Let's go for quick understanding on overview of G6PD deficiency

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https://youtu.be/DZS-diLCa1g

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Foods to Avoid By People With G6PD Deficiency

Foods to Avoid By People With G6PD Deficiency

1. FAVA BEAN

 

• Also known as BROAD BEAN, HORSE BEAN, FILED BEAN OR BELL BEAN.
• Because this bean contain component known as vicine, divicine, convicine, Isouramil.
• All of this is oxidants. Oxidant is the transformation of oxygen into more harmful substances. 
• Which it will lead to oxidative stress and hemolytic anemia   

2. LEGUME/ SOYA BEAN

• Hemolytic reaction of people with g6pd deficiency differ from one patient to another 
• It may be best to avoid any or all types of legumes and soy beans, soy-containing foods and all its forms like tofu,bean curd, etc. 

 3.  MINT/ MENTHOL.

• Foods to avoid by people with g6pd deficiency include mint candies and menthol-flavored foods; substances containing menthol, like toothpaste, mouthwash, and bubble gums. 

4.  VITAMIN K

• Vitamin K – may cause brain damage to patients with g6pd deficiency. This is very critical in infants because aside from hemolysis, the breakdown of red blood cells; it may also lead to neonatal brain damage and neonatal death. 

5. SULPHITES/ SULFITES.

• These substances are sometimes added or sprayed to food sto make their shelf life longer, to make fruits & vegetables look fresh and to preserve foods. They can also be found in some medications. These are foods to avoid by people with g6pd deficiency. 

Pathophysiology of G6PD

G6PD enzyme is required by all cells to protect them from damage by oxidation. It catalyses the first step in the pentose phosphate pathway, whereby glucose-6-phosphate is oxidised to form 6-phosphogluconate. This reaction is linked to the reduction of NADP (nicotinamide adenosine phosphate) to NADPH (nicotinamide adenosine dinucleotide phosphate), which is used to generate reduced glutathione.

The diagram below shows role of G6PD enzyme in the pentose phosphate pathway

Role of glucose-6-phosphate dehydrogenase in the pentose phosphate pathway resulting in the generation of nicotinamide adenosine dinucleotide phosphate (NADPH) and reduced glutathione (GSH), products required to protect the red cell from oxidative stress

For the red cell, this is the sole source of protection against oxidative damage. Red blood cells are constantly challenged by oxidants in the form of free radicals generated by the conversion of oxyhaemoglobin to deoxyhaemoglobin and by peroxides generated by phagocytosing granulocytes.

 Normal red cells can increase generation of NADPH in response to oxidative stress; this capacity is impaired in patients with G6PD deficiency. Failure to withstand oxidative stress damages sulphydryl groups in haemoglobin and the red cell membrane and causes haemolysis. Cells in other tissues and organs have alternate pathways for the generation of NADPH and can thus withstand such oxidative stress. In contrast, red cells are metabolically extremely simple; they lack a nucleus and mitochondria, cannot carry out protein synthesis, and exclusively metabolise glucose for ATP production. The activity of all red cell enzymes, including G6PD, is highest in young red cells (reticulocytes), and progressively declines as the cell ages.

Following a haemolytic episode, the rate of red cell production is accelerated and the increased proportion of young red cells with higher levels of G6PD limits further red cell lysis. Haemolytic episodes are therefore usually self-limiting in those with moderately deficient (class III) variants (for example, G6PD A-) but may result in more severe and progressive anaemia in patients with severely deficient (class II) variants.

Complete lack of the enzyme is incompatible with life. Rarely, male patients with class I variants have profoundly low G6PD activity, such that the red cells are constantly undergoing lysis (chronic non-spherocytic haemolytic anaemia). Almost all deficient people, however, have one of the polymorphic enzyme variants (e.g., G6PD Mediterranean, class II, or G6PD A-, class III) that bestow sufficient residual activity to maintain the person in an asymptomatic state under non-stressed conditions. However, when provoked by oxidative stress (e.g., ingestion of certain drugs, exposure to certain chemicals or glycosides in broad beans, and infection), red cell lysis occurs.

Some drugs are known to pose an oxidative challenge to the red cells and to induce haemolytic anaemia in patients with G6PD deficiency. For some drugs (e.g., dapsone, primaquine, sulphonamides), the association is very clear and consistent, and haemolysis will be observed in virtually all patients, irrespective of residual enzyme activity.  For others (for example, aspirin) the clinical association is less consistent and reflects an interplay of inherited (e.g., residual enzyme activity, pharmacokinetics) and acquired (e.g., dose, absorption and drug metabolism, co-existing infection) factors. 

Causes of G6PD Deficiency

Glucose-6-phosphate dehydrogenase deficiency is a genetic disorder that occurs most often in males. This condition mainly affects red blood cells, which carry oxygen from the lungs to tissues throughout the body. In affected individuals, a defect in an enzyme called glucose-6-phosphate dehydrogenase causes red blood cells to break down prematurely. This destruction of red blood cells is called hemolysis

"G6PD deficiency is passed along in genes from one or both parents to a child."

This condition is inherited in an X-linked recessive pattern. The gene associated with this condition is located on the X chromosome, which is one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of the gene in each cell is sufficient to cause the condition. In females (who have two X chromosomes), a mutation would have to occur in both copies of the gene to cause the disorder. Because it is unlikely that females will have two altered copies of this gene, males are affected by X-linked recessive disorders much more frequently than females. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons.

Mutations in the G6PD gene cause glucose-6-phosphate dehydrogenase deficiency.

      The G6PD gene provides instructions for making an enzyme called glucose-6-phosphate dehydrogenase. This enzyme is involved in the normal processing of carbohydrates. It also protects red blood cells from the effects of potentially harmful molecules called reactive oxygen species. Reactive oxygen species are byproducts of normal cellular functions. Chemical reactions involving glucose-6-phosphate dehydrogenase produce compounds that prevent reactive oxygen species from building up to toxic levels within red blood cells.

   If mutations in the G6PD gene reduce the amount of glucose-6-phosphate dehydrogenase or alter its structure, this enzyme can no longer play its protective role. As a result, reactive oxygen species can accumulate and damage red blood cells. Factors such as infections, certain drugs, or ingesting fava beans can increase the levels of reactive oxygen species, causing red blood cells to be destroyed faster than the body can replace them. A reduction in the amount of red blood cells causes the signs and symptoms of hemolytic anemia.

    Researchers believe that carriers of a G6PD mutation may be partially protected against malaria, an infectious disease carried by a certain type of mosquito. A reduction in the amount of functional glucose-6-dehydrogenase appears to make it more difficult for this parasite to invade red blood cells. Glucose-6-phosphate dehydrogenase deficiency occurs most frequently in areas of the world where malaria is common.

Hello guys! Have a nice day. Did you ever heard about ANEMIA ? YES ? NO? If yes, how you get to know about anemia? If not, its okay we may help you to find the answer. Actually anemia is a condition marked by a deficiency of red blood cells or of hemoglobin in the blood, resulting in pallor and weariness. Seriously? What is blood cell? Hemoglobin? You getting lost right now? Its okay lets us help you find the answer.  

Here, the answer for all the question that keep running on your head every time. But, before that let we recap or tell you general about blood. Blood is a combination of plasma (watery liquid) and cells that float in it. It is a specialized bodily fluid that supplies essential substances and nutrients, such as sugar, oxygen, and hormones to our cells, and carries waste away from those cells, this waste is eventually flushed out of the body through urine, feces, sweat, and lungs (carbon dioxide). 

Plasma constitutes 55% of blood fluid in humans and other vertebrates (animals with a backbone, spinal column).

Apart from water, plasma also contains:

• Blood cells
• Carbon dioxide
• Glucose (sugar)
• Hormones
• Proteins

Have you know actually inside this red color liquid (blood) consist several type of cells that you cant ever see through your naked eyes. Its amazing right?

Types of blood cells:

• Red blood cells - also known as RBCs or erythrocytes. They are shaped like slightly indented, flattened disks or biconcave. These are the most abundant cells, and contain hemoglobin (Hb or Hgb). 
• 
• Hemoglobin is a protein which contains iron; it transports oxygen from the lungs to body tissues and cells. 97% of a human's red blood cell's dry content is protein.
  
  Each RBC has a life span of about 120 days or 4 months; at the end of their lives they are degraded by the spleen and the Kupffer cells in the liver. The body continuously replaces the ones that die.

• White blood cells (leukocytes) - these are the cells of our immune system; they defend the body against infections and foreign materials. There are 5 types of white blood cell which either have granule (granulocytes) or without granule (agranulocytes)
• 
• Platelets (thrombocytes) - are involved in the clotting (coagulation) of blood. When we bleed the platelets clump together to help form a clot.
• 
• You get some general idea regarding the blood right? Now, back to our main topic which is ANEMIA. As mentioned before, anemia is the condition when the number of healthy red blood cells in your body is too low. Red blood cells carry oxygen to all of the body’s tissues, so a low red blood cell count indicates that the amount of oxygen in your blood is lower than it should be. Many of the symptoms of anemia are caused by decreased oxygen delivery to the body’s vital tissues and organs. There are many types of anemia such as sickle cell anemia, megaloblastic anemia, iron-deficiency anemia, hemolytic anemia and so on. However,in our blog, mainly focus about anemia due to G6PD deficiency. 

• ABOUT G6PD DEFICIENCY
• G6PD is one of many enzymes that help the body process carbohydrates and turn them into energy. G6PD also protects red blood cells from potentially harmful byproducts that can accumulate when a person takes certain medications or when the body is fighting an infection.
  In people with G6PD deficiency, either the RBCs do not make enough G6PD or what is produced cannot properly function. Without enough G6PD to protect them, RBCs can be damaged or destroyed. Hemolytic anemia occurs when the bone marrow (the soft, spongy part of the bone that produces new blood cells) cannot compensate for this destruction by increasing its production of RBCs.

Introduction

Hello people! How are you today? 

Have you ever wonder how red blood cell can rupture or break down before their lifespan? What are the consequences that will occur? what will happen to your body when you have low red blood cell in the body? ⟹

We are students of Bachelor in Medical Sciences, Semester 5/2016 from Management & Science University (MSU) Shah Alam are creating this blog to give awareness about anemia.

Here, introducing our group members:

oNURUL AZLIN SHAIRA

oNOR ALYA ATIKAH

oSITI SYAHIRAH

oASNA NABILAH

oNOR ARDYLA

oNURHIDAYAH

oSITI NASUHA

Last but not least, we hope that you can gain a new beneficial information and knowledge regarding anemia. Stay healthy people and stay tuned for more updates from us!