The main function of hemoglobin is the transfer of oxygen to all cells of the body. It is a protein composed of four chains of gluten, each containing an implicit group containing iron called hem, which helps to bind the reversible oxygen with hemoglobin.
The four chains are made up of two alpha series and two alpha-type chains, thus forming three main types of hemoglobin:
1. Hemoglobin A: consists of two series of alpha type and two series of beta (α2β2) and accounts for 97% of hemoglobin in adult life
Hemoglobin A2: consists of two series of alpha type and two series of the type of delta (α222) and accounts for 2.5% of hemoglobin in adult life
3 – hemoglobin F: consists of two series of the type of alpha and two gamma series (α222) and less than 1% of hemoglobin in adult life, but it is the largest proportion in the life of the fetus is about 50 – 90% of hemoglobin because of the lack of production Prenatal beta series.
The manufacture of these chains depends on the genes. The alpha chains control the manufacture of four genes (two genes on each chromosome 16). Beta chains control their manufacture of genes (gene on each chromosome 11).
Thalassemia is a hereditary disease that has a hereditary chromosome inheritance (the parents must be carriers of the disease in order to appear in their children) resulting from an imbalance in the production of the constituent chains of hemoglobin. This imbalance may be a partial inability or total inability to produce one of the constituents of hemoglobin .
There are two types of Thalassemia namely:
Alpha-thalassemia (the imbalance in the production of alpha chains)
Beta-thalassemia (beta-chain imbalance)
1 – Thalassemia is a genetic disease resulting from the imbalance in the manufacture of chains constituents of hemoglobin
2. The disease is divided into two main types, Thalassemia Beta and Thalassemia Alpha, and spreads the disease in the Mediterranean region, Africa and Northeast Asia
3 – Symptoms of thalassemia beta appear after 3-6 months of birth while symptoms and signs of thalassemia alpha at birth
4- Diagnosis of the disease mainly on the electrolysis of blood
Prevention lies in prenuptial screening, early diagnosis during pregnancy and the possibility of termination
6. Thalassemia treatment includes iron avoidance, folic acid intake, blood transfusions, iron sclerosis, and complications of iron accumulation.
1. Oxford handbook of clinical medicine 8th edition
2. Dvidson’s principles and practice of medicine 21st edition
3. illustrated texte of pediatrics -Tom lissauer, Graham Clayden 3rdedition