Glucose 6 phosphate dehydrogenase g6pd deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain foods, drugs, infections or stress. This deficiency can cause hemolytic anemia, usually after exposure. Learn what this has to do with blood, the problems it can cause and what medicines you should avoid. Substitutes for some common foods on my avoid list. The clinical expression of g6pd variants encompasses a spectrum of hemolytic syndromes. Mutation of the xlinked g6pd gene approximately 127 have been reported with a single base substitute leading to amino acid replacements results in many variants of protein with varying enzyme activity that result in different. Dec 11, 2017 this video discusses the g6pd deficiency avoid list. Glucose 6phosphate dehydrogenase g6pd deficiency is highly prevalent in individuals originating from most parts of africa, from most parts of asia, from oceania, and from southern europe. Drugs to avoid in g6pd deficiency definite risk of haemolysis possible risk of haemolysis pharmacological class drugs pharmacological class drugs. Medications to avoid in patients with g6pd deficiency due to. Hemolysis can be triggered by infection, hyperglycemia, certain foods, and certain medications. This enzyme, which is active in virtually all types of cells, is involved in the normal processing of carbohydrates. Glucose6phosphatase dehydrogenase g6pd deficiency is the most common enzyme deficiency in humans, affecting 400 million people worldwide.
Caring for glucose6phosphate dehydrogenase g6pddeficient. People with low g6pd activity have g6pd deficiency. Started in 1995, this collection now contains 6767 interlinked topic pages divided into a tree of 31 specialty books and 732 chapters. Drugs to be avoided in g6pd deficiency drug group example comment. G6pd deficiency, an xlinked disorder, is the most common enzymatic disorder of red blood cells in humans, affecting more than 400 million people worldwide. The level of risk for hemolysis by each trigger depends on the g6pd deficiency variant type as well as the individuals within each g6pdd variant group. G6pd, or glucose6phosphate dehydrogenase deficiency, is a genetic condition that makes carriers more susceptible to hemolysis and resultant. Aminopyrine pyramidon, amidopyrine antazoline antistine arginine 2amino5guanidinopentanoic acid c 6 h 14 n 4 o 2. What to avoid with g6pd deficiency g6pd deficiency foundation. Fava beans some, but only some, prefer also to avoid red wine, henna see below, all legumes, blueberries also yogurts containing these, soya products, tonic water. G6pd is an enzyme that helps protect red blood cells.
Drugs associated with risk of haemolysis in g6pddeficient individuals. Pdf glucose6phosphate dehydrogenase g6pd deficiency is the most prevalent. Naphthol niridazole stibophen nitrofurans nitrofurantoin nitrofurazone quinolones ciprofloxacin moxifloxacin nalidixic acid norfloxacin ofloxacin chloramphenicol sulfonamides. Glucose6phosphate dehydrogenase deficiency wikipedia. Shaun wen huey lee, nathorn chaiyakunapruk, and nai ming lai. The level of risk for hemolysis by each trigger depends on the g6pd deficiency variant type as well. People with g6pd deficiency need to avoid certain food items like peanuts, legumes, products that contain menthol, etc. Glucose6phosphate dehydrogenase g6pd deficiency is the most prevalent enzymopathy in mankind. Wading through conflicting literature on g6pd deficiency. Medications to avoid in patients with g6pd deficiency due.
It is an xlinked recessive disorder that results in defective glucose6phosphate dehydrogenase enzyme. Coli and salmonella can also result in the appearance of the symptoms. Glucose6phosphate dehydrogenase g6pd deficiency is the most common enzyme deficiency in the world and renders those affected susceptible to potentially severe oxidative hemolysis. Consumption of any part of the fava bean plant can cause hemolytic anemia in g6pd deficient individuals, including inhalation of fava bean plant pollen. The g6pd gene provides instructions for making an enzyme called glucose6phosphate dehydrogenase. Drugs causing haemolysis in g6pd deficiency subjects. Glucose6phosphate dehydrogenase g6pd deficiency is the most common enzymopathy worldwide, affecting an estimated 400 million people. This is when the red blood cells break down faster than they are made.
G6pd deficiency is the most common enzymatic disorder of rbcs. However, they should be taught to avoid drugs and chemical exposures that can cause oxidant stress. Asians, africans and mediterranean and more prevalent in males more than females 1,2. An enzyme is a type of protein thats important for cell function. One who is g6pd deficient may be at risk for any known triggers. G6pd is the only source of nadph within the cell, so deficiency makes red blood cells susceptible to hemolysis if they are under oxidative stress. What to avoid with g6pd deficiency g6pd deficiency. Pdf glucose6phosphate dehydrogenase g6pd deficiency is the most common. It occurs when a person is missing or has low levels of the enzyme glucose6phosphate dehydrogenase. Scientists think there are about 400 million people in the world with g6pd deficiency.
The carbohydrates that cause the most problems include white flour, refined sugars, and high fructose corn syrup, additional food products that can cause g6pd deficiency syndromes to appear include tonic water, items that use blue food coloring, and products that contain sulfites, such as wine and dried fruits. Glucose6phosphate dehydrogenase g6pd deficiency is a wellknown, xlinked genetic disorder that most commonly affects up to 25% of primarily male patients from africa, asia, and the mediterranean. The following is a partial list of medications and chemicals that individuals with glucose6phosphate dehydrogenase g6pd deficiency should avoid. G6pd deficiency is more common in males than it is in females. G6p d deficiency, xlinked, pentose phosphate pathway ppp, hemolytic anemia, favism. Lack of the enzyme g6pd means that red blood cells can be damaged and destroyed when the person is exposed to some chemicals including some medicines or during some infections. A list of medications, foods and infections that may result in reaction. Some drugs should be avoided by all g6pddeficient patients table 1. Patients with g6pd deficiency should avoid exposure to oxidative drugs table 3 and ingestion of fava beans.
Glucose6phosphate dehydrogenase g6pd is an enzyme which protects the red blood cells and prevents them from being damaged. This can lead to moderate or severe haemolytic anaemia anaemia means low levels of red blood. Glucose6phosphate dehydrogenase g6pd deficiency the most common abnormality in this system is g6pd deficiency, which can lead to druginduced hemolytic anemia view in chinese oxidant injury may be exacerbated by glucose6phosphate dehydrogenase g6pd deficiency but can occur in individuals without g6pd deficiency as well. Pdf living with glucose6phosphate dehydrogenase deficiency. Glucose6phosphate dehydrogenase deficiency genetic and. While reading around the topic, i found that the lists of medications to avoid in g6pd deficiency vary from one source to another. A list is available in appendix a6, as well as an information card that patients with clinically significant g6pd deficiency should carry in their wallet to show to healthcare practitioners at their medical appointments. Cardiovascular drugs diagnostic agent for cancer detection gout preparations hormonal contraceptives nitrates vitamin k substance vitamins others please refer to the contents page for more summary tables. It can be useful to provide a list of contraindicated drugs to the medical file or. However, for the great majority of people with g6pd deficiency, if they are careful to avoid the foods, drugs and other items that might trigger the blood cell breakdown, they should be able to live full, healthy lives with a normal life expectancy. It plays a critical role in red blood cells, which carry oxygen from the lungs to tissues throughout the body. Avoid eating fava or broad beans and their products.
Depending on the specific mutation the severity of the condition may vary. Dec 04, 2018 most individuals with g6pd deficiency do not need treatment. G6pd deficiency glucose6phosphate dehydrogenase g6pd deficiency is an xlinked inherited disorder caused by a genetic defect in the red blood cell enzyme g6pd, which serves to protect red blood cells from oxidative injury. It is hereditary, which means it is passed down in families. Red blood cell breakdown may be triggered by infections, certain medication, stress, or foods such as fava beans. Pdf dietary restrictions for people with glucose6phosphate. Glucose6phosphate dehydrogenase deficiency department of. Search drug groups, ingredients and trade names access limited to medical personnel and paying members drug names in red are of high risk to all deficiency variants. It is highly common in certain ethnic groups such as. Diagnosis and management of g6pd deficiency american.
Medicine, specialist physician g6pd deficiency is an inherited condition in which the body doesnt have enough of the enzyme glucose. What medicines should be avoided with g6pd deficiency. Mims summary table drugs to avoid in g6pd deficiency pharmacological class drugs anthelmintics. Hemolysis can be triggered by infection, hyperglycemia, certain foods, and. Most people who have g6pd deficiency are healthy, but sometimes fever or certain medicines and foods can break down their red blood cells. The severity of hemolytic anemia varies among individuals with g6pd. Our knowledge of g6pd deficiency is limited although it is a fairly common inherited disease in malaysia. Antibiotics referred to as sulfa drugs should be avoided. The who recommends testing of drugs to predict the risk of haemolysis in g6pd deficient individuals if the drugs are to be prescribed in areas of high prevalence of g6pd deficiency preclinical in vitro tests fall in glutathione in g6pd def red cells andor hexoaemonophosphoate pathway in g6pd normal rbc. Glucose6phosphate dehydrogenase g6pd is a substance that helps the body use sugar for energy. Evaluate glucose 6phosphate dehydrogenase g6pd deficiency. Aminopyrine pyramidon, amidopyrine antazoline antistine arginine 2amino5guanidinopentanoic acid c 6. Guide to g6pd deficiency rapid diagnostic testing to support p.
Mims summary table g6pd deficiency ver01032006 nomenclature based on inn international nonproprietary name refer to. Not finding any results does not necessarily mean the drug is safe to take. Treatment may involve medicines to treat infection, stopping drugs that are causing red blood cell destruction, andor transfusions, in some cases 2. G6pd deficiency is a major risk factor for dangerous levels of bilirubin hyperbilirubinemia in newborns. The family had already been given information about g6pd and agreed to genetic counseling so an appointment had been made. Following are the foods that should be avoided in g6pd deficiency. This deficiency can cause hemolytic anemia, usually after exposure to certain medications, foods, or even infections. Anaemias treatment summary bnf content published by nice. Antimalarials primaquine pamaquine african a variant may take it at reduced dosage under close monitoring chloroquine may be used for prophylaxis or treatment. Populations at higher risk for g6pd include those who their ethnic heritage is from the mediterranean, africa, middle east and asia. The key to the g6pd deficiency diet is the avoidance of trigger foods. If not treated, such rare cases could become fatal. Oct 23, 2018 glucose6phosphate dehydrogenase g6pd deficiency is a genetic disorder that is most common in males.
Manage hemolysis by removing trigger, prbc transfusion as needed, supportive management. Most of the time, people with g6pd deficiency can live healthy lives as long as they avoid certain foods and medicines that cause red blood. A g6pd test measures the levels of glucose6phosphate dehydrogenase g6pd, an enzyme in your blood. Diagnosis and management of g6pd deficiency jennifer e. Drugs to avoid list g6pd deficiency avoid list all legumes prohibited chickpeas, lentils, peanuts, beans, beans, soybeans, soybean oil. So it is very important to avoid the growth of bacteria in foods. Here is a list of the foods that should be refrained from in your diet. The who recommends testing of drugs to predict the risk of haemolysis in g6pddeficient individuals if the drugs are to be prescribed in areas of high prevalence of g6pd deficiency preclinical in vitro tests fall in glutathione in g6pd def red cells andor hexoaemonophosphoate pathway in g6pdnormal rbc. G6pd deficiency the g6pd gene is located on the telomeric region of the long arm of xchromosome band xq28. Drugs and substances should be avoided by g6pd deficient. Our database includes the most commonly used drugs that might pose a risk to g6pd deficient subjects. Most individuals with g6pd deficiency do not need treatment.
Favism occurs primarily in the mediterranean variety of g6pd deficiency. What foods should people with g6pd deficiency avoid. Consumption of any part of the fava bean plant can cause hemolytic anemia in g6pddeficient individuals, including inhalation of fava bean plant pollen. G6pd deficiency is caused by mutations in the g6pd gene. G6pd deficiency have a completely normal life as lon g as they avoid certain foods and drugs. Tonic water containing quinine, a contraindicated drug. Favism, g6pd deficiency, glucosephosphate dehydrogenase. Glucose 6phosphate dehydrogenase g6pd deficiency is a genetic blood disorder where an acute attack of a haemolytic anaemia occurs. Heinz body preparation reveals the dena tured hemoglobin precipitates.
Search our database for drug names and trade names in all countries. Enter a drug name or trade name to view risk level. G6pd deficiency leads to free radicalmediated oxidative damage to red blood cells, which in turn causes hemolysis. Glucose6phosphate dehydrogenase g6pd deficiency is the most common. Glucose6phosphate dehydrogenase g6pd is an enzyme which protects the red blood. G6pd deficiency is an inherited condition in which the body doesnt have enough of the enzyme glucose6phosphate dehydrogenase, or g6pd, which helps red blood cells rbcs function normally.
Severe jaundice hyperbilirubinemia develops in about 0. Why we have to avoid some things and what things on the avoid list do to those with g6pdd. G6pd deficiency occurs when a person is missing or does not have enough of an enzyme called glucose6phosphate. It is an enzyme, which means it speeds up chemical changes when the body converts sugar to energy. These antibiotics are typically used to treat skin or urinary tract bladder infections. Glucose6phosphate dehydrogenase deficiency g6pd deficiency is an xlinked recessive hereditary disease characterized by abnormally low levels of glucose6phosphate dehydrogenase, a metabolic. Naphthol niridazole stibophen nitrofurans nitrofurantoin nitrofurazone quinolones ciprofloxacin moxifloxacin nalidixic acid norfloxacin ofloxacin. G6pd deficiency is an inherited disease passed on from previous generations, and cannot be caught by being in contact with someone else. Dec 18, 2018 the key to the g6pd deficiency diet is the avoidance of trigger foods. Which medications should be avoided in patients with g6pd. G6pd is an enzyme involved in the pentose monophosphate pathway.
First of all, a number of drugs, as listed in ta ble 2,3,s,6 can precipitate hemolysis in g6pddefi cient subjects. Just avoiding the triggers can help you avoid the adverse reactions from occurring. The encounter made me think about what advice could be given to the parents of g6pd deficient children. Pdf glucose6phosphate dehydrogenase g6pd deficiency. May 08, 2017 glucose 6 phosphate dehydrogenase g6pd deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain foods, drugs, infections or stress. Your child with g6pd deficiency should avoid certain medicines and foods. The risk of drug induced g6pd deficiency related hemolysis depends on a number of factors including the g6pd. Peripheral smear from a patient with heinz body hemolytic anemia. Patients should use caution with glutenfree flours and products made from gluten. It may then be necessary for males in our locality to know their g6pd status so that those found to be deficient can avoid the trigger factors for acute haemolytic attack such as indiscriminate ingestion of drugs. G6pd deficiency mainly affects red blood cells, which carry oxygen from the lungs to tissues throughout the body. It is when the body doesnt have enough of an enzyme called g6pd glucose6phosphate dehydrogenase.
Caring for glucose6phosphate dehydrogenase g6pddeficient patients. Patients should also avoid broad beans ie, fava beans. C 3 neonates should be screened for g6pd deficiency when family history, ethnic or geographic origin, or the timing of the appearance of neonatal jaundice suggests the possibility of g6pd deficiency. If your institution subscribes to this resource, and you dont have a myaccess profile, please contact your librarys reference desk for information on how to. The g6pd enzyme is found in all the bodys cells, but it is especially important in red blood cells. Discussion glucose 6phosphate dehydrogenase deficiency g6pd is an xlinked disease that has multiple mutations. However, if they suffer from a severe infection or are exposed to oxidant stress such as from certain drugs or chemicals, massive. Other disorders can contribute to neonatal jaundice and not all g6pd deficient newborns become jaundiced.
Sep 15, 2014 he was seen in the clinic the following day and another bilirubin was 10. Diagnosis is based on symptoms and supported by blood tests and genetic testing. People with g6pd deficiency can generally enjoy normal health. Diagnosis and management of g6pd deficiency american family. G6pd deficiency g6pd deficiency is an inherited disorder. People with g6pd deficiency can tolerate most antibiotics but should be aware of a select few that can initiate significant red blood cell breakdown leading to anemia. This test measures the activity of g6pd enzymes in the blood.
Glucose6phosphate dehydrogenase g6pd deficiency is a condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection. Glucose6phosphate dehydrogenase deficiency orphanet. Drugs and foods that present risk in individuals with. Patients with g6pd deficiency should avoid exposure to oxidative. Miscellaneous elements besides drugs that should be avoided. Acetylphenylhydrazine 2phynylacetohydrazide aldesulfone sodium sulfoxone c 14 h 14 n 2 na 2 o 6 s 3. Most people who have g6pd deficiency are healthy, but sometimes fever or certain medicines and foods can break down their red blood cells haemolysis and cause anaemia. About 1 in 10 african american males in the united states has it. G6pd is a critical antioxidanta deficiency can predispose to oxidation and subsequent hemolysis of the red blood cell. Glucose6phosphate dehydrogenase g6pd deficiency is an inherited disorder caused by a genetic defect in the red blood cell rbc enzyme g6pd, which generates nadph and protects rbcs from oxidative injury. G6pd deficiency is more common in some racial groups, for example, people with african, asian or mediterranean heritage. Soya products blueberries and foods containing them fava beans and all legumes and flour containing fava beans must be avoided tonic water containing quinine, a contraindicated drug in g6pd artificial blue food color and artificial ascorbic acid in.
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