mohsen kheradmand
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BackgroundDuchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are inherited X-linked disorders resulting from alterations in the dystrophin gene. Genotype-phenotype matching studies have revealed a link between disease severity, the amount of muscle dystrophin, and the extent of mutation/deletion on the dystrophin gene. This study aimed to assess the relationship between genetic alterations in the dystrophin gene and the clinical status of patients with dystrophinopathies among the Iranian population.MethodsThis cross-sectional study examined 54 patients with muscle weakness caused by abnormalities in the dystrophin gene at a hospital affiliated to Isfahan University of Medical Sciences, Isfahan, Iran, in 2021. The participants’ demographic information, including age, family history of muscle dystrophies, and family history of other medical diseases as well as the type of muscular dystrophy were recorded. Furthermore, the number and region of deleted exons based on dystrophy types were also evaluated using multiplex ligation-dependent probe amplification (MLPA). The patients’ gaits were also assessed as using a wheelchair, the presence of waddling gaits, or toe gaits. The patients’ clinical status and the coexistence of pulmonary, bulbar, and mental conditions were also examined and compared between the two groups of dystrophinopathies.ResultsIn this study, 54 patients with dystrophinopathy with the mean age of 16.63 ± 12.10 years were evaluated, of whom 22 (40.7%) and 30 (55.6%) patients were classified as BMD and DMD, respectively. The most affected regions with deleted exons were exons 45-47 (n = 5) and 45-48 (n = 4) in patients with BMD, while exons 45, 48-52, 51-55, and 53 (2 cases per exon) were the most common affected exons in patients with DMD. Further analyses revealed that deletions in exons 45-47 and 51-55 were significantly associated with older and younger ages at the onset of becoming wheelchair-bound in patients with dystrophy, respectively. The hotspot range in both BMD and DMD was within exons 45-55 (n = 15 for each group); 63% of the patients had alterations on the dystrophin gene within this range [30 patients (68.18%) in the BMD group, 15 patients (53.57%) in the DMD group].ConclusionExon deletion was the most common genetic alteration in patients with dystrophinopathies. No significant difference was observed between DMD and BMD regarding the number of deleted exons. Deletions in exons 45-47 and 51-55 were linked to later and earlier onset of becoming wheelchair-bound, respectively.Keywords: Duchenne Muscular Dystrophy, Phenotype, Genotype, Dystrophin, Iran
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Background
Cerebrovascular diseases comprise a significant portion of neurological disorders related to coronavirus disease 2019 (COVID-19). We evaluated the clinical and imaging characteristics of a cohort of COVID-19 patients with stroke and also identified patients with watershed infarcts.
MethodsIn this cross-sectional study, seventy-three COVID-19 patients with ischemic stroke were included between October 2020 and January 2021. Patients were evaluated based on the following clinical and imaging features: severity of COVID-19 (critical/non-critical), stroke type, presence/absence of clinical suspicion of stroke, medical risk factors, Fazekas scale, atherothrombosis, small vessel disease, cardiac pathology, other causes, and dissection (ASCOD) criteria classification, and presence or absence of watershed infarction. Clinical outcomes were assessed based on Modified Rankin Scale (MRS) and mortality.
ResultsMost cases of ischemic stroke were due to undetermined etiology (52.1%) and cardioembolism (32.9%). In terms of imaging pattern, 17 (23.0%) patients had watershed infarction. Watershed infarction was associated with the clinically non-suspicious category [odds ratio (OR) = 4.67, P = 0.007] and death after discharge (OR = 7.1, P = 0.003). Patients with watershed infarction had a higher odds of having high Fazekas score (OR = 5.17, P = 0.007) which was also shown by the logistic regression model (adjusted OR = 6.87, P = 0.030). Thirty-one (42%) patients were clinically non-suspected for ischemic stroke. Critical COVID-19 was more common among patients with watershed infarct and clinically non-suspicious patients (P = 0.020 and P = 0.005, respectively). Patients with chronic kidney disease (CKD) were more prone to having stroke with watershed pattern (P = 0.020).
ConclusionWatershed infarct is one of the most common patterns of ischemic stroke in patients with COVID-19, for which clinicians should maintain a high index of suspicion in patients with critical COVID-19 without obvious clinical symptoms of stroke.
Keywords: Covid-19, Stroke, brain, Magnetic Resonance Imaging, Clinical Characteristics, White Matter, Cerebrovascular Disorders -
Background
Total hip arthroplasty (THA) is an effective surgery for patients with end-stage hip joint degenerative arthritis. This study aimed to determine peri-operative factors that impact the length of stay (LOS) and design a formula to predict LOS in patients undergoing THA.
MethodsThis cross-sectional study was performed from September 2019 to January 2020. For this study, all patients who underwent THA over a period of 12 years since 2005 were included in the study. Data about the LOS and several variables including demographic variables, surgery-related variables, transfusion, intensive care unit (ICU) admission, past drug history, comorbidities, and laboratory data, were gathered. Qualitative variables are presented as numbers (%), and quantitative variables are presented as mean Mann± standard deviation. Mann Whitney test , Kruskal-Wallis test, and Spearman’s rank correlation test were also used.
ResultsA total of 524 patients were included in the study; 12 were excluded .261 (51%) were female and 251(49%) male. The mean age was 56.13±17.04 years. In the univariate analysis, the day of admission, surgery indication, transfusion, diabetes mellitus, oral anti-diabetic drugs, American Society of Anesthesiology (ASA) score, preoperative hemoglobin (Hb) level, and type of prosthesis showed significant relation with LOS. Significant variables entered to zero truncated negative binomial regression. Among them, the day of admission, ASA score, preoperative Hb level, and type of prosthesis showed significant relation with LOS (P < 0.05) and were used for model design.
ConclusionPreoperative Hb level, ASA score, day of admission, and prosthesis type have an impact on LOS and can predict LOS in patients who are candidates for THA.
Keywords: Length of Stay, Prediction, Total Hip Arthroplasty -
ارزیابی عملکرد سوخت در شرایط حادثه یک چالش مهم در آنالیز ایمنی به شمار می رود. بدین منظور، کدهای محاسباتی متعددی مختص بررسی عملکرد میله سوخت توسعه یافته اند. این کدها به بررسی پدیده های حاکم بر رفتار میله سوخت در حین حادثه شامل تغییر فاز غلاف، اکسید شدن زیرکونیوم، تغییر شکل خزشی و شکست آلیاژ غلاف می پردازند. هدف پژوهش حاضر، بررسی عملکرد میله سوخت راکتور VVER-1000 در حین حادثه از دست رفتن خنک کننده مدار اول با استفاده از مجموعه کدهای FRAPCON-4.0 و FRAPTRAN-1.5 می باشد. نمونه مورد بررسی میله سوخت راکتور VVER-1000 در آزمایش IFA-650.6 است. مقایسه نتایج بدست آمده با دیگر نتایج تیوری و تجربی منتشر شده، موید اعتبار کد در آنالیز ترمومکانیکی میله سوخت VVER می باشد. علاوه بر این، نتایج شبیه سازی صورت گرفته نشان داد که علاوه بر معیارهای ایمنی فعلی در طراحی سیستم های ایمنی حادثه LOCA لازم است شکست غلاف نیز به عنوان دیگر معیار ایمنی درنظر گرفته شود. این موضوع هم اکنون توسط کمیسیون نظام ایمنی آلمان (RSK) در تحلیل حادثه LOCA اعمال میگردد.
کلید واژگان: عملکرد میله سوخت, آنالیز ترمومکانیکی, کد FRAPCON, کد FRAPTRANEvaluating fuel performance in the event of an accident is an important challenge in safety analysis. To this end, several computational codes have been developed to evaluate the performance of the developed fuel rod. These codes examine the phenomena that governing the behaviour of the fuel rod during an accident include sheath phase alteration, zirconium oxidation, creep deformation, and sheath alloy failure. The aim of the present study is to investigate the performance of the VVER-1000 reactor fuel rod during an LOCA accident using FRAPCON-4.0 and FRAPTRAN-1.5 codes. The sample examined is the VVER-1000 reactor fuel rod in the IFA-650.6 test. Comparison of the obtained results with other published theoretical and experimental results confirms the validity of the code in thermo mechanical analysis of VVER fuel rod. In addition, the simulation results showed that the current safety criteria in the design of LOCA safety systems, it is necessary to consider sheath failure as another safety criterion. This is currently being addressed by the German Commission on Safety (RSK) in the analysis of the LOCA incident.
Keywords: Fuel rod performance, thermo mechanical analysis, FRAPCON code, FRAPTRAN code -
Background
Neurological manifestations of coronavirus disease 2019 (COVID‑19) have been highlighted. COVID‑19 potentially increases the risk of thromboembolism. We aimed to compare patients with COVID‑19 with and without new‑onset acute ischemic stroke (AIS).
Materials and MethodsIn this single‑center retrospective case–control study, demographics, clinical characteristics, laboratory findings, and clinical outcomes were compared between 51 patients with both COVID‑19 and AIS (group A) and 160 patients with COVID‑19 and without AIS (group B).
ResultsPatients in group A were significantly older, more likely to present with critical COVID‑19 (P = 0.004), had higher rates of admission in the intensive care unit (P < 0.001), more duration of hospitalization (P < 0.001), and higher in‑hospital mortality (P < 0.001). At the time of hospitalization, O2 saturation (P = 0.011), PH (P = 0.04), and HCO3 (P = 0.005) were lower in group A. White blood cell count (P = 0.002), neutrophil count (P < 0.001), neutrophil‑lymphocyte ratio (P = 0.001), D‑Dimer (P < 0.001), blood urea nitrogen (BUN) ( P < 0.001), and BUN/Cr ratio (P < 0.001) were significantly higher in patients with AIS.
ConclusionStroke in COVID‑19 is multifactorial. In addition to conventional risk factors of ischemic stroke (age and cardiovascular risk factors), we found that patients with more severe COVID‑19 are more prone to ischemic stroke. Furthermore, leukocyte count, neutrophil count, neutrophil‑lymphocyte ratio, D‑Dimer, BUN, and BUN/Cr ratio were higher in patients with AIS following COVID‑19 infection.
Keywords: Acute ischemic stroke, blood urea nitrogen, coronavirus disease 2019, D‑dimer -
بررسی مالیت منافع غیرمستوفات انسان آزاد براساس معیارهای معتبر شرعی در مال انگاری اشیاء، موضوعی است که نتیجه آن در مباحثی چون «ضمان فوت منافع انسان آزاد»، «اصل اولی در لزوم کارکردن مفلس»، «صداق قراردادن این منافع» و «اجاره انسان» نمایان می شود و تاثیرات خود را در روند بررسی ادله مرتبط به هرکدام از این فروع خواهد گذاشت. به منظور یافتن پاسخ این مسیله، شرایط مالیت شیی براساس ضوابط عرفی و شرعی تحلیل می شود و با اثبات وجود معیارهای شرعی مال در منافع موردبحث، این منافع مال به شمار می آید. در ادامه شش ایراد درباره مالیت منافع انسان مطرح و با استناد به تحقق شرایط مالیت در این منافع به اشکالات پاسخ داده می شود و در این میان به برخی از عبارات فقیهان نیز استشهاد شده است. این تحقیق با انضمام دو موید یعنی صحت مهر قراردادن این منافع و درستی اجاره آن، و نیز در ادامه به بحث مال انگاری این منافع نزد فقیهان و بیان ثمرات مترتب بر مالیت این منافع مانند ضمان فوت منافع انسان آزاد و امکان قراردادن این منافع در برابر مبیع پرداخته شده است. این پژوهش در جریان بحث، از پایبندی تمام فقهای شیعه به مالیت منافع انسان در فروعی مانند «مهر قراردادن منافع انسان آزاد» و «اجاره این منافع» و اختلاف نظر ایشان در مباحث نظری پیرامون مالیت این منافع آگاهی داده است.
کلید واژگان: منافع انسان آزاد, منافع مستوفات, مالThe main focus of this research is to examine the value of the might of human labor based on valid jurisprudential criteria for valuing things. The result of this discussion is in the discussions such as "the guarantee of the elimination of the might of human labor," "the principle of the necessity of working the debtor," "putting human labor against the dowry" and "leasing human labor" and showing its effects in The process of examining the reasons for any of these will be necessary and This explains the need for research on this subject. In order to achieve the result, the conditions of the value of the things were analyzed on the basis of customary and jurisprudential rules. And, with the proof of the existence of a valid jurisprudential value of the might of human labor, the value of this labor force was accepted. Then six Objectionable about the value of the might of human labor were expressed and, based on Existence of conditions of value of the might of human labor, those were answered. And Some of the jurists' words were also given to confirm the subject matter. Then, with the addition of two confirming of the value of might of human labor in the phrases of the jurists, and the expression of the fruits of the value of human labor, the discussion came to an end. The present study also discusses the commitment of all Shi'a jurisprudents to the value of the might of human labor in the "putting human labor against the dowry" and "the lease of these human labors," and their disagreement with the theoretical discussion of the value on these human labors.
Keywords: human ability to work, unused ability to work, value -
تبیین جایگاه فقهی شفافیت اطلاعات دولت، محور مطالعات این جستار بوده و به دنبال یافتن پاسخ به این سوال که «اقتضای اصول، قواعد و ادله فقهی پیرامون دسترسی آزاد به اطلاعات دولت چیست؟» این نوشتار به انجام رسید. گرچه در سالهای اخیر، برخی محققین به موضوع شفافیت در منابع دینی پرداخته اند، اما هنوز جوانب این بحث از منظر فقه به شکل کامل روشن نگردیده است و همین امر ضرورت تحقیق فقهی در اطراف این موضوع را روشن میسازد. پژوهش پیش رو مبتنی بر فرضیه محدودیت شفافیت اطلاعات حکومت انجام شد و تحقیقات صورت گرفته به روش اجتهادی مرسوم در آیات کتمان، مشورت، نهی از منکر و ادای امانت، روایات واردشده پیرامون اظهار علم، مشورت، پرهیز از حضور در مواضع تهمت، شیوه حکومتی امیر المومنین و...، سیره عملی معصومین: و قواعد فقهی بر خلاف پیشفرض نخست، لزوم شفافیت در معنای موسع را نتیجه داد. در ادامه با عداد استثناءات شفافیت، راه کار فقهی سنجش مرجحات باب تزاحم، به عنوان راه حل تزاحم پیش آمده در مصالح و مفاسد متقابل، ارائه گردید.
کلید واژگان: شفافیت اطلاعات, حکومت اسلامی, دسترسی آزاد به اطلاعاتThis essay seeks to elucidate the jurisprudential status of the Islamic State’s information transparency in order to provide an answer to the question “what do the jurisprudential principles, rules and arguments require regarding free access to the data and information provided by the Islamic State?” Although in recent years some researchers have dealt with the issue of transparency in religious sources, various aspects of the issue have not yet been thoroughly clarified from the jurisprudential perspective. This makes clear the fact that it is essential to make a research into this subject from the jurisprudential point of view. The present research, being based on the thesis of limitation in access to the data provided by the government, has been conducted drawing upon the so-called established individual reasoning – as regards to the Qur’anic verses dealing with concealment, consultation, forbidding the evil, maintaining trust, the traditions regarding acknowledgement of knowledge, avoiding false accusations, the Commander of the Faithful’s way of governance and the practical life-style of the Immaculate Imams (peace be upon them) and jurisprudential rules – unlike the first presupposition -- result in the idea of necessity of transparency in its wider sense. Apart from making a reference to a number of exceptions of transparency, the article goes on to present the jurisprudential strategy for the measurement of preferences between contradicting jurisprudential issues as the solution for the conflict between the interests and whatever which violates the essential values of religion.
Keywords: information transparency, Islamic government, free access to information -
معرفی و گسترش رمز ارزها، زمینه طرح سوال های فقهی متعددی را فراهم آورده است. در این مقاله با استفاده از روش تحلیلی به تحلیل فقهی فعالیت های مورد انجام در فرایند تولید (استخراج) و مبادله این ارزها، با تمرکز بر شبکه بیت کوین می پردازیم. بر اساس یافته های پژوهش، تجارت رمزارزها در صورت رعایت شروط عمومی معاملات تجاری، از نظر فقهی مجاز است. مالکیت رمز ارزها را می توان در قالب «تلاش برای رمزگشایی و حیازت پاداش دریافتی از شبکه» تفسیر کرد. در معامله این ارزها امکان مراعات شرایط متعاقدین، عوضین و انشاء معاطاتی تملیک وجود دارد. برخی اشکالات طرح شده پیرامون تجارت رمزارزها و از جمله بیت کوین؛ همچون «پول شویی»، «ایجاد حباب ارزی»، «کاهش ارزش پول ملی»، «اکل مال به باطل بودن»، «غرری و ضرری بودن خرید آن»، نمی تواند مبنای عدم مشروعیت تجارت رمزارزها باشد. با این وجود، استفاده از رمز ارزها با چالش هایی همچون مصرف برق دولتی و مشارکت با محجورین در استخرهای ماینینگ روبرو است. حاکم شرع می تواند با احراز اثرات منفی توسعه رمز ارزها در بازار و مفاسد اقتصادی، اخلاقی و امنیتی احتمالی، استخراج و خرید و فروش آن را با صدور حکم حکومتی محدود یا ممنوع کند.
کلید واژگان: رمز ارز, ارز مجازی, ارز دیجیتال, بیت کوینThe popularity of the concept of crypto-currency in today's economy has raised many juridical questions. Using an analytical-juridical method, this paper analyzes the activities performed in the process of mining and exchanging of these currencies, with a focus on Bitcoin network. Based on the research findings, juridically, the trading of crypto-currency is permissible if the general conditions of commercial transactions are met. The crypto-currency ownership can be justified on the grounds of an effort for decoding and possessing the receiving rewards from the network. Observing the conditions of the contractors, considerations, and the creation of possession through sale by conduct in these currencies is possible. Some objections rose against crypto-currency trading, including Bitcoin, money laundering, creating a currency bubble, devaluation of national currency, obtaining property through illegitimate and illegal means, and the possibility of being deceitful or harmful, cannot be the basis for the illegality of the trade of crypto-currency. However, using crypto-currency faces challenges such as consumption of government electricity, partnerships with miners the incapacitated individuals in mining pools. The religious ruler may limit or prohibit the extraction and sale of such goods by issuing a government order, with the adverse effects of currency development on the market and possible economic, moral and security corruption. Sharia ruler can restrict or prohibit mining crypto-currency trade in case of negative effects of crypto-currency in the market, or in case of economic, moral or security problems.
Keywords: Crypto-currency, Virtual currency, Digital currency, Bitcoin -
BackgroundMultiple sclerosis (MS) is an autoimmune inflammatory disease that affects the central nervous system. Except tremor, other movement disorders such as Parkinsonism are less frequent in MS. However, some investigations have shown inflammatory and autoimmune aspects of Parkinson’s disease. In this study, we report eight new cases of MS who present Parkinson’s disease.MethodsThis retrospective population-based study was carried out on Isfahan MS society between April 2003 and July 2012. A total of 3792 patients with MS were surveyed for Parkinson’s disease. Parkinson’s disease was approved according to “UK Parkinson disease Brain Bank” criteria. Eligible cases were invited to have an interview with a neurologist. MRI was carried out according to the baseline.ResultsWe identified eight patients (three men and five women) who present MS and Parkinson’s disease. The mean (±SD) age of onset was 33.3 ± 6.5 (range: 24–42) years for MS and 39.5 ± 8.6 (range: 30–55) years for Parkinson’s disease patients. In all patients, MS was preceded Parkinson’s disease. Parkinson’s disease was developed within mean duration of 6.1 ± 3.4 (range 2–13) years after MS. Focal lesions was shown in six cases, lesions of basal ganglia (BG) in four, lesions of the thalamus in two and lesions of mid-brain in five of cases. In four cases, Parkinsonism occurred before age 40 that is considered as Young-Onset Parkinson’s disease.ConclusionWe reported eight patients with MS and Parkinson’s disease. To the best of our knowledge, 34 cases of Parkinsonism associating with MS are reported so far. Parkinsonism is a movement disorder, defines as deep gray matter disorder which lead to dopamine deficiency in BG. Investigations have shown that MS could affect deep gray matter structures. Demyelinated lesions in MS and consequence axonal loss in BG and/or nigrostriatal pathway may be responsible for Parkinsonism manifestations in such cases.Keywords: Extrapyramidal Signs, Magnetic Resonance Imaging, Movement Disorders, Multiple Sclerosis, Parkinsonism
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