<prompt> Severe hypoglycemia, increased uric acid and renal failure are seen in?Carbohydrate metabolic disorderGlycogen storage disorderLipoprotein deficiency disorderProtein folding disorder  <response>: ANSWER: B Glycogen storage disorderREF: Nelson 18th ed table 87-1, Harper 27th ed chapter 33See APPENDIX 18 GLYCOGEN STORAGE DISORDERSVON GIERKES DISEASEPurine overproduction and hyperuricemia in von Gierkes disease glucose-6-phosphatase deficiency occurs secondary to enhanced generation of the PRPP precursor ribose 5- phosphate. An associated lactic acidosis elevates the renal threshold for urate, elevating total body urates.Von Gierkes Disease Features:Severe fasting hypoglycemiaFatty liver with hepato and renomegalyProgressive renal diseaseGrowth retardation and delayed pubertyHyperlacticacidemia, hyperlipidemia, hyperuricemia APPENDIX - 18Glycogen Storage Disorders:NumberEnzymedeficiencyEponymHypoglycemiaHepatomegalyHyperlipidemiaMusclesymptomsDevelopmentprognosisOthersymptomsGSD type Iglucose-6-phosphatasevonGierkesdiseaseYesYesYesNoneGrowthfailureLacticacidosis,hyperuricemiaGSD type IIadd maltasePompesdiseaseNoYesNoMuscleweaknessDeath by age 2 years infantile variantheart failureGSD type IIIglycogendebrancherCoris disease or Forbes diseaseYesYesYesMyopathy GSD type IVglycogenbranchingenzymeAndersendiseaseNoYes,alsocirrhosisNoNoneFailure to thrive, death at age 5 years GSD type Vmuscle glycogen phosphorylaseMcArdlediseaseNoNoNoExercise-inducedcramps,Rhabdo-myolysis Renal failure by myoglobinuriaGSD type VIliver glycogen phosphorylaseHersdiseaseYesYesNoNone GSD type VIImusclephospbo-fructokinaseTaruisdiseaseNoNoNoExercise- induced muscle cramps and weaknessgrowthretardationHaemolyticanaemiaGSD type DCphosphorylase kinase, PHKA 2 NoNoYesNoneDelayedmotordevelopment,Growthretardation GSD type XIglucose transporter, GLUT 2Fanconi-BickelsyndromeYesYesNoNone GSD type XIIAldolase ARed cell aldolase deficiency???Exerciseintolerance,cramps GSD type XIIIb-enolase ???Exerciseintolerance,crampsIncreasing intensity of myalgias over decadesSerum CK: Episodic elevations; Reduced with restGSD type 0glycogensynthase YesNoNoOccasionalmusclecramping 