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G6PD Deficiency

G6PD is an enzyme in the red blood cells. It acts to protect the red blood cells from hemolysis when exposed to certain medications, food or infections.

G6PD, which stands for Glucose-6-Phosphate Dehydrogenase is an enzyme in the red blood cells. It acts to protect the red blood cells from hemolysis (breakage of the red blood cells) when exposed to certain medications, food or infections. G6PD deficiency occurs when there is reduced activity of the enzyme in the red blood cells.

Diagnosis

G6PD is very common in the Asian region. In Malaysia, all newborn babies are screened for G6PD deficiency using the blood from the umbilical cord at birth. G6PD deficiency is a hereditary condition that is determined by the X chromosome. This explains why most of those affected are males while females are usually carriers.

Symptoms

Most people with G6PD deficiency are asymptomatic. Newborn babies known to have this condition by screening will be admitted for a certain duration of time in the ward since they can develop jaundice (yellowish discolouration of the skin and sclera) rapidly, due to excessive hemolysis.

Signs and symptoms of hemolysis include:

  1. Due to anaemia (low level of red blood cell):
    • looks pale
    • lethargic or tiredness in the older child
    • dizziness
    • rapid heart beat (palpitations)
    • difficulty in breathing
    • chest pain
  2. Due to hemolysis:
    • jaundice (yellowness of skin and white part of the eyes)
    • tea coloured appearance of urine

If this happens, do seek medical treatment immediately. G6PD deficiency is a lifelong condition. Although there is no treatment available to reverse it, it does not have any negative effects and does not affect the growth and development of children.

Medications and things to avoid

Adults and children with G6PD deficiency need to avoid certain medications and other things that can lead to hemolysis (Diagram 1).

Breastfeeding is encouraged and mothers with babies who have G6PD deficiency should continue to breastfeed, but they are advised to avoid taking medications/substances in Diagram 1 as its may be present in the breast milk in small amounts.

Infections can also lead to hemolysis.

Inform your doctor if you or your children have G6PD deficiency to ensure that the medications you receive are safe for consumption and will not cause excessive hemolysis.


Picture 1 : Infant undergoing phototherapy


Picture 2 : Fava beans

Diagram 1 : Medications/substance that can lead to excessive hemolysis

Foodstuff and herbs to avoid
Medications that can be given in therapeutic doses
  • Fava beans
  • Chinese medication
    • Chuen Lin
    • San Chi
    • 13 herbs
    • 12 herbs
  • Other traditional medication (ask your doctor)

Things to avoid

  • Moth balls
  • Mosquito coils containing pyrethium

Medications to avoid

  • Acetanilide
  • Doxorubicin
  • Furazolidene
  • Methylene Blue
  • Nalidixic acid
  • Niridazole
  • Nitrofurantoin
  • Phenozopyridine
  • Primaquine
  • Sulfamethoxazole
  • Bactrim
  • Paracetamol
  • Ascorbic acid
  • Aspirin
  • Chloramphenicol
  • Chloroquine
  • Colchicine
  • Diphenhydramine
  • Isoniazid
  • Phenacetin
  • Phenylbutazone
  • Phenytoin
  • Probenecid
  • Procainamide
  • Pyrimethamine
  • Quinidine
  • Streptomycin
  • Sulfisoxazole
  • Trimethoprim
  • Tripelennamine
  • Vitamin K
  • Mefloquine

 

Last reviewed : 28 August 2020
Writer : Dr. Fazila Kutty
Reviewer : Dr. Zainab bt. Kusiar

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