Cysteamine

半胱胺
  • 文章类型: Systematic Review
    患者报告的结果(PRO)描述了患者报告的整个医疗护理过程中患者经历的衡量标准(Mercieca-Bebber等。在患者关系结果测量中,2018)。存在各种PRO工具。鉴于缺乏组织框架,选择合适的仪器是具有挑战性的,该组织框架描述了皮肤病外科中所有可测量的PRO,并代表了哪些仪器测量了哪些结果。我们的目标是系统地审查皮肤病外科中所有经过验证的PRO仪器,并使用定性分析来开发代表PRO措施和仪器的组织框架。PubMed/MEDLINE,Embase,CINAHL,PsycINFO,和Cochrane数据库被搜索以检索皮肤科手术人群中经过验证的PRO仪器.使用持续的定性分析比较方法来开发代表皮肤病外科所有PRO的组织框架。所有仪器都被分类到这个框架中。搜索确定了3195篇文章;提取并定性分析了35种经过验证的仪器。组织框架将所有仪器分类为与美国国立卫生研究院患者报告结果测量信息系统(GershonRC,RothrockN,HanrahanR,etal(2010)在临床研究中使用PROMIS和评估中心提供患者报告的结果测量)。措施分为四类(期望,满意,生活质量,需求)描述患者如何体验这些结果以及研究人员可以通过它们进行评估的镜片。总之,我们提出了一个组织框架,用于选择经过验证的工具来开发和回答PRO研究问题。
    Patient-reported outcomes (PROs) describe measures of a patient\'s experience throughout medical care as reported by the patient (Mercieca-Bebber et al. in Patient Relat Outcome Meas, 2018). Various PRO instruments exist. It is challenging to select appropriate instruments given the absence of an organizational framework which describes all measurable PROs in dermatologic surgery and represents which instruments measure which outcomes. Our objective was to systematically review all validated PRO instruments in dermatologic surgery and use qualitative analysis to develop an organizational framework representing PRO measures and instruments. PubMed/MEDLINE, Embase, CINAHL, PsycINFO, and Cochrane databases were searched to retrieve validated PRO instruments in the dermatologic surgery population. The constant comparative method of qualitative analysis was used to develop an organizational framework representing all PROs in dermatologic surgery. All instruments were sorted into this framework. The search identified 3195 articles; 35 validated instruments were extracted and qualitatively analyzed. The organizational framework sorted all instruments into 36 PRO measures aligned with the National Institutes of Health Patient-Reported Outcomes Measurement Information System (Gershon RC, Rothrock N, Hanrahan R, et al (2010) The use of PROMIS and assessment center to deliver patient-reported outcome measures in clinical research). Measures were grouped into four categories (expectations, satisfaction, quality of life, needs) describing how patients experience these outcomes and lenses through which researchers can evaluate them. In conclusion, we have proposed an organizational framework for use in choosing validated instruments to develop and answer PRO research questions.
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  • 文章类型: Journal Article
    为了可持续发展,更好的性能,瘤胃发酵过程中的气体污染较少,有必要为反刍动物找到一种绿色和安全的饲料添加剂。半胱胺(CS)是哺乳动物细胞中天然产生的生物化合物。由于其能够控制激素分泌,因此被广泛用作反刍动物的生长促进剂。它主要控制生长抑素的循环浓度和增强生长激素的产生,导致增长业绩改善。CS以有利于动物和环境的方式调节瘤胃发酵过程,导致更少的甲烷产生和养分损失。使用CS的另一个有益效果是它改善了动物对营养素的可用性并增强了它们的吸收。CS还可以作为抗氧化剂,保护细胞免受氧化损伤。此外,CS对反刍动物的细菌和真菌α多样性没有不良影响。CS的膳食补充增强了有益微生物的群体。尽管如此,没有关于反刍动物繁殖性能使用CS的数据,因此,有必要评估使用CS在长期饲养动物中的效果。在这次审查中,根据最近公布的数据更新了CS的作用模式,以突出反刍动物使用CS的有益效果.
    For sustainable development, better performance, and less gas pollution during rumen fermentation, there is a need to find a green and safe feed additive for ruminants. Cysteamine (CS) is a biological compound naturally produced in mammalian cells. It is widely used as a growth promoter in ruminants because of its ability to control hormone secretions. It mainly controls the circulating concentration of somatostatin and enhances growth hormone production, leading to improved growth performance. CS modulates the rumen fermentation process in a way beneficial for the animals and environment, leading to less methane production and nutrients loss. Another beneficial effect of using CS is that it improves the availability of nutrients to the animals and enhances their absorption. CS also works as an antioxidant and protects the cells from oxidative damage. In addition, CS has no adverse effects on bacterial and fungal alpha diversity in ruminants. Dietary supplementation of CS enhances the population of beneficial microorganisms. Still, no data is available on the use of CS on reproductive performance in ruminants, so there is a need to evaluate the effects of using CS in breeding animals for an extended period. In this review, the action mode of CS was updated according to recently published data to highlight the beneficial effects of using CS in ruminants.
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  • 文章类型: Meta-Analysis
    BACKGROUND: Various topical agents have been used to treat melasma; however, a large-scale evaluation among the currently available treatment is lacking.
    OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of topical agents for melasma.
    METHODS: The MEDLINE, Embase, Web of Science, Cochrane, and Alt-Healthwatch databases were searched in November 2021. Original studies that reported pre- and post-treatment Melasma Area Severity Index (MASI)/modified Melasma Area Severity Index (mMASI) scores and/or adverse effects (AEs) were eligible for inclusion. The main outcome was the efficacy analyzed by the changes in the pre- and post-treatment with standardized mean difference (SMD) of MASI/mMASI scores; the AEs were calculated with incidence proportion by the reported percentage of skin irritations.
    RESULTS: A total of 45 studies (2359 patients) and 55 studies (4539 patients) met the inclusion criteria for efficacy and AEs, respectively. Hydroquinone (HQ) monotherapy (SMD -1.3, 95% CI [-1.6 to -1.0]), HQ-containing combination therapy (-1.4, [-1.7 to -1.1]), cysteamine (-1.6, [-2.0 to -1.2]), tranexamic acid (-1.5, [-2.0 to -1.1]), azelaic acid (-1.3, [-1.7 to -1.0]), and kojic acid (-0.9, [-1.3 to -0.5]) demonstrated comparable efficacy, while zinc sulfate did not exhibit statistically significant improvement (-1.2, [-2.7 to 0.4]). HQ-containing combination therapy (50.9%) and cysteamine (42.2%) demonstrated the highest incidence of irritation, while azelaic acid (18.7%), kojic acid (5.3%), and tranexamic acid (0.8%) revealed a lower risk.
    CONCLUSIONS: In this meta-analysis, non-HQ agents except zinc sulfate may be considered as an alternative to HQ-containing agents. However, treatment should be guided by patient\'s tolerance, availability, and physicians\' experience.
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  • 文章类型: Meta-Analysis
    Melasma is a recurrent hypermelanosis disorder characterized by the appearance of brownish and symmetrical spots on the skin. It affects the quality of life and is resistant to available treatment approaches. Cysteamine has been reported as a promising depigmenting agent for melasma treatment and following formulation enhancement, its use is being reported. This review aimed to evaluate the efficacy of the use of depigmenting formulations containing 5% cysteamine in the treatment of patients with melasma. A systematic search was performed in PubMed, Science Direct, and Scielo databases until December 27, 2021, based on criteria selected by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Statistical analysis was performed with Review Manager 5.4 software. The risk of bias was assessed using the Cochrane Risk of Bias Tool for Randomized Trials. A total of six studies containing 120 melasma patients treated with 5% cysteamine were included in this meta-analysis. The meta-analysis demonstrated that 5% cysteamine is effective for the treatment of patients with melasma (MD 6.26 [95% CI 3.68-8.83], p < 0.0001, I2  = 86%). In this review, through meta-analysis allows concluding that 5% cysteamine is effective in the treatment of melasma and presents a low probability of side or adverse effects.
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  • 文章类型: Journal Article
    化妆品的安全性评估考虑了“普通消费者”的暴露,不是理发师的职业暴露。这篇综述旨在收集和评估有关盐酸半胱胺(盐酸半胱胺;CAS号。156-57-0),聚乙烯吡咯烷酮(PVP;CASno.9003-39-8),PVP共聚物(CAS号28211-18-9),月桂基醚硫酸钠(SLES;CAS号9004-82-4),椰油酰胺二乙醇胺(椰油酰胺DEA;CAS号68603-42-9),和椰油酰胺丙基甜菜碱(CAPB;CAS号61789-40-0)。共识别出298篇文章,其中包括70个。荟萃分析显示,与不是理发师的对照组相比,理发师对CAPB发生接触过敏的风险增加了1.7倍。与消费者相比,理发师由于工作职责而可能有更高的风险获得对半胱胺HCl的量子敏化。关于cocamideDEA,这种表面活性剂的潜在刺激性不容忽视。PVP的原创文章,PVP共聚物,缺乏SLES。该系统评价表明,当前的标准不能有效地解决与美发师使用美发化妆品相关的职业风险。头发化妆品中使用的物质具有相当大的刺激性和/或过敏性潜力,应促使对当前的风险评估实践进行重新评估。
    The safety assessment of cosmetics considers the exposure of a \'common consumer\', not the occupational exposure of hairdressers. This review aims to compile and appraise evidence regarding the skin toxicity of cysteamine hydrochloride (cysteamine HCl; CAS no. 156-57-0), polyvinylpyrrolidone (PVP; CAS no. 9003-39-8), PVP copolymers (CAS no. 28211-18-9), sodium laureth sulfate (SLES; CAS no. 9004-82-4), cocamide diethanolamine (cocamide DEA; CAS no. 68603-42-9), and cocamidopropyl betaine (CAPB; CAS no. 61789-40-0). A total of 298 articles were identified, of which 70 were included. Meta-analysis revealed that hairdressers have a 1.7-fold increased risk of developing a contact allergy to CAPB compared to controls who are not hairdressers. Hairdressers might have a higher risk of acquiring quantum sensitization against cysteamine HCl compared to a consumer because of their job responsibilities. Regarding cocamide DEA, the irritant potential of this surfactant should not be overlooked. Original articles for PVP, PVP copolymers, and SLES are lacking. This systematic review indicates that the current standards do not effectively address the occupational risks associated with hairdressers\' usage of hair cosmetics. The considerable irritant and/or allergenic potential of substances used in hair cosmetics should prompt a reassessment of current risk assessment practices.
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  • 文章类型: Journal Article
    黄褐斑是一种慢性和获得性色素疾病,主要影响女性并损害患者的满意度和信心。黄褐斑主要影响女性,占所有病例的90%。它影响到所有种族的人,特别是那些患有IV型和V型皮肤的人,他们生活在有大量紫外线辐射的地区。根据研究,黄褐斑病变见于整个面部中心面,Malar,和下颌图案。前额上的黄褐斑病变,脸颊,鼻子,上唇,和/或下巴是最普遍的中心面部图案。黄褐斑病变也可以沿着眶周区域检测到,尤其是亚洲人。黄褐斑对治疗有明显的抵抗力,许多患者只是暂时缓解和复发。结合针对多种病理成分的疗法,包括光损伤,炎症,异常血管分布,异常的色素沉着,通常导致最戏剧性的治疗改进。黑眼圈的治疗包括局部脱色药物,如氢醌,曲酸,壬二酸,和局部视黄酸,和物理治疗,如化学剥离,手术调整,和激光治疗。治疗的目的应该是弄清楚是什么导致了色素沉着过度以及是什么导致了色素沉着过度。本文综述了黄褐斑的治疗方法以及甲伊咪唑和半胱胺治疗黄褐斑的疗效。
    Melasma is a chronic and acquired pigmentary condition that primarily affects women and undermines patient satisfaction and confidence. Melasma mostly affects females, accounting for 90% of all cases. It affects people of all races, particularly those with skin types IV and V who live in areas with lots of UV radiation. According to the studies, Melasma lesions are seen throughout the face in centrofacial, malar, and mandibular patterns. Melasma lesions on the forehead, cheeks, nose, upper lip, and/or chin are the most prevalent centrofacial pattern. Melasma lesions can also be detected along the periorbital area, especially in Asian people. Melasma is notably resistant to treatment, with many patients experiencing only temporary relief and relapses. Combining therapies that target numerous pathologic components, including photodamage, inflammation, aberrant vascularity, and abnormal pigmentation, generally results in the most dramatic therapeutic improvements. Treatments for dark circles include topical depigmenting medicines like hydroquinone, kojic acid, azelaic acid, and topical retinoic acid, and physical treatments such as chemical peels, surgical adjustments, and laser therapy. The objective of therapy should be to figure out what is causing the hyperpigmentation and what is contributing to it. This article provides an overview of melasma therapies and the efficacy of methimazole and cysteamine for melasma therapy.
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  • 文章类型: Journal Article
    新生儿筛查(NBS)计划被认为是儿童期最成功的二级预防措施之一,可通过早期疾病识别和随后开始治疗来预防或降低发病率和/或死亡率。然而,虽然许多罕见疾病现在可以通过适当的诊断在早期阶段被发现,引入新的目标疾病需要对整个筛查过程进行详细分析,包括强大的科学背景,分析,信息技术,和物流。此外,伦理,融资,并且需要考虑所需的医疗措施,以便在比其潜在危害更高的水平上评估筛查的益处。婴儿肾病性膀胱炎(INC)是一种非常罕见的溶酶体代谢紊乱。随着20世纪80年代初引入半胱胺治疗以及婴儿期肾脏替代治疗的可能性,患有膀胱炎的患者现在可以达到成年。对胱氨酸病的早期诊断仍然很重要,因为这可以尽早启动半胱胺,以支持肾脏和患者的生存。使用分子技术,一项试点项目已经证明了筛查胱氨酸病的可行性。这篇综述旨在提供对NBS的见解,并讨论其使用分子技术对肾病性膀胱炎的重要性。
    Newborn screening (NBS) programmes are considered to be one of the most successful secondary prevention measures in childhood to prevent or reduce morbidity and/or mortality via early disease identification and subsequent initiation of therapy. However, while many rare diseases can now be detected at an early stage using appropriate diagnostics, the introduction of a new target disease requires a detailed analysis of the entire screening process, including a robust scientific background, analytics, information technology, and logistics. In addition, ethics, financing, and the required medical measures need to be considered to allow the benefits of screening to be evaluated at a higher level than its potential harm. Infantile nephropathic cystinosis (INC) is a very rare lysosomal metabolic disorder. With the introduction of cysteamine therapy in the early 1980s and the possibility of renal replacement therapy in infancy, patients with cystinosis can now reach adulthood. Early diagnosis of cystinosis remains important as this enables initiation of cysteamine at the earliest opportunity to support renal and patient survival. Using molecular technologies, the feasibility of screening for cystinosis has been demonstrated in a pilot project. This review aims to provide insight into NBS and discuss its importance for nephropathic cystinosis using molecular technologies.
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  • 文章类型: Case Reports
    BACKGROUND: Melasma remains a recurrent, chronic, therapeutically challenging, and psychologically burdening condition. Several different modalities and approaches have been utilized, and some with notable success to experimentally manage the condition. Cysteamines, with their depigmentation properties, have only recently been intensely studied. One such formulation is the topical 5% cysteamine hydrochloride, the structure of which is notably more stable and with a less foul odor than its prior counterparts. We, therefore, aimed to assess the efficacy of the mentioned formulation in the treatment of melasma.
    METHODS: The PubMed, SCOPUS, ISI Web of Science, and Embase, Cochrane, and Proquest databases were thoroughly searched for English studies evaluating the effects of the topical agent mentioned.
    RESULTS: Eight studies (five RCTs, two case reports, and one case series) were included after three rounds of screening, most of which were carried out in Iran. Statistical significance was noted when assessing decreased melanin content and satisfaction rates.
    CONCLUSIONS: It appears that the cysteamine cream could be comparably efficient in treating melasma while accompanied only by minor and transient adverse events. However, current evidence is limited by insufficient sample size, long-term follow-up, and only to epidermal melasma, highlighting the need for appropriately designed randomized controlled clinical trials to draw a conclusive image of the cysteamine\'s role in treating this recalcitrant condition.
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  • 文章类型: Journal Article
    To evaluate safety and efficacy of topical cysteamine ophthalmic solution for corneal cystinosis.
    Seven databases were searched (PubMed, OVID, EMBASE, Web of Science, Cochrane Central, Google Scholar, and ClinicalTrials.gov) for relevant studies, using appropriate keywords. Comparative observational studies and randomized controlled trials comparing cysteamine with control or other formulations for treatment of corneal or ophthalmic cystinosis were included. Outcome measurements were improvement or response to therapy, change in corneal cystine crystal score (CCCS), in vivo confocal microscopy score (IVCM), cystine crystal depth, contrast sensitivity (CS), photophobia score, and safety.
    Systematic review and meta-analysis.
    Seven studies were included. Compared to placebo and control, the cysteamine arm was better in terms of improvements and responses to therapy (2 studies showed a risk ratio [RR] of 16; 95% confidence interval [CI]: 2.30-111.37) and crystal density score (1 study showed a mean difference [MD] of -0.80; 95% CI: -1.56 to -0.04). No significant differences were observed in terms of improvement in CS (1 study showed an RR of 7.00; 95% CI: 0.47-103.27). Compared to cystamine, cysteamine showed benefits in terms of crystal density score (MD -0.94; 95% CI: -1.64 to -0.24). Compared to a newer formulation, the standard formulation (cysteamine [Cystaran]; 0.55% cysteamine hydrochloride + benzalkonium chloride 0.01%) performed better in terms of decreasing CCCS. Another newer, viscous formulation, Cystadrops, performed better than the standard formulation in terms of change in CCCS, IVCM score, corneal crystal depth, and photophobia score; however, local adverse effects and blurring were higher in the group receiving Cystadrops.
    Conventional cysteamine (0.1% to 0.3%) performed better than placebo (control) in terms of response to therapy. In terms of decreasing corneal cystine density, cysteamine (0.55%) was better than cystamine (0.55%), and the viscous Cystadrops (0.55%) was better than the standard formulation (0.1%).
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  • 文章类型: Journal Article
    虽然肾病性膀胱炎通常被认为是一种儿童疾病,通过改进治疗,患者通常活到成年。我们对有关该人群随着年龄增长而面临的并发症的现有文献进行了系统回顾。几乎每个器官系统都会受到胱氨酸病的影响,要么是因为疾病本身,要么是因为肾移植的后遗症。虽然已知半胱胺可以延缓终末期肾病的发作,其对其他膀胱炎并发症的影响尚不明确。更常见的成人发病并发症包括肌病,糖尿病,和甲状腺功能减退。一些不太常见的并发症,比如神经功能障碍,仍然可以对患有胱氨酸病的人产生深远的影响。该领域的进一步研究领域包括半胱胺对膀胱炎非肾脏表现的影响的其他研究。以及新的和新颖的治疗方法的可能途径。
    While nephropathic cystinosis is classically thought of as a childhood disease, with improved treatments, patients are more commonly living into adulthood. We performed a systematic review of the literature available on what complications this population faces as it ages. Nearly every organ system is affected in cystinosis, either from the disease itself or from sequelae of kidney transplantation. While cysteamine is known to delay the onset of end-stage kidney disease, its effects on other complications of cystinosis are less well determined. More common adult-onset complications include myopathy, diabetes, and hypothyroidism. Some less common complications, such as neurologic dysfunction, can still have a profound impact on those with cystinosis. Areas for further research in this area include additional study of the impact of cysteamine on the nonrenal manifestations of cystinosis, as well as possible avenues for new and novel treatments.
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