简介：Waardenburgsyndromeisararediseasecharacterizedbysensorineuraldeafnessinassociationwithpigmentarydefects.Dependingonadditionalsymptoms,WShavebeenclassifiedintofourtypes.Waardenburgsyndrometype4,alsocalledasWaardenburgShahSyndromeisaveryrarecongenitaldisorderwithastoundingvariableclinicalexpression,characterizedbypigmentaryabnormalitiesofthehair(Awhiteforelockofhair,prematuregraying)andpigmentarychangesoftheirissuchasheterochromiaorhomochromiairides,sensorineuraldeafnessandHirschsprungdisease.ThreegeneshavebeenbestowedsofarinconsociationwithEDNRB,EDN3,andSOX10genes.ThepatternofinheritanceismultifariouswiththeSOX10mutationaffiliationwithautosomaldominantinheritancewhereastheEDNRBandEDN3genesarepasseddowninanautosomallyrecessivepattern.

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简介：Littoral-cellangioma(LCA),aprimaryangiomawhichclinicallybelongstosplenichemangioma,canbemostlyfoundinnormalspleenredsinusshorecellsofreticuloendothelialcellsystem.ThecellsofLCAstronglyexpressendothelialandtissuecellassociatedantigensthatindicateadualdifferentiationcharacteristic;whereasonlyendothelialcellmarkersarepositiveinnormalspleenredsinusshorecells.DiagnosisofLCAreliesonhistopathology.Regularfollow-upisneededtomonitorrecurrenceandmetastasis.

简介：AbstractBackground:Endolymphatic sac tumor (ELST) is one of neuroectodermal tumor which arising from endolymphatic sac and duct. It is actually quite rare, with less than 200 cases reported. Although ELST presents benign appearance in histopathology, it can present aggressive destructive behavior in clinical. The cornerstone of treatment for ELST is complete surgical excision. However, it is almost impossible to completely resect the advanced stage tumor. There is still controversy about other treatments, such as radiotherapy and gamma knife surgery.Case presentation:A 47-year-old man was admitted in The First Affiliated Hospital of Fujian Medical University with a 7-year history of progressive hearing loss and near 6-month repeated attacks of headache. Preoperative CT revealed a massive intracranial lesion and associated hydrocephalus. MR scanning demonstrated a 7.2 cm × 4.6 cm × 4.2 cm bulky mass located in left-sided posterior cranial fossa and temporo-occipital region which showed hyperintensity on T1-weighted images and mixed signal intensity on T2-weighted images. There was no neither clinical manifestation nor family history of Von Hippel-Lindau syndrome (VHL).Due to the mass that was large and invading the bone of skull base, it was difficult to extirpate surgically, so the ventriculoperitoneal shunt combined with local biopsy was performed. The postoperative pathology and immunohistochemical findings confirmed the lesion was an endolymphatic sac tumor. After operation, the patient regularly received radiotherapy.Conclusion:The widely accepted management of ELST is complete surgical resection. However, it is difficult for surgeons to achieve radical resection with late-stage ELST. Currently, there is much dispute about the role of radiotherapy for the management of ELST in academic circles. In this case where the mass cannot be surgical removed, radiotherapy has the curative effect for ELST in terms of disease control and quality of life.

简介：A78-year-oldwomanpresentedwithfever,severeabdominalpain,anddistension.Shehadbeeninstitutionalizedfordepressionandseniledementia.Laboratoryexaminationsdisclosedaleucocytosis(WBC:12500/μL)andelevatedlevelsofserumC-reactiveprotein(2.8mEq/L).Diagnosisofacutececalvolvuluswasmadefroma'coffeebeansign'onanabdominalcomputedtomographyanda'beaksign'onagastrographinenema.Anemergentlaparotomyconfirmedthediagnosisandanileo-colectomywithprimaryanastomosiswascarriedout.Thepatientrecoveredafterintensiverespiratorycareandfluidtherapy,andthenreturnedtoherformerinstitution.AreviewofJapaneseliteraturedisclosedthat:(1)amarkedincreaseofagedpatientswithmentaldisabilitypresentingwithcecalvolvulus,(2)adoptionofileo-colectomyasthestandardsurgicalprocedure,and(3)improvedsurvivalofthepatients,wereobservedinthelastdecade.

简介：Congenitalcholesteatoma(CC)isararelyseenbenigntumorofthetemporalbone.Therearefivegeneralsitesofextraduraloccurrence:themiddleear,externalauditorymeatus,mastoid,squamousportionandthepetrousapexofthetemporalbone.CCgrowsslowlyandpresentsnosymptomsattheearlystage.Delayedandmis-diagnosisarecommonwiththiscondition~1.CasereportA10-year-oldboypresentedwitha3-monthhistoryofhearinglossonrightside.Therewasnohistoryofotorrhea,facialpalsy,previousotologicalproceduresortrauma.Otoscopyrevealedabulgingposterosuperiorquadrantintheotherwiseintactrighttympanicmembrane(Fig.1).Puretoneaudiometryshowedanaveragethresholdof51dBfor500,1000,2000and4000Hz,witha40dBair-bonegap,suggestingamoderateconductivehearingloss(Fig.4).CTscanofthetemporalboneshowedanisolatedsofttissuedensitylesioninthemiddleear(Fig.2).

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简介：AbstractIntroduction:Nicolau syndrome is a rare complication following intramuscular injections of various drugs. It is characterized by severe pain, skin and sometimes muscle necrosis after drug injection. However the pathogenesis of Nicolau syndrome remains unclear.Here, we report a case of Nicolau syndrome following metamizole injection.Case presentation:A 35-year-old female presented with an painful necrotic ulcer who developed Nicolau syndrome after an intramuscular injection of metamizole in her right thigh due to reccurent episodes of back pain. This reaction is typical for Nicolau syndrome.Discussion:The most common finding of Nicolau syndrome in the early period was severe pain at the injection site. Other common findings were skin discoloration, tenderness, and swelling; indurated livedoid plaques; and erythematous, ecchymotic and purpuric patches. The histopathological results of the case with Nicolau syndrome showed formation of ulceration and fat necrosis.Conclusion:Although Nicolau syndrome is an uncommon cutaneous adverse reaction, clinicians should be aware of this complication to prevent or minimize the severity of Nicolau syndrome.

简介：ObjectiveTostudyclinicalcharacteristicsofendolymphaticsactumor(ELST)anditsdiagnosisandtreatment.MethodsELSTwasdiagnosedin6casesbasedonsurgicalandhistologicalfindings.Thesecaseswerereviewedfortheirclinicalmanifestations,differentialdiagnosisandsurgicaltreatmenttechniques.ResultsTherewere1maleand5femalesinthisgroup,agedfrom28to59years(meanage=38.7years).Thetumorwasinleftearin4casesandinrightearintheother2cases.Diseasecoursesrangedfrom5to30years(meanduration=12.6years).Clinicalpresentationsincludedsensorineuralhearingloss(n=4),otorrheaandtinnitus(n=2),tinnitusandfacialspasm(n=1),otorrheawithfacialparalysis(n=1),andhearinglosswithtinnitus(n=2).NoneofthecaseswasdiagnosedasELSTpreoperatively.Twocasesweremisdiagnosedasglomusjugularetumor,2aschronicsuppurativeotitismedia,1assweatglandadenomaonbiopsyand1astemporalbonetumor.Tumorsweresurgicallyresectedinall6casesviathemastoidectomy(n=2)orcombinedoto-cervicalorcranio-oto-cervicalapproaches(n=4).Postoperativecerebrospinalfluidotorrheaoccurredin1case.Thetumorswereconfirmedonhistologicalexaminationtobealow-gradeadenocarcinoma.Allpatientshavesurvivedatthetimeofthispaper.ConclusionELSTisrareandcommonlymisdiagnosedandinadequatelytreated.Itsprognosisisrelativelyfavorablebecauseofitsslowgrowthrate.

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简介：Objective:Toobserveclinicaleffectofcombinedmixtureoftraditionalchinesesandwesternmedicine"PIANTANNING"intreatmentofischemiccerebrovasculardiseasesandchangeofbrainatlas.Methods:In6660patientswithischemiccerebrovasculardisease,thecurativeeffectof"PIANTANNING"andbrainatlaswereobservedandother30caseswerecomparedwithtreatmentofDextran.Results:Aftertreatment,patient'sclinicalsymptomsandrecovereddegreeofmyodynamiaandbrainatlasimprovedsignificantly.Totaleffectiveratewas96.7%.Butthecontralgroupwas80.0%.Therewasasignificantdifferencebetweenthem(p＜0.05).Conclusion:The"PIANTANNING"producedagoodclinicalcurativeeffectintreatmentofischemiccerebrovasculardiseasesandthesideeffectwasonlyafew.Soitcanbeappliedverywellinpatientswithischemiccerebrovasculardisease.Theappliedvalueofbrainatlaswasverygood.

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简介：Hygienesupervisionandcontroloverthemanufacturingplantssupplyingflightfood,thecabinservicedepartmentofAirChinaandfoodsupplyonaircrafthavebeenimplementedbytheBeijingAirportHealthandQuarantineBureau.Fifty—threespecificproblemsofvariouskindshavebeendetectedina12monthexperimentalperiodfromAugustof1991toAugustof1992.Thispaperhasdiscussedthelegalbasis,supervisionunit,workproceduresandworkcontentofsupervisionandcontroloveraircatering.

简介：Alongwiththeincreasinglyurgentmanagementneedsofintranetterminalsinhospital,andlargescaleddeploymentofterminalmanagementsystem,terminalaccesscontrolhasbecomeoneofthestandardfunctionsofterminalmanagement.Thispapermainlyaimsatsomesimpleresearchforthesystemconstructionofhospitalintranetterminalaccesscontrol.

简介：Theadditionofenvironmentalsensing,intheformofdepthsensing,tothecontrolofpowered,lower-limbprostheseswasproposed.Currently,thepredictionofwhichlocomotionmode-basedcontrollertousehasbeenperformedusingEMGfromtheresiduallimbandkineticsandkinematicsfromtheprosthesis.However,duetothevariabilityofEMG,kinetics,andkinematicsacrosstrials,days,andsubjects,itisdifficulttoproducerobustpredictionswithlowerrorrates.Thisworkfocusedonthede-

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简介：Chlamydia,ahumanpathogenthatmediateschronic,persistentandrepeatedinfectionprogress,iscapableofinflictingpermanentscarformation.Atypicalreticularbodyisfoundinpersistentinfections.ChlamydialHsp60,interferon(IFN)-gammaandothercytokinesfunctioninthecourseofrepeatedinfection.Immunopathologicalfactorsmediatechronicinfection.