Adult hereditary fructose intolerance-中国期刊网

摘要 Hereditaryfructoseintolerance(HFI)isanunderrecognized,preventablelife-threateningcondition.ItisanautosomalrecessivedisorderwithsubnormalactivityofaldolaseBintheliver,kidneyandsmallbowel.Symptomsarepresentonlyaftertheingestionoffructose,whichleadstobriskhypoglycemia,andanindividualwithcontinuedingestionwillexhibitvomiting,abdominalpain,failuretothrive,andrenalandliverfailure.AdiagnosisofHFIwasmadeina50-year-oldwomanonthebasisofmedicalhistory,responsetofructoseintolerancetest,demonstrationofaldolaseBactivityreductioninduodenalbiopsy,andmolecularanalysisofleukocyteDNAbyPCRshowedhomozygosityfortwodosesofmutantgene.HFImayremainundiagnoseduntiladultlifeandmayleadtodisastrouscomplicationsfollowinginadvertentfructoseorsorbitolinfusion.SeverallethalepisodesofHFIfollowingsorbitolandfructoseinfusionhavebeenreported.Thediagnosiscanonlybesuspectedbytakingacarefuldietaryhistory,andthiscanpresentseriouscomplications.

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Adult hereditary fructose intolerance

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Adult hereditary fructose intolerance

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