<prompt> Deficiency of Acid lipase:-Fabry diseaseGaucher diseaseFarber diseaseWolman disease  <response>: Deficiency Disease Group Acid maltase Pompes disease Glycogen storage disease Acid lipase Wolman disease Lysosomal storage disease Wolman disease Not a sphingolipidosis. Cholesterol ester storage disease Lysosomal acid lipase deficiency Triglyceride CF Vomiting Watery green diarrhea Hepatosplenomegaly Calcification of adrenals characteristic feature Rx- Enzyme replacement therapy Gauchers disease It is the most common lysosomal storage disorder. It is characterized by the following clinical features: Painless hepatomegaly and splenomegaly Liver cirrhosis Hypersplenism and pancytopenia Bleeding tendencies Bone pain Osteoporosis, pathological fractures Developmental delay, hypeonia, strabismus, supranuclear gaze palsy. Treatment : Enzyme replacement therapy is available in the form of Human recombinant acid b-glucosidaseImiglucerase Velaglucerase alfa Taliglucerase alfa Note:Neurological involvement is not seen in type I Gauchers disease. 