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    University of Perpetual Help

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    Jayvee Novenario Casaljay
    G6PD deficiency was discovered through research on hemolytic anemia caused by antimalarial drugs, which found that some individuals lacked the enzyme glucose-6-phosphate dehydrogenase (G6PD) and had nonimmune hemolytic anemia in response to certain medications, foods, or infections. G6PD deficiency is an X-linked genetic disorder characterized by abnormally low levels of the G6PD enzyme, which protects red blood cells from oxidative damage, leading those with the disorder to develop hemolytic anemia from oxidative stresses like medications or fava beans. Clinical studies on prisoner volunteers in the past contributed to understanding why certain drugs cause hemolysis and the discovery

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    University of Perpetual Help

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    Jayvee Novenario Casaljay
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    G6PD deficiency was discovered through research on hemolytic anemia caused by antimalarial drugs, which found that some individuals lacked the enzyme glucose-6-phosphate dehydrogenase (G6PD) and had nonimmune hemolytic anemia in response to certain medications, foods, or infections. G6PD deficiency is an X-linked genetic disorder characterized by abnormally low levels of the G6PD enzyme, which protects red blood cells from oxidative damage, leading those with the disorder to develop hemolytic anemia from oxidative stresses like medications or fava beans. Clinical studies on prisoner volunteers in the past contributed to understanding why certain drugs cause hemolysis and the discovery

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    G6PD deficiency was discovered through research on hemolytic anemia caused by antimalarial drugs, which found that some individuals lacked the enzyme glucose-6-phosphate dehydrogenase (G6PD) and had nonimmune hemolytic anemia in response to certain medications, foods, or infections. G6PD deficiency is an X-linked genetic disorder characterized by abnormally low levels of the G6PD enzyme, which protects red blood cells from oxidative damage, leading those with the disorder to develop hemolytic anemia from oxidative stresses like medications or fava beans. Clinical studies on prisoner volunteers in the past contributed to understanding why certain drugs cause hemolysis and the discovery

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    56 views12 pages

    University of Perpetual Help

    Uploaded by

    Jayvee Novenario Casaljay
    G6PD deficiency was discovered through research on hemolytic anemia caused by antimalarial drugs, which found that some individuals lacked the enzyme glucose-6-phosphate dehydrogenase (G6PD) and had nonimmune hemolytic anemia in response to certain medications, foods, or infections. G6PD deficiency is an X-linked genetic disorder characterized by abnormally low levels of the G6PD enzyme, which protects red blood cells from oxidative damage, leading those with the disorder to develop hemolytic anemia from oxidative stresses like medications or fava beans. Clinical studies on prisoner volunteers in the past contributed to understanding why certain drugs cause hemolysis and the discovery

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
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    G6PD deficiency was discovered as an outgrowth of an investigation of hemolytic anemia
    occurring in some individuals treated for malaria with 6-methoxy-8-aminoquinoline drugs.
    Favism is a disorder characterized by hemolytic anemia in response to ingestion of fava beans.
    Favism as a diagnosis has been known since antiquity. One theory for the Pythagoreans'
    avoidance of beans is avoidance of favism, but more likely, this was a philosophical matter,
    such as the belief that beans and humans were created from the same material.
    It is worth noting that the clinical investigations needed to understand why 8-amino
    quinoline antimalarials cause hemolysis could not have been carried out without the
    participation of prisoner volunteers serving sentences in the Illinois State Penitentiary at Joliet.
    Today such studies could not be performed. If the idea that it was improper to study prisoner
    volunteers had been in vogue then as it is now, these men would have been deprived of
    making a contribution to society, and the discoveries that were to be made would have been
    held back for years. The discovery of G6PD deficiency is a clear example of how clinical
    studies carried out on prisoner volunteers can benefit society and save lives without harm to
    anyone.
    Glucose-6-phosphate dehydrogenase deficiency is an X-linked recessive hereditary
    disease characterised by abnormally low levels of glucose-6-phosphate dehydrogenase
    (abbreviated G6PD or G6PDH), a metabolic enzyme involved in the pentose phosphate
    pathway, especially important in red blood cell metabolism. Individuals with the disease may
    exhibit nonimmune hemolytic anemia in response to a number of causes, most commonly
    infection or exposure to certain medications or chemicals.
    Function of Blood
    ë Blood transport gases, nutrients, waste products, processed molecules and
    regulatory molecules
    ë Blood regulates pH, fluid, and ion balance\
    ë Blood is involved with temperature regulation and protects against foreign
    substances.
    ë Blood clotting prevents fluid and cell loss and is part of tissue repair
    Composition of Blood
    ë 55% plasma and 45% formed elements
    ë Plasma contains protein (7%), water (91%), and other solutes (2%)
    ë Formed elements contains platelets, white blood cells and red blood cells.
    Red Blood Cells (Erythrocytes)
    ë Red blood cells are disk-shape cells containing hemoglobin, giving its red color.
    Hemoglobin transports oxygen and carbon dioxide
    ë Worn out RBC are phagocytized by macrophages in the spleen or liver.
    White blood Cells (Leukocytes)
    ë WBC protects the body against microorganism and remove dead cells and debris
    ë Granulocytes are leukocytes that contain cytoplasmic granules. There are 3 types of
    it, neutrophils are small phagocytic cells, basophil promote inflammation and
    eosinophils reduces inflammation.
    ë Agranulocytes are leukocytes that have very small granules and there are 2 types:
    lymphocytes are involved in antibody production and other immune response and
    the monocytes that became macrophages that ingest microorganism and cellular
    debris.
    Platelet (Thrombocytes)
    ë Platelet are cell fragments involved with preventing blood loss
    Defected X
    chromosome

    Build up of
    Low G6PD glucose Diabetes

    Hemolytic
    Decreased
    Anemia
    protection to
    RBC against RBC was taken to
    oxidants Hemolysis of circulation and
    RBC destroyed to spleen

    oxidants Hemoglobin
    metabolized to
    bilirubin
    Infection or
    medication
    or diet jaundice
    s Diagnostic Test
    Liver enzymes (to exclude other causes of jaundice);
    Lactate dehydrogenase (elevated in hemolysis and a
    marker of hemolytic severity)
    Haptoglobin (decreased in hemolysis);
    s Other tests that may be done include:
    Bilirubin level
    Complete blood count, including red blood cell count
    Hemoglobin - blood
    Hemoglobin - urine
    Haptoglobin level
    LDH test
    Methemoglobin reduction test
    Reticulocyte count
    The most important measure is prevention - avoidance
    of the drugs and foods that cause hemolysis. Vaccination
    against some common pathogens (e.g. hepatitis A and
    hepatitis B) may prevent infection-induced attacks.
    In the acute phase of hemolysis, blood transfusions
    might be necessary, or even dialysis in acute renal failure.
    Blood transfusion is an important symptomatic measure,
    as the transfused red cells are generally not G6PD
    deficient and will live a normal lifespan in the recipient's
    circulation.
    Some patients may benefit from removal of the spleen
    (splenectomy), as this is an important site of red cell
    destruction. Folic acid should be used in any disorder
    featuring a high red cell turnover. Although vitamin E and
    selenium have antioxidant properties, their use does not
    decrease the severity of G6PD deficiency.
    s As nurse, it is very important that the patient is oriented and
    educated about the disorder.
    s Educate the client about foods and medications that may
    cause hemolysis.
    s Explain to the patient the importance of antioxidant to their
    diet. Antioxidants play an important role in preventing
    hemolysis since red blood cells that are G6PD Deficient
    cannot defend themselves from oxidative stress. Make them
    a daily addition to your diet.
    s If infant, instruct the mother that best milk for the baby is
    breast milk.
    s In some agency, the patient should be instructed to wear
    MedicAlert bracelets that identify that they have G6PD.
    s Provide a sterile environment to avoid infection induced
    hemolysis.
    s Vaccination and Immunization is needed to prevent other
    communicable disease. This is also to avoid hemolytic
    attacks.

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