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1、上面是英文，下面是中文History:Twenty-year-oldman.Shownbelowarefat-satT1WenhancedGREimages.Herenowarefat-satT2WandDualechoFSPGRimages.（5-7）Findings:ThereismarkedlydecreasedT2signalintensitywithintheliver.Theliverisnormalinsizeandotherwisenormalinappearance.Thespleenissurgicallyabsent.Thepancreasandkidneysareno
2、rmalinsizeandshownormalsignalcharacteristics.Diagnosis:Secondaryhemochromatosis(hemosiderosis).DiscussionThispatienthashadhemolyticanemia,thoughttobehereditary,sincebirth.Etiologyisuncertainbutthereisafamilyhistory.Hehashadmultiplehospitalizationsandbloodtransfusionsandhadsplenectomyatage5.Serumiro
3、nstudiesrevealedahemoglobinlevelof10.4gm/dL,ahematocritof34%,amarkedlyelevatedferritinlevelof3055ng/mL,anelevatedironlevelof216mcg/mL,adecreasedTIBCof216mcg/mL,andanelevatedironsaturationlevelof95%.MRIwasorderedtoevaluateforironoverloadintheliver.Onnon-enhancedCT,aliverwithincreasedironcontentshows
4、diffuselyincreasedattenuation,usuallygreaterthan75HU.Thecontrastbetweenparenchymaandvasculatureisincreased.Thesefindingscanalsobepresentinglycogenstoragedisease,patientsonamiodaronetherapy,goldtherapy,andinWilsondisease.MRismoresensitiveandspecificfordetectionofincreasedhepaticirondeposition.MRimag
5、ingofaliverwithincreasedironcontentshowsacharacteristicT2signallossduetothemagneticsusceptibilityartifactofironwhichcausesspindephasing.T2-weightedgradientechoimagesarethemostsensitive.OnMR,increasedironaccumulationinthelivercanbediagnosediftheliverdemonstratessignalintensityequaltoorlowerthanthato
6、fskeletalmuscle,suchastheparaspinalmuscles,oneitherT2-weightedgradientecho,orT2-weightedspinechoimages.Primaryhemochromatosisisanautosomalrecessivediseasewhichresultsinabnormallyincreasedironabsorptionfromthegut,andsubsequentdepositionintoorgansincludingtheliver,heart,pancreas,pituitary,joints,ands
7、kin.ThiscanresultinorgandysfunctionincludinglivercirrhosisanditscomplicationsincludingHCC,skinpigmentation,andtype1diabeteswhichgenerallyoccurlateinthedisease.Mostpatientswithprimaryhemochromatosisdonothave
