Crohn＇s病的肠外表现特征及其临床意义

目的探讨Crohn＇s病（CD）患者肠外表现的特点和临床意义。方法回顾性收集有肠外并发症表现的51例CD患者的临床资料,分析患者出现肠外并发症的特征表现,结合文献报道对比复习这些肠外并发症在Crohn＇s的诊断和治疗中的意义。结果51例患者肠外表现依次为骨关节病变、口腔溃疡、肝胆病变、皮肤病变、眼部、代谢性骨病、血管闭塞等。出现一种肠外病变的29例,两种肠外病变的为13例,三种或以上病变的为9例。原发CD病灶的受累范围越广,其肠外表现的种类越多。结论肠外表现与Crohn＇s病的累及部位与严重度有一定关系,肠外并发症对其原发Crohn＇s病诊断和治疗均有一定的影响。     

小儿心因性疾病临床探讨

目的　探讨儿童心因性疾病发病诱因、临床表现、治疗及预后。方法　分析总结 6 6例儿童心因性疾病临床与辅助资料。结果　 6 6例患儿大多为学龄儿童 ,且均有不良精神因素刺激 ,其中校园因素占 31例(47% ) ,家庭因素占 18例 (2 7% ) ,躯体疾病占 11例 (17% ) ,其他 6例 ,如惊吓、胆怯、意外事故等。临床表现各异 ,以瘫痪和抽搐最多 ,各占 36 %和 18%。治疗以心理治疗和暗示治疗为主 ,效果明显 ,预后良好。结论　儿童心因性疾病并不少见 ,其发病与社会及家庭环境关系密切 ,故家长和老师应加强对学龄儿童精神心理教育 ,以降低该病的发生。同时该病表现多样 ,易误诊 ,应引起儿科医生的重视     

合并甲状腺病变的嗜酸性筋膜炎1例及文献复习

目的 报告1例合并甲状腺病变的嗜酸性筋膜炎(EF)病例，为今后临床诊治提供参考。方法 兰州大学第二医院2021年4月2日收治的1例合并甲状腺疾病的女性EF病例，对相关文献进行复习。结果 女，52岁。双肩关节皮肤肿痛2月，双前臂皮肤肿胀、僵硬1月，入院检查时发现甲状腺结节。双前臂“沟槽征”，双前臂核磁共振成像示筋膜炎症性改变，外周血嗜酸性粒细胞计数0.55×109/L。甲状腺组织病理活检提示甲状腺乳头状癌、结节性甲状腺肿、桥本甲状腺炎。综上诊断为EF、甲状腺恶性肿瘤(乳头状癌)、桥本甲状腺炎、结节性甲状腺肿。给予醋酸泼尼松片和甲氨蝶呤，3月后皮损基本痊愈；并行双侧甲状腺切除+左侧中央区清扫，随访3月无复发及转移。结论 EF可能作为系统性疾病的皮肤表现，遇到此类病人除积极筛查自身免疫系统、血液系统相关疾病等常见合并症，也应该高度警惕实体肿瘤等少见合并症，早期识别各种潜在疾病。     

社区常见口腔黏膜疾病的诊治

口腔黏膜病是指发生在口腔黏膜及软组织上的疾病总称,与免疫功能、饮食生活习惯、遗传因素、精神因素等相关。本文对口腔黏膜病中常见的阿弗他溃疡、扁平苔藓、白斑的病因、临床表现和治疗进行了详细的阐述。     

Common and uncommon features of rheumatoid arthritis and chronic obstructive pulmonary disease: clues to a future therapy

Over the last decade it has become apparent that common pathogenic mechanisms are shared between many human chronic inflammatory diseases of unrelated pathology and manifestation. These mechanisms include common inflammatory networks that control tissue destructive and repair processes and their study is of major therapeutic potential as recently demonstrated for TNFalpha. Thus, early studies in rheumatoid arthritis defined TNFalpha as a major therapeutic target, the blockade of which was subsequently proved to be of great efficacy in the clinic. This paved the way for the successful blockade of TNFalpha in various other diseases including Crohn's disease, psoriasis, spondyloarthropathies and juvenile arthritis, although no similar networks with anti-TNFalpha at their apex had previously been demonstrated. In this article, we review the current knowledge of the pathogenic mechanisms involved in rheumatoid arthritis and chronic obstructive pulmonary disease with particular emphasis on the role of inflammatory cytokines, chemokines, and tissue degrading enzymes as revealed by studies in the laboratory and the clinic. Direct comparison of these mechanisms may provide clues for a future therapy for these painful and incurable diseases.     

Enzyme-replacement therapy: Problems and prospects

Several diseases can, at least in theory, be treated by the administration of an enzyme, the deficiency of which is the cause of the disease. Various attempts have been made to correct enzymatic deficiencies responsible for the clinical manifestation of diseases for which prevention cannot be achieved by modification of the diet or by supportive therapy with drugs. Except for treating certain digestive disorders, enzyme-replacement therapy has not yet found a broad application. In this review article a compilation is given of the problems and prospects of enzyme-replacement therapy in diseases caused by the deficiency of an enzyme.     

以血细胞减少为特征的非血液系统疾病临床分析

目的：分析以血细胞减少为特征的非血液系统疾病特点,开阔临床医师思维,避免误诊、漏诊。方法对108例以血细胞减少为特征的非血液系统疾病患者的临床特征、实验室检查及诊断进行回顾性分析。结果所有患者中感染性疾病31例,其中疟疾28例,流行性出血热2例,伤寒1例;脾功能亢进21例;结缔组织病21例,以系统性红斑狼疮最为多见;骨髓转移癌18例;甲状腺机能减退症7例;甲状腺机能亢进症5例;胃肠道肿瘤5例。结论以血细胞减少为特征的非血液系统疾病临床以感染性疾病最为多见,其次为脾功能亢进、结缔组织病和骨髓转移癌,应引起临床医师重视,减少误诊、漏诊。     

Treatment approach to connective tissue disease-associated interstitial lung disease

Interstitial lung disease (ILD) is a common manifestation in connective tissue diseases (CTD), such as rheumatoid arthritis (RA), systemic sclerosis (SSc), and inflammatory myositis (IM). ILD is associated with significant morbidity and mortality in nearly all CTD highlighting the critical need for effective treatment strategies in this patient population. In this review, we will summarize the approach to treatment when there is concern for CTD-ILD and highlight recent advancements in therapeutics within various forms of CTD-ILD.     

Viral vectors for neurotrophic factor delivery: A gene therapy approach for neurodegenerative diseases of the CNS

The clinical manifestation of most diseases of the central nervous system results from neuronal dysfunction or loss. Diseases such as stroke, epilepsy and neurodegeneration (e.g. Alzheimer's disease and Parkinson's disease) share common cellular and molecular mechanisms (e.g. oxidative stress, endoplasmic reticulum stress, mitochondrial dysfunction) that contribute to the loss of neuronal function. Neurotrophic factors (NTFs) are secreted proteins that regulate multiple aspects of neuronal development including neuronal maintenance, survival, axonal growth and synaptic plasticity. These properties of NTFs make them likely candidates for preventing neurodegeneration and promoting neuroregeneration. One approach to delivering NTFs to diseased cells is through viral vector-mediated gene delivery. Viral vectors are now routinely used as tools for studying gene function as well as developing gene-based therapies for a variety of diseases. Currently, many clinical trials using viral vectors in the nervous system are underway or completed, and seven of these trials involve NTFs for neurodegeneration. In this review, we discuss viral vector-mediated gene transfer of NTFs to treat neurodegenerative diseases of the central nervous system.     

放疗导致心脏损伤的发生机制及防治进展

摘要：放疗作为恶性肿瘤的主要治疗方法之一，广泛应用于乳腺癌、食管癌、肺癌、纵隔肿瘤和淋巴瘤等疾病。 接受放疗患者长时间在X线照射下很容易导致心脏损伤，引起不同程度的放射性心脏病（RIHD），RIHD可以破坏心 脏的任何结构，因此心脏损伤在癌症幸存者中成为危及生命的疾病，而有效的临床管理及防治仍然具有很大的挑战 性。本文对放疗后心脏损伤的表现、发生机制、心脏相关指标监测及防治进展等方面作一综述。     

X线胸片对新生儿常见肺疾患诊断和预后评估的价值

目的：分析新生儿常见肺疾患的临床和胸片特征，探讨X线胸片在该类疾病诊断及预后评估中的价值。方法：对50例新生儿湿肺症(WLD)、45例胎粪吸入综合征(MAS)、31例呼吸窘迫综合征(NRDS)、25例肺出血(NPH)X线诊断结果与临床资料、病理报告进行对比分析。结果：本组影像诊断总准确率为96．7％。WLD病程短，预后良好，X线表现演变迅速；NRDS症状出现早，病程长，萎陷的肺野内大量含气支气管影；MAS临床无特异性，影像表现复杂，肺野呈现羊水异物影；NPH起病急，发展快，以出血性肺水肿为主要表现。结论：新生儿常见肺疾患具有支气管充气征、蝴蝶征和肺野异物等多种特征性胸片表现，并可以通过对胸片征象及其演变规律的分析，判定病变严重程度及预后转归。     

Therapeutic potential of endothelin receptor antagonists and nitric oxide donors in pulmonary hypertension

Pulmonary hypertension can occur idiopathically as a primary disorder of the pulmonary circulation or more commonly, it can exist as a haemodynamic manifestation of a wide variety of pulmonary and cardiovascular diseases, including acute lung injury, chronic obstructive lung disease, congenital heart disease, mitral stenosis, chronic left-sided congestive heart failure and connective tissue diseases such as scleroderma. Pulmonary hypertension is associated with changes in vascular tone as well as vascular structure, with the relative contribution of each dependent upon the aetiology of the increased pulmonary vascular resistance. Most currently available treatments utilise anticoagulants as well as vasodilator drugs that only attenuate the vasoconstrictive component of the disease. The latter category includes oral calcium channel blockers, iv. and aerosolised prostacyclin analogues and inhaled nitric oxide but all three classes of vasodilators have disadvantages and limitations. Treatment with vasodilators is often ineffective in patients with longstanding pulmonary hypertension in which structural changes contribute significantly to the pulmonary hypertension, blood flow obstruction and right heart failure. In view of the immense clinical need, new therapies are being developed by pharmaceutical companies to treat pulmonary hypertension. This update will focus on the current development status of endothelin receptor antagonists and nitric oxide donors for the treatment of pulmonary hypertension.     

Pomegranate seed oil nanoemulsions for the prevention and treatment of neurodegenerative diseases: the case of genetic CJD

Neurodegenerative diseases generate the accumulation of specific misfolded proteins, such as PrP^Sc prions or A-beta in Alzheimer's diseases, and share common pathological features, like neuronal death and oxidative damage. To test whether reduced oxidation alters disease manifestation, we treated TgMHu2ME199K mice, modeling for genetic prion disease, with Nano-PSO, a nanodroplet formulation of pomegranate seed oil (PSO). PSO comprises large concentrations of a unique polyunsaturated fatty acid, Punicic acid, among the strongest natural antioxidants. Nano-PSO significantly delayed disease presentation when administered to asymptomatic TgMHu2ME199K mice and postponed disease aggravation in already sick mice. Analysis of brain samples revealed that Nano-PSO treatment did not decrease PrP^Sc accumulation, but rather reduced lipid oxidation and neuronal loss, indicating a strong neuroprotective effect. We propose that Nano-PSO and alike formulations may be both beneficial and safe enough to be administered for long years to subjects at risk or to those already affected by neurodegenerative conditions.From the Clinical EditorThis team of authors report that a nanoformulation of pomegranade seed oil, containing high levels of a strong antioxidant, can delay disease onset in a mouse model of genetic prion diseases, and the formulation also indicates a direct neuroprotective effect.     

妊娠期合并外科急腹症的误诊分析及外科处理策略探讨

目的探讨妊娠合并急腹症患者的临床表现、误诊原因及确诊经验分析,为临床妊娠并发急腹症诊治提供可借鉴依据。方法收集我院2006年6月～2011年6月132例妊娠期并发急腹症患者的资料,分析各疾病误诊率及各疾病在妊娠早、中、晚期误诊率,分析其误诊原因并探讨其确诊经验。结果常见误诊疾病包括卵巢囊肿蒂扭转(10.61%),异位妊娠(10.61%),酮症酸中毒(7.58%),急性胰腺炎(7.58%),急性阑尾炎(6.82%),浆膜下子宫肌瘤扭转(6.82%)等。妊娠各期误诊疾病分布各有特点,以酮症酸中毒、急性阑尾炎、泌尿系结石及异位妊娠、卵巢囊肿蒂扭转等疾病多见。结论妊娠期合并急腹症有较高误诊率,妊娠各期误诊疾病分布各有特点,常可因妊娠期腹内变化导致急腹症诊断困难而延误治疗,提高对罕见疾病的警惕性,积极慎重处理,可及早确诊及降低流产、早产及母孕死亡的相关风险。     

Minimal renal dysfunction in inflammatory bowel disease is related to disease activity but not to 5-ASA use

BACKGROUND: Conflicting data exist about proteinuria in inflammatory bowel diseases. It is still unclear whether the occurrence of proteinuria in inflammatory bowel disease patients is an extra-intestinal manifestation of disease or the result of adverse effects to medication, especially to aminosalicylates (ASA). METHODS: A total of 95 patients (51 with Crohn's disease and 44 with ulcerative colitis) were enrolled in the study. Disease activity was assessed by Crohn's Disease Activity Index (CDAI) or the Truelove index, respectively. Urine was collected over 24 h and protein excretion of specific marker proteins for tubular (alpha 1-microglobulin-alpha 1-MG) and glomerular (albumin-Alb, Immunoglobulin G-IgG) dysfunction was measured using a highly sensitive immunoluminometric assay. RESULTS: Out of 51 Crohn's disease patients, 20 showed elevated urinary alpha 1-MG. The amount of alpha 1-MGuria was strongly correlated to the CDAI (r=0.6, P < 0.001). Only four Crohn's disease patients showed slightly elevated values for glomerular proteins in urine. Similar results were obtained for ulcerative colitis: whereas only two ulcerative colitis patients showed albuminuria, tubular proteinuria was detected in 28 out of 44 ulcerative colitis patients. Proteinuria was strongly dependent on disease activity (P < 0.01) but was not related to ASA treatment. CONCLUSIONS: Proteinuria of tubular marker proteins occurs in the majority of inflammatory bowel disease patients and is related to disease activity rather than to ASA treatment. Tubular proteinuria seems to reflect a renal extra-intestinal manifestation of inflammatory bowel disease and may serve as a new relevant marker of disease activity.     

非胆囊性疾病胆囊壁的超声声像图分析及其鉴别

目的探讨非胆囊性疾病胆囊变化及其临床意义。方法观察肝硬化腹水、黄疸型肝炎和结核性腹膜炎患者胆囊壁超声像图特点,并分析其异同。结果三种疾病胆囊壁均存在不同程度的水肿和增厚、毛糙欠光滑或不光滑;此外,肝硬化腹水胆囊壁超声多呈“双边影”,并多伴肠管飘浮征和脾大;结核性腹膜炎患者胆囊壁超声多表现呈单层薄壁,且多伴腹水分隔征,脾脏多不增大;黄疸型肝炎患者胆囊壁超声多表现为胆囊腔缩小、腔中回声差、甚至不透声。结论对患者胆囊壁超声声像图进动态观察,结合实验室检查及临床表现,对诊断和鉴别腹部疾病病因具有重要的临床意义。     

The lung in immune-mediated disorder: rheumatoid arthritis

Various pleuro-pulmonary abnormalities are known to complicate vascular collagen diseases, particularly, rheumatoid arthritis. Each component of the respiratory system is affected, either separately or in combination. Although most pulmonary complications appear in an established case of collagen vascular disease, in certain conditions, the lung disease precedes the more typical manifestation. While some complications are asymptomatic and tend to be resolved spontaneously (for e.g. pleuritis and rheumatoid nodules), others may cause severe or fatal conditions (interstitial pneumonia and constrictive bronchiolitis). The incidence of interstitial lung disease is increasing in vascular collagen disease. This may be mainly attributed to the increase use of invasive techniques such as bronchoscopy and video-assisted thoracoscopic surgery and in part due to the use of high resolution computed tomography, and functional pulmonary tests.     

原发性肠道淋巴瘤11例临床分析

目的提高对原发性肠道淋巴瘤的诊治水平。方法回顾性分析近5年11例原发性肠道淋巴瘤的临床资料，总结原发性肠道淋巴瘤的临床、实验室检查特点及误诊原因。结果原发性肠道淋巴瘤临床表现无特异性，术前诊断困难，漏诊、误诊率高。结论医师应充分重视该病，对疑似病例尽早行全肠道内镜检查，病变区即使是微小病变，也要常规取活检行病理组织学检查，内镜检查仍不能确诊的病例，应及时在影像学监视下行腹腔包块穿刺或剖腹探查，以进一步明确诊断。     

老年人缺血性结肠炎的临床及内镜特点

目的研究老年人缺血性结肠炎的发病因素、临床及内镜特点,探讨早期诊断方法,减少误诊发生。方法对笔者所在医院2008年1月～2012年2月确诊的20例老年人缺血性肠炎的临床资料进行回顾性分析。结果 20例老年患者多有心血管疾病、高血压、糖尿病、血液病等基础疾病。临床表现主要为腹痛、便血、腹泻。早期内镜表现主要为结肠黏膜高度水肿、充血、出血、糜烂及溃疡。结论结肠镜是早期诊断缺血性结肠炎的重要手段。     

急性心肌梗死30例临床误诊分析

目的 对30例急性心肌梗死误诊原因进行分析总结.方法 对本院门诊、急诊收治的急性心肌梗死30例患者临床资料进行回顾性分析,对首诊误诊及救治效果进行总结分析.结果 12例误诊为COPD、肺心病、气胸、急性肺栓塞等呼吸系统疾病急性发作;6例误诊为脑血管急症;6例误诊为消化道急腹症;3例误诊为牙周炎等;2例误诊为颈椎病;1例误诊为肋软骨炎.经紧急抢救,痊愈23例,死亡7例,死亡率为23.3%.结论 急性心肌梗死患者早期临床表现不典型,容易导致误诊,延误治疗导致患者死亡,医生应根据症状体征、ECG及血生化等结果进行综合诊断,减少误诊发生.