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  • 简介:AbstractObjective:Scoping review of published literature to establish clinical characteristics and audiologic outcomes in patients diagnosed with Susacs Syndrome(SS) who have undergone cochlear implantation (CI).Data sources:All published studies of CI in SS and contribution of two of our own patients who have not been reported previously.Methods:A comprehensive search of MEDLINE (via PubMed) was carried out in March 2020 using the following keywords and related entry terms: Susacs Syndrome, Cochlear Implantation. Results: Our search identified a total of five case reports of CI in SS. With the addition of our two patients reported here, we analyzed characteristics and outcomes in seven patients. Mean age at implantation was 30 years old (range 19-46), with six women and one man implanted. Mean time from onset of hearing loss to implantation was 17 months (range three months to four years). Best reported postoperative speech understanding was reported via different metrics, with six of seven patients achieving open set speech scores of 90% or better, and one subject performing at 68%. Vestibular symptoms were present preoperatively in four of seven patients (57%), with vestibular testing reported in two patients, and showing vestibulopathy in one patient. No complications were reported following cochlear implantation.Conclusion:Cochlear implantation is a viable option for hearing rehabilitation in patients with SS, with levels of attainment of open set speech comparable to other populations of CI candidates.

  • 标签: Susac’s syndrome Cochlear implantation Scoping review
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  • 简介:Symptomatichepato-diaphragmaticinterpositionofabowellooporChilaiditi'ssyndromeisapeculiaranatomicalconditionmostoftenfoundbychance.Itsdescribedsymptomsrangefromintermittent,mildabdominalpainanddyspepsiatoacuteintestinalobstruction.Wereportacaseofhepato-diaphragmaticmigrationofthehepaticflexureofthecolonassociatedtoanunusual,heretoforeunreported,angina-likepainexclusivelyevokedbytheleftlateraldecubitus.Tomaximizethechanceofobservinganatomicalchangesindifferentpostures,computedtomographyofthechestandabdomenwasperformedafterairinsufflationintothecolon.Whilefrankherniationintothechestwasexcluded,thescanshowedthatthehepaticflexure-withtheinterpositionofthediaphragm-cameincontactwiththerightsideoftheheartintheleftlateral,butnotinthesupine,decubitus.Thisfindingwasreproducedbyechocardiographywhichalsoshowedvirtuallyunalteredhemodynamicsafterthechangeofposture.ECG,leftandrightventricularglobalandregionalfunctionaswellascardiacinjurymarkersalsoremainedunchangedduringthemaneuver,indicatingthatthepainevokedbythelatterwasunlikelyduetomyocardialischemia.ThiscasesuggeststhatChilaiditi'ssyndromeshouldbeincludedamongthepossible,althoughrare,causesofunexplainedangina-likesymptoms.

  • 标签: 契莱迪特综合症 膈肌下结肠嵌入综合症 临床表现 病理机制 咽痛
  • 简介:Reiter'ssyndromeisasecondarydiseaseofurethrainfection.Itconsistsofurethritis,nonsuppurativearthritis,conjunctivitisandcutaneousmucosalesions.YoungmalesaremorelikelytogetReiter'ssyndromethanfemales.Thusfar,therearenoreportsofwomeninChinawithReiter'sSyndrome.ThisreportdocumentstwofemaleswithReiter'sSyndrome.

  • 标签: REITER综合征 尿道感染 尿道炎 关节炎 结膜炎 病例分析
  • 简介:AIM:Todeterminetheclinicalfeatures,diagnosisandtreatmentoftheprimarySjogrensyndrome(SS)relatedopticneuritis.METHODS:Theclinicaldataof8patients(12eyes)withprimarySSrelatedopticneuritiswereanalyzedretrospectively.RESULTS:Eightof128consecutivepatientswithopticneuritisresultedfromvariedcausesfulfilledthediagnosticcriteriafortheprimarySS.Theypresentedinitiallywiththesignsandsymptomsofnon-specificopticneuritis,and5patientspresentingwithoutdrynessshowedachronicinflammationofsubmandibularglandorparotidgland,andlymphocyteinfiltrationwasdemonstratedbylabialglandbiopsyin2patients.Therewereserumpositivetitersforanti-SjogrensyndromeA(SSA)in7patientsandanti-SjogrensyndromeB(SSB)in8patients.Anti-aquaporin-4(AQP4)antibodywasnegativeinallthe8patients.Bothglucocorticoidsandimmunosuppressiveagentwereadministered,andvisualacuityelevatedin8eyes(66.7%),3patients(37.5%)recurredinthefollow-up.CONCLUSION:PrimarySSrelatedopticneuritisislesscommonandeasilymisdiagnosed.Theconventionaltherapiesforopticneuritiscouldnotcontroltherecurrence.

  • 标签: OPTIC NEURITIS PRIMARY Sjgren’s SYNDROME anti-aquaporin-4
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  • 简介:Sweet’s综合征(Sweet’sSyndrome,SS),又称急性发热性嗜中性皮病,是一种与免疫因素相关的伴有全身症状的少见皮肤病。临床分为经典型(特发型)、肿瘤相关型及药物诱导型三种类型,其中肿瘤相关型与急性白血病最常见,合并骨髓增生异常综合征(MDS)的SS国内已有10例报道,但均尚未见报道同时合并肝脾肺多系统受累且有过敏性紫癜样皮损的病例,现报道1例如下并作文献复习。

  • 标签: 文献复习 骨髓增生异常综合征 急性发热性嗜中性皮病 MDS 少见皮肤病 急性白血病
  • 简介:Tourette'ssyndromeistreatedbybehavioralorpharmacologicaltherapy.However,patientswithmalignantTourette'ssyndromealsoexhibitlife-threateningsymptoms,whichareunresponsivetoconservativetreatmentsorneurosurgicalprocedures,suchasdeepbrainstimulation.Inrecentyears,mesenchymalstemcells(MSCs)haveshowntherapeuticpotentialinmanyneurologicaldiseases.Therefore,thepresentstudyproposedtouseMSCtransplantationasanoveltherapyforTourette'ssyndrome.StereotypicbehaviorsinTourette'ssyndromeratsdecreasedsignificantlyat21daysafterhumanMSCstransplantationintothestriatum.ImmunohistochemistryanalysesrevealedsurvivaloftransplantedhumanMSCsanddifferentiationintoneuronsandastrocytesintheratbrain.ResultssuggestthatintrastriataltransplantationofhumanMSCscouldprovidetherapeuticpotentialforTourette'ssyndrome.

  • 标签: 骨髓间质干细胞 体细胞移植 综合征 动物模型 骨髓基质干细胞 药物治疗
  • 简介:客观:与严重煽动性的反应症候群(先生)在创伤的病人调查甲状腺荷尔蒙的引申。方法:有严重先生的五十个创伤的病人被注册并且根据他们是否介绍了multiorgandysfunction症候群(MODS)把组划分了成二。甲状腺荷尔蒙大小被拿,包括totaltriiodothyronine(TT3),全部的甲状腺素(TT4),免费triiodothyronine(FT3),免费甲状腺素(FT4)和甲状腺刺激荷尔蒙(TSH)。尖锐生理学和长期的健康评估Ⅱ(APACHEⅡ)20根据临床的数据被计算。恢复或恶化的结果被记录,以及从到时间甲状腺荷尔蒙的先生的发作的时间的长度被测量。结果:Euthyroid病了的症候群(S字)在45cases.TT3水平被介绍否定地与APACHEH分数被相关(r=-0.330,P<0。05),并且TT3/TT4value否定地与先生的持续时间被相关(r=-0.316,P<0.05)。没有MODS,在MODS病人的TT3,TT4和FT3levels是比那些显著地低的(P<0.05)。没有MODS,给低TT4或FT4的MODS病人比那些经常铺平更多(P<0.05)。与在正常TSH组的病人相比,有有的减少的TSH的病人降低T3,T4,恢复率和更高的APACHEⅡ分数,MODS发生,但是二个组之间没有差别(P>0.05).Conclusions:有严重先生的损伤病人有高可能性得到S字,它更经常并且严重地发生在MODS病人。它在甲状腺轴上显示出先生的影响。Withthe坚持和先生的恶化,有甲状腺荷尔蒙的进步减小。

  • 标签: 甲状腺功能 创伤 免疫反应 病理机制
  • 简介:共分析了50篇丈章,分别采用单纯针剌法,针剌结合其他疗法,耳穴贴压疗法和其他疗法进行治疗。有39篇文章有明确的诊断标准,其中《实用中西医结合诊断治疗学》,Kupperman评分和《中医病证诊断疗效标准》。治疗组涉及病例总数2946例,临床疗效最高达100%,最低为58、1%。有16篇设立了对照组。15篇进行了辨证分型,共27种证型,有肝肾阴虚,睥肾阳虚,肾阳虚,肾阴虚,心肾不交和肝郁等。针灸治疗更年期综合征具有一定效果,但缺少严格的随机对照试验(RCT)。

  • 标签: 针刺疗法 耳穴贴压疗法 更年期综合征 针灸 中医 TCM
  • 简介:少年polyposis症候群是多重不同少年息肉在胃肠的道和colorectal癌症的增加的风险描绘的稀罕正染色体的主导的症候群。colorectal癌症的累积一生风险是39%,相对风险是34。少年息肉把特殊组织学被有煽动性的房间和膀胱地扩大的腺的薄板propria排队了由的许多edematous描绘了对有反应变化的圆柱的上皮立方形。临床上,少年polyposis症候群被5的存在或更多的少年息肉在colorectum,在整个胃肠的道的少年息肉或少年息肉的任何数字和少年polyposis的积极家庭历史定义。在大约与少年polyposis症候群诊断的病人的50%-60%,在SMAD4或BMPR1A基因的一个germline变化被发现。两基因在表明小径的BMP/TGF-beta起一个作用。在少年polyposis的癌症可以通过反常stromal环境导致邻近的上皮并且最后的肿瘤的转变的所谓的landscaper机制发展,这被建议了侵略的癌。这稀罕混乱的识别关于治疗为病人和他们的家庭是重要的,后续并且屏蔽在风险个人。与少年息肉的诊断面对的每位临床医生应该因此考虑少年polyposis症候群的可能性。另外,少年polyposis症候群提供一个独特模型一般来说学习colorectal癌症致病并且在癌症的分子的基因基础给卓见。这评论讨论少年polyposis症候群的临床的表明,遗传,致病和管理。

  • 标签: 幼年性 综合征 SMAD4蛋白 分子遗传基础 发病机制
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  • 简介:Gutmicrobiotaexertsasignificantroleinthepathogenesisofthemetabolicsyndrome,asconfirmedbystudiesconductedbothonhumansandanimalmodels.Gutmicrobialcompositionandfunctionsarestronglyinfluencedbydiet.Thiscomplexintestinal'superorganism'seemstoaffecthostmetabolicbalancemodulatingenergyabsorption,gutmotility,appetite,glucoseandlipidmetabolism,aswellashepaticfattystorage.Animpairmentofthefinebalancebetweengutmicrobesandhost’simmunesystemcouldculminateintheintestinaltranslocationofbacterialfragmentsandthedevelopmentof'metabolicendotoxemia',leadingtosystemicinflammationandinsulinresistance.Dietinducedweight-lossandbariatricsurgerypromotesignificantchangesofgutmicrobialcomposition,thatseemtoaffectthesuccess,ortheinefficacy,oftreatmentstrategies.Manipulationofgutmicrobiotathroughtheadministrationofprebioticsorprobioticscouldreduceintestinallowgradeinflammationandimprovegutbarrierintegrity,thus,amelioratingmetabolicbalanceandpromotingweightloss.However,furtherevidenceisneededtobetterunderstandtheirclinicalimpactandtherapeuticuse.

  • 标签: GUT MICROBIOTA METABOLIC syndrome OBESITY DIABETES
  • 简介:AbstractMetabolic syndrome (MetS) describes a set of risk factors that can eventually lead to the occurrence of cardiovascular and cerebrovascular disease. A detailed understanding of the MetS mechanism will be helpful in developing effective prevention strategies and appropriate intervention tools. In this article, we discuss the relationship between the clinical symptoms of MetS and differences in the gut microbial community compared with healthy individuals, characterized by the proliferation of potentially harmful bacteria and the inhibition of beneficial ones. Interactions between gut microbiota and host metabolism have been shown to be mediated by a number of factors, including inflammation caused by gut barrier defects, short-chain fatty acids metabolism, and bile acid metabolism. However, although we can clearly establish a causal relationship between gut microbial profiles and MetS in animal experiments, the relationship between them is still controversial in humans. Therefore, we need more clinical studies to augment our understanding of how we can manipulate the gut microbiota and address the role of the gut microbiota in the prevention and treatment of MetS.

  • 标签: Metabolic syndrome Gut microbiota Inflammation Short-chain fatty acids Bile acids
  • 作者: Zuo Yu Shi Hui Li Chun Knight Jason S.
  • 学科: 医药卫生 >
  • 创建时间:2020-08-10
  • 出处:《中华医学杂志(英文版)》 2020年第08期
  • 机构:Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA,Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China,Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
  • 简介:AbstractAntiphospholipid syndrome (APS) is a thromboinflammatory disease with a variety of clinical phenotypes. Primary thrombosis prophylaxis should take an individualized risk stratification approach. Moderate-intensity vitamin K antagonist such as warfarin remains the primary strategy for secondary thrombosis prophylaxis among APS patients, especially for patients with predominantly venous disease. For now, direct oral anti-coagulants should be avoided in most APS patients, especially those with history of arterial manifestations. Obstetric APS management should be tailored based on an individual patient’s antiphospholipid antibody profile, and obstetric and thrombotic history. Pharmacological agents beyond anticoagulants may be considered for the management of microthrombotic and nonthrombotic manifestations of APS, although more data are needed. A relatively recent discovery in the area of APS pathogenesis is the implication of neutrophil extracellular traps in thrombin generation and initiation of inflammatory cascades. APS is a complex thromboinflammatory disease with a broad clinical spectrum. Personalized therapy according to an individual’s unique thrombosis and obstetric risk should be advocated.

  • 标签: Antiphospholipid syndrome Antiphospholipid antibodies Treatment
  • 简介:Thecutaneousnerveentrapmentsyndromeisnamedthat,thecutaneousnerve'sfunctionaldisordercausedbysomechronicentrapment,moreoverappearsaseriesofnerve'sfeelingobstacle,vegetativenervefunctionobstacle,nutritionobstacle,evenmotorfunctionobstacleinvariousdegree.

  • 标签: 皮肤神经诱导综合症 病因 临床表现 类型
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