<prompt> Enzyme replacement therapy is most commonly used forGauchers diseaseFabrys diseaseNiemenn picks diseasePompe disease  <response>: Gaucher disease is a multisystemuc lipidosis characterized by hematological abnormalities,organomegaly and,skeletal involvement.It has 3 clinical subtypes . It occurs due to the deficient activity of acid beta glucosidase . Thus glucosylceramide accumulate in reticule end I the loam cells. Treatment is mainly enzyme replacement therapy with recombinant acid beta glucosidase.Most extra skeletal symptoms are reversed by 60 IUkg enzyme iv infusion every other week. Monthly maintenance improves skeletal manifestations. Reference: Nelsons TB of pediatrics 19th edit,pg 487 