In subjects affected by favism there is a deficit of an enzyme involved in the biogenetic pathway of pentose-phosphates, glucose-6-phosphate dehydrogenase (G6PD): the deficiency of the enzyme has serious consequences at the level of erythrocytes (red blood cells), since G6DP is essential for their correct functioning and survival.
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The term "favism" is however improper, since in some fabic subjects the clinical haemolytic reaction can also occur independently of the consumption of broad beans and peas.
However, in most cases, affected people cannot eat these legumes; furthermore, other substances, such as naphthalene and certain drugs, such as analgesics, antipyretics, antimalarials, salicylates, certain chemotherapy drugs, quinidine, methylene blue etc., which can aggravate the pre-existing condition, cannot be taken or inhaled.
The deficiency of the enzyme glucose-6-phosphate dehydrogenase, as we have seen, is the triggering cause that causes acute hemolysis with jaundice. In fact, the role played by this enzyme as a defense factor of erythrocytes from oxidation is known.
The drugs and substances mentioned above alter the oxidative balance, acting as pro-oxidants.
The subject affected by favism, after 12-48 hours from the ingestion of peas, broad beans or particular drugs, manifests a yellowish complexion that sometimes tends to green, the ocular sclera appear intense yellow, the urine dark. severe form, the subject could have a cardiovascular collapse: jaundice is caused by a high concentration in the blood of bilirubin, a catabolic (waste) product of the hemoglobin contained in red blood cells. If the jaundice progresses, the disease could develop into the more severe form of kernittero (bilirubin encephalopathy: bilirubin is deposited in the brain, resulting in brain damage and possible mental retardation).
Furthermore, haemolytic anemia could occur not only following the intake of the banned substances in this disease, but also as a consequence of pneumonia, viral hepatitis, malaria and diabetic ketoacidosis.
There are various forms of favism and, based on the severity, the WHO (World Health Organization) distinguishes five levels:
- the first two express a severe deficiency (chronic haemolytic anemia / acute renal failure and intermittent haemolysis),
- the third represents a mild deficiency (haemolysis manifested only in case of contact with oxidizing substances - beans, peas, analgesic drugs, naphthalene etc.),
- while the last two do not involve any dangerous clinical effect.
, NSAIDs and some antibiotics.
Blood transfusions, in the case of an acute hemolytic crisis, and dialysis for people with kidney failure, are vital.
In some serious cases, the removal of the spleen may be the only solution: it is in fact at the level of the spleen that the red blood cells are destroyed.
The careful and scrupulous diagnosis of the disease is essential before adopting any treatment: the diagnostic test searches for the glucose-6-phosphate dehydrogenase enzyme inside the erythrocytes, highlighting even the slightest deficiencies.