主动脉疾病-英文

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1、DiseasesoftheAortaFredWu,M.D.April19,2006CategoriesofaorticdiseaseAneurysmPseudoaneurysmDissectionPenetratingatheroscleroticulcerIntramuralhematomaAtheroscleroticdiseaseCoarctationThoracicaorticaneurysmsAortopathyduetoheritablediseases(>20%ofaneurysms)Marfansyndro

2、meEhlers-DanlossyndromeFamilialAnnuloaorticectasiaTurnersyndromeNoonansyndromeOsteogenesisimperfectaBicuspidaorticvalveCoarctationoftheaortaMetabolicdisorders(eg,homocystinuria,familialhypercholesterolemia)Aorticarteritis(Takayasu’s,giant-cell)TraumaPregnancySyphi

3、lis/infectiousCrackcocaineuseIatrogeniccauses(eg,cardiaccatheterization)AtherosclerosisHypertensionMarfanSyndromeAutosomaldominantinheritanceMissensemutationinthefibrillingeneonchromosome15Prevalenceis1in10,000(25%sporadic)Leadingcauseofprematuredeathisaorticroota

4、neurysmleadingtodissection(cysticmedialdegeneration)MarfanSyndromeGhentCriteriaIndexcaseMajorcriteriain2differentorgansystemsandinvolvementinathirdorgansystemFamilyhistoryMajorcriterioninfamily/geneticsand1majorcriterioninanorgansystemandinvolvementinasecondorgans

5、ystemMarfanSyndromeSkeletalMajor(atleast4items)PectuscarinatumPectusexcavatumrequiringsurgeryDecreaseduppertolowersegmentratioORincreasedarmspamtoheightratioWristandthumbsignsScoliosis>20degreesDecreasedelbowextensionPesplanusProtrusioacetabulaeMinorPectusexcavatu

6、m(moderate)JointhypermobilityHigharchedpalatewithcrowdingofteethFacialappearanceOcularMajorEctopialentisMinorAbnormallyflatcorneaIncreasedaxiallengthofglobeHypoplasticirisorciliarymuscleSkinandintegumentMinoronlyStriaenotassociatedwithweightchange,pregnancyorstres

7、sRecurrentorincisionalherniasPulmonaryMinoronlySpontaneouspneumothoraxApicalblebsDuraMajoronlyLumbosacralduralectasiaCardiovascularMajorDilatationoftheascendingaortainvolvingatleastthesinusesofValsalvaDissectionoftheascendingaortaMinorMitralvalveprolapsewithorwith

8、outMRMitralannularcalcificationbeforeage40Dilationordissectionofdescendingaortabeforeage50DilationofthePAwithoutcausebeforeage40Familial/geneticMajoronl