Critical Care

Moving the critical care research agenda forward in Saudi Arabia Yaseen M Arabi, Yasser Mandourah, Fahad M Al-Hameed, Khalid Maghrabi, Mohammed S ALshahrani, Musharaf Sadat Saudi Critical Care Journal 2019 3(1):1-2

1999–2019: Twenty years of watershed moments for patient safety M Sofia Macedo, Yasser Mandourah, Anita Moore, Abdulelah AlHawsawi Saudi Critical Care Journal 2019 3(1):3-11 The case for patient safety is obvious; no one would argue in favor of harming patients. Since the launch of the paper To Err is Human, patient safety has been on the forefront of public health policymakers' priorities. Yet, 20 years later, while progress has been made, harm to patients is still a reality, daily, in health systems over the world. As countries reform their health systems, the national health programs must ensure not only the integration of universal health coverage (UHC) but also that the health coverage provided is safe. To this point, new models of care must be designed and implemented, and organizations should aim to achieve high-reliability care, similar to other industries that keep a solid safety record. This can be achieved by aiming for high-reliability organization principles, ensuring empowerment of patients as codesigners of health care, workforce safety to ensure safety of patients, and UHC without harm and proper regulation of digital health to avoid unintended adverse consequences. Since the past 20 years, the knowledge gap on patient safety has been shortened and therefore the health-care community holds a firm foundation for starting to implement evidence-based strategies that ensure safe care. The Jeddah Declaration on Patient Safety, 2019, is an actionable document that provides guidance to policy- and decision-makers globally that aim to achieve UHC free of harm. Nevertheless, given the high-level of complexity of health-care systems and its vulnerability to error, the question is what is the way forward toward a safer provision of care? How can the year 2019 be the watershed moment for the health-care industry?

Management of carbon monoxide poisoning-induced cardiac failure and multiorgan dysfunction with combined respiratory and circulatory extracorporeal membrane oxygenation AA Rabie, A Asiri, M Alsherbiny, W Alqassem, M Rajab, S Mohamed, W Hazem I Alenazi, L Ariplackal Saudi Critical Care Journal 2019 3(1):12-14 Carbon monoxide (CO) is an odorless, colorless, and nonirritant gas; it is the most common cause of poisoning and poisoning-related death. The main mechanism of CO toxicity is ischemic hypoxia secondary to hypoxemia. The heart is the major target organ of acute CO poisoning. Cardiac failure is the most common cardiac presentation; however, other cardiovascular manifestations include arrhythmia, pulmonary edema, and myocardial infarction. Recovery time from CO-induced cardiomyopathy varies from 4 days to 6 weeks. To our knowledge, there are a limited number of reported cases that demonstrated successful extracorporeal membrane oxygenation (ECMO) in adult and pediatric patients with CO poisoning and multiple organ failure. We present our experience with a case we think that it is the first case to be published for a patient with acute CO poisoning received both circulatory and respiratory support (hybrid venoarterial-venous ECMO).

Fluid administration strategies in traumatic brain injury Abdulrahman Alharthy, Waleed Tharwat Aletreby, Ibrahim Soliman, Fahad AlFaqihi, Waseem Alzayer, Nassir Nasim Mahmoud, Lawrence Marshall Gillman, Dimitrios Karakitsos Saudi Critical Care Journal 2019 3(1):15-18 Fluid restriction strategies may reduce morbidity and mortality in critical care patients and are currently trending as preliminary data showed encouraging results. A positive fluid balance was related to increase morbidity and mortality in a variety of disorders (i.e., sepsis, acute respiratory distress syndrome, and postsurgical cases) as well as resulted in an increased rate of complications observed in the intensive care unit setting. Traumatic brain injury (TBI) has been managed thus far in terms of fluid resuscitation under the concept of general trauma resuscitation recommendations that favored euvolemia above all fluid balance states. Notwithstanding, scarce data exist to clarify details about fluid management strategies in TBI such as the desirable fluid balance per se and/or its impact on patients' outcomes. We, therefore, reviewed previously published data and concluded in an observational manner (by creating a visual display model) that a highly positive and/or a negative fluid balance may have a detrimental impact on the prognosis of TBI patients. Accordingly, well-designed randomized controlled trials are clearly required to investigate further and in detail the most efficacious fluid administration strategies in TBI contributing thus in the rapidly expanding field of neurocritical care.

King Saud Medical City Intensive Care Unit: A critical and cost-focused appraisal Abdulrahman Alharthy, Dimitrios Karakitsos Saudi Critical Care Journal 2019 3(1):19-23 Intensive care unit (ICU) cost analysis has not been extensively addressed in the Kingdom of Saudi Arabia. We have implemented cost analysis (2015–2016) at the largest polyvalent ICU of the Kingdom (King Saud Medical City). Our block model analysis assimilated both modified Therapeutic Intervention Scoring System (TISS) and Omega scoring points to evaluate the overall cost; while, specific utilization elements were included in such as medication, procedural, laboratory, radiology, physiotherapy, nursing/physician, and overhead/other costs. The overall cost (Saudi Riyals [SAR]/ICU patient/day) averaged for TISS/Omega scores and adjusted for 2015–2016 inflation rates was approximately 23.269 (TISS: 167 points; Omega: 173 points generating predictive costs scores which were approximating the aforementioned score [R2 validated 0.91 and 0.90, respectively, all P < 0.005). Thereafter, we have applied effective antibiotic stewardship program and control of procedural supplies, novel administration policies, diversification of the ergonomy and clinical orientation, early mobilization of patients, increase of by-the-bed critical care ultrasound applications and decrease in the length of stay. The cost was reduced to 19.800 SAR (15%) in 2017–2018 that is comparable to international standards. Preliminary follow-up cost analysis (2019) is confirming projections of stabilizing the ICU cost <18.000 SAR (4790 USD)/patient/day. Our budget-cut policy has provided the department with a vital investment space to integrate new therapeutic technologies.

Prevention of pressure injury in the intensive care unit Hasan M Al-Dorzi Saudi Critical Care Journal 2019 3(1):24-28 Pressure injury (PI) is common in critically ill patients and is largely preventable. Prevention of PI in the intensive care unit (ICU) depends on routine risk assessment and implementation of preventive measures, such as adequate nutritional support, proper positioning and repositioning, mobilization, proper skin care, use of appropriate pressure-redistributing surfaces, and application of skin protective dressings. The available evidence suggests that a multifaceted approach is usually required. In addition, there is a need for high-quality studies to guide PI prevention in ICU patients.

Machine learning applications in critical care Mohammed Al Dhoayan, Huda Alghamdi, Yaseen M Arabi Saudi Critical Care Journal 2019 3(1):29-32 The use of machine learning (ML) applications in the intensive care units (ICUs) has surged over the last two decades. This is the result of the digital transformation that many health-care organizations have implemented. Data that are generated in the process of intensive care have more volume, velocity, and value than data generated in any other general hospital's department. This characteristic of ICUs makes them attractive environments for developing models that require rich dataset. ML has been used to develop clinical decision support system (CDSS) that could make informative decisions without requiring prior in-depth knowledge about the roots of the disease or common characteristics of the patients. The adoption of ML-based CDSS in ICUs is continuously increasing as ML algorithms achieve high levels of accuracy in descriptive, diagnostic, predictive, and prescriptive decisions. This article reviews some of the applications of ML in ICUs. This article will show examples of how ML was used for outcome predictions, such as predicting mortality and readmission. Examples in this article also include using ML for diagnostic and image recognition purposes. This review will discuss the use of ML for monitoring ICU patients, whether monitoring their physical safety with artificial intelligence vision detection algorithms, monitoring their continuous bedside measurements, or monitoring the administration and dosage of their medications. All these examples show that ML-based CDSS are on the path for a journey full of innovative and creative solutions that will increase the quality, efficiency, and effectiveness of critical care.

Neuromuscular-blocking agent use in critically ill patients Shmeylan A Al Harbi, Hind S Almodaimegh, Yaseen M Arabi Saudi Critical Care Journal 2019 3(1):33-37 Neuromuscular-blocking agents (NMBAs) are a cornerstone in the management of critically ill patients. There is evidence supporting short course (<48 h) of paralysis for patients with moderate-to-severe acute respiratory distress syndrome with PaO2/FiO2 ratio <150. Proper knowledge of these agents and their evidence-based use is fundamental. Health-care providers can play an important role in the regulation and the use of NMBAs in critically ill patients.

Management of heavy smokers in the intensive care unit Jawaher Grmaish Saudi Critical Care Journal 2019 3(1):38-42 Quitting smoking abruptly can precipitate the nicotine withdrawal syndrome, characterized by psychological and physical components. Data from critically ill patients have shown that active smokers are more likely to suffer from psychomotor agitation, self-removal of tubes and catheters, need for physical restraint, and therefore usually require higher doses of sedatives, neuroleptics, and analgesic agents. Furthermore, smokers admitted to the intensive care unit (ICU) experience delirium or agitation, which increases the length of hospital stay and the cost of medical care. Nicotine replacement therapy (NRT) has been shown to be safe and effective in the outpatient setting in smokers who intended to quit. However, the management of nicotine withdrawal symptoms in critically ill patients is controversial. Several studies have identified that treating nicotine withdrawal symptoms with NRT can be beneficial while others suggest that it can potentially increase mortality in critically ill patients. In the absence of high-quality data, NRT cannot currently be recommended for routine use to prevent delirium or to reduce hospital or ICU mortality in critically ill smokers. From the currently available data, it seems that the use of NRT in critically ill patients should be limited to selected patients where the potential benefit clearly outweighs the risk. To establish definitive conclusions regarding the use of NRT in smokers admitted to the ICU, it is necessary to carry out well-designed prospective studies with a sample of adequate size to limit the confounding factors and biases present in the current retrospective observational studies.

Central line-associated bloodstream infections in the Kingdom of Saudi Arabia Raymond M Khan, Jawad Subhani, Yaseen M Arabi Saudi Critical Care Journal 2019 3(1):43-48 Healthcare-associated infections (HAI) are a preventable cause of morbidity and mortality in the Kingdom of Saudi Arabia and internationally. They are associated with increased length of stay, mortality, antibiotics cost, and overall hospital cost. About 250,000 central line-associated bloodstream infections (CLABSI) occur in the US yearly, with a rate of 0.8 per CL-days and attributed mortality of 12%–25%. CLABSI constitutes 14.2%–38.5% of HAIs in the Kingdom, with rates varying from 2.2 to 29.7/1000 CL-days and crude device-associated mortality of 16.8%–41.9%. This article highlights the scope of the problem and outlines preventive strategies.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

Anesthesiology

Neurological complications after cardiac surgery: anesthetic considerations based on outcome evidence Purpose of review Neurological complications after cardiac surgery remain prevalent. This review aims to discuss the modifiable and outcome-relevant risk factors based on an up-to-date literature review, with a focus on interventions that may improve outcomes. Recent findings There is a close relationship between intraoperative blood pressure and postoperative neurological outcomes in cardiac surgical patients based on cohort studies and randomized controlled trials. Adopting an optimal and personalized blood pressure target is essential; however, the outstanding issue is the determination of this target. Maintaining cerebral tissue oxygen saturation at least 90% patient's baseline during cardiac surgery may be beneficial; however, the outstanding issues are effective intervention protocols and quality outcome evidence. Maintaining hemoglobin at least 7.5 g/dl may be adequate for cardiac surgical patients; however, this evidence is based on the pooled results of thousands of patients. We still need to know the optimal hemoglobin level for an individual patient, which is of particular relevance during the decision-making of transfusion or not. Summary The available evidence highlights the importance of maintaining optimal and individualized blood pressure, cerebral tissue oxygen saturation and hemoglobin level in improving neurological outcomes after cardiac surgery. However, outstanding issues remain and need to be addressed via outcome-oriented further research. Correspondence to Wei Mei, MD, Department of Anesthesiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jiefang Road, Wuhan 430030, China. Tel: +86 27 83662673; e-mail: wmei@hust.edu.cn Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Anesthesia and brain tumor surgery: technical considerations based on current research evidence Purpose of review Anesthetics may influence cancer recurrence and metastasis following surgery by modulating the neuroendocrine stress response or by directly affecting cancer cell biology. This review summarizes the current evidence on whether commonly used anesthetics potentially affect postoperative outcomes following solid organ cancer surgery with particular focus on neurological malignancies. Recent findings Despite significant improvement in diagnostic and therapeutic technology over the past decades, mortality rates after cancer surgery (including brain tumor resection) remains high. With regards to brain tumors, interaction between microglia/macrophages and tumor cells by multiple biological factors play an important role in tumor progression and metastasis. Preclinical studies have demonstrated an association between anesthetics and brain tumor cell biology, and a potential effect on tumor progression and metastasis has been revealed. However, in the clinical setting, the current evidence is inadequate to draw firm conclusions on the optimal anesthetic technique for brain tumor surgery. Summary Further work at both the basic science and clinical level is urgently needed to evaluate the association between perioperative factors, including anesthetics/technique, and postoperative brain tumor outcomes. Correspondence to Daqing Ma, Anaesthetics, Pain Medicine and Intensive Care, Department of Surgery and Cancer, Faculty of Medicine, Imperial College London, Chelsea & Westminster Hospital 369 Fulham Road, London SW10 9NH, UK. Tel: +44 203315 8495; fax: +44 203315 5109; e-mail: d.ma@imperial.ac.uk Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Neuroanesthesia and outcomes: evidence, opinions, and speculations on clinically relevant topics Purpose of review The objective of this review is to identify outstanding topics most relevant to neuroanesthesia practice and patient outcomes. We discuss the role of awake craniotomy, choice of general anesthetic agents, monitoring of anesthetic 'depth', mannitol-induced diuresis, neurophysiological monitoring, hyperventilation, and cerebral hypoperfusion. Recent findings Awake craniotomy, although a technique likely underused, is associated with enhanced recovery after surgery and prolonged survival after brain tumor resection compared with surgery under general anesthesia. The choice of general anesthetic must balance patient and surgical factors. Although propofol may be associated with favorable oncologic outcomes, currently available retrospective evidence does not specifically address neurosurgical patients. Both the definition and monitoring of anesthetic 'depth' remains elusive. Neuroanesthesiologists need to recognize and manage intraoperative light anesthesia in a timely fashion. Further evidence related to the optimal management of mannitol-induced diuresis and hyperventilation in neurosurgical patients is needed. Contemporary neurophysiological monitoring can reasonably detect intraoperative neurologic injury; however, its effect on patient outcome is unclear. Finally, cerebral hypoperfusion without stroke may be common; however, the clinical significance requires further investigation. Summary We provide an overview of several topics that are relevant to neuroanesthesia practice and patient outcomes based on evidence, opinions, and speculations. Our review highlights the need for further outcome-oriented studies to specifically address these clinically relevant issues. Correspondence to Lingzhong Meng, MD, Professor and Division Chief, Department of Anesthesiology, Yale University School of Medicine, 333 Cedar Street, TMP 3, PO Box 208051, New Haven, CT 06520, USA. Tel: +1 203 785 2802; e-mail: lingzhong.meng@yale.edu Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Anaesthesia for stroke thrombectomy: technical considerations based on outcome evidence Purpose of review Stroke is the second leading cause of death and the third leading cause of disability worldwide. Treatment is time limited and delays cost lives. This review discusses modern stroke management, during a time when treatments and guidelines are rapidly evolving. Recent findings Stroke thrombectomy has become the therapy of choice for large vessel occlusion (LVO) strokes. Perfusion imaging techniques, both computed tomography (CT) and MRI, now allow treatment beyond a set time window in specific patients. Both general anaesthesia and conscious sedation are options for patients undergoing stroke thrombectomy. Summary An individualized approach to the patient's anaesthetic management is optimal, and depends on close communication with the neurointerventionalist regarding patient and procedure-specific variables. No specific anaesthetic agent is preferred. Guiding principles are minimization of time delay, and maintenance of cerebral perfusion pressure. Correspondence to David L. McDonagh, MD, 5323 Harry Hines Blvd., Dallas, TX, USA. Tel: +1 214 648 6400; e-mail: David.mcdonagh@utsouthwestern.edu Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Neurophysiological monitoring during neurosurgery: anesthetic considerations based on outcome evidence Purpose of review This article reviews the recent outcome studies that investigated intraoperative neurophysiological monitoring (IONM) during spine, neurovascular and brain tumor surgery. Recent findings Several recent studies have focused on identifying which types of neurosurgical procedures might benefit most from IONM use. Despite conflicting literature regarding its efficacy in improving neurological outcomes, many experts have advocated for the use of IONM in neurosurgery. Several themes have emerged from the recent literature: the entire perioperative team must always work together to ensure adequate communication and intervention; systems and checklists, in which each member of the perioperative team has a clearly defined role, can be useful in the event of a sudden intraoperative changes in electrophysiological signals; regardless of the IONM modality used, any sudden change in electrophysiological signal should prompt an immediate and appropriate intervention; a multimodal IONM approach is often, but not always, advantageous over a single IONM approach. Summary For neurosurgical procedures that can be complicated by neural injury, the use of IONM should be considered according to specific patient and surgical factors. Future studies should focus on improving IONM technology and optimizing sensitivity and specificity for detecting any impending neural damage. Correspondence to Shaun E. Gruenbaum, MD, PhD, Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Jacksonville, FL 32224, USA. Tel: +1 904 956 3398; e-mail: gruenbaum.shaun@mayo.edu Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Awake craniotomy: anesthetic considerations based on outcome evidence Purpose of review This review highlights anaesthesia management options for awake craniotomy and discusses the advantages and disadvantages of different approaches, intraoperative complications and future directions. Recent findings For lesions located within or adjacent to eloquent regions of the brain, awake craniotomy allows maximal tumour resection with minimal consequences on neurological function. Various techniques have been described to provide anaesthesia or sedation and analgesia during the initial craniotomy, and rapid return to consciousness for intraoperative testing and tumour resection; there is no evidence that one approach is superior to another. Although very safe, awake craniotomy is associated with some well recognized complications; most are minor and self-limiting or easily reversed. In experienced hands, failure of awake craniotomy occurs in fewer than 2% of cases, irrespective of anaesthesia technique. Although brain tumour surgery remains the most common indication for awake craniotomy, the technique is finding utility in other neurosurgical procedures. Summary Several anaesthetic approaches are available for the management of patients during awake craniotomy. The choice of technique should be based on individual patient factors, location and duration of surgery, and anaesthesiologist expertise and experience. Appropriate patient selection and excellent multidisciplinary team working is associated with high levels of procedural success and patient satisfaction. Correspondence to Martin Smith, MBBS, FRCA, FFICM, Department of Neuroanaesthesia and Neurocritical Care Unit, The National Hospital for Neurology and Neurosurgery, University College London Hospitals, Queen Square, London WC1N 3BG, UK;. E-mail: martin.smith@ucl.ac.uk Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Anesthesia and airway management for gastrointestinal endoscopic procedures outside the operating room Purpose of review To review the anesthestic and airway management for gastrointestinal procedures outside of the operating room. Recent findings The number of gastrointestinal endoscopic procedures performed is steadily increasing worldwide. As complexity, duration and invasiveness of procedures increase, there is ever greater requirement for deeper sedation or general anesthesia. A close relationship between anesthetic practitioners and endoscopists is required to ensure safe and successful outcomes. The American Society of Gastrointestinal endoscopy and the British Society of Gastroenterology have recently released guidelines for sedation and general anesthesia in gastrointestinal endoscopy, highlighting the need for careful monitoring for all cases, and anesthetic expertise in complex cases. The recent advances in high-flow nasal oxygenation in sedation may provide alternative options for oxygenation during gastrointestinal sedation, especially in deep sedation and this may reduce the need for general anesthesia. Summary The advances in gastrointestinal endoscopic intervention have increased the requirement for deep sedation and anesthetic involvement outside of the operating room. Careful titration of anesthetic intervention and close monitoring are required to ensure patient safety. Correspondence to Jaideep J. Pandit, St John's College, Oxford OX1 3JP, UK. Tel: +44 1865 221590; e-mail: jaideep.pandit@dpag.ox.ac.uk Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Anesthesia practice for endovascular therapy of acute ischemic stroke in Europe Purpose of review Anesthetic assistance is often required during endovascular therapy (EVT) of large vessel occlusion in patients with acute ischemic stroke. It is currently debated whether EVT should be performed under general anesthesia or conscious sedation. This review will summarize the recent literature with emphasis on the influence of anesthesia method on neurological outcome. Recent findings Recent randomized trials have reported no difference in outcome after EVT performed under either conscious sedation or general anesthesia. This is in contrast to a substantial number of retrospective studies, which found that EVT performed under general anesthesia was associated with a worse neurologic outcome compared with conscious sedation. Anesthetic drugs affect vessel tone and the level of blood pressure may influence outcome. The most favorable choice of anesthetic agents and ventilatory strategy is still debated. Summary The optimal anesthetic practice for EVT remains to be identified. Currently, conscious sedation is often an easy first-line strategy, but general anesthesia can be considered an equal and safe alternative to conscious sedation when there is a carefully administered anesthetic that maintains strict hemodynamic control. Attention to ventilation is advocated. The presence of a specialized neuroanesthesiologist or otherwise dedicated anesthesia personnel is highly recommended. Correspondence to Mads Rasmussen, Section of Neuroanesthesia, Department of Anesthesia, Aarhus University Hospital, Nørrebrogade 44, 8000 Aarhus C, Denmark. Tel: +45 30566977; e-mail: mads.rasmussen@vest.rm.dk Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Nonoperating room anesthesia education: preparing our residents for the future Purpose of review Nonoperating room anesthesia (NORA) is the fastest growing segment of anesthetic practice. This review provides an overview of knowledge and trends that will need to be introduced to residents as part of their education. Recent findings Topics for the future include, but are not limited to, new medications, artificial intelligence and big data, monitoring depth of hypnosis, translational innovation and collaboration, demographic changes, financial driving forces, destination hubs, medical tourism, and new approaches to education training and self-management. Summary Implementing new medical technologies for anesthesia outside the operating room will help to successfully master this ever evolving subspecialty. Anesthesiologists require specific preparation for the diverse settings that they will encounter during their training. In this rapidly changing field, cognitive fitness must be factored into teaching and evaluation of residents. We describe the most important topics to consider when educating anesthesiology residents, and highlight research that addresses upcoming challenges. Correspondence to Steven D. Boggs, MD, MBA, Department of Anesthesiology, The University of Tennessee School of Health Sciences Memphis, Chandler Building, Suite 600, 877 Jefferson Avenue, Memphis, TN 38103, USA. Tel: +1 901 448 5988; e-mail: sboggs6@uthsc.edu Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Anesthesia-administered sedation for endoscopic retrograde cholangiopancreatography: monitored anesthesia care or general endotracheal anesthesia? Purpose of review The decision to undertake monitored anesthesia care (MAC) or general endotracheal anesthesia (GEA) for patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) is influenced by many factors. These include locoregional practice preferences, procedure complexity, patient position, and comorbidities. We aim to review the data regarding anesthesia-administered sedation for ERCP and identify the impact of airway management on procedure success, adverse event rates and endoscopy unit efficiency. Recent findings Several studies have consistently identified patients at high risk for sedation-related adverse events during ERCP. This group includes those with higher American Society of Anesthesiologists class and (BMI). ERCP is commonly performed in the prone position, which can make the placement of an emergent advanced airway challenging. Although this may be alleviated by performing ERCP in the supine position, this technique is more technically cumbersome for the endoscopist. Data regarding the impact of routine GEA on endoscopy unit efficiency remain controversial. Summary Pursuing MAC or GEA for patients undergoing ERCP is best-approached on an individual basis. Patients at high risk for sedation-related adverse events likely benefit from GEA. Larger, multicenter randomized controlled trials will aid significantly in better delineating which sedation approach is best for an individual patient. Correspondence to Zachary L. Smith, DO, University Hospitals Digestive Health Institute, 11100 Euclid Ave, Wearn 2nd Floor, Cleveland, OH 44106, USA. Tel: +1 216 844 6172; fax: +1 216 844 7480; e-mail: zachary.smith2@uhhospitals.org Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

AIDS and Behavior

HIV Prevalence and Sexual Behaviors Among Transgender Women in Tehran, Iran Abstract To date, no study has looked at the prevalence of HIV and the high-risk behaviors among transgender women in Iran. Between May 2013 and February 2014, 104 transgender women were recruited for participation in this study. Inclusion criteria consisted of having an official letter from the Tehran Psychiatric Institute, or a well-known psychiatrist, that showed a diagnosis of gender dysphoria and/or completed Gender-Affirming Surgery at least 6 months prior to this study. Of the 104 participants, 2 were diagnosed with HIV, which translates to a HIV prevalence of 1.9%. Condom use with a non-paying partner, casual partner, and paying partner was respectively 39.7%, 34.6%, and 53.3%. A high percentage of transgender women in Tehran engage in high-risk sexual behaviors including condomless receptive anal sex, which is of particular concern given the low rates of HIV testing. Targeted public intervention programs and research are desperately needed for this high-risk group.

Undiagnosed and Untreated HIV Infection Among Persons Who Inject Drugs: Results of Three National HIV Behavioral Surveillance Surveys, San Francisco, 2009–2015 Abstract We examined three waves of National HIV Behavioral Surveillance surveys of persons who inject drugs (PWID) in San Francisco to assess meeting UNAIDS 90–90–90 targets. Diagnosis of PWID living with HIV increased from 64.4% in 2009 to 80.5% in 2015. Antiretroviral treatment among those diagnosed did not improve (63.8% in 2009, 62.9% in 2015). Programs in San Francisco have not achieved the first two UNAIDS targets for PWID by 2015. In a context of a rising opioid epidemic, there is urgent need for increased case finding of PWID living with HIV who are undiagnosed with rapid linkage to treatment.

Estimated Population Size of Men Who Have Sex with Men, San Francisco, 2017 Abstract Current population size estimates of communities at risk for HIV are needed to adequately plan interventions and care. We update the estimated number of men who have sex with men (MSM) living in San Francisco. Multiple data sources and approaches, including population growth, registered HIV cases, wisdom of the crowd, and a published estimate on the proportion of adults who are MSM were used for estimation. The several estimates were synthesized into a final figure with acceptable bounds using a Bayesian method. We project 69,974 (acceptable bounds 65,523–74,323) MSM are living in San Francisco as of the beginning of 2017. The population of MSM in San Francisco has increased by 19.4% since 2014.

Alcohol Use and Sexual Risk Behavior in Young Women: A Qualitative Study Abstract Alcohol use and sexual behavior co-occur frequently in young women, increasing risk for HIV and other sexually transmitted infections. To inform preventive interventions, we used qualitative methods to better understand how women think about the contribution of alcohol use to sexual risk-taking. Young women (N = 25; M = 22.8 years; 64% White) were recruited from a community-based reproductive health clinic to attend focus groups; a semi-structured agenda was used to investigate both a priori explanatory mechanisms as well as participant-driven explanations for the alcohol-sex association. Women reported that alcohol reduced their social anxiety, helped them to feel outgoing and confident, and lowered inhibitions and other barriers to sexual encounters (consistent with alcohol expectancies). During drinking events, women described being less concerned with risks, less discriminating regarding sexual partners, and less likely to insist on safer sex practices (consistent with alcohol myopia). These empirical findings support previous theory-based guidance for tailoring preventive programs for alcohol use and sexual risk reduction for young women.

Approaches to Identify Unknown HIV-Positive Men Who Have Sex with Men in Nairobi, Kenya Abstract Kenya has been home to one of the most severe HIV/AIDS epidemics in Sub-Saharan Africa. This persistent epidemic requires interventions tailored to affected populations, particularly men who have sex with men (MSM). Given the resource constraints of many clinics and ecological challenges of Kenya, such as the illegality of sex among MSM, interventions to address HIV must strategically engage this population. This quasi-experimental pilot study of N = 497 sought to explore differences in discovering previously unknown HIV-positive MSM in Nairobi, Kenya. The study used four clinical sites to compare a social and sexual network index testing (SSNIT) strategy compared to traditional HIV screening. Clinics using the SSNIT strategy had significantly higher incidence rates of HIV diagnoses than control clinics (IRR = 3.98, p < 0.001). This study found that building upon the social and sexual networks of MSM may be one promising strategy while discovering critical cases of HIV.

The Association Between Condomless Anal Sex and Social Support Among Black Men Who Have Sex With Men (MSM) in Six U.S. Cities: A Study Using Data from the HIV Prevention Trials Network BROTHERS Study (HPTN 061) Abstract We assessed how egocentric (i.e., self-generated descriptions of a person's social contacts) network structure and composition corresponded with reported instances of condomless receptive and insertive anal intercourse with men who were reportedly HIV-infected or of unknown HIV serostatus in a sample of black men who have sex with men (MSM) in six U.S. cities. Ratings showing a higher percentage of network members who provided social participation and medical support were positively associated with reporting condomless sex. There were also significant positive associations between stimulant use and condomless insertive and receptive anal sex. Future research should examine the social processes that underlie these associations and explore ways that social support can affect HIV prevention efforts for black MSM.

Syndemic Classes, Stigma, and Sexual Risk Among Transgender Women in India Abstract Syndemic theory could explain the elevated HIV risk among transgender women (TGW) in India. Using cross-sectional data of 300 TGW in India, we aimed to: identify latent classes of four syndemic conditions (Depression-D, Alcohol use-A, Violence victimization-V, HIV-positive status), test whether syndemic classes mediate the association between stigma and sexual risk, and test whether social support and resilient coping moderate the association between syndemic classes and sexual risk. Four distinct classes emerged: (1) DAV Syndemic, (2) AV Syndemic, (3) DV Syndemic, and (4) No Syndemic. TGW in the DAV Syndemic (OR 9.80, 95% CI 3.45, 27.85, p < 0.001) and AV Syndemic classes (OR 2.74, 95% CI 1.19, 6.32, p < 0.01) had higher odds of inconsistent condom use in the past month than the No Syndemic class. Social support significantly moderated the effect of DAV Syndemic class on inconsistent condom use. DAV Syndemic was found to be a significant mediator of the effect of transgender identity stigma on sexual risk. HIV prevention programs among TGW need to: (a) incorporate multi-level multi-component interventions to address syndemic conditions, tailored to the nature of syndemic classes; (b) reduce societal stigma against TGW; and (c) improve social support to buffer the impact of syndemics on sexual risk.

Role of Social Network Sexual Norms and Behaviors on the HIV Sexual Risk Behaviors of People Who Inject Drugs in HPTN 037 Abstract This study examined the effect of social network descriptive sexual norms and behaviors on the sexual behaviors of people who inject drugs (PWID). Data from HPTN037 of 232 PWID (egos) and 464 network members (alters) were used in multilevel multivariate logistic regression models. Egos whose alters reported multiple sex partners had greater odds of multiple sex partners (aOR 2.20, 1.13–4.29). Egos' norms of condomless sex with primary (aOR 2.67, 1.15–6.17) and casual (aOR 2.38, 1.01–5.59) partners and egos' norms of giving (aOR 5.52, 1.87–16.25) and receiving (aOR 7.38, 1.34–40.66) money/drugs for sex were associated with the egos' respective behaviors. History of sex between an ego and alter was not associated with increased influence of alters' norms and behaviors on egos' sexual behavior. Findings provide support for developing interventions that target descriptive norms and selective network behavioral characteristics to decrease sexual HIV risk behavior among PWID.

The Intersection of HIV, Social Vulnerability, and Reproductive Health: Analysis of Women Living with HIV in Rio de Janeiro, Brazil from 1996 to 2016 Abstract Comprehensive care for sexual and reproductive health (SRH) and social needs for women living with HIV remains limited globally. We aimed to assess trends in baseline sociodemographic, clinical, sexual, and reproductive characteristics among a cohort of HIV-infected women in Rio de Janeiro from 1996 to 2016. Participants were stratified into four time periods based on year of enrollment; we compared cross-sectional data from each period. Of 1361 participants (median age 36), most were black or mixed race (60.1%), unemployed (52.1%), and without secondary education (54%). Adolescent pregnancy was common (51.5%), and 18.3% reported sexual debut at < 15 years old. Nearly half (45.2%) had < 5 lifetime sexual partners, yet prior syphilis and oncogenic human papillomavirus prevalence were 10.9% and 43.1%, respectively. Lifetime prevalence of induced abortion was 30.3%, and 16% used no contraceptive method. Future research should explore interactions between social vulnerability, HIV, and poor SRH outcomes and healthcare models to alleviate these disparities.

Dual Unsafe Injection and Sexual Behaviors for HIV Infection Among People Who Inject Drugs in Iran Abstract We used two national surveys (2010: N = 1597; 2013: N = 1057) of people who inject drugs (PWID) in past-month to assess the prevalence and population size of PWID with either safe or unsafe injection and sex behaviors, overall and by HIV status. In 2013, only 27.0% (vs. 32.3% in 2010) had safe injection and sex, 24.6% (vs. 23.3% in 2010) had unsafe injection and sex, 26.4% (vs. 26.5% in 2010) had only unsafe injection, and 22.0% (vs. 18.0% in 2010) had unsafe sex only. Among HIV-positive PWID in 2013, only 22.1% (~ 2200 persons) had safe injection and sex, 14.2% (~ 1400 persons) had unsafe injection and sex, 53.1% (~ 5200 persons) had unsafe injection, and 10.6% had unsafe sex (~ 1100 persons). Among HIV-negative PWID in 2013, only 27.5% (~ 22,200 persons) had safe injection and sex, 25.9% (~ 20,900 PWID) had unsafe injection and sex, 23.2% (~ 18,700 persons) had unsafe injection, and 23.3% (~ 18,800 persons) had unsafe sex. HIV-positive and -negative PWID in Iran continue to be at risk of HIV acquisition or transmission which calls for targeted preventions services.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

Health Sciences

Modeling and designing indices of talent identification in the field of basketball based on physical-motor, psychological, anthropometric, and physiological parameters Keshavarz Loghman, Farahani Aboalfazl, Zarei Bidsorkhi Ali International Archives of Health Sciences 2019 6(2):59-64 Aims: The purpose of this study was to modeling and designing indices of talent identification (TID) in the field of basketball based on physical-motor, psychological, anthropometric, and physiological parameters. Materials and Methods: The research method was qualitative (grounded theory) that was done on the field by interpretive analysis of deep interviews, including 21 experts in; TID (among professional clubs), basketball coaches (among premier league), and university professors (among professors of Tehran University, Shahid Beheshti University, and Kharazmi University). The validity of the interviews was confirmed by verifying the views of experts in the final results, and the reliability of the interviews was also confirmed through process audits and intrasubject agreement of two coders (84%). The analysis of interviews was done through interpretive analysis technique using open coding, axial coding, and selective coding. Results: As a result of the analysis of the interviews, 10 categories, 30 subcategories, and 101 key concepts were extracted. After axial and selective coding of the categories, a paradigm model was presented for TID in basketball. Conclusions: Finally, further explanations and suggestions are provided for each of the categories.

Taxation for reducing purchase and consumption of sugar-sweetened beverages: A systematic review Seyyed Reza Sobhani, Mina Babashahi International Archives of Health Sciences 2019 6(2):65-72 Aims: Noncommunicable diseases (NCDs) are common in worldwide and lead to a dramatic rise in mortality. Excess consumption of sugar due to dietary changes can lead to arising calorie intake that contributes to weight gain, adiposity, and NCDs. Taxes, subsidies, and other economic executive policy have a key role in discouraging the consumption of unhealthy food. This study was aimed at a systematic review of recent research evidence about the tax impacts on the purchase and consumption of sugar-sweetened beverages (SSBs). Materials and Methods: Five databases, including PubMed, Scopus, Science Direct, CENTERAL, and EMBASE, were systematically searched from 2000 to May 2017. Results: Seven studies were included in this review. In six of the seven experimental studies reviewed demonstrated that consumers can be responsive to changes in food and drink prices. Taxing SSBs effectively could decrease food purchases, increasing SSBs price, and reducing consumption. Reducing the consumption of these beverages results in reduced overweight, obesity, and body mass index among populations. Conclusion: The current evidence base appears to converge and suggests that the fiscal strategy is likely to reduce purchases of high sugar products at least in the short term and likely can lead to decreasing calorie intake.

Extranodal nasofacial natural killer/T-Cell lymphoma often missed by clinician Santosh Kumar Swain, Prabodh Kumar Das, Mahesh Chandra Sahu International Archives of Health Sciences 2019 6(2):73-77 Aim: This study aims to review the current literature and to focus on etiopathogenesis, clinical profile, diagnosis, and treatment of extranodal nasofacial natural killer (NK)/T-cell lymphoma. Materials and Methods: It is based upon the available literatures from PubMed, Scopus, and Google scholar with the keywords: etiopathogenesis, clinical pictures, diagnostic methods, and current treatment of extranodal nasofacial NK/T-cell lymphoma from 2002 to 2017. Results: Primary nasofacial lymphoma is a rare form of malignancy in head and neck area. Extranodal nasofacial NK/T-cell lymphoma is an unusual clinical entity, which is an aggressive entity of non-Hodgkin's lymphoma with distinct clinicopathological pictures. It is possibly associated with Epstein–Barr virus infection. It is highly aggressive disease with poor prognosis. Nasofacial NK/T-cell lymphoma or lethal midline granuloma is often associated with destruction of midface and surrounding areas such as orbit, paranasal sinuses, and palate. The clinical picture is highly variable, often missed by clinician and depends on location and histopathological type of the lesion. Histopathological and immunohistochemistry are important tools for diagnosis of nasofacial NK/T-cell lymphoma. Histopathological picture shows angiocentric and angiodestructive pattern of tumor cells which often mimic vasculitis. Radiotherapy is the treatment of choice which improves quality and longevity of life whereas addition of chemotherapy gives additional benefit to the patients. Conclusion: Practicing physicians and otorhinolaryngologist need to be aware of this nonspecific presentation of lesion to prevent delay in diagnosis. Early diagnosis and intervention prolongs the survival of the patients.

Evaluation of evolutionary status of 4–60-month-old children in Kashan and Aran-Bidgol and its related factors in 2016–2017 Seyed-Alireza Moraveji, Hamideh Ghaffarian, Fatemeh Atoof, Davarkhah Rabbani, Maryam Ahmadishad International Archives of Health Sciences 2019 6(2):78-83 Background: Paying attention to Children's evolutionary status help to improve their mental capacity and make them more intelligent and creative adults. The present study aimed to investigate the developmental status in 4–60-month-old children of Kashan and Aran-Bidgol and its related factors in 2016–2017. Methods: This descriptive cross-sectional study was performed on 656 children aged 4–60 months in Kashan and Aran-Bidgol that were recruited by a stratified random sampling method. Data were collected through self-report using Ages and Stages Questionnaire (ASQ) and demographic and clinical questionnaire. The ASQ consists of 30 questions in five domains including communication, gross motor, fine motor, problem-solving, and personal-social skills. The inter-rater agreement coefficient of 93%, the Cronbach's alpha coefficient of 0.51–0.87, and the 2-week test–retest correlation of 0.75 are reported for this questionnaire. Data were analyzed using descriptive statistics as well as Chi-square, ANOVA, and t-test. The significance level was considered <0.05. Results: The results showed that 527 (80.3%) of the 656 children were healthy, and the rest had a disorder at least in one of the developmental domains, including 1.5% disorders in communication skills, 6.2% in gross motor, 1.2% in fine motor skills, 1.2% in problem-solving, and 0.9% in the personal-social area. There was a significant relationship between developmental disorders with the child's gender and birth weight. Among maternal characteristics, there was a significant relationship between the mother's job and age with developmental delay. Conclusion: Despite the normal evolution in most children of Kashan and Aran-Bidgol, a significant number of them had a disorder at least in one of the evolutionary domains. It is essential to sensitize the families about the developmental process of their children and encourage them to alleviate the risk factors to prevent early complications of these disorders in the future.

Burnout among the Nurses of Kashan Beheshti Hospital During 2014 Manijeh Kadkhodaei, Gholam Abbass Moosavi, Roya Seyyedi International Archives of Health Sciences 2019 6(2):84-88 Aims: Research suggests that burnout levels have always been higher in the health-care populations and the nurses were at high risk too. It can increase staff turnover and reduce the quality of care. Hence, this study was performed with the purpose of evaluating the rate of professional burnout in the nurses of Beheshti hospital's staff. Materials and Methods: The study population comprised 230 nurses of the Beheshti hospital of Medical Sciences using sampling in 2014. Maslach Burnout Inventory and staff demographic characteristics questionnaire were used in this study. Data were analyzed using the SPSS software, Pearson's independent, Fisher's exact test, ANOVA, and Chi-square test. Results: Most of the participants were in mild-to-moderate levels of exhaustion. they had mild level until severe in depersonalization, and mild in reduced personal accomplishment. however, burnout was higher in women . Health-care staff nurses had a higher level of job burnout than that of administrative staffs (P < 0.001). Conclusions: The findings suggest the research focused could yield important advances in understanding burnout in this group and yield potential interventions to buffer burnout and its consequences.

Comparing Two Instructing Methods: Instructor-Based (Traditional Lecture) and Student-Based (Class Conference), in Healthcare Learning in Kashan Medical Science University Ebrahim Koochaki, Monika Motaghi International Archives of Health Sciences 2019 6(2):89-92 Aims: Evaluating different methods of profound learning and efficient memorizing is a vital solution for medical science students because of the importance of this science. The present study overviews the comparison between the traditional instructing and the class conference methods. Materials and Methods: This quasi-experimental study was conducted on 50 students of Kashan in 2016 and 2017 selected by available sampling method. For the first group (25 students), instructing was performed via traditional lecture, and the second (25 students) experienced class conference approach. At the end of each semester, the satisfaction questionnaire assessment and knowledge of the students distributed among the subjects and filled out, then analyzed by SPSS 16 software. Results: The mean scores obtained from traditional lecture and class conference approaches were 95 with 4.2 ± 0.41 and 96 with 4.44 ± 0.36, respectively (T = 0.6, P = 0.06), which demonstrated no significant difference. Furthermore, the students' satisfaction level of lecture and conference was measured as 3.87 ± 0.41 and 4.58 ± 0.25, respectively, which revealed a significant difference (P = 0.04). Conclusion: Although the two instructing approaches used in this study did not considerably affect the final scores of the students, and no statistically significant difference was observed among the students for the two methods, class conference could mostly draw attention of the students. Therefore, extended research on applying student-based rather than instructor-based approaches is suggested.

Relationship between developmental assets and addiction potential with regard to mediating role of alexithymia in adolescents of Arak, Iran Akram Mazloomi, Hossein Davoudi, Hasan Heidari, Mohammad Asgari International Archives of Health Sciences 2019 6(2):93-100 Aims: This study aimed at presenting a model for addiction potential based on developmental assets mediating by alexithymia in adolescents living in Arak, Iran. Materials and Methods: Five hundred members were selected as samples (n = 500) among female and male second-grade high school students at the tenth and eleventh academic grades in Arak using cluster random sampling. To collect data, Iranian Addiction Potential Scale, Toronto Alexithymia Scale and Developmental Assets Profile were used. The data were analyzed using Structural Equation Modeling and LISREL Software. Results: Model fit indices had suitable model fit with data. Internal developmental assets with path coefficient of B = −0.48 were more effective in reducing addiction potential compared with external developmental assets with path coefficient of B = −0.27. Positive identity, empowerment, and social competency were the most effective components of developmental assets in reducing addiction potential. Internal assets could explain addiction potential in adolescence more than external assets. Conclusion: Direct effect of developmental assets on addiction potential was confirmed and its indirect effect with mediation of alexithymia was significant. Moreover, results showed that only internal developmental asset had an effect on addiction potential in adolescents mediating by alexithymia, so the effect of external developmental asset on addiction potential in adolescents based on the mediation of alexithymia was rejected.

The effect of acceptance and commitment therapy on the conflict resolution styles of incompatible marital women Maryam Farahanifar, Hasan Heidari, Hosein Davodi, Seyed Ali Aleyasin International Archives of Health Sciences 2019 6(2):101-107 Aim: Acceptance and commitment therapy (ACT) is one of the third-generation behavioral therapies in which it is attempted to increase the psychological relationship of an individual with his or her thoughts and feelings instead of changing cognition. The purpose of this study was the effectiveness of ACT on conflict resolution styles of incompatible marital women. Methods: This is a semi-experimental, pretest and posttest design and a 3-month follow-up. The statistical population of the study consisted of all incompatible women who referred to counseling centers in Arak in 2018. Therefore, 24 participants were selected by convenience sampling method and randomly assigned to one test groups and one control group of 12 participants for each. Data were collected in the pretest, posttest and follow-up stages with (the Conflict Resolution Styles Scale or Rahim Organizational Conflict Inventory-II). The test group received a therapeutic intervention based on ACT for twelve 90-min sessions, but no therapy was provided for the control group. After the completion of treatment sessions, both groups were subjected to posttest. The data were analyzed using analysis of variance with repeated measures and Bonferroni post-hoc test. Results: The results showed that there was a statistically significant difference between the posttest scores of the test group compared with that of the control group (P < 0.05), and the difference was suitably sustainable during the time. Conclusion: ACT is considered as an effective intervention in improving conflict resolution styles.

The effect of visual training on the rate of performance accuracy in girl soccer players Azam Afshar, Jaleh Baqerli, Morteza Taheri International Archives of Health Sciences 2019 6(2):108-113 Aims: The purpose of this study was to determine the effect of visual training on the performance of shooting skills of soccer girl players. Materials and Methods: The research method was quasi-experimental, in which the participants were assigned into experimental and control groups, with pre-test and post-test design. The participants were girl soccer players in Alborz province. In this study, among the community, 45 athletes with an average age of 19.3 ± 1.4 years were selected from club in Alborz province. The research tool was based on Raven and Gibor's vision exercises and a researcher-made exercise protocol (colored gates, colored caissons). Specific visual training and researcher-made sports vision exercises were performed for 2 weeks (three sessions per week and 12 min each session). The covariance analysis and Bonferron's post hoc test were used to analyze the data. Results: The results of the study showed that there was a significant difference between the rate of performance accuracy of soccer players in visual training, athletic-based visual training, and control groups (P = 0.000). It was also indicated that visual training had no significant effect on the performance of soccer girls' shots (P = 0.003). However, sports–visual exercises improved their shooting skills (P = 0.002). Conclusion: Specific visual training could be more beneficial in improving the performance of soccer girls' shots.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

Acta Neurologica

MRI findings in liquor hypotension syndrome

Dynamic thiol/disulphide homeostasis in children with Duchenne muscular dystrophy Abstract Duchenne muscular dystrophy (DMD) is a disorder that alter the expression of the dystrophin protein. Dystrophin deficiency alters the structural integrity of the contractile apparatus/sarcolemmal integrity, leading to dystrophic changes. Dystrophin deficiency results in an increase in oxidative stress. We aimed to investigate the thiol/disulfide balance as an oxidative stress marker in children with DMD. We included 24 DMD, and 22 healthy control group subjects in the study. The total thiol, native thiol, and disulphide levels were measured and the disulphide/native thiol, disulphide/total thiol and native thiol/total thiol ratios were calculated in DMD patients and healthy subjects. The mean age distribution of the patients and the healthy control group subjects was similar. The total thiol, native thiol, and disulfide levels were lower in DMD group than the healthy controls. In conclusion, the markers and ratios were measured and calculated in the blood, and we detected that the total thiol, and native thiol levels were lower in DMD group than the healthy controls. These results indicate that dynamic thiol-disulphide homeostasis can be used as a marker of oxidative stress in clinical trials with DMD.

Distal 1q21.1 and proximal 1q21.2 microduplication in a child with attention-deficit hyperactivity disorder

Central-variant posterior reversible encephalopathy syndrome in a young patient with systemic lupus erythematosus

Serum troponin T in patients with amyotrophic lateral sclerosis

Brain abscess due to Nocardia infection in an immunocompetent patient with asymptomatic pulmonary alveolar proteinosis

Writer's cramp: a new dystonic feature in spinocerebellar ataxia type 3

Predictors of amyotrophic lateral sclerosis mimic syndrome Abstract The term amyotrophic lateral sclerosis mimic syndrome (ALSms) includes pathologies that present signs or symptoms similar to those caused by amyotrophic lateral sclerosis (ALS), which can lead to misdiagnosis. In general, any kind of misdiagnosis can result in negative clinical, psychological and economic consequences as well diagnostic and treatment delay. The objectives were to determine the frequency and to compare the demographic and clinical characteristics of patients with ALS and ALSms in our ALS clinic. We retrospectively studied all patients evaluated from 2007 to 2017 including only patients with a definite final diagnosis. Out of 368 patients with motor neuron disease symptomatology, 43 (11.7%) had an ALSms. The most frequent etiology was compressive myelopathy (32.6%). Multivariate analysis considering positive associations was statistically significant for patients having only upper or lower motor neuron signs in the physical examination, a non-compatible electromyogram (EMG), as well as atypical first symptoms. ALS misdiagnosis is an ongoing and not infrequent problem. From our series of patients, atypical symptoms, absence of EMG pathological findings or isolated upper or lower motor neuron disease should prompt suspicion of a differential diagnosis.

Letter to the editor: phenytoin-induced rhabdomyolysis

Psychotic polyembolokoilamania secondary to left parietal lobe glioblastoma

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

International Organizations

The service economy: U.S. trade coalitions in an era of deindustrialization Abstract Services dominate the US economy and are increasingly traded across borders yet little is known about service firms' trade policy objectives or lobbying activities. We fill this gap by examining services' political engagement on trade policy as manifested through lobbying, public positions on trade, and reports issued by U.S. Industry Trade Advisory Committees. We document for the first time that service firms are highly active in the politics of US trade agreements and, compared to firms in goods-producing industries, are much less likely to disagree over trade. Instead, service firms are almost uniformly supportive of US trade agreements, which we explain by focusing on the stark US comparative advantage in services. Service firms are therefore a key constituency for deeper international economic cooperation, helping to explain the present era of global integration despite tough times for uncompetitive US manufacturing. We expect service producers to join the defense of global economic order against emergent populism.

Popular non-support for international organizations: How extensive and what does this represent? Abstract This paper investigates popular non-support for international organizations (IO), asking two questions. First, are attitudes within the mass public becoming less supportive of IOs? Second, how can we explain these IO attitudes, especially when the mass public appears to know relatively little about specific international institutions? Using survey data from the International Social Survey Programme's National Identity module, fielded across multiple countries in 1995, 2003, and 2013, it reports that on average and within most countries, citizen attitudes about IOs have become less positive over time. To explain these attitudes, this paper argues that citizens tend to group things that appear as "international" such as cross-border economic flows and IOs. While citizens might feel positively or negatively about these international factors, this grouping implies that they view them similarly, based on what they can feel from the international level related to their job and income. Thus, less (more) skilled citizens who are hurt by (who benefit from) economic globalization should express more negative (positive) views about IOs. Controlling for cultural attitudes socialized through education, we find that skill is a statistically significant and substantively strong predictor of IO attitudes. We also show how this individual-level skill difference gets larger in countries that are more and/or less-favorably exposed to economic globalization.

Hello, goodbye: When do states withdraw from international organizations? Abstract Under what conditions do states withdraw from intergovernmental organizations (IGOs)? Recent events such as Brexit, the US withdrawal from UNESCO, and US threats to withdraw from NAFTA, NATO, and the World Trade Organization have triggered widespread concern because they appear to signify a backlash against international organizations. Some observers attribute this recent surge to increasing nationalism. But does this explanation hold up as a more general explanation for IGO withdrawals across time and space? Despite many studies of why states join IGOs, we know surprisingly little about when and why states exit IGOs. We use research on IGO accession to derive potential explanations for IGO withdrawal related to domestic politics, IGO characteristics, and geo-politics. We quantitatively test these potential explanations for withdrawal using an original dataset of 493 IGOs since 1945, documenting about 200 cases of withdrawal. We find that nationalism is not the key driver of IGO withdrawals in the past. Instead, we show that geo-political factors – such as preference divergence and contagion – are the main factors linked to IGO withdrawals, followed by democracy levels in the country and organization. These findings have important implications for research on the vitality of international organizations, compliance, and the liberal world order.

Global value chains and the political economy of WTO disputes Abstract This paper investigates how the rise of global value chains (GVCs) in international trade affects the political economy of trade disputes. It addresses the gap between the domestic and international politics of trade disputes, which is especially relevant in these times as populist nationalism favors protectionist forces. We advance the argument that firms face institutional disadvantages in opposing protectionist forces at home, as observed in how contesting firms fare in US anti-dumping cases. As a consequence, they have incentive to seek cross-border cooperation with firms along the supply chain to escalate the adoption of anti-dumping measures to WTO disputes. The paper implements a two-stage empirical strategy. First, we examine the political contestation around US anti-dumping cases ongoing in the WTO era that takes place in International Trade Commission (ITC) hearings. We observe that while these cases see significant opposition from firms relying on the imports of intermediate goods, most cases end in favor of petitioners supporting the imposition of anti-dumping duties. In a second step, we quantitatively analyze the effect of intermediate and GVC goods trade in products that are the subjects of anti-dumping cases on the incidence of a formal WTO dispute. The results suggest that the high costs of litigation at the WTO are often prohibitive relative to the volume of trade in question and pose an equally difficult challenge for firms seeking to overturn protectionist measures.

"Take back control"? The effects of supranational integration on party-system polarization Abstract In this paper, we examine the relationship between supranational integration and domestic party-system polarization (extremism). We first construct a theoretical argument that uncovers the key trade-off between the "output legitimacy" of a supranationally integrated party system and the inevitable loss of "input legitimacy" caused by externally imposed policy constraints. This translates into a strategic tradeoff between responsibility and responsiveness at the party level of electoral competition. We hypothesize that while moderate supranational policy constraints can initially speed-up platform convergence, ever-closer political integration may reverse the trend towards higher levels of party-system polarization and party extremism. We apply our framework to the case of EU integration and test our key non-monotonic prediction both at the party-system level of polarization and at the party level of ideological extremism. Finally, we apply to synthetic control method (SCM) for causal inference in comparative case studies to study how political integration and supranational policy constraints have affected their overall level of party-system polarization over time. Our overall empirical analysis strongly corroborates our theoretical argument.

International organizations in a new era of populist nationalism Abstract This article introduces the special issue on International Organizations in a New Era of Populist Nationalism. The special issue aims to clarify the stakes for and the politics of international organizations in a time of rising populist nationalism around the world. In this introductory essay, we attempt to disentangle the rise of populism and a resurgence of nationalism as distinct processes and concepts. While neither force is new, we observe significant variation across countries in the type of level of nationalist and populist objections to international institutions. We develop a typology for thinking about how and when populism, nationalism, or their combination might have different effects on international cooperation and organizations. Finally, we review the specific article contributions to the special issue and how they fit with the themes developed in this essay. The final section concludes with questions and ideas for future research on the topic that will enhance our understanding of the complex challenges – and potential opportunities – for international cooperation and organizations in the years ahead.

Euroscepticism and government accountability in the European Union Abstract The European Union has become a contested issue amongst voters in Europe. I analyze how the increasingly salient attitudes toward European integration have affected how voters hold their governments accountable for their policy decisions at the EU-level. I argue that attitudes toward the EU have become an important source of electoral accountability that complement attitudes on the left-right dimension, but they matter differently for pro- and anti-European voters. Whereas Eurosceptic voters are likely to use their attitudes toward the EU to hold their governments accountable, pro-European voters tend to rely on their specific attitudes toward particular policies to assess the responsiveness of their politicians. The paper presents the results of a conjoint experiment in a survey of 2,540 German citizens to analyze how pro- and anti-European voters' attitudes influence their assessment of typical signals of government responsiveness.

The rise of modern taxation: A new comprehensive dataset of tax introductions worldwide Abstract This article describes the new Tax Introduction Dataset (TID). Listing the year and the mode of the first permanent introduction of six major taxes (inheritance tax, personal income tax, corporate income tax, social security contributions, general sales tax and value added tax) in 220 countries, 1750–2018, TID is the most comprehensive dataset of its kind. The comprehensiveness of our measure is of critical value to empirical work on the causes of tax innovation and its consequences for state, society and economy. In this paper, we explain the selection of our tax sample and the structure of the dataset, descriptively map temporal and regional patterns of tax introductions around the world, and draw on TID to investigate associations between tax introductions and economic development, war, and democratization.

Publisher Correction to: The KOF Globalisation Index – revisited The article listed above was initially published with incorrect copyright information. Upon publication of this Correction, the copyright of this article changed to "The Author(s)". The original article has been corrected.

Social standards in trade agreements and free trade preferences: An empirical investigation Abstract Free trade generates macroeconomic gains but also creates winners and losers. Historically, to reconcile this tension, governments compensated globalization losers with social spending in exchange for support for free trade, known as the embedded liberalism compromise. In the neoliberal era, what other policies can governments pursue to strengthen support for globalization? We assess the effect of social standards in preferential trade agreements (PTAs) on individual preferences for free trade. We analyze data from an original survey experiment and find that respondents in advanced industrialized countries have greater support for free trade when PTAs include social standards. Differences do exist in how these social standards are perceived: while we do find evidence of an embedded liberalism compromise recast, fair trade norms have the most salience. An external validity check using the PEW global attitudes survey confirms the hypothesis. Our analysis has serious implications for the legitimacy of the global trading system suffering from neo-mercantilist creep.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com

Clinical Immunology

Different Phenotypic Presentations of X-Linked Lymphoproliferative Disease in Siblings with Identical Mutations

Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome Abstract Objectives Mutations affecting the TMEM173 gene cause STING-associated vasculopathy with onset in infancy (SAVI). No standard immunosuppressive treatment approach is able to control disease progression in patients with SAVI. We studied the efficacy and safety of targeting type I IFN signaling with the Janus kinase inhibitor, ruxolitinib. Methods We used DNA sequencing to identify mutations in TMEM173 in patients with peripheral blood type I IFN signature. The JAK1/2 inhibitor ruxolitinib was administered on an off-label basis. Results We identified three patients with SAVI presenting with skin involvement and progressive severe interstitial lung disease. Indirect echocardiographic signs of pulmonary hypertension were present in one case. Following treatment with ruxolitinib, we observed improvements of respiratory function including increased forced vital capacity in two patients, with discontinuation of oxygen therapy and resolution of echocardiographic abnormalities in one case. Efficacy was persistent in one patient and only transitory in the other two patients. Clinical control of skin complications was obtained, and one patient discontinued steroid treatment. One patient, who presented with kidney involvement, showed resolution of hematuria. One patient experienced increased recurrence of severe viral respiratory infections. Monitoring of peripheral blood type I interferon signature during ruxolitinib treatment did not show a stable decrease. Conclusions We conclude that targeting type I IFN receptor signaling may represent a promising therapeutic option for a subset of patients with SAVI syndrome and severe lung involvement. However, the occurrence of viral respiratory infection might represent an important cautionary note for the application of such form of treatment.

Clinical and Laboratory Features of 184 Italian Pediatric Patients Affected with Selective IgA Deficiency (SIgAD): a Longitudinal Single-Center Study Abstract Purpose Selective IgA deficiency (SIgAD) is the most common humoral primary immunodeficiency. Long-term follow-up data in large cohort of pediatric patients are scarce. Methods We report on a single-center cohort of 184 pediatric patients affected with selective IgA deficiency and describe the characteristics at diagnosis and during follow-up. Results Respiratory infections were the most common clinical finding leading to the initial diagnosis (62%). Positive family history for antibody deficiencies (selective IgA deficiency, common variable immunodeficiency) led to SIgAD diagnosis in 16% of cases. During follow-up, while the incidence of respiratory infections was not particularly high, gastrointestinal symptoms were reported in 27% of patients. Allergic manifestations were found in 23% at diagnosis and an additional 16% of patients during follow-up, leading to a prevalence of atopy of 39% among SIgAD patients. Autoimmune manifestations, excluding celiac disease, were found in 9% of affected patients during follow-up. Celiac disease was found in a high prevalence (14%). Increase of serum IgA levels to partial deficiency (9%) and normal serum levels for age (4%) was observed during follow-up. A small percentage of patients (2%) progressed to common variable immunodeficiency (CVID). Conclusions In conclusion, this is the first study to describe a large single-center pediatric cohort of patients affected with SIgAD, revealing that overall most patients do well with regard to infections. Many develop CD, at a rate much higher than the general population. A few normalize their IgA levels. A few progress to CVID. Thus, careful follow-up is suggested to diagnose and treat potential complications earlier for avoiding potential morbidities.

Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity Abstract Live-attenuated vaccines (LAVs) can protect humans against 12 viral and three bacterial diseases. By definition, any clinical infection caused by a LAV that is sufficiently severe to require medical intervention attests to an inherited or acquired immunodeficiency that must be diagnosed or identified. Self-healing infections can also result from milder forms of immunodeficiency. We review here the inherited forms of immunodeficiency underlying severe infections of LAVs. Inborn errors of immunity (IEIs) underlying bacille Calmette-Guérin (BCG), oral poliovirus (OPV), vaccine measles virus (vMeV), and oral rotavirus vaccine (ORV) disease have been described from 1951, 1963, 1966, and 2009 onward, respectively. For each of these four LAVs, the underlying IEIs show immunological homogeneity despite genetic heterogeneity. Specifically, BCG disease is due to inborn errors of IFN-γ immunity, OPV disease to inborn errors of B cell immunity, vMeV disease to inborn errors of IFN-α/β and IFN-λ immunity, and ORV disease to adaptive immunity. Severe reactions to the other 11 LAVs have been described yet remain "idiopathic," in the absence of known underlying inherited or acquired immunodeficiencies, and are warranted to be the focus of research efforts. The study of IEIs underlying life-threatening LAV infections is clinically important for the affected patients and their families, as well as immunologically, for the study of the molecular and cellular basis of host defense against both attenuated and parental pathogens.

Autoinflammation Masquerading as Autoimmunity in an Adult with Heterozygous p.E250K PSTPIP1 Mutation

Variable Responses to Tocilizumab in Four Patients with Schnitzler Syndrome

Role of Allogeneic Hematopoietic Stem Cell Transplant for Chronic Granulomatous Disease (CGD): a Report of the United States Immunodeficiency Network Abstract Purpose Chronic granulomatous disease (CGD) is a primary immunodeficiency for which allogeneic hematopoietic stem cell transplant (HSCT) offers potential cure. Direct comparison of HSCT to non-HSCT management in the North American population was performed to identify clinical factors associated with overall survival (OS) and transplant-related survival (TRS). Methods Retrospective review of CGD subjects enrolled in the United States Immunodeficiency Network. Survival was estimated by the Kaplan-Meier method and modeled by proportional hazards regression. Results We identified 507 patients (66% CYBB mutants) diagnosed in 1953–2016. Fifty underwent allogeneic HSCT. Median follow-up was 9.1 years after diagnosis (0–45.8 years). OS was negatively associated with CYBBmutation (HR = 6.25; p = 0.034) and not associated with HSCT (88% v. 85% ± HCT) (HR = 1.26; p = 0.65). Transplant at ≤ 14 years old was associated with improved TRS (93% v. 82% at T + 60 months) (HR = − 4.51; p = 0.035). Patients transplanted before 15 years old had fewer severe infections pre-HSCT (mean 0.95 v. 2.13; p = 0.047). No mortality was reported in patients receiving stem cells from matched siblings. Infection incidence declined post-HSCT in subjects with greater than or equal to four infections pre-HSCT (p = 0.0010). Compared to non-HSCT patients ≥ 15 years old, post-transplant survivors had higher mean performance score (93.2 v. 85.9; p = 0.0039) and lower frequency of disability (11% v. 52%; p = 0.014). Conclusion Allogeneic HSCT was associated with reduced infection incidence and improved functional performance, but not with a change in overall survival. Transplant-related survival was elevated in patients undergoing HSCT before 15 years old. Consider HSCT prior to late adolescence in patients with severely diminished reactive oxygen intermediate synthesis, particularly if a matched sibling is available.

Enterovirus-Associated HLH: Addition of Anakinra to IVIG and Corticosteroids

Fifteen Years of the J Project

Recurrent Salmonella typhi Infection and Autoimmunity in a Young Boy with Complete IL-12 Receptor β1 Deficiency

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com