sclerosis

硬化
  • 文章类型: Case Reports
    硬皮病是一种以炎症和血管异常为特征的多系统疾病,和过度纤维化。进行性系统性硬化症(PSS)主要随皮肤进展,接头,肺,心,和肾脏受累。在局部硬皮病和PSS中,脑血管的受累很少见。短暂性脑缺血发作和中风是硬皮病的罕见并发症。
    我们介绍了一位60岁的中风患者,患有局限性硬皮病,表现为言语障碍,忘记单词,偶尔会暂时失忆.
    在我们介绍的情况下,在缺血性危险因素方面进行的临床和实验室检查中未发现病理.皮肤表现包括挛缩,皮肤活检结果,与硬皮病相关的抗体阳性。鉴于硬皮病目前的发病机制,病人怀疑中风。
    UNASSIGNED: Scleroderma is a multisystemic disorder characterised by inflammatory and vascular anomalies, and excess fibrosis. Progressive systemic sclerosis (PSS) mainly progresses with skin, joint, lung, heart, and kidney involvement. Involvement of cerebral vessels is rare in both localised scleroderma and PSS. Transient ischemic attack and stroke are rare complications of scleroderma.
    UNASSIGNED: We present a 60-year-old stroke patient with localised scleroderma presenting with impaired speech, forgetting words, and occasional temporary memory loss.
    UNASSIGNED: In the case we present, no pathology was found in the clinical and laboratory tests performed in terms of ischemic risk factors. Skin findings included contracture, skin biopsy results, and antibody positivity related to scleroderma. Given the current pathogenesis of scleroderma, the patient was suspected of having a stroke.
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  • 文章类型: Journal Article
    阴茎Mondor病(PMD)是一种罕见的综合征,其特征是阴茎浅静脉的浅表血栓性静脉炎后硬化。PMD最常见的外观是招标,明显的,痛苦,有时在阴茎背面可见的绳索。其发病机制尚不清楚,标准化治疗尚未建立。
    一名54岁男性患者出现左侧间接复位腹股沟疝。患者接受Lichtenstein腹股沟疝修补术。术后第十天,他返回时,经多普勒超声检查证实为PMD。每天用4000UI低分子量肝素(LMWH)治疗三周,缓解了症状,但轻微的静脉扩张只是阴茎的近端部分仍然存在。
    PMD的确切原因尚不清楚,但是各种研究已经确定了与这种疾病风险增加相关的某些因素。在各种可能引发PMD的潜在因素中,腹股沟疝的修复仅有一次报道。治疗可能涉及疼痛管理,抗炎药,抗凝剂,and,在某些情况下,手术。
    开放式疝修补术后的PMD是一种非常罕见的良性疾病。正确的诊断和及时的治疗可以缓解症状。残余静脉扩张除了外观外观外没有临床意义。
    UNASSIGNED: Penile Mondor\'s disease (PMD) is a rare syndrome characterized by sclerosis after superficial thrombophlebitis of the superficial penile veins. The most usual appearance of PMD is a tender, palpable, painful, and sometimes visible cord on the dorsal surface of the penis. Its pathogenesis is still unclear, and a standardized treatment has not been established.
    UNASSIGNED: A 54-year-old male patient presented with a left-sided indirect reducible inguinal hernia. The patient underwent Lichtenstein\'s procedure for inguinal hernia repair. On the tenth postoperative day, he returned with PMD confirmed by Doppler ultrasonography examination. Treatment with 4000 UI low molecular weight heparin (LMWH) daily for three weeks resolved the symptoms, but mild venous ectasia just to the proximal part of the penis remained.
    UNASSIGNED: The exact cause of PMD is not well understood, but various studies have identified certain factors associated with an increased risk of the condition. Out of various potential factors that could trigger PMD, the repair of an inguinal hernia has been reported only once. Treatment may involve pain management, anti-inflammatory medications, anticoagulants, and, in some cases, surgery.
    UNASSIGNED: PMD after open hernia repair surgery is a very rare benign condition. Correct diagnosis and prompt treatment allowed symptom resolution. Residual venous ectasia has no clinical significance other than a cosmetic appearance.
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  • 文章类型: Journal Article
    股骨头坏死(FHN)是股骨颈骨折(FNF)后的严重并发症,通常与螺旋路径周围的硬化有关。我们的研究旨在使用整合的蛋白质组学和代谢组学分析揭示FHN和硬化症的蛋白质组学和代谢组学基础。我们确定了三组之间的差异表达蛋白(DEP)和代谢物(DEM):FNF患者(A组),硬化症(B组),和FHN(C组)。使用京都基因和基因组百科全书和基因本体论富集分析,我们研究了这些蛋白质和代谢物的作用。我们的发现强调了不同群体之间的显著差异,在硬化组和FNF组之间确定了218个DEP和44个DEM,FHN和硬化症组之间的247DEP和31DEM,FHN和FNF组之间有682个DEP和94个DEM。与碳酸盐脱水酶和水解酶相关的活性在FHN和硬化组中相似,而FHN和FNF组普遍存在胞外区域和溶酶体。我们的研究还强调PI3K-Akt途径参与硬化和FHN。此外,关键的代谢途径与甘油磷脂代谢和内源性大麻素的逆行信号有关.使用西方印迹,我们证实了特定基因/蛋白质如ITGB5,TNXB,CAII,硬化中的CAIII和FHN中的酸性磷酸酶5和组织蛋白酶K。这种全面的分析阐明了硬化和FHN背后的分子机制,并提出了潜在的生物标志物和治疗靶点。为改进治疗策略铺平道路。有必要进一步验证研究结果,以加强结果的稳健性和可靠性。
    Femoral head necrosis (FHN) is a serious complication after femoral neck fractures (FNF), often linked to sclerosis around screw paths. Our study aimed to uncover the proteomic and metabolomic underpinnings of FHN and sclerosis using integrated proteomics and metabolomics analyses. We identified differentially expressed proteins (DEPs) and metabolites (DEMs) among three groups: patients with FNF (Group A), sclerosis (Group B), and FHN (Group C). Using the Kyoto Encyclopedia of Genes and Genomes and Gene Ontology enrichment analyses, we examined the roles of these proteins and metabolites. Our findings highlight the significant differences across the groups, with 218 DEPs and 44 DEMs identified between the sclerosis and FNF groups, 247 DEPs and 31 DEMs between the FHN and sclerosis groups, and a stark 682 DEPs and 94 DEMs between the FHN and FNF groups. Activities related to carbonate dehydratase and hydrolase were similar in the FHN and sclerosis groups, whereas extracellular region and lysosome were prevalent in the FHN and FNF groups. Our study also emphasized the involvement of the PI3K-Akt pathway in sclerosis and FHN. Moreover, the key metabolic pathways were implicated in glycerophospholipid metabolism and retrograde endocannabinoid signaling. Using western blotting, we confirmed the pivotal role of specific genes/proteins such as ITGB5, TNXB, CA II, and CA III in sclerosis and acid phosphatase 5 and cathepsin K in FHN. This comprehensive analyses elucidates the molecular mechanisms behind sclerosis and FHN and suggests potential biomarkers and therapeutic targets, paving the way for improved treatment strategies. Further validation of the findings is necessary to strengthen the robustness and reliability of the results.
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  • 文章类型: Journal Article
    目的:本研究旨在分析癫痫和手术变量与术后记忆表现之间的关系,术后因海马硬化(HS)引起的难治性内侧颞叶癫痫(MTLE)。
    方法:回顾了201例MTLE/HS手术患者的即时和晚期随访的逻辑记忆(LM)和视觉记忆(VM)评分。用年龄和教育程度相匹配的54名健康个体的对照组对分数进行标准化。计算可靠变化指数(RCI)以验证晚期LM和VM分数的个体记忆变化。使用LM和VM评分以及临床变量对RCI进行多元线性回归分析。
    结果:总共112例(56%)患者有正确的HS。右HS组的RCI显示6例(7%)患者在晚期LM中表现出改善,而5例(6%)患者表现出降低的评分;对于晚期VM,7例(8%)患者出现改善,2例(3%)患者的评分较差。左HS组的RCI显示3(3%)个人得分提高,而5例(4%)患者的晚期LM评分恶化;对于晚期VM,3例(3%)患者得分较高,6例(5%)得分较低。左HS和首次癫痫发作时的高龄是晚期LM丢失的预测因素(p<0.05)。
    结论:左MTLE/HS和高龄时癫痫发作是晚期LM恶化的预测因素。我们观察到左侧HS组的基线LM功能较差,而一些切除右侧MTL的患者的LM改善。正确的HS组患者的VM和LM评分的术后可靠改善百分比更高。
    OBJECTIVE: This study was performed with the purpose of analysing the relationship between epileptological and surgical variables and post-operative memory performance, following surgery for refractory mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS).
    METHODS: Logical memory (LM) and visual memory (VM) scores for immediate and late follow-up of 201 patients operated for MTLE/HS were reviewed. Scores were standardized with a control group of 54 healthy individuals matched for age and education. The Reliable Change Index (RCI) was calculated to verify individual memory changes for late LM and VM scores. A multiple linear regression analysis was carried out with the RCI, using LM and VM scores as well as the clinical variables.
    RESULTS: A total of 112 (56%) patients had right HS. The RCI of the right HS group demonstrated that 6 (7%) patients showed improvement while 5 (6%) patients showed decreased scores in late LM; for late VM, 7 (8%) patients presented improvement, and 2 (3%) patients showed poorer scores. RCI of the left HS group showed that 3 (3%) individuals showed improved scores, while scores of 5 (4%) patients worsened for late LM; for late VM, 3 (3%) patients presented higher scores and 6 (5%) showed lower scores. Left HS and advanced age at onset of the first epileptic seizure were predictors of late LM loss (p<.05).
    CONCLUSIONS: Left MTLE/HS and seizure onset at advanced ages were predictive factors for the worsening of late LM. We observed poorer baseline LM function in the left HS group and improvement of LM in some patients who had resection of the right MTL. Patients in the right HS group showed a higher percentage of reliable post-operative improvement for both VM and LM scores.
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  • 文章类型: Case Reports
    作者描述了一例推定的猫科动物皮下脂肪硬化,以前(1987年)仅报告过一次,并通过验尸诊断。我们描述了射线照相,计算机断层扫描和组织病理学发现,对口服甲基强的松龙和维生素E的部分反应
    The authors describe a case of presumptive feline subcutaneous fat sclerosis, a condition reported only once previously (in 1987) and diagnosed with postmortem examination. We describe radiographic, computed tomography scan and histopathological findings, and partial response to oral methylprednisolone and Vitamin E.
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  • 文章类型: Case Reports
    围手术期饮水(PIWD)是颞叶癫痫的一种罕见的植物性表现,没有明确的侧向值。我们报告了一名22岁的阿曼男性脑震荡后综合征和癫痫患者的PIWD病例,该患者被送往马斯喀特的三级护理医院,阿曼,2021年用于评估阵发性事件。他的PIWD行为被家人误解,直到在癫痫监测单位被定性为癫痫的表现,并接受了医学治疗。据作者所知,这是该地区报告的第二例病例。
    Peri-ictal water drinking (PIWD) is a rare vegetative manifestation of temporal lobe epilepsy without a definite lateralisation value. We report a case of PIWD in a 22-year-old Omani male patient with post-concussion syndrome and epilepsy presented to a tertiary care hospital in Muscat, Oman, in 2021 for evaluation of paroxysmal events. His behaviour of PIWD was misinterpreted by his family until characterised in the epilepsy-monitoring unit as a manifestation of epilepsy that was treated medically. To the best of the authors\' knowledge, this is the second reported case in the region.
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  • 文章类型: Journal Article
    目的:本研究旨在表征慢性硬化性唾液腺炎的组织病理学免疫组织化学特征,强调IgG4相关疾病。
    方法:对17例慢性硬化性唾液腺炎进行组织病理学检查,(炎症,纤维化,腺体薄壁组织,和淋巴滤泡)和免疫组织化学(BCL2,CD3,CD20,CD34,CD163,p63,细胞周期蛋白D1,肥大细胞,SMA,S100A4,IgG,和IgG4)评分。研究IgG4相关疾病特征。还收集了人口统计学和临床数据。
    结果:男性占主导地位(10:7),平均病灶大小3.9cm。常见的组织病理学发现包括腺泡实质减少,淋巴滤泡形成,和导管增殖。CD3阳性T淋巴细胞以及CD34和SMA阳性基质成纤维细胞丰富。9例(53%)显示唾液酸,3例符合IgG4相关疾病的标准。
    结论:下颌下腺的CSS代表反应型而不是IgG4-RD,因为只有3例似乎与IgG4-RD有关。免疫组织化学谱显示大量的CD3阳性T淋巴细胞,与细胞周期蛋白D1等调节蛋白相反,这表明CD34和SMA阳性基质成纤维细胞的群体有助于CSS的纤维化特征。此外,我们的研究结果为CSS的研究及其与IgG4-RD的关系提供了全面的见解.
    OBJECTIVE: This study aimed to characterize the histopathological immunohistochemical features of chronic sclerosing sialadenitis, emphasizing the IgG4-related disease.
    METHODS: Seventeen cases of chronic sclerosing sialoadenitis were examined for histopathological aspects, (inflammation, fibrosis, glandular parenchyma, and lymphoid follicles) and immunohistochemistry (BCL2, CD3, CD20, CD34, CD163, p63, cyclin D1, mast cell, SMA, S100A4, IgG, and IgG4) which were scored. IgG4-related disease features were investigated. Demographic and clinical data were also collected.
    RESULTS: Males predominated (10:7), with an average lesion size of 3.9 cm. Common histopathological findings included reduced acinar parenchyma, lymphoid follicle formation, and ductular proliferation. CD3-positive T lymphocytes and CD34- and SMA-positive stromal fibroblasts were abundant. Nine cases (53%) showed sialoliths and three cases met the criteria for IgG4-related disease.
    CONCLUSIONS: CSS of the submandibular gland represents a reactive pattern rather than IgG4-RD as only 3 cases seemed to be related to IgG4-RD. The immunohistochemical profile revealed an abundant population of CD3-positive T lymphocytes, as opposed to regulatory proteins such as cyclin D1, demonstrating that populations of CD34- and SMA-positive stromal fibroblasts contribute to the fibrosis characteristic of CSS. In addition, our results provide a comprehensive insight into the study of CSS and its relationship with IgG4-RD.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    背景:海马硬化(HS)是成人癫痫手术队列中常见的手术基质,但在各种儿科队列中的报道不同。
    目的:我们旨在研究癫痫表型,放射学和病理学变异性,在接受手术的颞叶前部有或没有其他细微信号变化的情况下,患有耐药性癫痫和海马硬化(HS)的儿童的癫痫发作和神经认知结局。
    方法:这项回顾性研究纳入了患有耐药性局灶性癫痫和海马硬化的儿童,这些儿童在颞叶前颞叶接受了前颞叶切除并进行了杏仁核海马切除术,并伴有或不伴有微小的T2-Fluid衰减反转恢复(FLAR)/质子密度(PD)信号变化。他们的临床,脑电图,神经心理学,回顾并总结了放射学和病理学资料。
    结果:确定了36名合格患者。癫痫发作的平均年龄为3.7岁;25%的人在手术时每天发作。在22例(61.1%)中发现了孤立的HS,在14例(38.9%)中,同侧颞叶有其他细微的信号变化。与规范人口相比,该组在智力功能和大多数听觉和视觉记忆任务方面的平均表现明显低于标准样本。手术平均年龄为12.3岁;22例患者(61.1%)进行了左半球手术。在平均随访时间为2.3年后,28例(77.8%)患者出现了ILAE1级结局。32例(88.9%)在病理上发现海马硬化;2型(54.5%)是主要亚型,可以进一步分类。在11例(30.6%)中发现了其他病理异常;与孤立的海马硬化/神经胶质增生的儿童相比,这些癫痫发作的发生率相似(63.6%vs84%,p=0.21)。在手术后的个体水平上,在听觉和视觉记忆任务中观察到了显着的可靠变化。
    结论:在大多数孤立的放射学海马硬化患儿中可以看到良好的癫痫发作结局。与孤立的海马硬化/神经胶质增生的儿童相比,具有其他病理异常的患者的癫痫发作自由率相似。
    BACKGROUND: Hippocampal sclerosis (HS) is a common surgical substrate in adult epilepsy surgery cohorts but variably reported in various pediatric cohorts.
    OBJECTIVE: We aimed to study the epilepsy phenotype, radiological and pathological variability, seizure and neurocognitive outcomes in children with drug-resistant epilepsy and hippocampal sclerosis (HS) with or without additional subtle signal changes in anterior temporal lobe who underwent surgery.
    METHODS: This retrospective study enrolled children with drug-resistant focal epilepsy and hippocampal sclerosis with or without additional subtle T2-Fluid Attenuated Inversion Recovery (FLAR)/Proton Density (PD) signal changes in anterior temporal lobe who underwent anterior temporal lobectomy with amygdalohippocampectomy. Their clinical, EEG, neuropsychological, radiological and pathological data were reviewed and summarized.
    RESULTS: Thirty-six eligible patients were identified. The mean age at seizure onset was 3.7 years; 25% had daily seizures at time of surgery. Isolated HS was noted in 22 (61.1%) cases and additional subtle signal changes in ipsilateral temporal lobe in 14 (38.9%) cases. Compared to the normative population, the group mean performance in intellectual functioning and most auditory and visual memory tasks were significantly lower than the normative sample. The mean age at surgery was 12.3 years; 22 patients (61.1%) had left hemispheric surgeries. ILAE class 1 outcomes was seen in 28 (77.8%) patients after a mean follow up duration of 2.3 years. Hippocampal sclerosis was noted pathologically in 32 (88.9%) cases; type 2 (54.5%) was predominant subtype where further classification was possible. Additional pathological abnormalities were seen in 11 cases (30.6%); these had had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis (63.6% vs 84%, p=0.21). Significant reliable changes were observed across auditory and visual memory tasks at an individual level post surgery.
    CONCLUSIONS: Favourable seizure outcomes were seen in most children with isolated radiological hippocampal sclerosis. Patients with additional pathological abnormalities had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis.
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  • 文章类型: Journal Article
    纤维化是50多种不同疾病的共同特征,也是全球35%以上死亡的原因。哪个肝脏,肾,皮肤,心和,最近,肺部受到最多关注。组织变化,导致器官功能丧失,是疾病和结果的原因和结果。纤维化是由细胞外基质蛋白的过度沉积引起的,随着时间的推移会导致器官功能受损和器官衰竭,导致成纤维细胞活化增加的途径有很多。这篇叙述性综述调查了纤维化的共同点,成纤维细胞,和成纤维细胞的激活,为了应对肝脏中过量的能量消耗,肾,心,皮肤和肺纤维化。成纤维细胞是肺器官功能丧失的主要驱动因素,肝脏,皮肤,心脏和肾脏疾病。响应于过量能量消耗的成纤维细胞活化导致一系列胶原蛋白的过度生产,哪些类型的I,III和VI似乎是疾病进展的重要驱动因素。成纤维细胞活化可以在血清中定量,能够对患者进行分析和选择。成纤维细胞的激活导致胶原蛋白的过度生产,使器官功能恶化。患者血清中成纤维细胞活性的分析,量化为胶原蛋白的产生,可以识别器官死亡轨迹,更好地识别用于不同代谢干预的正确治疗方法。由于代谢激活的患者患肾脏的风险很高,肝脏和心脏衰竭,首先确定要治疗哪个器官,并监测器官状态以纠正治疗方案。与此直接对齐,确定正确的患者与正确的器官恶化轨迹,以纳入临床研究是至关重要的。
    Fibrosis is a common feature of more than 50 different diseases and the cause of more than 35% of deaths worldwide, of which liver, kidney, skin, heart and, recently, lungs are receiving the most attention. Tissue changes, resulting in loss of organ function, are both a cause and consequence of disease and outcome. Fibrosis is caused by an excess deposition of extracellular matrix proteins, which over time results in impaired organ function and organ failure, and the pathways leading to increased fibroblast activation are many. This narrative review investigated the common denominator of fibrosis, fibroblasts, and the activation of fibroblasts, in response to excess energy consumption in liver, kidney, heart, skin and lung fibrosis. Fibroblasts are the main drivers of organ function loss in lung, liver, skin, heart and kidney disease. Fibroblast activation in response to excess energy consumption results in the overproduction of a range of collagens, of which types I, III and VI seem to be the essential drivers of disease progression. Fibroblast activation may be quantified in serum, enabling profiling and selection of patients. Activation of fibroblasts results in the overproduction of collagens, which deteriorates organ function. Patient profiling of fibroblast activities in serum, quantified as collagen production, may identify an organ death trajectory, better enabling identification of the right treatment for use in different metabolic interventions. As metabolically activated patients have highly elevated risk of kidney, liver and heart failure, it is essential to identify which organ to treat first and monitor organ status to correct treatment regimes. In direct alignment with this, it is essential to identify the right patients with the right organ deterioration trajectory for enrolment in clinical studies.
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