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What is Which of the following enzyme is absent in Wolman's disease? and explain in detail?
Ans. D. Acid lipase (Ref. H-17th/ pg. 2454; Table 355-1)WOLMAN'S 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 Therapy)Stored MaterialClinical Types (Onset)Inheri-tanceNeuro- logic featuresLiver Spleen Enlarge- mentSkeletal DysplasiaEyeHemato logicUnique featuresWolman diseaseAcid lysoso- mal Lipase (BMT)Choles- terol esters, triglyce ridesInfantileARMild mental retarda- tion+++NoneNoneNoneAdrenal 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 (135)Acid -gluco sidase (ET)Glyco- genInfantile late onsetARNeuro- muscu- lar+-NoneNoneNoneMyocar- diopathy