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Information on G6PD Deficiency


Description

Glucose-6-phosphatase dehydrogenase (G-6-PD) deficiency is the most common disease-producing enzymopathy in humans. Inherited as an X-linked disorder, G-6-PD deficiency affects 400 million people worldwide.

The G6PD enzyme catalyzes the oxidation of glucose-6-phosphate to 6-phosphogluconate while concomitantly reducing the oxidized form of nicotinamide adenine dinucleotide phosphate (NADP+) to nicotinamide adenine dinucleotide phosphate (NADPH). NADPH, a required cofactor in many biosynthetic reactions, maintains glutathione in its reduced form.

Reduced glutathione acts as a scavenger for dangerous oxidative metabolites in the cell. With the help of the enzyme glutathione peroxidase, reduced glutathione also converts harmful hydrogen peroxide to water. Red blood cells rely heavily upon G-6-PD activity because it is the only source of NADPH that protects the cells against oxidative stresses; therefore, people deficient in G-6-PD are not prescribed oxidative drugs because their red blood cells undergo rapid hemolysis under this stress.

Those with Glucose-6-phosphate dehydrogenase (G6PD) deficiency should avoid vitamin K. Those with G6PD deficiency experience a serious breakdown of red blood cells (called hemolysis) when exposed to certain infections or medications, including vitamin K.

Ascorbic acid is involved in modulating iron absorption and transport. It is highly unlikely that healthy individuals who take supplemental vitamin C will have any problem with iron overload. On the other hand, those with hemochromatosis, thalassemia, sideroblastic anemia, sickle cell anemia and erythrocyte G6PD deficiency might have such a problem if they use large amounts of vitamin C

Vitamin E injections are used for thalassemia, orally for glucose-6-phosphate dehydrogenase deficiency [G6PD] anemia and anemia caused by kidney dialysis.

DHEA blocks G6PD (glucose-6-phosphate-dehydrogenase)

Vitamin E and folic acid (both anti-oxidants) may help decrease hemolysis in G6PD-deficient individuals.

Causes

G6PD deficiency is caused by one copy of a defective G6PD gene in males or two copies of a defective G6PD gene in females. Hemolytic anemic attacks can be caused by oxidants, infection, and or by eating fava beans.

Sudden attacks of G6PD deficiency can be caused by any serious illness and certain medicines such as:

Medicines for the treatment of malaria: Chloroquine, Primaquine, Pamaquine, Pentaquine, Plasmoquine, Quinine, Quinocide

Medicines for fever and pain: Acetanilid, Acetylsalicylic acid, Aminopyrine, Antipyrine

Sulphonamides: Sulphanilamide, Sulphacetamide, Sulphapyridine, Sulphamethoxypyradizine

Sulphones: Sulphoxone, Thiazolsulfone, Diaminodiphenyl Sulphone

Nitrofurans: Nitrofurantonin, Furazolidone, Nitrofurazone

Others: Phenylhydrazine, Acetylphenylhydrazine, Probenecid, Dimercaprol, Methylene blue, Naphthalene, Vitamin K, Aminosalicylic acid, Chloramphenicol, Vitamin C (only in very high doses), Neosalvarsan



Conventional Labs

Measure the actual enzyme activity of G6PD rather than the amount of G-6-PD protein. Performing assay for G-6-PD during hemolysis and reticulocytosis may affect levels and not reflect baseline values.

Perform a CBC count with reticulocyte count to determine the level of anemia and bone marrow function.

Indirect bilirubinemia occurs with excessive hemoglobin degradation and can produce clinical jaundice.

Serum haptoglobin levels serve as an index of hemolysis and will be decreased.



Imaging Studies:

Abdominal ultrasound may be useful in assessing for splenomegaly and gallstones.

Histologic Findings: Acute hemolysis from G-6-PD deficiency is associated with formation of Heinz bodies, which consist of denatured hemoglobin.



Specialty Lab Tests

Oxidative Stress Analysis, Urine
Detoxification Profile, Standard


Products to consider

Ultimate E, 60gc
Detoxification Factors, 60 Caps


Natural Therapies

Most natural health web sites put a list of herbs, vitamins and nutritional supplements along with citations from scientific studies.

Have you found this to be confusing, since there are so many to choose from?

A far simpler way is to choose a supplement from the appropriate body system, such as liver support, detoxification, etc.
A well formulated supplement works a lot better than separate vitamins and usually costs a lot less.
Please call me at (239) 659-2684 if you would like some assistance in choosing an appropriate product.

I can't diagnose or treat diseases by phone, but I can assist you in making better choices. I also can send some relevant articles to read. The first ten minutes are free (as long as you purchase from my web site).

What's your next step?

The next step, after you have been diagnosed with a disease, is to find out what is the specific cause, and then choose supplements that address it. Phone consults are my specialty. Please call my at (239) 659-2684 to schedule a consultation.

The choice is yours

I recommend that you make an informed choice, and the goal of this web site is to provide you with the information to make a wise choice when it comes to your health and wellness.

As you can see, I don't sell any vitamins on this web site. There are thousands of vitamin stores that will sell you whatever vitamin you want, and many of them offer discounts.

If you are interested in some high-quality basic nutritional supplements, I recommend visiting the Store You will find my recommendations for a multiple, tasty chewable fiber wafers, high-potency probiotics, and fish oils.

Good luck in your journey towards health and wellness!!

Why don't you tell me what vitamins to take for this disease?

Unfortunately, that would be a clear violation of FDA regulations. Also, one of the most important aspect of naturopathy is: "Treat the person, not the disease". This is a profound statement, and many people have spent thousands of dollars on vitamins listed in books or web sites for a specific disease. This is what I call "vending machine medicine". It rarely works! The solution is to find a naturopathic physician, such as myself, to assist you.


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Ronald Steriti, ND, PhD
Natural Health Coach and Consultant
Call today (239) 659-2684

Information on this site is provided for informational purposes only and is not meant to substitute for the advice provided by your own physician or other medical professional. You should not use the information contained herein for diagnosing or treating a health problem or disease, or prescribing any medication. If you have or suspect that you have a medical problem, promptly contact your health care provider. Information and statements on this site have not been evaluated by the Food and Drug Administration.

© 2002