Pompe glycogen storage and hydroxychloroquine

Discussion in 'Plaquenil' started by SeeJey, 26-Feb-2020.

  1. Dmitri007 Moderator

    Pompe glycogen storage and hydroxychloroquine

    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.

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    Pompe Disease, a glycogen storage most notably in skeletal muscle disease type II is an autosomal recessive disorder caused by deficiency of acid α glucosidase the lysosomal enzyme. It occurs due to mutation in acid α-glucosidase gene which is located on chromosome 17q25.3. Hydroxychloroquine is used to treat lupus erythematosus and rheumatoid arthritis. It’s also used to prevent and treat malaria. How it works. Hydroxychloroquine is an antimalarial drug. 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

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  6. Glycogen storage disease GSD is a rare condition that changes the way the body uses and stores glycogen, a form of sugar or glucose. Glycogen is a main source of energy for the body. Glycogen is stored in the liver. When the body needs more energy, certain proteins called enzymes break down glycogen into glucose.

    • Glycogen Storage Disease Johns Hopkins Medicine.
    • Pompe Disease Symtoms, Causes, Treatments.
    • Glycogen storage disease type 2 Genetic and Rare..

    Synonyms glycogen storage disease type II; acid maltase deficiency Pompe's disease is a glycogen storage disorder. Deficiency of the lysosomal enzyme alpha-1,4-glucosidase acid maltase leads to the accumulation of glycogen in many tissues 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 –. 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.

  7. Learnet New Member

    Suppression: 400 mg (310 mg base) orally on the same day every week Comments: -Suppressive therapy should begin 2 weeks prior to exposure; however, failing this, an initial dose of 800 mg (620 mg base) may be taken in 2 divided doses (6 hours apart). Side Effects of Plaquenil Hydroxychloroquine, Warnings, Uses Hydroxychloroquine 200 mg Tablets Generic Plaquenil Hydroxychloroquine 200mg Brands Prices, Side-effects, Uses.
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  9. Notebookhp Moderator

    Plaquenil Oral Uses, Side Effects, Interactions, Pictures. Find patient medical information for Plaquenil Oral on WebMD including its uses, side effects and safety, interactions, pictures, warnings and user ratings. If you miss a dose, take it as soon.

    Hydroxychloroquine Oral Route Proper Use - Mayo Clinic