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: 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: Journal Article
    目的:本研究旨在阐明放化疗前后弹性变化与肛门直肠功能的关系。
    方法:这是一项单中心前瞻性队列研究(肿瘤外科,东京大学)。我们建立了一种使用经肛门超声和实时组织弹性成像将肛门内括约肌硬度量化为弹性的技术。纳入2019-2022年期间27例放化疗后直肠癌患者。在放化疗前后进行经肛门超声实时组织弹性成像,以测量肛门内括约肌硬度为“弹性”(最硬(0)至最软(255);弹性降低表明硬化变化)。放化疗前后弹性的增加或减少与最大静息压之间的关系,最大挤压压力,Wexner评分是结果指标。
    结果:放化疗后,16/27(59.3%)患者的弹性下降。放化疗后弹性下降的患者包括肛门内括约肌硬化和非硬化组,分别。在肛门内括约肌硬化组中,放化疗后的最大静息压力显着升高(63.0mmHg与47.0mmHg),大多数人的韦克斯纳得分恶化(60.0%vs.18.2%)与非硬化组相比。弹性降低(肛门内括约肌硬化)与较高的最大静息压力相关(r=0.36);在弹性变化程度和最大挤压压力之间没有观察到相关性。
    结论:放化疗引起的肛门内括约肌硬化可能与肛门直肠功能障碍有关。
    OBJECTIVE: This study aimed to clarify the relationship between changes in elasticity and anorectal function before and after chemoradiotherapy.
    METHODS: This is a single-center prospective cohort study (Department of Surgical Oncology, The University of Tokyo). We established a technique to quantify internal anal sphincter hardness as elasticity using transanal ultrasonography with real-time tissue elastography. Twenty-seven patients with post-chemoradiotherapy rectal cancer during 2019-2022 were included. Real-time tissue elastography with transanal ultrasonography was performed before and after chemoradiotherapy to measure internal anal sphincter hardness as \"elasticity\" (hardest (0) to softest (255); decreased elasticity indicated sclerotic changes). The relationship between the increase or decrease in elasticity pre- and post-chemoradiotherapy and the maximum resting pressure, maximum squeeze pressure, and Wexner score were the outcome measures.
    RESULTS: A decrease in elasticity was observed in 16/27 (59.3%) patients after chemoradiotherapy. Patients with and without elasticity decrease after chemoradiotherapy comprised the internal anal sphincter sclerosis and non-sclerosis groups, respectively. The maximum resting pressure post-chemoradiotherapy was significantly high in the internal anal sphincter sclerosis group (63.0 mmHg vs. 47.0 mmHg), and a majority had a worsening Wexner score (60.0% vs. 18.2%) compared with that of the non-sclerosis group. Decreasing elasticity (internal anal sphincter sclerosis) correlated with a higher maximum resting pressure (r = 0.36); no correlation was observed between the degree of elasticity change and maximum squeeze pressure.
    CONCLUSIONS: Internal anal sphincter sclerosis due to chemoradiotherapy may correlate to anorectal dysfunction.
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  • 文章类型: Journal Article
    潜在致命的真菌性蝶窦炎(FSS)会导致视觉损伤。然而,很少有研究报道其视力损害和预后。收集了在北京同仁医院接受治疗的51111例眼部并发症的FSS患者,并确定了临床特征和视力结果。511例患者中有32例(6%)有视力障碍,13和19例患者有侵入性和非侵入性FSS,分别。18例患者(56.25%)患有糖尿病,2例患者(6.25%)长期全身使用抗生素(n=1)和皮质类固醇(n=1)。所有患者均有视力障碍,侵入性FSS比非侵入性FSS更严重。骨壁缺损和硬化19例(59.38%),11例患者(34.38%)在计算机断层扫描(CT)上有蝶窦炎的微钙化。经过5年的随访,3例(9.38%)死亡。非侵入性FSS患者的视力改善率高于其他患者。在多变量分析中,CT显示蝶窦窦壁硬化与较好的视觉预后相关。FSS可导致视力丧失并伴有持续性头痛,尤其是糖尿病患者。CT显示蝶窦壁硬化,表明有视力障碍的FSS具有更好的视力预后。
    Potentially fatal fungal sphenoid sinusitis (FSS) causes visual damage. However, few studies have reported on its visual impairment and prognosis. Five hundred and eleven FSS patients with ocular complications treated at Beijing Tongren Hospital were recruited and clinical features and visual outcomes were determined. Thirty-two of the 511 patients (6%) had visual impairment, with 13 and 19 patients having invasive and noninvasive FSS, respectively. Eighteen patients (56.25%) had diabetes and 2 patient (6.25%) had long-term systemic use of antibiotics (n = 1) and corticosteroids (n = 1). All patients had visual impairment, which was more severe in invasive FSS than in noninvasive FSS. Bony wall defects and sclerosis were observed in 19 patients (59.38%), and 11 patients (34.38%) had microcalcification in their sphenoid sinusitis on computed tomography (CT). After a 5-year follow-up, three patients (9.38%) died. Patients with noninvasive FSS had a higher improvement rate in visual acuity than their counterparts. In the multivariate analysis, sphenoid sinus wall sclerosis on CT was associated with better visual prognosis. FSS can cause vision loss with persistent headaches, particularly in those with diabetes. CT showed the sphenoid sinus wall sclerosis, indicating a better visual prognosis in FSS with visual impairment.
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  • 文章类型: Journal Article
    背景:我们旨在分析可能影响术后癫痫发作的潜在预后因素,通过跨侧裂入路选择性杏仁核海马切除术(SAHE)后,由于海马硬化(HS)引起的内侧颞叶癫痫(MTLE)患者队列的神经心理学和精神病学结局。
    方法:使用单变量和多变量logistic回归模型评估了1994年至2019年间接受SAHE的171例耐药MTLE合并HS患者(88例女性)的临床变量,调查哪一个解释参数可以最好地预测结果。
    结果:在手术后12.3±6.3年的最后一次随访中,114例患者(67.9%)无癫痫发作。左半球MTLE在术后第一年与更差的术后癫痫发作结果相关(OR=0.54,p=0.01),女性性别-在第2年(OR=0.52,p=0.01)和第5年(OR=0.53,p=0.025)复发,术前抗癫痫药物试验次数较多-在第2年(OR=0.77,p=0.0064),而没有创伤性脑损伤病史的患者在第一年的术后癫痫发作结局更好(OR=2.08,p=0.0091).所有预测因子在长期过程中都失去了预测价值。HS类型对预后无影响。与左侧相比,右侧手术的患者在言语记忆方面表现更好(VLMT1-5p<0.001,VLMT7p=0.001)。与无癫痫患者相比,无癫痫患者的抑郁症发生率较低(BDI-IIZ=-2.341,p=0.019)。
    结论:SAHE改善了术后癫痫发作的机会,HS引起的MTLE患者的精神病学和神经心理学结局。短期结果的预测因素不能预测长期结果。
    BACKGROUND: We aimed to analyze potentially prognostic factors which could have influence on postoperative seizure, neuropsychological and psychiatric outcome in a cohort of patients with mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS) after selective amygdalohippocampectomy (SAHE) via transsylvian approach.
    METHODS: Clinical variables of 171 patients with drug-resistant MTLE with HS (88 females) who underwent SAHE between 1994 and 2019 were evaluated using univariable and multivariable logistic regression models, to investigate which of the explanatory parameters can best predict the outcome.
    RESULTS: At the last available follow-up visit 12.3 ± 6.3 years after surgery 114 patients (67.9%) were seizure-free. Left hemispheric MTLE was associated with worse postoperative seizure outcome at first year after surgery (OR = 0.54, p = 0.01), female sex-with seizure recurrence at years 2 (OR = 0.52, p = 0.01) and 5 (OR = 0.53, p = 0.025) and higher number of preoperative antiseizure medication trials-with seizure recurrence at year 2 (OR = 0.77, p = 0.0064), whereas patients without history of traumatic brain injury had better postoperative seizure outcome at first year (OR = 2.08, p = 0.0091). All predictors lost their predictive value in long-term course. HS types had no prognostic influence on outcome. Patients operated on right side performed better in verbal memory compared to left (VLMT 1-5 p < 0.001, VLMT 7 p = 0.001). Depression occurred less frequently in seizure-free patients compared to non-seizure-free patients (BDI-II Z = - 2.341, p = 0.019).
    CONCLUSIONS: SAHE gives an improved chance of achieving good postoperative seizure, psychiatric and neuropsychological outcome in patients with in MTLE due to HS. Predictors of short-term outcome don\'t predict long-term outcome.
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  • 文章类型: Journal Article
    目的:本研究旨在确定海马T2高强度是否可预测高热性癫痫持续状态的后遗症,包括海马萎缩,硬化症,和内侧颞叶癫痫。
    方法:在高热状态后平均4.4(SD=5.5,中位数=2.0)天内获得了>200名婴儿的急性磁共振成像(MRI),并在大约1、5和10年进行了MRI随访。海马大小,形态学,和T2信号强度由不了解临床细节的神经放射科医师进行视觉评分。海马容积法提供了定量测量。在发生两次或更多次无缘无故的癫痫发作时,受试者被重新评估癫痫.使用总脑体积将海马体积标准化。
    结果:22例急性海马T2高强度患者中有14例返回随访MRI,10人发展为明确的海马硬化,持续了10年的随访。最初看起来正常的海马在视觉检查中保持正常。然而,在海马体正常的受试者中,体积表明男性,但不是女性,海马比对照组小,但是在发热状态下没有看到海马不对称的增加。44名受试者发展为癫痫;六个发展为内侧颞叶癫痫,六个,两个有明确的,两个人模棱两可,两个没有海马硬化。只有一名受试者在没有初始高强度的情况下发展了内侧颞叶癫痫,那个受试者有海马旋转不良。所有类型癫痫的十年累积发病率,包括内侧颞叶癫痫,在初始T2高强度的受试者中最高,在信号正常且无其他脑异常的受试者中最低。
    结论:高热性癫痫持续状态后海马T2高强度预测海马硬化和颞叶内侧癫痫的可能性。在急性发作后MRI中,海马外观正常,随后外观保持正常。对称增长,降低癫痫的风险。体积测量检测到发热状态男性海马体积轻度减少。
    OBJECTIVE: This study was undertaken to determine whether hippocampal T2 hyperintensity predicts sequelae of febrile status epilepticus, including hippocampal atrophy, sclerosis, and mesial temporal lobe epilepsy.
    METHODS: Acute magnetic resonance imaging (MRI) was obtained within a mean of 4.4 (SD = 5.5, median = 2.0) days after febrile status on >200 infants with follow-up MRI at approximately 1, 5, and 10 years. Hippocampal size, morphology, and T2 signal intensity were scored visually by neuroradiologists blinded to clinical details. Hippocampal volumetry provided quantitative measurement. Upon the occurrence of two or more unprovoked seizures, subjects were reassessed for epilepsy. Hippocampal volumes were normalized using total brain volumes.
    RESULTS: Fourteen of 22 subjects with acute hippocampal T2 hyperintensity returned for follow-up MRI, and 10 developed definite hippocampal sclerosis, which persisted through the 10-year follow-up. Hippocampi appearing normal initially remained normal on visual inspection. However, in subjects with normal-appearing hippocampi, volumetrics indicated that male, but not female, hippocampi were smaller than controls, but increasing hippocampal asymmetry was not seen following febrile status. Forty-four subjects developed epilepsy; six developed mesial temporal lobe epilepsy and, of the six, two had definite, two had equivocal, and two had no hippocampal sclerosis. Only one subject developed mesial temporal epilepsy without initial hyperintensity, and that subject had hippocampal malrotation. Ten-year cumulative incidence of all types of epilepsy, including mesial temporal epilepsy, was highest in subjects with initial T2 hyperintensity and lowest in those with normal signal and no other brain abnormalities.
    CONCLUSIONS: Hippocampal T2 hyperintensity following febrile status epilepticus predicted hippocampal sclerosis and significant likelihood of mesial temporal lobe epilepsy. Normal hippocampal appearance in the acute postictal MRI was followed by maintained normal appearance, symmetric growth, and lower risk of epilepsy. Volumetric measurement detected mildly decreased hippocampal volume in males with febrile status.
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  • 文章类型: Journal Article
    终板硬化是脊柱退化或老化的一个值得注意的方面,但机制尚不清楚。这里,我们报告说,衰老的巨噬细胞在腰椎不稳定(LSI)或衰老的雄性小鼠模型的硬化终板中积累。具体来说,巨噬细胞中cdkn2a(p16)的敲除消除了LSI或老化诱导的终板中的血管生成和硬化。此外,体内和体外研究均表明,IL-10是衰老相关分泌表型(SASP)的主要升高细胞因子。机械上,IL-10增加内皮细胞中的pSTAT3,导致pSTAT3直接与Vegfa的启动子结合,Mmp2和Pdgfb鼓励他们的生产,导致血管生成。这项研究提供了有关了解免疫衰老与终板硬化之间联系的信息,这可能对治疗方法有用。
    Endplate sclerosis is a notable aspect of spine degeneration or aging, but the mechanisms remain unclear. Here, we report that senescent macrophages accumulate in the sclerotic endplates of lumbar spine instability (LSI) or aging male mouse model. Specifically, knockout of cdkn2a (p16) in macrophages abrogates LSI or aging-induced angiogenesis and sclerosis in the endplates. Furthermore, both in vivo and in vitro studies indicate that IL-10 is the primary elevated cytokine of senescence-related secretory phenotype (SASP). Mechanistically, IL-10 increases pSTAT3 in endothelial cells, leading to pSTAT3 directly binding to the promoters of Vegfa, Mmp2, and Pdgfb to encourage their production, resulting in angiogenesis. This study provides information on understanding the link between immune senescence and endplate sclerosis, which might be useful for therapeutic approaches.
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