Favism

Favism.

From Biochemistry Department Book :

- Favism is a type of Congenital Haemolytic Anaemia or Hereditary Haemolyti Anaemia.

- Congenital Hemolytic Anaemia is occurs due to congenital deficiency of Glucose 6 Phosphate Dehydogenase ( G6PD ).

- It is X-linked condition.

- Deficiency of G6PD result in :

 * Decrease in the level of NADPH+H

 * Hence, a decrease in the concentration of Reduced Glutathione ( GSH ), which is the main defense mechanism facing the dangerous effect of Hydrogen Peroxide and other oxidant radicals.

- Hydrogen Peroxide may decrease the life span of Erythrocytes and increase the rate of oxidation of Haemoglobin ( Hb ) into Methaemoglobin.

- The deficiency is manifested only after intake of certain oxidant drugs  like primaquine, the antimalarial drug or after ingestion of fava beans ( broad beans ).

- Oxidizing drugs and sulfa drugs, in absence of reduced glutathione ; may distort the membrane of RBCs resulting in hemolysis.

* The urine turns black

* Jaundice is developed

* Hb contentof blood drops sharply.


- In some cases; massive destruction of RBCs causes death.

- This disease could be treated by :

* Administration of antioxidants

* Regular transfusion of packed RBCs.


Meaning of Favism :


Dorland's Pocket Medical Dictionary 28th Edition.

- An acute hemolytic anemia precipitated by fava beans ( ingestion or inhalation of pollen ), usually caused by G6PD in erythrocytes.


Oxford Medical Dictionary.

- Inherited defect in the enzyme G6PD causing the red blood cells ( RBC ) to become sensitive to a chemical in fava beans.

Eating this bean result in destruction of RBC ( Haemolysis ), which may lead to severe anaemia, requiring blood transfusion. Favism occur most commonly in the Mediterranean and Middle East.

Fava Beans ( Broad Beans )

Wallahua'lam.

-MrHope-

25 October 2012.
7:04 a.m