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1、ClinicalCorrelationofAutonomicNervousSystemPDFgeneratedusingtheopensourcemwlibtoolkit.Seehttp://code.pediapress.com/formoreinformation.PDFgeneratedat:Tue,19Feb201315:10:35UTCContentsArticlesHirschsprung'sdisease1Familialdysautonomia8Raynaud'sphenomenon
2、14Horner'ssyndrome21Botulism25Lambert–Eatonmyasthenicsyndrome32ReferencesArticleSourcesandContributors37ImageSources,LicensesandContributors38ArticleLicensesLicense39Hirschsprung'sdisease1Hirschsprung'sdiseaseHirschsprung'sdiseaseClassificationandexter
3、nalresourcesHistopathologyofHirschsprungdisease.Enzymehistochemistryshowingaberrantacetylcholineesterase(ACHE)-positivefibres(brown)inthelaminapropriamucosae.ICD-10Q43.1[1]ICD-9751.3[2]OMIM142623[3]DiseasesDB5901[4]MedlinePlus001140[5]eMedicinemed/1016
4、[6]MeSHD006627[7]Hirschsprung'sdisease(HD)isadisorderoftheabdomenthatoccurswhenallorpartofthelargeintestinehasnonervesandthereforecannotfunction.Duringnormalfetaldevelopment,cellsfromtheneuralcrestmigrateintothelargeintestine(colon)toformanetworkofnerv
5、escalledAuerbach'splexus.InHirschprung'sdisease,themigrationisnotcompleteandpartofthecolonlacksthesenervebodiesthatregulatetheactivityofthecolon.Theaffectedsegmentofthecoloncannotcontractandthereforepassstoolthroughthecolon,creatinganobstruction.[8]Inm
6、ostaffectedpeople,thedisorderaffectsthepartofthecolonthatisnearesttheanus.Inrarecases,thelackofnervebodiesinvolvesmoreofthecolon.Infivepercentofcases,theentirecolonisaffected.Hirschsprung'sdiseaseisalsosometimescalledcongenitalaganglionicmegacolon.Hirs
7、chsprung'sdiseaseoccursinaboutonein5,000children(U.S.andJapan).Itisusuallydiagnosedinchildren,andaffectsboysmoreoftenthangirls.Hirschsprung'sdisease2HistoryanddescriptionThefirstreportofHirschsprungdiseasedatesbackto1691,[9]however,thediseaseisnamedaft
8、erHaraldHirschsprung,theDanishphysicianwhofirstdescribedtwoinfantswhodiedofthisdisorderin1888.[10][11]Hirschsprung’sdiseaseisacongenitaldisorderofthecoloninwhichcertainnervecells,knownasganglioncells,areabsent,causingchronicconstipation
