manifestations

表现
  • 文章类型: Journal Article
    假性剥脱综合征(PEX)是一种长期、与年龄相关的细胞外基质状况,导致异常的原纤维假性剥脱物质(PXM)在各种身体组织中积聚。眼睛的前部是这种疾病最常见的地方。它影响整个身体。大多数情况下,它可以在老年人身上看到,通常是50岁以上的人。纤维沉积是假性剥脱综合征的症状,在眼睛的前部发现。可见纤维白色片状材料的沉积。晶状体囊,角膜,睫状上皮,晶状体上皮,虹膜色素上皮,小带,眼眶软组织,小梁网,虹膜血管,据报道,虹膜基质和虹膜基质都显示出这种沉积。皮肤,心,肺,肝脏,肾脏,据报道,其他器官也含有这些沉积物。不对称和双侧疾病都是可能的。心肌梗塞,脑血管意外,和全身性高血压都与之有关。首先报道了假性剥脱情况,并在晶状体前囊上发现了白色或灰色薄片的特征。青光眼的患病率随着年龄的增长而上升,它存在于大约50%的眼睛中。几十年后,假性剥脱症这个术语是为了区别于真正的剥脱综合症。真正的剥脱综合征的特征是晶状体囊的层状分层,是由暴露于红外辐射引起的。它常见于玻璃鼓风机。一旦一个人达到70岁,年龄是PEX的风险因素。PEX的症状包括眼压升高,乳头周围透视缺陷,潜在的青光眼视神经损伤,可怜的扩张,Sampaolesi线,瞳孔边界和纤维状白色片状沉积物。同时,晶状体前囊(HoarfrostRing)上的纤维状白色片状沉积物和色素分散综合征不是病理性的。
    Pseudoexfoliation syndrome (PEX) is a long-term, age-related extracellular matrix condition that causes aberrant fibrillary pseudoexfoliative material (PXM) to accumulate in various body tissues. The anterior portion of the eye is where this disorder most frequently presents. It affects the entire body. Most frequently, it is seen in older people, usually those over 50. Fibrillar deposits are a symptom of the pseudoexfoliation syndrome and are found in the anterior part of the eye. Deposition of fibrillary white flaky material is seen. The lens capsule, cornea, ciliary epithelium, lens epithelium, iris pigment epithelium, zonules, orbital soft tissues, trabecular meshwork, iris blood vessels, and iris stroma have all been reported to show such depositions. The skin, heart, lungs, liver, kidneys, and other organs have also been reported to contain these deposits. Asymmetrical and bilateral illnesses are both possible. Myocardial infarction, cerebrovascular accidents, and systemic hypertension have all been linked to it. The pseudoexfoliative condition was first reported with the characteristic findings of white or grey flakes on the anterior lens capsule, the prevalence of glaucoma rising with age, and its presence in about 50% of eyes. A few decades later, the term pseudoexfoliation was given to differentiate it from the true exfoliation syndrome. True exfoliation syndrome is characterized by lamellar delamination of the lens capsule and is caused by exposure to infrared radiation. It is commonly seen in glassblowers. Age is a risk factor for PEX once a person reaches 70. Symptoms of PEX include elevated intraocular pressure, peripapillary transillumination deficiencies, potential glaucomatous optic nerve damage, poor dilatation, Sampaolesi line, and fibrillar white flaky deposits along the pupillary border. Meanwhile, fibrillar white flaky deposits on the anterior lens capsule (Hoarfrost Ring) and pigment dispersion syndrome are not pathognomonic.
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  • 文章类型: Systematic Review
    猴痘是一种人畜共患疾病,中部和西部非洲地区的地方病,并重新出现,目前导致截至2022年5月的疫情爆发。在这次系统审查中,我们的目标是描述疾病的当前面貌,对皮肤粘膜进行了详细的分类,以及该疾病的全身症状。我们搜索了四个主要的在线数据库,关键字为“猴痘”和“正痘病毒”。共收录46篇文章,累计1984年确诊病例。患者主要是与男性发生性关系的男性,他们大多在30多岁,有无保护的性接触或国际旅行史。在皮肤粘膜表现中,肛门生殖器病变是最常见的,然后是四肢的病变,脸,树干,和手掌或鞋底。在病变类型中,膀胱脓疱,脓疱或假脓疱,泡状膜和丘疹性病变是最常见的,主要是异步呈现,每个患者的病变少于10个。几乎所有患者也报告了全身表现,即发烧,淋巴结病,疲劳,肌痛,头痛,咽炎,和直肠炎。性接触是本次疫情的主要传播途径,体液中的病毒脱落起着关键作用。我们已经将这些新爆发的特殊发现与以前的爆发进行了比较。我们还从我们纳入的研究中收集并分类了图像,为这个“新的”猴痘面孔制作了一个“临床地图集”,这对临床医生来说是最重要的,并对猴痘的鉴别诊断有清晰的了解。
    Monkeypox is a zoonotic disease, endemic in central and west African regions, and has re-emerged, currently causing an outbreak as of May 2022. In this systematic review, we aimed to characterize the current face of the disease, with a detailed categorization of mucocutaneous, as well as systemic symptoms of the disease. We searched four main online databases with the keywords \"monkeypox\" and \"Orthopoxvirus\". A total of 46 articles were included, with a cumulative number of 1984 confirmed cases. Patients were predominantly men who have sex with men, who were mostly in their 30s, with a history of unprotected sexual contact or international travel. Among mucocutaneous manifestations, anogenital lesions were the most commonly observed, followed by lesions on the limbs, face, trunk, and palms or soles. Among lesion types, vesiculopustular, pustular or pseudo-pustular, vesicular-umbilicated and papular lesions were the most common, mainly presenting asynchronously, with less than 10 lesions on each patient. Almost all patients also reported systemic manifestations, namely fever, lymphadenopathy, fatigue, myalgia, headaches, pharyngitis, and proctitis. Sexual contact is the main pathway of transmission in the current outbreak, with viral shedding in bodily fluids playing a key role. We\'ve compared these idiosyncratic findings of the new outbreak with previous outbreaks. We\'ve also gathered and categorized images from our included studies to make a \"clinical atlas\" for this \"new\" face of monkeypox, which can be of utmost importance for clinicians to be familiarized with, and have a clear picture of monkeypox for their differential diagnoses.
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  • 文章类型: Case Reports
    2020年3月11日,由严重急性呼吸道综合症冠状病毒(SARS-CoV-2)引起的2019年冠状病毒病(COVID-19)被世界卫生组织(WHO)宣布为大流行。随着COVID-19大流行在全球肆虐,孩子们没有受到影响。从成人的疾病经验中收集的信息有助于儿童的疾病检测和治疗策略。成人文献中已经描述了许多关于COVID-19血液学表现的病例。本病例系列旨在报告儿童COVID-19和多系统炎症综合征患者的几种血液学表现(MIS-C,导致严重COVID-19疾病的免疫介导反应)有或没有原发性血液病。
    On 11 March 2020, coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus (SARS-CoV-2) was declared as a pandemic by the World Health Organization (WHO). As the COVID-19 pandemic has ravaged worldwide, children have not been unaffected. Information gleaned from adult experience with the disease has aided in disease detection and treatment strategies in children. Numerous cases have been described in adult literature about hematologic manifestations of COVID-19. This case series aims to report several hematologic presentations in patients with COVID-19 and multisystem inflammatory syndrome in children (MIS-C, an immune-mediated reaction leading to severe COVID-19 illness) with and without a primary hematologic disorder.
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  • 文章类型: Journal Article
    传染病被定义为由任何感染微生物引起的一组疾病,这些感染微生物高度有效地严重影响人类生活。从严重感染到死亡,结局可能有所不同。据报道,大多数传染病的传播速度很快。马尔堡病毒病是一种通常表现为出血热的传染性病毒性疾病。世卫组织确认的最新报告的马尔堡病毒病病例是2021年8月6日在几内亚西南部省份。马尔堡病毒病表现出与埃博拉病毒感染相似的表现。尽管没有广泛传播以成为大流行,马尔堡病毒病仍然严重威胁着人类的生命。这篇综述强调了通过与马尔堡病毒感染有关的各种研究确定的新的当前事实。从这些有希望的理论中,review试图提出各种研究结论的重要性,这些结论可能在不久的将来对卫生部门产生重大影响。
    Infectious diseases are defined as a group of diseases caused by any infecting microorganism which are highly potent to severely affect human life. The end can vary from critical infection to mortality. Most infectious diseases are reported with a rapid rate of transmission. Marburg virus disease is a kind of infectious viral disease usually manifested as hemorrhagic fever. The latest reported case of Marburg virus disease confirmed by WHO was on 6th August 2021 in the south-western province of Guinea. Marburg virus disease exhibit similar manifestations to that of infection with the Ebola virus. Though not widely spread to emerge as a pandemic, Marburg virus disease remains a serious threat to human life. This review emphasizes the novel current facts determined through various studies related to Marburg virus infection. From these promising theories, the review tries to put forward the importance of various study conclusions, which are likely to have a major impact on the health sector in the near future.
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  • 文章类型: Journal Article
    为了表征人口统计,遗传,临床,西班牙北部地区抗3-羟基-3-甲基戊二酰辅酶A还原酶(HMGCR)免疫介导的坏死性肌病(IMNM)患者的血清学特征。
    在西班牙北部一家参考医院进行的5年期间所有诊断为抗HMGCRIMNM的患者的研究。除了临床和实验室数据,我们分析了HLA基因的遗传影响和SLCO1B1基因中的rs4149056(c.521T>C)单核苷酸多态性(SNP)。
    8名患者(5名女性,3名男性),平均±SD年龄为64.9±7.3岁,符合抗HMGCRIMNM的标准。发病率为0.6/100.000人年,患病率为3/100.000人口。所有患者都暴露于他汀类药物。他们都有明显的下肢近端和对称肌无力,2人严重,血清CK水平升高,中位数[IQR]为4488[2538-9194]IU/L。血清25-羟基维生素D水平在所有确定的患者中均降低。先前诊断为甲状腺功能减退症的3例患者在诊断时TSH水平异常。所有患者使用不同的免疫抑制治疗方案均有所改善。值得注意的是,8例患者中有7例携带HLA-DRB1*11等位基因。SLCO1B1基因中rs4149056C等位基因的频率(12.5%)与普通人群相似。
    在西班牙北部,抗HMGCRIMNM优先影响50岁以上HLA-DRB1*11等位基因携带者并服用他汀类药物的人群。低维生素D水平和甲状腺功能减退可能在该疾病的发展中起潜在的诱发作用。
    To characterize the demographic, genetic, clinical, and serological features of patients with anti-3‑hydroxy-3-methylglutaryl-CoA reductase (HMGCR) immune-mediated necrotizing myopathy (IMNM) in a region of northern Spain.
    Study of all patients diagnosed with anti-HMGCR IMNM during a 5-year period at a reference hospital in northern Spain. Besides clinical and laboratory data, we analyzed the genetic influence of HLA genes and the rs4149056 (c.521T>C) single nucleotide polymorphism (SNP) in the SLCO1B1 gene.
    8 patients (5 women, 3 men) with a mean ± SD age of 64.9 ± 7.3 years, fulfilled the criteria for anti-HMGCR IMNM. The incidence rate was 0.6 per 100.000 person-years and the prevalence 3 per 100.000 population. All patients had been exposed to statins. All of them had predominant lower limb proximal and symmetric muscle weakness that was severe in 2 and had elevated serum CK levels with a median [IQR] of 4488 [2538-9194] IU/L. Serum 25‑hydroxy vitamin D levels were decreased in all patients in whom it was determined. The 3 patients with a previous diagnosis of hypothyroidism had abnormal levels of TSH at the time of diagnosis. All patients experienced improvement with different schemes of immunosuppressive therapy. Noteworthy, 7 of 8 patients carried the HLA-DRB1*11 allele. The frequency of the rs4149056 C allele in the SLCO1B1 gene (12.5%) was similar to that of the general population.
    In northern Spain, anti-HMGCR IMNM preferentially affects people over 50 years of age who are carriers of the HLA-DRB1*11 allele and take statins. Both low vitamin D levels and hypothyroidism may play a potential predisposing role in the development of this disease.
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  • 文章类型: Journal Article
    自2019年爆发以来,冠状病毒病(COVID-19)已经成为一种流行病,影响超过5200万人,并导致全球超过100万人死亡。目前的研究揭示了广泛的疾病表现和行为,包括体内多个器官系统和巨大的全身性炎症。这些都在这篇综述中进行了总结。来自一些科学评论的数据,研究文章,案例系列,观察性研究,病例报告是通过使用Cochrane等在线搜索引擎检索的,PubMed,和Scopus从2019年12月到2020年11月。体征和症状患病率的数据,使用SPSS第25版分析了潜在的疾病机制和合并症。这篇综述将讨论迄今为止记录的各种COVID-19临床表现,以及目前对潜在的一般以及系统特异性病理生理学的理解,和致病途径。这些包括病毒通过ACE2受体直接渗透到宿主细胞中,通过病毒蛋白诱导炎症和免疫反应,以及全系统炎症和细胞因子产生的启动。此外,外周器官损伤和潜在的共病,可导致短期和长期的疾病,对身体的可逆和不可逆损伤也进行了研究。我们得出的结论是,潜在的合并症及其对身体的病理影响在很大程度上决定了患者的疾病严重程度和死亡率。目前还没有批准用于治疗COVID-19的药物,但是现在正在使用多种疫苗,更多的研究正在进行中。
    Since its outbreak in 2019, the coronavirus disease (COVID-19) has become a pandemic, affecting more than 52 million people and causing more than 1 million mortalities globally till date. Current research reveals a wide array of disease manifestations and behaviors encompassing multiple organ systems in body and immense systemic inflammation, which have been summarized in this review. Data from a number of scientific reviews, research articles, case series, observational studies, and case reports were retrieved by utilizing online search engines such as Cochrane, PubMed, and Scopus from December 2019 to November 2020. The data for prevalence of signs and symptoms, underlying disease mechanisms and comorbidities were analyzed using SPSS version 25. This review will discuss a wide range of COVID-19 clinical presentations recorded till date, and the current understanding of both the underlying general as well as system specific pathophysiologic, and pathogenetic pathways. These include direct viral penetration into host cells through ACE2 receptors, induction of inflammosomes and immune response through viral proteins, and the initiation of system-wide inflammation and cytokine production. Moreover, peripheral organ damage and underlying comorbid diseases which can lead to short term and long term, reversible and irreversible damage to the body have also been studied. We concluded that underlying comorbidities and their pathological effects on the body contributed immensely and determine the resultant disease severity and mortality of the patients. Presently there is no drug approved for treatment of COVID-19, however multiple vaccines are now in use and research for more is underway.
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  • 文章类型: Journal Article
    To establish practical recommendations for the management of patients with psoriatic arthritis (PsA) with particular clinical situations that might lead to doubts in the pharmacological decision-making. A group of six expert rheumatologists on PsA identified particular clinical situations in PsA. Then, a systematic literature review (SLR) was performed to analyse the efficacy and safety of csDMARDs, b/tsDMARDs in PsA. In a nominal group meeting, the results of the SLR were discussed and a set of recommendations were proposed for a Delphi process. A total of 65 rheumatologists were invited to participate in the Delphi. Agreement was defined if ≥ 70% of the participants voted ≥ 7 (from 1, totally disagree to 10, totally agree). For each recommendation, the level of evidence and grade of recommendation was established based on the Oxford Evidence-Based Medicine categorisation. Particular clinical situations included monoarthritis, axial disease, or non-musculoskeletal manifestations. The SLR finally comprised 131 articles. A total of 16 recommendations were generated, all but 1 reached consensus. According to them, it is crucial to carefully analyse the impact of individual manifestations on patients (disability, quality of life, etc.), but also to recognise the impact of each drug singularities on selected clinical phenotypes to adopt the most appropriate treatment strategy. Early diagnosis and treatment to target approach, along with a close risk management, is also necessary. These recommendations are intended to complement gaps in national and international guidelines by helping health professionals address and manage particular clinical situations in PsA.
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  • 文章类型: Journal Article
    Behçet\'s Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis-major vessel and life-or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.
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  • 文章类型: Journal Article
    2019年12月新型冠状病毒-严重急性呼吸系统综合症-冠状病毒2(SARS-CoV2)-起源于武汉,中国,并迅速传播到世界各地。这篇文献综述强调了COVID-19传播和呈现的动态性质。分析截至5月1日的59篇相关文章,2020年反映,COVID-19大流行的主要报告临床表现是发热和呼吸道受累。此外,目前的文献显示了COVID-19的各种不同和非典型表现。SARS-CoV2传播的明确途径是呼吸道飞沫,然而,在粪便和尿液标本中也检测到病毒核酸。COVID-19症状的严重程度和结果因患者的医学背景而异,年龄,性别,和并发的医疗条件(例如怀孕)。这是第一次对COVID-19表现的所有要点进行分类,一目了然,通过更好地了解诊断和管理来改善患者的预后。
    In December 2019 novel coronavirus-Severe Acute Respiratory Syndrome-Corona Virus2 (SARS-CoV2)-originated from Wuhan, China, and spread rapidly around the world. This literature review highlights the dynamic nature of COVID-19 transmission and presentation. Analyzing 59 relevant articles up to May 1st, 2020 reflects that the main reported clinical manifestation of COVID-19 pandemic is fever and respiratory involvement. Also, current literature demonstrates a wide spectrum of different and atypical presentation(s) of COVID-19. The definite route of SARS-CoV2 transmission is respiratory droplets, however, virus nucleic acid has been detected in the stool and urine specimens as well. The severity of symptoms and outcomes of COVID-19 vary based on the patient\'s medical background, age, sex, and concurrent medical conditions (e.g. pregnancy). This is the first review that classifies all essential points regarding COVID-19 manifestations at a glance to improve the outcome of the patients by a better insight into diagnosis and management.
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  • 文章类型: Journal Article
    UNASSIGNED: Human coronavirus (HCoVs) are a group of viruses with recognized neurotropic and neuroinvasive capabilities. The reports on the neurological and ocular findings are increasing day after day and several central and peripheral neurological manifestations are already described. However, none specifically describes the neuro-ophthalmological manifestation of HCoVs. This is the first article specifically reviewing neuro-ophthalmological manifestations of HCoVs infection.
    UNASSIGNED: PubMed and Google Scholar databases were searched using the keywords: coronaviridae, coronavirus, COVID-19, SARS-CoV-2, SARS-CoV-1, MERS, ocular, ophthalmology, ophthalmological, neuro-ophthalmology, neurological, manifestations. A manual search through the reference lists of relevant articles was also performed. There were no restrictions concerning language or study type and publications not yet printed but available online were considered.
    UNASSIGNED: Coronavirus eye involvement is not frequent and includes mostly a typical viral follicular conjunctivitis. Recently, retinal anatomical alterations were described using optic coherence tomography. Neuro-ophthalmological symptoms and signs can appear isolated or associated with neurological syndromes. The manifestations include headache, ocular pain, visual impairment, diplopia, and cranial nerve palsies secondary to Miller Fisher syndrome, Guillain-Barré syndrome, or encephalitis, and nystagmus.
    UNASSIGNED: Neurological and neuro-ophthalmological syndromes, symptoms, and signs should not be neglected and a complete ophthalmological examination of these patients should be performed to fully describe ocular manifestations related to HCoVs. We believe that major ocular and neuro-ophthalmological manifestations reports lack due to safety issues concerning detailed ophthalmological examination; on the other hand, in a large number of cases, the presence of life-threatening coronavirus disease hinders ocular examination and ophthalmologist\'s visit to the intensive care unit.
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