<prompt> Glucoseramide with enlarged liver and spleen is found in -GaucherTay sachsFabryNiemanpick  <response>: Ans. is Gaucher Gauchers o Autosomal recessive, o Deficiency of glucocerebrosidase. o Accumulation of glucosyl ceramides in cells of RES. o Spleen enlarged with So hypersplenism leukopenia thrombocytopenia, o Liver enlarged, o Expansion of bone marrow. Tay sachs o Autosomal recessive, o Deficiency of hexosaminidase, o Accumulation of ganglioside GM2 in nervous system, o CF Apathy Hypotomia Visual defect Develop mental defect o Fundus Cherry red spot hepatosklema o Fabry o X-link recessive. o Dificiency of ceramide trihexosidase. o Accumulation of trihexose in CNS, skin, kidney. Blood vessel, heart, sweat, comeabone marrow, o Recurrent episodes of burning pain and paresthesia, o Skin lesion, o Corneal opacity cataract, o Necrosis of femoral head, o Hyperkalemia. o Renal failure, hepatospleno megaly o. 