr.2020;20(5):245-64.

The Rumpel-Leede phenomenon is when the distal dermal capillaries of an extremity rupture in response to local mechanical pressure, such as an inflated blood pressure cuff or a compression band, disrupting the capillary membrane. This article presents an image of the rumpel-leede phenomenon after transulnar percutaneous coronary intervention.

Background: As elective surgical services recover from the COVID-19 pandemic a movement towards day-case surgery may reduce waiting lists. However, evidence is needed to show that day-case surgery is safe for many ENT operations including endoscopic sinus surgery (ESS). We aimed to investigate the safety of ESS in England. Methods: This was an observational, secondary analysis of administrative data. Participants were all patients in England undergoing elective ESS procedure aged ≥ 17 years during for the five years from 1st April 2014 to 31st March 2019. The exposure variable was day-case or overnight stay. The primary outcome was emergency readmission within 30 days post-discharge. Results: Data were available for 49,223 patients operated on across 129 NHS hospital trusts. In trusts operating on more than 50 patients in the study period, rates of day-case surgery varied from 100% to 20.6%. Rates of day-case surgery increased from 64.0% in 2014/15 to 78.7% in 2018/19. Day-case patients had lower rates of 30-day emergency readmission (odds ratio 0.71, 95% confidence interval 0.62 to 0.81). For secondary outcomes measures, there was no evidence of poorer outcomes for day-case patients. Outcomes for patients operated on in trusts with ≥80% day-case rates compared to patients operated on in trusts with <50% rates of day-case surgery were similar. Conclusions: ESS can safely be performed as day-case surgery at current rates. There is a potential to increase rates of day-case ESS in England, especially in departments that currently have low rates of day-case ESS.

Abstract Objectives The aim of this study was to evaluate whether adding an extra perichondrium patch to enhance the cartilage graft during endoscopic myringoplasty can improve the healing rate and post-operative hearing of patients with poor prognostic factors (eustachian tube dysfunction, large perforations, subtotal perforations and marginal perforations). Methods This retrospective study analyzed a total of 80 patients (40 females and 40 males, median age of 40.55 years) who had received an extra patch during endoscopic cartilage myringoplasty. Patients were followed up for six months. Healing rates, complications, preoperative and postoperative pure-tone average (PTA) and air-bone gap (ABG) were analyzed. Results At six months follow-up, the healing rate of tympanic membrane was 100% (80/80). The mean preoperative pure-tone average (PTA)（43.18± 14.57 dB HL）significantly decreased (27.08±9.36 dB HL) six months after the operation (P=0.002). Similarly, the mean preoperative ABG（19.05±5.72 dB HL）reduced to（9.36±3.75 dB HL） (P=0.0019) at sixth month. Major complications were not observed during follow-up. Conclusions The usage of an extra patch during endoscopic cartilage myringoplasty for large, subtotal and marginal tympanic membrane perforations achieved a high healing rate and a statistically significant hearing gain with low incidence of complications.

Schizencephaly is a rare developmental disorder of the central nervous system (CNS) marked by abnormal clefts or slits spreading from the lateral ventricle to the cerebral hemispheres.Developmental venous anomaly (DVA) is a benign venous anomaly draining cerebral parenchyma. DVAs are usually asymptomatic and do not cause any complications.

The Internet of Things (IoT) is a self-configuring, intelligent system in which things connect to the Internet and communicate with each other. As “things” are autonomous and rely on a significant amount of autonomy to carry out their individual and collective tasks, it is possible that the autonomous environment of IoT may raise privacy concerns. IoT encounters significant privacy and security challenges, including inaccurate device updates, a lack of efficient privacy solutions, user unawareness, and famed active device monitoring capabilities. In this paper, the authors describe the background of IoT systems and privacy and security measures, (a) approaches to preserving privacy in IoT-based systems, (b) existing privacy solutions, and (c) recommending privacy models for different layers of IoT applications. Based on the results of our study, it is clear that new methods such as Blockchain, Machine Learning, Data Minimization, and Data Encryption can greatly impact privacy issues to ensure security and privacy. Moreover, it makes sense that users can protect their personal information easier if there is less data to collect, store, and share by smart devices. Thus, the use of Data Minimization methods in these networks can be very beneficial for privacy-preserving, which is useful to route researchers to

RAD51 is a core factor for homologous recombination (HR) to repair DNA double strand breaks and overexpressed in breast cancer cells. Truncated peptide BRC4 (1523-1537) was obtained by computer simulation which had the highest binding free energy targeting RAD51. To enhance the binding affinity to the target protein, six nicotinic acid derivatives were modified at the N-terminal of BRC4 (1523-1537) by Fmoc solid-state synthesis to obtain nicotinamide-modified peptides. The interaction of RAD51 (181-200) with BRC4 (1523-1537) and nicotinamide-modified peptides were verified by circular dichroism (CD) spectroscopy and fluorescence spectroscopy. In conclusion, modifying small molecule pharmacophores can improve binding ability. According to spectral results, 2-chloro-5-fluoronicotinic acid modified BRC4 (1523-1537) has the most significant influence on the secondary structure of RAD51 (181-200); binding constant is 1.1×10 4 L·mol -1. Cell experiments showed that BRC4 (1523-1537) modified with nicotinic acid N-oxide had the best inhibitory effect on the proliferation of MDA-MB-231 cells.

A 30-year-old man with a structurally normal heart was referred to us with a 2-year history of recurrent episodes of rapid paroxysmal palpitations. A few episodes required hospitalization and were terminated with intravenous diltiazem. During electrophysiology (EP) study done twice before in other hospitals, the patient was diagnosed as typical atrioventricular nodal reentrant tachycardia (AVNRT) and underwent radiofrequency ablation of the slow pathway. However, the episodes recurred.  Because of the patient’s persistent symptoms, an EP study was performed again. Tachycardia was easily induced using atrial extrastimuli, ventricular extrastimuli and with rapid atrial pacing.

Arrhythmogenic cardiopathy is a genetic disease that mainly affects young men and mainly involves the right ventricle. It is responsible for up to 25% of sudden deaths in children under 35 years of age [1]. To make its diagnosis, certain criteria are required, such as the characteristic electrocardiographic alterations in sinus rhythm, the presence of documented ventricular tachycardia and structural abnormalities especially in the right ventricle [2]. We present the case of a 25-year-old male patient with a confirmed diagnosis of arrhythmogenic cardiopathy who underwent ablation with a ventricular tachycardia catheter with an endocardial -epicardial approach.

A 37-year-old woman with a history of incomplete atrioventricular canal and an extensive history of corrective cardiac surgeries presented with recurrent episodes of acute decompensated heart failure in the presence of junctional bradycardia with chronotropic incompetence. The initial plan was to proceed with implantation of a dual chamber pacemaker but given high index of suspicion for the presence of atrial standstill in the context of her extensive cardiac surgical history, we performed electroanatomical mapping and echocardiography at the time of pacemaker implantation that confirmed atrial standstill. Islands of electrically functional atrial myocardium were detected. Pacing from these locations resulted in local atrial capture, but failed to result in global atrial electrical capture, mechanical atrial contraction, or atrioventricular conduction. In light of the dismal outcomes of right ventricular pacing in patients with atrial standstill, we implanted a left bundle branch pacing lead that resulted in improvement of patient's cardiac output and prevented future admissions for decompensated heart failure in one year follow up.

Understanding the long-term characteristics of vegetation variations and their relationship to climate and human activities is important for regional sustainable development and ecological construction. Herein, the normalized difference vegetation index (NDVI) was selected as a proxy, related method and algorithm were applied to obtain the nonlinear characteristics of long-term interannual NDVI in China. Partial least squares-structural equation modeling was employed to separate the effects of climate and human activities on vegetation greening. Further, geographically weighted regression was applied to explore the spatial correlations among comprehensive forces and vegetation growth and achieve the partitioning of driving forces. The results suggested that vegetation growth in China experienced an abrupt change in 1995, there was obvious vegetation browning during 1990–1995, and noticeable vegetation recovery from 1996 to 2018. Climate was a directly main driving force for vegetation increasing in China. The positive effect of climate was the most obvious in south China, with a path coefficient of 0.348. However, climate was significantly negative to vegetation growth in northwest China (-0.049). Improving socio-economic conditions had a slightly negative impact on vegetation greening, while ecological policy played a direct and obvious role in promoting vegetation growth, especially in northwest China, with a path coefficient of 0.295. Furthermore, ecological policy would directly affect the microclimate in northwest China, strengthen the restraint effect of water resources on vegetation, and then indirectly hinder vegetation increasing. Therefore, the implementation of ecological policies should be adjusted according to regional climatic conditions, to avoid the traditional way of increasing forest (grassland) area, and reduce the contradiction between water, soil and vegetation. Actually, the indirect effect of socio-economic conditions and ecological policy on vegetation growth was far greater than its direct impact in some cases; therefore, research attention should be paid to the indirect effects of driving forces on vegetation growth

In the French Mediterranean plain, the northern extreme of its native range, the Iberian grey shrike, Lanius meridionalis, predominantly feeds on arthropods. Its type of loral plumage plays a key role in protecting its eyes while transporting large prey. The aims is to understand the role played by feathers in protecting the animal from various types of defensive prey. We combine an inspection of large insect prey types found on larders with a review of bird specimens found in museum collections to examine the morphometric characteristics of rictal feathers and culmen. In addition, precision photographs are used to observe the posture of the plumage in natura. We could identify four categories of protective feathers: clustered bristles, semi-bristles, semi-plumes distributed in the loral area, and semi-plumes above the eyes. Our results suggest that the Iberian grey shrike has a complex structure of loral feathers, specific to its foraging activity and prey types. In France, local species have longer beaks than their Spanish counterparts which suggests a more insect-based diet.

CT-imaging vs. high-density mapping in ischemic cardiomyopathy VT ablation: in whom do we trust?Thomas Fink, MD1, Vanessa Sciacca, MD1, Philipp Sommer, MD11Clinic for Electrophysiology, Herz- und Diabeteszentrum NRW, Ruhr-Universität Bochum, Bad Oeynhausen, Germany.Disclosures: PS is advisory board member of Abbott, Biosense Webster, Boston Scientific and Medtronic.Funding: (None)

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