Pompe glycogen storage and hydroxychloroquine

Discussion in 'Hydroxychloroquine' started by gooold, 15-Mar-2020.

  1. yaqwer Well-Known Member

    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 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. Pompe disease, also known as type II glycogenosis, is a progressive autosomal recessive glycogen storage disease caused by deficiency of lysosomal acid alpha glucosidase GAA primarily in skeletal and cardiac muscle with age of onset ranging from infancy through adulthood. Glycogen storage disease IIIa hypotonia, cardiomegaly muscle weakness, elevated creatine kinase CK, non-ketotic hypoglycemia* with more dramatic liver involvement than usually seen in Pompe disease. Autosomal recessive. Glycogen storage disease type IV hypotonia, cardiomegaly, muscle weakness, elevated CK.

    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

    Dietary Management of the Ketogenic Glycogen Storage., Pompe Disease Literature Review and Case Series

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  5. 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.

    • HEX4 - Clinical Glucotetrasaccharides, Urine.
    • Pompe Disease Glycogen storage disease, type II; Acid..
    • Glycogen storage disease type II - Wikipedia.

    A glycogen storage disease GSD, also glycogenosis and dextrinosis is a metabolic disorder caused by enzyme deficiencies affecting either glycogen synthesis, glycogen breakdown or glycolysis glucose breakdown, typically within muscles and/or liver cells. GSD has two classes of cause genetic and acquired. 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. Pompe disease glycogen storage disease type II a or acid maltase deficiency is a lysosomal disorder in which acid α-glucosidase GAA deficiencies lead to intralysosomal accumulations of glycogen in all tissues, most notably in skeletal muscles. Pompe disease was first described by Dr. J. C. Pompe in a 7-month-old girl with cardiomyopathy.

     
  6. builder Guest

    Plaquenil (hydroxychloroquine) belongs to a group of medicines called quinolines. Alcohol and RA" Rheumatoid Arthritis Community - Support Group Can i Drink Alcohol While Taking Plaquenil Side Effects of Plaquenil Hydroxychloroquine, Warnings, Uses
     
  7. Andy.B Moderator

    When ‘Collateral Damage’ Strikes the Cornea Vortex keratopathy, or corneal verticillata, is a common side effect of a number of systemic medications—e.g. amiodarone, aminoquinolones, indomethacin, tamoxifen, atovaquone and tilorone—and results from the intralysosomal accumulation of lipids. This condition presents with golden-brown deposits in a whorl-like pattern normally seen.

    Early Plaquenil Toxicity Detected without Bull’s Eye.