Orotic aciduria refers to an excessive excretion of orotic acid in the urine. Orotic acid is an intermediate product produced during the ...SpotlightPracticePearlsWomen'sHealthZosterClinicalRoleSeeAll>HospitalOncologyPharmacyTechnicianRetailSpecialtyPharmacyStudentClinicalSeeAll>AllergyBrainHealthBreastCancerCardiovascularHealthCoronavirusCoughandColdDiabetesFluHematologyImmunizationInfectiousDiseaseNeurologyPainManagementVitaminsandSupplementsAboutUsAdvertiseContactUsPrivacyPolicyTerms&ConditionsDoNotSellMyInformation©2021MJHLifeSciences™andPharmacyTimes.Allrightsreserved.WhatIsHereditaryOroticAciduria?October12,2015TheFDArecentlyapprovedanewtreatmentforarareconditioncalledhereditaryoroticaciduria.TheFDArecentlyapproveduridinetriacetate(Xuriden),atreatmentforarareconditioncalledhereditaryoroticaciduria.Oroticaciduriareferstoanexcessiveexcretionoforoticacidintheurine.Oroticacidisanintermediateproductproducedduringthepyrimidinesynthesispathway.Theinabilitytoconvertoroticacidresultsinthebodybeingunabletonormallysynthesizeuridine,anecessarycomponentofRNA,whichultimatelyleadstoafailuretothrive.1Hereditaryoroticaciduriaisarareautosomalrecessivedisorder.Aheterozygouspatientmaynothavesymptoms,buttherehavebeenreportsofheterozygouspatientsexperiencingsymptoms.2Thesymptomsofthediseaseincludementalretardation,growthretardation,megaloblasticanemia,immunodeficiency,andhighlevelsoforoticacidinthepatient’surine,approximately500to1000timesthenormalamount.MegaloblasticanemiadoesnotrespondandcannotbecuredbyadministrationofvitaminB12orfolicacid.2,3UridinetreatmentiseffectivebecauseitcaneasilybeconvertedintoausableformandthenusedbythebodyforRNAformation.Xuridenisavailableinsingle-usepacketsthatcontainoralgranulesandcanbeadministeredwithmilk,formula,orsoftfoodsuchasapplesauce,pudding,oryogurt.Therecommendeddoseis60mg/kgdaily,whichcanbetitratedto120mg/kgdailyifneeded(maximumof8g/day).Reasonstoincreasethedoseinclude:Oroticacidlevelsintheurineremainabovenormalorincreaseabovetheusualorexpec