<prompt> Which of the following enzyme is absent in Wolmans disease?Cholestrol ester hydrolaseAcid hydrolaseAcyl-CoA carnitineAcid lipase  <response>: Ans. D. Acid lipase Ref. H-17th pg. 2454; Table 355-1WOLMANS DISEASE It is a lysosomal storage disorder, that occurs due to acid lipase deficiency. Features: increased LDL levels, hypercholesterolemia, atherosclerosis, adrenal calcification and death in infancy itself.Disorders of Neutral LipidsDisorderEnzyme Deficiency Specific TherapyStored MaterialClinical Types OnsetInheri-tanceNeuro- logic featuresLiver Spleen Enlarge- mentSkeletal DysplasiaEyeHemato logicUnique featuresWolman diseaseAcid lysoso- mal Lipase BMTCholes- terol esters, triglyce ridesInfantileARMild mental retarda- tionNoneNoneNoneAdrenal calcifi- cationCholes- teryl ester storage diseaseAcid lysoso- mal lipaseCholes- teryl estersChild- hoodARNoneHepato- megalyNoneNoneNoneCirrhosisFarber diseaseAcid cerami daseCeramideInfantile JuvenileAROccasio- nal men- tal retar- dation-NoneMacular degene- rationNoneArthropa thy, subcu- taneous nodulePompe disease 135Acid -gluco sidase ETGlyco- genInfantile late onsetARNeuro- muscu- lar-NoneNoneNoneMyocar- diopathy 