G6PD deficiency (Glucose-6-Phosphate Dehydrogenase deficiency) G-6-PD缺乏症(蠶豆症)(英文) · Definition · Symptoms and signs · Prevention and Precaution.衛教單張HealthEducation首頁衛教單張G6PDdeficiency(Glucose-6-PhosphateDehydrogenasedeficiency)G-6-PD缺乏症(蠶豆症)(英文)衛教資訊衛教單張衛教影片衛教海報衛教諮詢正確用藥教育中心衛教講座衛教講座報名爸媽教室哺乳聚會孕產課程健康城市中醫講座正確用藥G6PDdeficiency(Glucose-6-PhosphateDehydrogenasedeficiency)G-6-PD缺乏症(蠶豆症)(英文)文章分享首頁分享至FaceBook分享至LineQrcode列印A-A+G6PDdeficiency(Glucose-6-PhosphateDehydrogenasedeficiency)G-6-PD缺乏症(蠶豆症)(英文)2021/1/27DefinitionGlucose-6-PhosphateDehydrogenase(G6PD)deficiencyisthemostcommonhumanenzymedeficiencyintheworld;itaffectsanestimated400millionpeople.G6PDdeficiencyisalsoknownas"favism,"sinceG6PDdeficientindividualsarealsosometimesallergictofavabeans.G6PDdeficiencyisanallelicabnormalitywhichisinheritedinanX-linkedrecessivefashion.WhensomeonehasG6PDdeficiency,complicationscanarise;hemolyticanemiaandprolongedneonataljaundicearethetwomajorpathologiesassociatedwithG6PDdeficiency.Bothoftheseconditionsaredirectlyrelatedtotheinabilityofspecificcelltypestoregeneratereducednicotinamideadeninedinucleotidephosphate(NADPH);thisreactionisnormallycatalyzedbytheG6PDenzyme.AconsequenceofthisisthatG6PDdeficientindividualsareresistanttothemalariacausingparasite.SymptomsandsignsInG6PDdeficientindividuals,anemiaisusuallycausedbycertainoxidativedrugs,infections,orfavabeans.Whenanyoneoftheseagents,ortheirmetabolites,entersaG6PDdeficientredbloodcell,hemoglobinbecomesdenatured,thusdestroyingitsfunctionastheprincipaloxygencarryingmolecule.AchildwithG6PDdeficiencywhoisexposedtoamedicationorinfectionthattriggersthedestructionofRBCsmayhavenosymptomsatall.Inmoreseriouscases,achildmayexhibitsymptomsofanemia(alsoknownasahemolyticcrisis),including:paleness(indarker-skinnedchildrenpalenessissometimesbestseeninthemouth,especiallyonthelipsortongue),extremetiredness,rapidheartbeat,rapidbreathingorshortnessofbreath,jaundice,oryellowingoftheskinandeyes,particularlyinnewborns,anenlargedspleen,dark,andtea-coloredurine.