Thalassemia (mediterranean anemia)

General or Other | General Practice | Thalassemia (mediterranean anemia) (Disease)


Description

Thalassemia is an inherited disease characterized by low production of hemoglobin. Hemoglobin is a molecule in red blood cell composition, also called erythrocytes, which is to carry oxygen to organs. Even if the diagnosis seems alarmingly in the last two decades treatment methods have improved, so that the quality and life expectancy of children suffering from this disease increased significantly.

Causes and Risk factors

Hemoglobin is made of two proteins: Alpha globin and beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of one of these proteins.

There are two types of thalassemia: alpha and beta-thalassemia, named according to the chain of proteins affected. Hemoglobin contains two different protein chains, called alpha and beta. Any deficiency in these chains causes abnormalities in the formation, size and shape of red blood cells.

Thalassemia causes insufficient production and / or inefficient red blood cells. As a result, children with thalassemia have a reduced number of red blood cells. Condition is called anemia and affect the transport of oxygen to cells and tissues. In addition, thalassemia can cause red blood cells to be smaller than normal or hemoglobin in red blood cells to be less than normal. Children who suffer from various forms of thalassemia encounter health problems. In some cases it is all too easy for anemia without serious damage, while in other cases, medical treatment only regulat. Talasemia is a genetic disease, which is inherited from parents. If a child received thalassemia gene from one parent, he could be a simple carrier of the gene, without the disease to manifest. In many families are carriers of the gene, but this goes unnoticed by doctors, just because the disease is not evident. Only when one of these carriers meets another, then the child will be born who will suffer talasemy.

Children with alpha-thalassemia are not considered, only carriers of the disease. They have very serious health problems. Can easily be anemic, hence the slightly prolonged fatigue. This fatigue can be misinterpreted as being caused by a lack of the iron . Beta-thalassemy is the most common form of thalassemia. May be, in turn, of three types: minor, intermediate and major.

Diagnosis and Treatment

Treatment for thalassemia major often involves regular blood transfusions and folate supplements. If you receive blood transfusions, you should not take iron supplements. Doing so can cause a high amount of iron to build up in the body, which can be harmful.

Persons who receive significant numbers of blood transfusions need a treatment called chelation therapy to remove excess iron from the body. A bone marrow transplant may help treat the disease in some patients, especially children. ...