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| Thalassemia (Mediterranean Anemia) (image 1) |
Thalassemia (Mediterranean Anemia)
THALASSEMIA (MEDITERRANEAN ANEMIA)
What Is Thalassemia?
Is seen in most Mediterranean countries, but
spread all over the world due to migration and the education of a hereditary
blood disease is easily preventable with screening tests.
Thalassemia.
Thalassemia occurs as a result of the disorder
in the production of hemoglobin. The hemoglobin molecule carries oxygen from
lungs to tissues and regulating. In the blood erythrocytes (red blood cells) is
located. ‘Both’ and ‘globin’ there are two parts to it. The two chains called
Alpha and beta Globin fragment. This chain production is controlled by genes .
Thalassemia causes the lack or deficiency of one of the chain of production.
Eat deficiency Alpha thalassemia Alpha chain, beta chain causes beta
thalassemia deficiency.
The Frequency Of Thalassemia:
Turkish Hematology Association, according to
data from a carrier of thalassaemia in our country, 1.3 million, is about 4500
thalassemia patients. The number of the carrier 266 million all over the world.
Other Asian countries the most common Alpha-thalassemia. Seen in thalassemia
type beta thalassemia in the Mediterranean countries and Turkey. The most
common talasemin our country, provinces, Antalya, Adana, Denizli and Izmir.
Clinical beta thalassemia has three forms which differ:
1) thalassemia trait (thalassemia minor)
2) thalassemia intermedia
3) thalassemia major
Healthy except for the symptoms of mild anemia
in thalassemia for the person just carries the gene for thalassemia disease.
However, two carrier of the child to be born, when she married a 25%
probability in patients with a 50% chance carrier, 25% chance there will be.
The possibility of two defective genes coming together of consanguineous
marriages , thereby increases the probability of their children becoming ill.
Community education for prevention with the prevention of consanguineous
marriages is required. Hemoglobin analysis by people before they get married
again especially in risky areas should have the screening test for thalassemia
carrier determine whether it should be. There is an obstacle in the marriage of
two individuals who are thalassemia carriers. Just when they got to the
children of these couples, a prenatal diagnosis Center for genetic diagnosis and
genetic counseling to learn whether the baby will be sick by contacting your
should receive.
Cerebral thalassemia in the mother and the
Father is the carrier. In patients with moderate anaemia and symptoms in
advanced age will occur. In these patients the treatment is much less than in
thalassemia major and blood in need of a transplant.
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| Thalassemia (Mediterranean Anemia) (image 2) |
Again thalassemia majord father and the mother
is a carrier, however, is more than a disorder of globin synthesis. Severe
symptoms of the disease begins 6 months after birth. Patients weakness, pallor,
loss of appetite, restlessness, abdominal swelling as a result of enlargement
of the liver and spleen, is seen in the expansion of bones. We face broad and
prominent forehead flattened shapes of the nose is abnormal. The symptoms of
anaemia is very severe and need a blood transfusion within 3-4 weeks.
Thalassaemia major with severe symptoms, is a progressive disease which leads
to various complications in time. These patients are required to seek regular
treatment throughout their lives. In patients without regular treatment in the
body, malformations, growth retardation, diabetes, heart failure and
osteoporosis are seen.
How Is It Diagnosed Thalassemia:
Complete blood count, peripheral smear,
hemoglobin electrophoresis, with the degree of anemia, is determined by the
type of the disease. By measuring serum Iron levels anemia to rule out the
possibility of interference with other. Iron deficiency anemia is low iron in
the blood, high in iron talasemide. This distinction is very important because
a patient's condition worsens iron deficiency anemia thalassemia treated as
such.
Diagnosis is confirmed with molecular
diagnostics and DNA analysis methods also.
The Treatment Of Thalassemia:
After a diagnosis of thalassemia patient, the
treatment process is an exhausting and costly , a challenging life awaits you.
So the treatment, which is much more important to prevent the emergence of
diseased individuals.
Inpatient treatment should be performed in
centers with the ability to thalassemia. Hemotolog sick, cardiologist,
endocrine specialist, psychologist and social worker must be under the
supervision.
Treatment is done according to need a blood
transfusion within 3-4 weeks. The purpose of blood transfusion is the provision
of correction of anemia and the oxygen supply to tissues. Thus, the normal
physical appearance, normal growth, sexual development, psychosocial support
and quality of life will be given.
However, Frequent blood transfusions can
accumulate iron in various organs including the heart and liver as a result of.
This accumulation and reduce heart and liver Iron to chelation therapy that
binds with drugs would do to prevent the damage is done.
Again, transfusion hepatitis due to hepatitis B
vaccination and immune monitoring should be done against the possibility of
contamination.
In advanced age the patient's blood are taken
from the spleen of the person to reduce the need for transfusion. To prevent
infections that may occur after the spleen is removed in patients each year,
pneumococcal, meningococcal, and Haemophilus influenza vaccines should be made.
I mean the only final and lasting treatment of
the disease a bone marrow transplant is a stem cell transplant. From an early
age (especially before age 7) with a bone marrow transplant patient completely
recovers. The most appropriate is the brother of the patient transmitter.
The diet of the iron in thalassemia patients-the
poor should be rich in calcium. Diabetes diabetes diet for growers is applied.
The Most Effective Methods For Control Of Thalassemia :
1) organization of training to raise community
awareness about the disease
2) premarital screening programs for detection
of carriers. (Especially in risky areas wedding, pre-screening tests must be
constructed. )
3) carrier and prenatal diagnosis genetic
counseling to the families of sick new giving by using the methods of prenatal
diagnosis amniocentesis or chorionic villus biopsy is the prevention of the
birth of a baby.. unborn baby is determined by whether or not carry the
diseased gene, and genetic counseling with the baby's fate is decided.
4) the most advanced method to this day before
pregnancy occurs with IVF (preimplantation phase ) with the selected cells with
the gene intact cells the creation of pregnancy. This is a method of
preimplantation genetic diagnosis in thalassemia and hereditary diseases can be
detected with this method except.
Reduced the number of cases where such means is
under the control of talasemin there are countries where even entirely averted.
Diagnosis of thalassemia was established by the Ministry of health and US
Center for Applied thalassemia trait screening program by providing support and
obedience and we have to contribute to the prevention of disease.
