CHAPTERHEMOLYTICANEMIAS:REDBLOODCELLMEMBRANEANDMETABOLICDEFECTS
REVIEWQUESTIONS
1.Amanrecentlydiagnosedwithglucose-6-phosphatedehydrogenase(G6PD)deficiencyafteranepisodeofhemolysisaftertakingchloroquine氯喹malaria疟疾prophylaxis预防beforevisitingBenin贝宁（位于非洲西部）.Hehasmanyquestions,includingaboutstartingafamily.Attheendofthevisit,youprovideseveralsuggestions.Theseincludeallofthefollowingexcept
A.alwaysinformhealthcareprovidersthatheisG6PDdeficient,particularlybeforetheyprescribeanymedication.
B.seekmedicalattentionifheisfeelingtired,shortofbreath,andexperiencingpalpitations心悸andifdark-coloredurineisobserved.
C.heshouldavoideatingfavabeans蚕豆andfavaplant–derivedfood.
D.ifhiswifedoesnothaveG6PDdeficiency,allsonswillbeunaffected,andalldaughterswillbecarriers.
E.ifhiswifeisaG6PDcarrier,oneoftwodaughterswillbeG6PDdeficient,oneoutoftwodaughterswillbeG6PDcarriers,andhissonswillnotbeaffected.
Answer:EGlucose-6-phosphatedehydrogenase(G6PD)deficiencyisinheritedinanX-linkedmanner.Thus,ifhiswifedoesnothaveG6PDdeficiency,allsonswillbeunaffected,andalldaughterswillbecarriers(answerD).However,ifhiswifeisaG6PDcarrier,oneoftwodaughterswillbeG6PDdeficient,oneoftwodaughterswillbeG6PDcarriers,onesonoftwowillbeG6PDdeficient,andonesonoftwowillbeunaffected.WiththediagnosisofG6PDdeficiency,heshouldalwaystellhishealthcareproviders,especiallywhentheyareprescribingmedications,andheshouldavoidfavabeansandallfava-derivedproducts.Finally,heshouldseekmedicalattentionwhenexperiencingthesymptomsofahemolyticreaction,tiredness,shortnessofbreath,palpitations,anddark-coloredurine.
2.Youhavejustdiagnoseda23-year-oldpatientwithhereditaryspherocytosis球形红细胞贫血症.Whichofthefollowingareappropriateactions?
A.Obtainultrasonographyofthespleenandgallbladder.
B.Counselthepatienttoavoidoxidant-inducingfoodsandmedications.
C.Determineifotherfamilymemberscouldbeaffectedandshouldbeevaluatedforthepresenceofhereditaryspherocytosis.
D.Counselregardingpossibleoccurrenceofhemolyticandaplasticcrises再障危象.
E.A,B,andC
F.A,C,andD
G.Alloftheabove
Answer:FAssessingspleensizeandthebiliarytractforthepresenceofcholelithiasis胆石症(answerA),determiningifotherfamilymemberscouldbeaffectedafterdiagnosingapatientwithhereditaryspherocytosis(answerC),andcounselingregardinghemolyticandaplasticcrises(answerD)areallappropriateactionsafterdiagnosingapatientwithhereditaryspherocytosis.Avoidanceofoxidant-inducingfoodandmedications(answerB),whichisappropriateinpatientswithmetabolicdiseaseoftheerythrocyte,especiallyglucose-6-phosphatedehydrogenasedeficiency,isnotnecessaryinhereditaryspherocytosis.
3.Youareaskedtoseeajaundiced62-year-oldmanwithcirrhoticliverdisease肝硬化疾病白癫疯医院白癜风的危害
