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. Dejar de tomar plaquenil Plaquenil 200mg tablets 100 Glycogen storage diseases GSDs are a heterogeneous group of inherited disorders caused by inborn errors of glycogen metabolism. These disorders most commonly affect the muscle and liver where glycogen is the most abundant. 1 For GSD I, secondary metabolic disturbances include fasting hyperlactatemia, hyperuricemia, and hyperlipidemia. Glycogen storage disease III is caused by reduced activity of the debrancher enzyme, GSD VI by phosphorylase, and GSD IX by phosphorylase kinase. Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. 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. 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 HEX4 - Clinical Glucotetrasaccharides, Urine, Glycogen storage disease type II - Wikipedia Bad side effects of plaquenilChloroquine functional groups Glycogen Storage Disorders GSDs are a group of inherited disorders. They are caused by enzymes that do not work properly. Certain enzymes in the body are involved in the buildup and break down of a sugar called glycogen. NEWBORN SCREENING. Pompe Disease Symtoms, Causes, Treatments. Perioperative management of children with glycogen storage.. Glycogen storage diseases GSDs are a group of inherited genetic disorders that cause glycogen to be improperly stored in the body. Children with glycogen storage diseases have a buildup of abnormal amounts or types of glycogen in their tissues. 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. 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.