This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Glycogen Metabolism and Glycogen Storage, Subject name: Biochemistry
Severe hypoglycemia, increased uric acid and renal failure are seen in? 
Select and explain. 
A. Carbohydrate metabolic disorder, B. Glycogen storage disorder, C. Lipoprotein deficiency disorder, D. Protein folding disorder
B: Glycogen storage disorder
Answer: B: Glycogen storage disorder 
Explanation: ANSWER: (B) Glycogen storage disorderREF: Nelson 18th ed table 87-1, Harper 27th ed chapter 33See APPENDIX 18 "GLYCOGEN STORAGE DISORDERS"VON GIERKE'S DISEASEPurine overproduction and hyperuricemia in von Gierke's 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 Gierke's Disease Features:Severe fasting hypoglycemiaFatty liver with hepato and renomegalyProgressive renal diseaseGrowth retardation and delayed pubertyHyperlacticacidemia, hyperlipidemia, hyperuricemia APPENDIX - 18Glycogen Storage Disorders:NumberEnzymedeficiencyEponymHypoglycemiaHepatomegalyHyperlipidemiaMusclesymptomsDevelopment/prognosisOthersymptomsGSD type Iglucose-6-phosphatasevonGierke'sdiseaseYesYesYesNoneGrowthfailureLacticacidosis,hyperuricemiaGSD type IIadd maltasePompe'sdiseaseNoYesNoMuscleweaknessDeath by age ~2 years (infantile variant)heart 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 phosphorylaseHers'diseaseYesYesNoNone GSD type VIImusclephospbo-fructokinaseTarui'sdiseaseNoNoNoExercise- 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