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: Case Reports
    该病例以一名76岁男性为中心,患有劳力性呼吸困难和咯血,具有以复发性肺栓塞和慢性阻塞性肺疾病(COPD)为特征的病史。值得注意的是,他居住在组织胞浆菌病流行区。计算机断层扫描(CT)肺栓塞扫描显示明显的发现,包括扩大的右下肺动脉,血管充血,肺不张,和对右下肺静脉施加压力的肿块。活检结果确定肿块为纤维化纵隔炎,可能归因于组织胞浆菌病。经胸超声心动图显示右心室扩张,功能受损,右心室收缩压为63mmHg.在右心导管插入术中,患者在左右两侧显示不同的肺动脉楔压(PAWP).这种差异与从左心房到导管的迟钝的背波有关,由肺静脉压迫引起。虽然这是一种罕见的现象,记录的PAWP不对称性在指导准确的患者管理方面起着至关重要的作用.左侧缺乏随后的PAWP评估可能会改变治疗计划,可能会延迟适当的患者护理。该病例强调在临床症状允许的情况下进行右心导管彻底探查的必要性,强调标准化做法在此类程序中的重要性。
    This case centers on a 76-year-old male experiencing exertional dyspnea and hemoptysis, with a medical history marked by recurrent pulmonary embolism and chronic obstructive pulmonary disease (COPD). Notably, he resides in a histoplasmosis-endemic area. A computed tomography (CT) pulmonary embolism scan revealed notable findings, including an enlarged right lower pulmonary artery, vascular congestion, atelectasis, and a mass exerting pressure on the right lower pulmonary vein. Biopsy results identified the mass as fibrosing mediastinitis, likely attributed to histoplasmosis. A transthoracic echocardiogram indicated right ventricular dilatation, impaired function, and a right ventricular systolic pressure of 63 mm Hg. During right heart catheterization, the patient displayed disparate pulmonary artery wedge pressures (PAWPs) between the right and left sides. This discrepancy was linked to a blunted back wave from the left atrium to the catheter, induced by pulmonary vein compression. Although an infrequent phenomenon, the recorded asymmetry in PAWPs played a crucial role in guiding accurate patient management. The absence of subsequent evaluation of PAWP on the left side could have altered the treatment plan, potentially delaying appropriate patient care. This case emphasizes the necessity of thorough exploration with right heart catheterization when clinical symptoms warrant, highlighting the importance of standardized practices in such procedures.
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  • 文章类型: Case Reports
    腺样囊性癌(SACC)的硬化性变体是腺样囊性癌(ACC)的罕见变体,文献中只有2例。在这篇文章中,我们报告了2例SACCs病例,并试图解释硬化的原因及其与疾病结局的关系.
    第一位患者是一名43岁的德拉威德印度女性,主诉右后颊区域疼痛和肿胀,持续时间为四个月。第二名患者是一名8岁男孩,左颊区域有明确的肿胀。
    这两种情况均被诊断为ACC的硬化变体。
    两名患者的肿瘤均通过手术切除。
    两名患者都没有残留疾病/复发的迹象。
    我们认为致密的硬化基质可能在肿瘤细胞生长和进展中具有阻止作用。
    UNASSIGNED: Sclerosing variant of adenoid cystic carcinoma (SACC) is a rare variant of adenoid cystic carcinoma (ACC) with only two cases in the literature. In this article, we report two cases of SACCs and attempt to explain the reason for sclerosis and its association with the disease outcome.
    UNASSIGNED: The first patient is a 43-year-old Dravidian Indian female with a chief complaint of pain and swelling in the right posterior cheek region of four months\' duration. The second patient is an 8-year-old boy with a well-defined swelling in the left cheek region.
    UNASSIGNED: Both these cases were diagnosed as sclerosing variants of ACC.
    UNASSIGNED: The tumour was surgically excised for both patients.
    UNASSIGNED: Both the patients had no signs of residual disease/recurrence.
    UNASSIGNED: We opine that the dense sclerotic stroma may have a preventing role in tumour cell growth and progression.
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  • 文章类型: Case Reports
    硬化性苔藓(LS)是一种罕见的,慢性,炎性皮肤粘膜疾病主要见于病因不明的女性。它表现为通常位于肛门生殖器区域的白色硬化斑块。婚外LS不那么普遍,影响口腔粘膜的LS非常罕见,文献中仅报道了39例活检确诊病例。由于它的几个模仿条件,组织学检查通常需要明确的诊断,特别是在口服LS患者中。目前尚无口服LS的循证治疗建议;然而,大多数病例在使用局部或病灶内皮质类固醇治疗后倾向于改善。我们报告了一例从耳鼻喉科转诊的58岁女性,用于评估存在1年的下唇粘膜上无症状的白色硬化斑块。穿刺活检后,患者被诊断为唇粘膜LS。局部和病灶内皮质类固醇治疗2个月后,病情改善。本病例报告提高了对口腔LS的认识,并有助于了解这种罕见疾病。
    Lichen sclerosus (LS) is an uncommon, chronic, inflammatory mucocutaneous disorder found predominantly in females with unknown etiology. It presents as a white sclerotic plaque commonly located on the anogenital area. Extragenital LS is less prevalent, and LS affecting the oral mucosa is extremely rare, with only 39 biopsy-confirmed cases reported in the literature. Due to its several mimicking conditions, histological examination is usually required for a definitive diagnosis, particularly in patients with oral LS. Current evidence-based treatment recommendations for oral LS are unavailable; however, most cases tend to improve after treatment with topical or intralesional corticosteroids. We report a case of a 58-year-old female referred from the otolaryngology department for evaluating an asymptomatic whitish sclerotic plaque on the lower lip mucosa that had existed for 1 year. Following a punch biopsy, the patient was diagnosed with LS of labial mucosa. The condition improved after 2 months of treatment with topical and intralesional corticosteroids. The present case report raises awareness in recognizing oral LS and contributes to knowledge of this rare disorder.
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  • 文章类型: Case Reports
    硬化性多囊性腺瘤(SPA)是一种罕见的唾液腺硬化性肿瘤,好发于腮腺,镜下主要由增生的微囊、腺泡及导管样结构组成,腺上皮细胞细胞质呈泡沫状及空泡状,具有轻度非典型性和顶浆分泌性。由于SPA较罕见,易误诊为其他肿瘤。本文报道1例左侧腮腺SPA的病理变化及其鉴别诊断,旨在提高病理医师对SPA的认知及诊断水平。.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    硬化性多囊腺瘤(SPA)是一种罕见的肿瘤性唾液腺病变,迄今为止,全世界仅报道了约100例。该病变与几种恶性和其他良性肿瘤相混淆,例如大汗腺导管内癌(IC),涎管癌(SDC),慢性硬化性唾液腺炎,多囊性遗传性疾病(PDD),多形性腺瘤(PA),腺泡细胞癌(ACC),和粘液表皮样癌(MEC)。我们介绍了一名23岁男性患者的SPA病例,该患者表现为腮腺肿块缓慢增长。进行细针抽吸(FNA),然后完全切除肿瘤,图像与SPA一致。我们讨论了案例的发现,并简要回顾了有关SPA的文献。
    Sclerosing polycystic adenoma (SPA) is a rare neoplastic salivary gland lesion with only about 100 cases reported worldwide so far. The lesion is confused with several malignant and other benign tumors such as apocrine intraductal carcinoma (IC), salivary duct carcinoma (SDC), chronic sclerosing sialadenitis, polycystic dysgenetic disease (PDD), pleomorphic adenoma (PA), acinic cell carcinoma (ACC), and mucoepidermoid carcinoma (MEC). We present a case of SPA for a 23-year-old male patient presenting with a slowly growing parotid mass. Fine needle aspiration (FNA) followed by total excision of the tumor was performed and the picture was consistent with SPA. We discuss the findings of the case and briefly review the literature on SPA.
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  • 文章类型: Case Reports
    三角骨增生,也称为凝聚性骨炎(OCI),是一种罕见的情况,主要发生在女性身上,病因不明.这种疾病是一种与髂骨纤维化相关的硬化性疾病,一种非炎症性和自限性疾病。这种情况主要影响骶髂关节的髂关节部分,有时影响毗邻的骨骼,比如骶骨,腰椎,和髂骨.这是许多人的偶然发现,但由于骶髂关节受累而很少与下背痛相关。它主要是用物理治疗和药物治疗。OCI应被认为是没有强直性脊柱炎或炎性关节炎的女性慢性背痛的原因。在这里,一名47岁的女性患有骨炎,在中线和两个骶髂区域出现间歇性下背痛的主要主诉,据报道,没有任何强直性脊柱病的证据,骨盆的X射线和磁共振成像(MRI)显示OCI的特征。该病例通过药物和运动进行管理,患者正在接受定期随访。
    Hyperostosis triangularis ilii, also called osteitis condensans ilii (OCI), is a rare condition, mostly occurring in females, and the etiology is unknown. This disease is a sclerotic disorder associated with iliac fibrosis, a noninflammatory and self-limiting disorder. This condition primarily affects the iliac part of the sacroiliac joints and sometimes the adjoining bones, such as the sacrum, lumbar vertebrae, and iliac bones. It is an incidental finding in many individuals but rarely associated with lower back pain due to sacroiliac joint involvement. It is mainly treated with physical therapy and medications. OCI should be considered a cause of chronic back pain in females not having ankylosing spondylitis or inflammatory arthritis. Herein, a case of osteitis condensans ilii in a 47-year-old female, presenting with the chief complaints of intermittent lower back pain in the midline and on both sacroiliac regions, without any evidence of ankylosing spondylosis and the X-ray of the pelvis and magnetic resonance imaging (MRI) showing features of OCI, is being reported. The case was managed with medications and exercise, and the patient is under regular follow-up.
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  • 文章类型: Case Reports
    背景:纤维纵隔炎(FM)是一种罕见的纵隔病变,其特征是纵隔内纤维组织增生。以前的报道表明,这种病变可能是由组织胞浆菌病和结核病引起的。在极少数情况下,FM也可由自身免疫性疾病如抗中性粒细胞胞浆抗体相关血管炎和大血管动脉炎引起。
    方法:在我们的例子中,我们报道了一名重症肌无力(MG)患者在机器人胸腺切除术后发现的意外纤维性纵隔炎.术前影像学显示纵隔无明显病变,患者否认组织胞浆菌病和结核病史。手术后,在胸腺中可以发现纤维组织和异位生发中心(GC)的增殖。
    结论:这一罕见病例可能丰富了我们对FM与自身免疫性疾病之间关系的认识。
    BACKGROUND: Fibrous mediastinitis (FM) is a rare mediastinal lesion characterized by proliferation of fibrous tissue within the mediastinum. Previous reports have shown that this lesion can be caused by histoplasmosis and tuberculosis. In extremely rare cases, FM can also be caused by autoimmune diseases such as antineutrophil cytoplasmic antibody-associated vasculitis and large-vessel arteritis.
    METHODS: In our case, we report unexpected fibrous mediastinitis found after robotic thymectomy in a patient with myasthenia gravis (MG). The preoperative imaging indicated no obvious lesion in the mediastinum and the patient denied histories of both histoplasmosis and tuberculosis. After the operation, both proliferation of fibrous tissue and ectopic germinal centres (GCs) could be found in the thymus.
    CONCLUSIONS: This rare case might enrich our knowledge of the relationship between FM and autoimmune diseases.
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