<prompt> A child presents with hepatosplenomegaly and pancytopenia. Bone marrow shows crumbled tissue paper appearance. It is due to accumulation of:GlucocerebrosideSphingomyelinGangliosideGalactocerebroside  <response>: Ans. A. GlucocerebrosideRef: Nelson 20e, Chapter 86.4 LipidosesSphingolipidosesEnzyme deficiencySphingolipid accumulatedFarber diseaseCeramidaseCeramideFabry diseasea-GalactosidaseGlobotriaosyl CeramideGM1 gangliosidosisb-GalactosidaseGM1 GangliosideGM2 gangliosidosisTay-Sachs diseaseb-Hexosaminidases AGM2 GangliosideSandhoff diseaseb-Hexosaminidases A and BGM2 GangliosideGaucher diseaseGlucocerebrosidase b GlucosidaseGlucosylceramideNiemann-Pick DiseaseSphingomyelinaseSphingomyelinMetachromatic leukodystrophyArylsulfatase A Sphingolipid activator Protein SAP-1Sulfogalactosyl CeramideKrabbe diseaseb-Galactosidaseb-GalactocerebrosidaseGalactosyl CeramideGauchers DiseaseMost common lysosomal storage disorderGlucocerebrosidase defectLysosomes filled with glucocerebrosideNo cherry red spot in the maculaNo mental deterioration Type IHematological features-Pancytopenia, bleeding manifestationHepatosplenomegalyBone pain and pathological fractures of long bonesX-ray Femur-Erlenmeyer flask deformityBone marrow biopsy-Gaucher cell with Wrinkled paper appearancecrumbled tissue paper appearance. 