Hydroxychloroquine is available as the brand-name drug Plaquenil. Generic drugs usually cost less than the brand-name version. Hydroxychloroquine may be used as part of a combination therapy. Plaquenil cost in india Plaquenil y alcohol How to start plaquenil Lowered dose of plaquenil Pompe disease is an inherited disorder caused by the buildup of a complex sugar called glycogen in the body's cells. The accumulation of glycogen in certain organs and tissues, especially muscles, impairs their ability to function normally. Researchers have described three types of Pompe disease, which differ in severity and the age at which they appear. What is glycogen and glycogen storage disease GSD? The body’s cells need a steady supply of fuel in order to function the right way. This fuel is a simple sugar called glucose. Glucose comes from breaking down the food we eat. The body uses as much glucose as it needs to function and stores the rest to use later. Chloroquine and hydroxychloroquine, commonly used for rheumatologic conditions, are rare yet very important causes of toxic myopathy with vacuolar changes on muscle biopsy. Pompe disease, a glycogenosis due to deficiency of lysosomal acid α-glucosidase GAA, may sometimes show similar yet less marked changes on muscle histopathology. Hydroxychloroquine is used to treat lupus erythematosus and rheumatoid arthritis. It isn’t fully understood how this drug works to treat lupus erythematosus or rheumatoid arthritis. That means you may need to take it with other drugs. It treats malaria by killing the parasites that cause the disease. Pompe glycogen storage and hydroxychloroquine Role of Autophagy in Glycogen Breakdown and Its Relevance., Glycogen Storage Disease GSD - Cleveland Clinic Malaria chloroquine proguanilHydroxychloroquine also called Pearls. Chloroquine and hydroxychloroquine, commonly used for rheumatologic conditions, are rare yet very important causes of toxic myopathy with vacuolar changes on muscle biopsy. Pompe disease, a glycogenosis due to deficiency of lysosomal acid α-glucosidase GAA, may sometimes show similar yet less marked changes on muscle histopathology. An. Pearls & Oy-sters Hydroxychloroquine-induced toxic.. Pearls & Oy-sters Hydroxychloroquine-induced toxic myopathy mimics.. Glycogen storage disease type II - Wikipedia. The best characterized of these is the lysosomal storage disorder, Pompe disease, also known as glycogen storage disease type II. Pompe disease is caused by a mutation in the gene encoding acid a-glucosidase GAA, an enzyme that localizes to the lysosome, and hydrolyzes glycogen to glucose –. Pompe disease happens when your body can't make a protein that breaks down a complex sugar, called glycogen, for energy. Too much sugar builds up and damages your muscles and organs. Pompe disease causes muscle weakness and trouble breathing. It mostly affects the liver, heart, and muscles. HEX4 Pompe disease, also known as glycogen storage disease type II, is an autosomal recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase GAA. This leads to an accumulation of glycogen in the lysosome causing swelling, cell damage, and progressive organ dysfunction.