idiopathic

特发性
  • 文章类型: Case Reports
    腹茧综合征(ACS),作为机械性肠梗阻的罕见原因,可分为原发性/特发性vs.次要类型。原发性ACS通常无症状,仅在剖腹探查术中诊断。手术的主要治疗方法可能具有挑战性。由于肠壁和腹膜紧密粘附,肠穿孔可能发生在粘连松解术期间。因此,有一个有经验的外科医生来进行手术是很重要的。
    作者介绍了一个50岁男性的主要ACS病例。患者入院时表现出难以忍受的上腹痛。计算机断层扫描(CT)扫描显示严重的肠梗阻。需要剖腹探查术,导致ACS的诊断,排除次要因素后被认为是特发性的。成功地进行了粘连分解。注意,在手术期间测量的整个肠仅为2.1m。无术后并发症。患者顺利康复。
    原发性ACS的病因未知。发病率相对较低,男女之间被认为是平等的。作为肠梗阻的罕见原因,应加强对诊断的怀疑。包括粘连松解术和肠切除术的手术仍然是主要的治疗方法。如果粘连溶解失败,肠切除将是不可避免的。将测试外科医生的知识和经验。
    应进一步探讨原发性ACS的病因。肠梗阻的鉴别诊断应涵盖ACS,以便外科医生在手术前做好准备。
    UNASSIGNED: Abdominal cocoon syndrome (ACS), as a rare cause of mechanical intestinal obstruction, can be divided into primary/idiopathic vs. secondary type. The primary ACS is often asymptomatic and only diagnosed in exploratory laparotomy. The major treatment of surgery can be challenging. Since the gut wall and peritoneum are densely adhered, gut perforation might occur during adhesiolysis. Thus, it is important to have an experienced surgeon to perform the surgery.
    UNASSIGNED: The authors present a primary ACS case of a 50-year-old man. The patient demonstrated an unbearable upper abdominal pain upon admission. A computed tomography (CT) scan showed a severe bowel obstruction. An exploratory laparotomy was indicated, leading to the diagnosis of ACS, which was considered idiopathic after ruling out secondary factors. An adhesiolysis was performed successfully. Note that the entire intestine measured was only 2.1 m during the surgery. There was no post-surgical complication. The patient was recovered uneventfully.
    UNASSIGNED: The aetiology of primary ACS is unknown. The incidence is comparatively low and considered equal between men and women. As a rare cause of gut obstruction, the suspicion of the diagnosis should be strengthened. Surgery including adhesiolysis and bowel resection remains the major treatment. If adhesiolysis fails, bowel resection will be inevitable. The knowledge and experience of surgeon will be tested.
    UNASSIGNED: The aetiology of primary ACS should be further explored. And the differential diagnosis of bowel obstruction should cover ACS in order for the surgeon to be prepared before surgery.
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  • 文章类型: Case Reports
    特发性肺动脉瘤是临床上罕见的疾病,其特征是病因不明和偶发。尽管它很罕见,特发性肺动脉瘤给患者带来潜在风险。目前,缺乏关于其管理的既定临床指南和共识,导致治疗策略的持续争议。特别是,解决主肺动脉的最佳方法,它的分支,肺动脉瓣仍不确定。一名57岁的女性患者出现胸痛和胸闷,排除其他潜在原因后导致诊断为特发性肺动脉瘤。随后,她接受了手术治疗。然而,在手术过程中,肺动脉壁非常脆弱,促使我们采用一种手术方法,包括使用人工移植物包裹自体血管。通过总结近年来报道的几乎所有手术治疗策略,包括肺动脉血管和肺动脉瓣的管理,我们制定了治疗流程图。此流程图可作为管理未来出现类似挑战的案例的宝贵指南,为临床医生提供有价值的见解和循证建议。
    Idiopathic pulmonary aneurysm is a clinically rare condition characterized by an unknown etiology and episodic occurrence. Despite its rarity, idiopathic pulmonary artery aneurysm poses potential risks to patients. Currently, there is a lack of established clinical guidelines and consensus regarding its management, leading to ongoing controversies in treatment strategies. Particularly, the optimal approach for addressing the main pulmonary artery, its branches, and the pulmonary artery valve remains uncertain. A 57-year-old female patient presented with chest pain and tightness, leading to the diagnosis of idiopathic pulmonary artery aneurysm after excluding other potential causes. Subsequently, she underwent surgical treatment. However, during the surgery, the pulmonary artery wall was found to be extremely weak, prompting us to employ a surgical approach involving the utilization of autologous vessel wrapping with artificial grafts. By summarizing almost all surgical treatment strategies reported in recent years, including the management of pulmonary artery vessels and the pulmonary valve, we have developed a treatment flow chart. This flowchart serves as a valuable guide for the management of future cases presenting similar challenges, offering clinicians valuable insights and evidence-based recommendations.
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  • 文章类型: Journal Article
    背景:血小板减少症是原发性干燥综合征(pSS)的血液学表现之一。本研究的目的是评估抗血小板糖蛋白自身抗体在原发性干燥综合征(pSS)血小板减少症发病机制中的可能作用。
    方法:血浆抗糖蛋白Ib水平,在36个无血小板减少症的pSS患者和35个有血小板减少症的pSS患者中,IIIa和IIb/IIIa自身抗体,对36例特发性血小板减少性紫癜(ITP)患者和39例正常对照者进行酶联免疫吸附试验(ELISA)检测。
    结果:抗GPIb的水平,GPIIIa,有血小板减少症的pSS中的GPIIb/IIIa自身抗体(A490)显着高于无血小板减少症的pSS(0.813±0.161vs0.688±0.133;0.917±0.094vs0.802±0.070;0.911±0.125vs0.782±0.109)。反GPIb的发生率,GPIIIa,有血小板减少症的pSS中的GPIIb/IIIa自身抗体显着高于无血小板减少症的pSS(25.7%vs0%;65.7%vs11.1%;31.4%vs0%)。在pSS患者中,抗GPIb的血小板计数较低,GPIIIa,GPIIb/IIIa自身抗体阳性患者((25.67±5.5)×10^9/Lvs(116.8±84.52)×10^9/L;29.04±11.33×10^9/Lvs(152.0±75.47)×10^9/L;(31.55±14.0)×10^9/Lvs(118.8±85.24)×10^9/L)。
    结论:血浆抗血小板糖蛋白自身抗体水平升高可能在pSS血小板减少症的发病机制中起作用。要点•抗GPIb水平,GPIIIa,血小板减少症pSS中的GPIIb/IIIa自身抗体(A490)增加。•anti-GPIb事件,GPIIIa,血小板减少症的pSS中的GPIIb/IIIa自身抗体增加。•pSS患者,抗GPIb的血小板计数较低,GPIIIa,GPIIb/IIIa自身抗体阳性患者。
    BACKGROUND: Thrombocytopenia is one of the primary Sjögren\'s syndrome (pSS) hematological manifestations. The objective of this study was to evaluate the possible roles of antiplatelet glycoprotein autoantibodies in the pathogenesis of thrombocytopenia in primary Sjögren\'s syndrome (pSS).
    METHODS: The level of plasma anti-glycoprotein Ib, IIIa and IIb/IIIa autoantibodies in 36 pSS patients without thrombocytopenia and 35 pSS patients with thrombocytopenia, 36 Idiopathic thrombocytopenic purpura (ITP) patients and 39 normal control were measured with enzyme-linked immunosorbent assay (ELISA).
    RESULTS: The level of anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies (A490) in the pSS with thrombocytopenia was significantly higher than that of pSS without thrombocytopenia (0.813 ± 0.161 vs 0.688 ± 0.133; 0.917 ± 0.094 vs 0.802 ± 0.070; 0.911 ± 0.125 vs 0.782 ± 0.109). Incidences of the anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies in the pSS with thrombocytopenia was significantly higher than that of pSS without thrombocytopenia (25.7% vs 0%; 65.7% vs 11.1%; 31.4% vs 0%). In patients with pSS, there was a lower platelet count in anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies positive patients ((25.67 ± 5.5) × 10^9/L vs (116.8 ± 84.52) × 10^9/L; 29.04 ± 11.33 × 10^9/L vs (152.0 ± 75.47) × 10^9/L; (31.55 ± 14.0) × 10^9/L vs (118.8 ± 85.24) × 10^9/L).
    CONCLUSIONS: Elevated plasma levels of anti-platelet glycoprotein autoantibodies may play a role in the pathogenesis of thrombocytopenia in pSS. Key Points • The level of anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies (A490) in the pSS with thrombocytopenia was increased. • Incidences of the anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies in the pSS with thrombocytopenia was increased. • In patients with pSS, there was a lower platelet count in anti-GPIb, GPIIIa, GPIIb/IIIa autoantibodies positive patients.
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  • 文章类型: Journal Article
    协助医生做出更好的治疗决定并改善患者预后,确定哪种治疗方式适用于各种形式的特发性肥厚性脑硬脑膜炎(IHCP)非常重要.所有病例均来自医院病历系统,通过电话随访收集了一些后续信息。共有26名患者,14男12女,年龄从20岁到73岁,平均年龄为47.42岁,包括在研究中。规则型比不规则型和结节型更不容易复发,局灶性类型比弥漫性类型更不可能复发,和皮质类固醇难治性类型比皮质类固醇敏感型更容易复发。病变的范围和形状以及对皮质类固醇的敏感性是可能影响复发的潜在因素。Futhermore,本文还提出将成纤维细胞作为一个新的治疗靶点,可能提高患者预后生存质量。
    To assist doctors in making better treatment decisions and improve patient prognosis, it is important to determine which therapy modalities are suitable for various forms of idiopathic hypertrophic cranial pachymeningitis (IHCP).
    All cases were received from the hospital medical record system, and some follow-up information was gathered through telephone follow-up.
    A total of 26 patients, 14 men and 12 women, with ages ranging from 20 to 73 years and a mean of 47.42 years, were included in the research. Regular types were less likely to recur than irregular and nodular types, focal types were less likely to recur than diffuse types, and corticosteroid-refractory types were more likely to recur than corticosteroid-sensitive types.
    The extent and shape of the lesion and susceptibility to corticosteroids are potential factors that could influence recurrence. Futhermore, this paper also proposes the fibroblasts as a new therapeutic target which may improve the quality of prognostic survival of patients.
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  • 文章类型: Journal Article
    特发性宫颈肌张力障碍(ICD)是肌张力障碍的最大亚组。心理压力作为一个触发因素早已被讨论,但缺乏详细的描述。我们报告了一组13例ICD患者和之前的过度心理压力(ICD发病年龄39.0±13.9岁,7位女性,6个男性)。观察期为7.8±5.0年。过度的心理压力包括伴侣冲突(离婚和分居,家庭暴力),特殊的家庭负担,法律纠纷和移民。它在ICD发作前8.3±3.9个月开始。在我们85%的患者(典型病例)中,ICD在5.8±4.4周内出现,然后持续了18.5±8.3个月,在发病后2.7±0.8年开始缓解至最大严重程度的54.5±35.3%之前。特发性肌张力障碍被认为是基于由表观遗传因素触发的遗传易感性。我们的研究表明,过度的心理压力可能是其中之一。病理生理因素只是模糊地确定,但可能包括内质网应激反应,小脑5HT-2A受体与热休克蛋白的代谢。虽然ICD的临床表现之前是过度的心理压力是典型的,其病程不典型,起效迅速,缓解迅速。
    Idiopathic cervical dystonia (ICD) is the largest subgroup of dystonia. Psychological stress as a triggering factor has long been discussed, but detailed descriptions are lacking. We report on a group of 13 patients with ICD and preceding excessive psychological stress (age at ICD onset 39.0 ± 13.9 years, 7 females, 6 males). The observation period was 7.8 ± 5.0 years. Excessive psychological stress included partner conflicts (divorce and separation, domestic violence), special familial burdens, legal disputes and migration. It started 8.3 ± 3.9 months before ICD onset. In 85% of our patients (typical cases), ICD developed within 5.8 ± 4.4 weeks, then lasted 18.5 ± 8.3 months, before it started to remit 2.7 ± 0.8 years after its onset to 54.5 ± 35.3% of its maximal severity. Idiopathic dystonia is thought to be based upon a genetic predisposition triggered by epigenetic factors. Our study suggests that excessive psychological stress could be one of them. Pathophysiologic elements are only vaguely identified, but could include the endoplasmic reticulum stress response, cerebellar 5HT-2A receptors and the metabolism of heat shock proteins. Whilst the clinical presentation of ICD preceded by excessive psychological stress is typical, its course is atypical with rapid onset and fast and substantial remission.
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  • 文章类型: English Abstract
    Occupational and environmental exposure can directly cause specific lung diseases, and can also induce autoimmune diseases that can lead to various types of interstitial lung diseases. In recent years, it was discovered that certain occupational and environmental exposure was related to the increased risk of Idiopathic pulmonary fibrosis (IPF) disease and progression, including metal and mineral dust, wood dust, organic dust, asbestos dust, silica dust, cigarette smoke and air pollution. IPF is a chronic progressive fibrotic lung disease of unknown etiology, with a characteristic imaging and histologic pattern called usual interstitial pneumonia. This article is a review based on the correlation and mechanism of occupational and environmental exposure in the pathogenesis and disease progression of IPF to improve the understanding of the disease and promote the formulation of treatment plans.
    职业和环境暴露会直接引起特定的肺部疾病,还可诱发自身免疫疾病从而继发各类间质性肺疾病。近年来,研究发现一些特定的职业和环境暴露与特发性肺纤维化(IPF)的发病和疾病进展的风险增加有关,主要暴露因素包括金属粉尘、木粉尘、其他有机粉尘、石棉粉尘、二氧化硅粉尘、烟草烟雾及空气污染物等。IPF是一种病因不明的慢性进行性纤维化性肺部疾病,其胸部影像和肺组织病理呈普通型间质性肺炎。本文对IPF发病和疾病进展过程中职业和环境暴露的相关性及作用机制进行综述,以期提高对该疾病的认识,推动治疗方案的制定。.
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  • 文章类型: Systematic Review
    多发性特发性宫颈根吸收(MICRR)的最新文献,一种罕见且激进的外部根部吸收形式,仅限于病例报告和系列。因此,我们对这种情况进行了系统回顾.全面搜索PubMed,Embase,WebofScience,科克伦图书馆,CNKI,WANFANG是使用与MICRR相关的关键术语进行的,辅以灰色文献检索。使用Cochrane和JoannaBriggs研究所的工具评估偏倚风险。共纳入36项研究,共47例。MICRR在年轻女性中更常见,可能与荷尔蒙变化和denosumab使用有关。最初,前磨牙通常会受到影响,但最终可能会涉及所有恒牙。建议使用锥形束计算机断层扫描来诊断和评估吸收性病变。管理的重点是完全去除和恢复吸收组织,以保持牙齿的结构完整性。然而,MICRR通常预后不良。由于其侵入性和攻击性行为,MICRR需要更多的关注。
    The current literature on multiple idiopathic cervical root resorption (MICRR), a rare and aggressive form of external root resorption, is limited to case reports and series. Therefore, we performed a systematic review of this condition. A comprehensive search of PubMed, Embase, Web of science, Cochrane Library, CNKI, and WANFANG was conducted using key terms relevant to MICRR, supplemented by a grey literature search. Risk of bias was assessed using Cochrane\'s and Joanna Briggs Institute\'s tools. A total of 36 studies with 47 cases were included. MICRR is more common among younger females and may be related to hormonal changes and denosumab use. Initially, the premolars are usually affected but all permanent teeth may eventually be involved. Cone-beam computed tomography is recommended for diagnosis and assessment of resorptive lesions. The management is focused on complete removal and restoration of the resorptive tissue to maintain the tooth\'s structural integrity. However, MICRR usually has a poor prognosis. Due to its invasive and aggressive behavior, MICRR requires greater attention.
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  • 文章类型: Journal Article
    背景:纤维性纵隔炎(FM)被认为是良性疾病,但如果进展导致肺门受压或心脏侵入,可能是致命的。这种疾病的超声心动图报告非常罕见。
    方法:我们介绍了一名14岁男性患者,其非增强胸部计算机断层扫描显示前上纵隔软组织致密性病变不清晰。超声心动图显示心脏被软组织病变广泛包裹。组织学证实FM。
    结论:当FM影响心脏时,超声心动图可以帮助表征疾病并帮助诊断。超声心动图应被视为跟踪该疾病进展并指导治疗方法的重要工具。
    BACKGROUND: Fibrosing mediastinitis (FM) is considered a benign disease, but it can be fatal if progression leads to compression of the hilum of the lungs or invasion of the heart. Echocardiographic reports of this disease are very rare.
    METHODS: We present a 14-year-old male patient whose non-enhanced chest computed tomography showed unclear soft-tissue dense lesions in the anterior superior mediastinum. Echocardiography showed the heart was extensively wrapped by soft tissue lesions. The histology confirmed FM.
    CONCLUSIONS: When FM affects the heart, echocardiography can help to characterize the disease and aid in the diagnosis. Echocardiography should be considered an important tool to follow the progression of this disease and guide the therapeutic approach.
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  • 文章类型: Case Reports
    外伤性神经瘤主要由手术或外伤引起的神经损伤引起。未经腹部手术的胆囊外伤性神经瘤极为罕见,我们称之为“特发性胆囊创伤性神经瘤”。“由于它的稀有性和缺乏特定的临床和放射学特征,最常误诊。在我们的案例中,患者因胆管癌入院。术前重复腹部对比增强计算机断层扫描显示肝门部胆管癌。由于未来肝脏残留不足,我们在多学科小组会诊的基础上,计划术前经皮肝穿刺胆管引流术和经皮肝穿刺门静脉栓塞术。然后患者在1个月后入院接受手术。由于术中强烈怀疑胆囊癌,我们进行了腹腔镜胆囊切除术和广泛的腹腔镜右肝切除术。然而,最终诊断为经病理检查证实的胆囊外伤性神经瘤。胆囊的外伤性神经瘤非常罕见,我们希望通过报道我们的病例和回顾关于这个主题的文献,为诊断提供一些参考,这样可以避免广泛的治疗,从而提高患者的生活质量。据我们所知,这是自1996年以来在英语文献中报道的首例未进行手术的创伤性神经瘤病例.
    Traumatic neuroma mostly results from nerve injury caused by surgery or trauma. Traumatic neuroma of the gallbladder without prior abdominal surgery is extremely rare, and we termed it \"idiopathic traumatic neuroma of the gallbladder.\" Due to its rarity and a lack of specific clinical and radiological features, it is most commonly misdiagnosed. In our case, the patient was admitted to our hospital for cholangiocarcinoma. Repeated abdominal contrast-enhanced computed tomography scans preoperatively indicated hilar cholangiocarcinoma. Due to insufficient future liver remnant, we planned preoperative percutaneous transhepatic cholangiodrainage and percutaneous transhepatic portal vein embolization based on multidisciplinary team consultation. The patient was then admitted 1 month later for surgery. We performed a laparoscopic cholecystectomy and an extensive laparoscopic right hepatectomy as gallbladder carcinoma was strongly suspected intraoperatively. However, the final diagnosis was traumatic neuroma of the gallbladder confirmed by pathological examination. Traumatic neuroma of the gallbladder is very rare, and we hope to provide some references for diagnosis by reporting our case and reviewing the literature on this topic so that extensive treatment can be avoided, thus improving patients\' quality of life. To the best of our knowledge, this is the first reported case of traumatic neuroma without prior surgery in the English literature since 1996.
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  • 文章类型: Journal Article
    非梗阻性无精子症(NOA),最严重的男性不育类型,影响全世界约1%的男性。然而,大多数NOA病例的病因不明确,这被定义为特发性NOA(INOA),在世界范围内构成临床难题。这些患者中的大多数必须接受供体精子治疗,直到出现显微切割睾丸精子提取(micro-TESE)。尽管该方法最近已成为INOA的有希望的治疗方法,低精子回收率和睾丸损伤促使我们探索合适的非侵入性分子生物标志物来预测术前精子恢复的结局。以前的研究已经确定了一系列生物标志物来解决不同组织中不同水平的这一具有挑战性的问题。如DNA,RNA,血液和精液中的蛋白质和类固醇水平。为了更好地了解和评估来自不同组织的不同分子生物标志物对INOA患者通过micro-TESE进行精子提取结果的预测价值,我们总结了最近的发现,并讨论了这些方法的潜在应用。本研究的最终目的是为进一步研究和临床管理提供参考。
    Non-obstructive azoospermia (NOA), the most severe type of male infertility, affects approximately 1% of men worldwide. However, the aetiology of most NOA cases is not definite, that is defined as idiopathic NOA (INOA), posing a clinical conundrum worldwide. Most of these patients must receive donor sperm treatment until the emergence of microdissection testicular sperm extraction (micro-TESE). Although this procedure has recently become a promising treatment for INOA, the low sperm retrieval rate and testicular trauma have prompted us to explore appropriate non-invasive molecular biomarkers to predict the outcomes of sperm recovery preoperatively. Previous studies have identified a spectrum of biomarkers to address this challenging issue at various levels in different tissues, such as DNAs, RNAs, protein and steroid levels in the blood and seminal fluid. To better understand and assess the predictive values of diverse molecular biomarkers from different tissues on the outcome of sperm retrieval by micro-TESE in patients with INOA, we summarised recent findings and discussed the potential applications of these methods. The ultimate goal of this study was to provide references for further studies and clinical management.
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