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[目的] 总结分析裘昌林教授救治重症肌无力危象的中医辨治思路。[方法] 通过跟师学习、整理医案、复习相关中医文献,整理总结裘师临床抢救重症肌无力危象的中医辨治经验,分析重症肌无力危象的中医认识,中医治疗原则和用药方法,并列举医案分析。[结果] 裘师认为,重症肌无力危象病情危重,属于中医大气下陷证,与脾肾密切相关,脾肾虚损为本,甚者脉微息弱、元气耗散而气脱,治疗重在急救脱陷、温肾敛阴,兼顾祛痰通腑,并且研用马钱子起沉疴。所举医案贯彻裘师辨治经验,遣方用药以健脾益气为主,补益肝肾为辅,佐以祛痰通腑,获得较好疗效。[结论] 裘师基于中医病因病机,对重症肌无力危象进行辨证论治,遣方用药从虚出发,随证加减,配合西医治疗,能够提高疗效,减少不良反应,充分体现了中医药治疗重症肌无力危象的优势,其经验具有推广价值。  相似文献   
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The adrenal cortex gives rise to a biologically heterogenous group of neoplasms, each with a distinct morphology, antigen expression and molecular profile. Adrenal cortical adenomas have excellent prognosis and are usually cured by surgical resection alone, while adrenal cortical carcinomas are very aggressive tumors with a poor prognosis regardless of therapy. These tumors are rare and often challenging for a pathologist to diagnose, as significant overlap exists between benign and malignant lesions in some cases. In this review, we attempt to summarize most important histologic and clinical features of adrenal cortical adenomas and carcinomas, clarify the use of different grading systems, the use of special stains and the differential diagnosis for practicing pathologists. Most relevant hereditary syndromes associated with adrenal cortical tumors are listed. Updates in molecular alterations in adrenal cortical neoplasms and hyperplastic diseases as well as their clinical significance and potential therapeutic implications are also discussed.  相似文献   
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TAFRO syndrome is a systemic inflammatory, lymphoproliferative disorder, but the pathophysiology of the disease is unknown. It is typically characterized by thrombocytopenia, anasarca, a fever, reticulin fibrosis, renal dysfunction, and organomegaly. However, other manifestations have been also reported. We encountered a 43-year-old man with TAFRO syndrome who showed mediastinal panniculitis, liver damage, and adrenal lesions in addition to the core signs. He achieved complete remission with combination therapy of corticosteroids, tocilizumab, and cyclosporin, and remission was maintained even after drug discontinuation at 15 months. Atypical manifestations and complete remission of TAFRO syndrome were remarkable features of our case.  相似文献   
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《Brain & development》2021,43(10):1023-1028
BackgroundAromatic L-amino acid decarboxylase (AADC) deficiency, caused by a pathogenic variant in the dopa decarboxylase (DDC) gene, is a rare neurometabolic disorder in which catecholamine and serotonin are not synthesized. From a large number of reports, it has been recognized that most affected patients show severe developmental delay in a bedridden state and are unable to speak. On the other hand, patients with a mild phenotype with AADC deficiency have been reported, but they number only a few cases. Therefore, the variation of phenotypes of the disease appears to be broad, and it may be challenging to diagnose an atypical phenotype as AADC deficiency.Case reportWe report novel compound heterozygous variants in DDC (c.202G > A and c.254C > T) in two sisters, whose main complaint was mild developmental delay, by whole-exome sequencing (WES). Additionally, we describe their clinical features and provide an image that shows the variants located at different sites responsible for the catalysis of AADC in a three-dimensional structure. The patients were prescribed a Monoamine oxidase (MAO) inhibitor after diagnosis.InterpretationOur cases indicate that a comprehensive genomic approach helps to diagnose AADC deficiency with atypical features, and underscore the significance of understanding the variations of this disorder for diagnosis and appropriate treatment.  相似文献   
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《Annales d'endocrinologie》2022,83(3):181-185
21-Hydroxylase deficiency (21OHD) is the most common cause of congenital adrenal hyperplasia. Increased production of adrenal-derived androgens and progesterone in 21OHD women interfere with their reproductive function and their fertility in many different ways, depending on the severity of the disease. Sexuality and fertility in women with classic 21OHD is impaired, due to several issues such as disrupted gonadotropic axis due to androgen and progesterone overproduction, and mechanical, psychological factors related to genital surgery. Fertility and fecundity in these women get better over the years. Subfertility seems contrariwise to be relative in non-classic 21OHD women. Before pregnancy, genotyping the partner and genetic counselling is mandatory.  相似文献   
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BackgroundThe opioid epidemic is an international public health concern. Pharmacists are in a strategic position to promote and implement effective opioid stewardship due to both their central role on health care teams and frequent interaction with patients. Despite this integral role, pharmacists do not have harmonized scopes of practice in opioid stewardship.ObjectivesThis scoping review was conducted to identify and critically review the role of pharmacists in opioid stewardship and identify future areas of study.MethodsThe scoping review was conducted according to the methodological framework proposed by Arksey and O'Malley, which was further modified by the Joanna Briggs Institute. Six databases were searched for original, peer-reviewed research; PubMed (MEDLINE), Ovid Embase, Ovid International Pharmaceutical Abstracts, Scopus, Cochrane Library, and APA PsycInfo.ResultsIn 92% of the included studies (n = 77), opioid stewardship interventions led by either a pharmacist or in an interdisciplinary team resulted in improvements in at least one outcome measure, with education and medication therapy adjustments being the most predominant activities. Other areas supported by evidence include community stakeholder education, policy and guideline setting, and risk assessment.ConclusionThis scoping review provides valuable insight into the various roles pharmacists can have in opioid stewardship. The findings from this review identified opioid stewardship activities that can make significant contributions towards reducing the impact of the opioid crisis. This review informs future research and has the potential to influence pharmacy practice on a national and international scale.  相似文献   
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AimTo identify factors contributing to Greek nursing students’ stress and the coping strategies used.BackgroundStress experienced by nursing students in the context of ongoing economic recession is as yet unexplored.DesignA cross-sectional study .MethodologyUndergraduate nursing students (n = 424) completed the Student Nurse Stress Index (short version) and the Brief COPE questionnaires during the year 2017.ResultsMost of the participants stated that their current financial status (81.7%) contributed towards their experience of stress, and they had higher scores on the interface worries subscale (p < 0.001). Female gender (p < 0.001) seniority (p = 0.005) and stress of transportation from home to university (p = 0.019) significantly increased the SNSI total score as well as most of the stress subscales scores. As far as coping subscales were concerned, higher means were shown in active/positive coping (mean 2.95), seeking support (mean 2.84), expression of negative feelings (mean 2.71) and avoidance (2.47). However, the use of higher maladaptive coping strategies was noted in cases presenting higher SNSI Total score, interface worries and personal problems.ConclusionThe introduction of stress management training and conflict management skills ought to be considered. Fund raising initiatives should be considered to provide financial assistance to those in need.  相似文献   
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