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2007-02-11 03:54:53 · 7 answers · asked by gopaljee j 1 in Science & Mathematics Medicine

7 answers

thalassemia is a hemolytic disorder..in this condition yher is defective globin synthesis( haemoglobin have two components. haem and globin . globin itself 2 types. alpha and beta which are just protein molecules..) 3 types of thalassemia are there - alpha , beta and intermediate type. based on the type ther may be bleeding manifestations and spleen and liver may be enlarged. sometimes in the severe form yoou have to give repeated blood transfusion as the haemoglobin that is produced in the body is defective..

2007-02-11 04:25:09 · answer #1 · answered by mmbaskr 3 · 0 0

No, it's not when cuts bleed too much -- that is hemophilia.

It's a disorder in which hemoglobin is misfolded. Normally, hemoglobin consists of two alpha subunits and two beta subunits that fold together into a protein that holds a heme (iron group) in the middle -- the heme is what picks up oxygen in the lungs. In thalassemias (of which there are several types), there is a mutation in one of the subunit genes such that the subunit protein doesn't fold properly -- therefore, the hemoglobin molecule doesn't assemble well, doesn't hold iron properly, and as a result, doesn't carry oxygen properly. The misfolded hemoglobin can also cause misshapen red blood cells that cause pain when going through capillaries.

2007-02-11 04:25:16 · answer #2 · answered by PasDeNom 1 · 0 0

Thalassemia is a genetic blood disorder. The red blood cells carry the haemoglobin component of our blood, which is responsible to carry the oxygen in the body. In Thalassemia there is an abnormal formation of the haemoglobin. This causes babies to be born with abnormal RBCs, which have a shorter lifespan than that of a healthy person’s. Thus, they are dependent on blood transfusion every few week as their body cannot produce enough on its own. If the transfusion is not given, more than 90% of them die before 2 years of age. To sustain their lives, blood transfusion is required every few weeks to maintain their haemoglobin levels.
There are 2 types of Thalassemia- Alpha Thalassemia and Beta Thalassemia. It depends on the type of haemoglobin chain affected.
Beta thalassemia is more common In India, eastern India, and Bangladesh. In some parts of south East Asia and china, Alpha Thalassemia is also present. But the management of both the types of thalassemia is almost the same.

2016-06-20 21:39:15 · answer #3 · answered by Narayana 2 · 0 0

Wat PasDeNom (watever it means!) said is right. It is a hemoglobinopathy where one of the chains is defective or deficient. It might be alpha or the beta chains. Another name is Coolie's anaemia or Mediterranean disease. So the oxygen carrying capacity is decreased resulting in hypoxia, fatigue etc.

2007-02-13 04:52:52 · answer #4 · answered by dharini 2 · 0 0

In thalessimia, if there is a cut, blood does not stop coming out of the body.

2007-02-11 04:01:06 · answer #5 · answered by deepak57 7 · 0 1

Thalassemia is an inherited condition. The genes received from one's parents before birth determine whether a person will have thalassemia. Thalassemia cannot be caught or passed on to another person. The clinical severity of thalassemia varies tremendously depending on the exact nature of the genes that a person inherits.
At the time of conception, a person receives one set of genes from the mother (egg) and a corresponding set of genes from the father (sperm). The combined effects of many genes determine some traits (hair color, and height for instance). Traits determined by a combination of genes often have gradations in magnitude (the difference between Michael Keaton and Kareem Abdul-Jabbar, for instance). Other characteristics are determined by a single gene pair (a person's sex, for instance). A person is either a biological male or female.
The inheritance pattern is complicated in patients with thalassemia because two sets of genes on different chromosomes cooperate to produce hemoglobin. A defect anywhere in this complex can produce thalassemia. The expression of thalassemia, therefore, more closely resembles that of height, with gradations in effect.

The genes involved in thalassemia control the production of a protein in red cells called hemoglobin. Hemoglobin binds oxygen in the lungs and releases it when the red cells reach peripheral tissues, such as the liver. The binding and release of oxygen by hemoglobin is essential for survival.
Each hemoglobin molecule contains four subunit proteins. Two of the subunit proteins are called alpha and two are called beta. Hemoglobin properly binds and releases oxygen only when two alpha subunits are connected to two beta subunits. A pair of genes located on chromosome #16 controls the production of the alpha subunits of hemoglobin. A single gene located on chromosome #11 controls the production of the hemoglobin beta subunit (Figure 1).

All cells contain pairs of idential chromosomes, one from the father and one from the mother. Each chromosome contains thousands of genes lined up in sequence. As shown in Figure 1, each person has two beta globin genes, one from the father and one from the mother. Since each chromosome #16 has two alpha globin genes, each person has a four of these genes. One chromosome #16 comes from the father, who therefore contributes two alpha globin genes to the offspring. One chromosome #16 comes from the mother who also contributes two alpha globin genes to the offspring.

A complete hemoglobin molecule has four subunits: two alpha and two beta. The two beta globin genes contribute equally to the production of beta globin subunit protein. The alpha globin genes together produce an amount of alpha globin protein that exactly equals the beta globin protein. Since there are four alpha globin genes compared to two beta globin genes, each alpha globin gene produces only about half as much protein as a beta globin gene. These keeps the overall production of subunits equal from each set of chromosomes .

Thalassemia occurs when one or more of the genes fails to produce protein, leading to a shortage of one of the subunits. If one of the beta globin genes fails, the condition is called beta thalassemia. Beta thalassemia, therefore, is due to a shortage of beta subunits. If an alpha globin gene fails, the condition is called alpha thalassemia. In this case, a shortage of alpha subunits occurs.

The fundamental abnormality in thalassemia is impaired production of either the alpha or beta hemoglobin chain.

Thalassemia is a difficult subject to explain, since the condition is not a single disorder, but a group of defects with similar clinical effects. More confusion comes from the fact that the clinical descriptions of thalassemia were coined before the molecular basis of the thalassemias were uncovered. As a result, the organizational structure is somewhat disorderly. Review of thalassemia is best approached by separately examining its genetic basis and clinical expression.
Genetic Classification of the Thalassemias
Thalassemia(1) includes disorders affecting the alpha hemoglobin chain genes and the beta hemoglobin chain gene (see Hemoglobin Overview for explanation of alpha and beta chains).

2007-02-11 12:47:29 · answer #6 · answered by Anonymous · 1 0

u need to get the spelling right
http://www.geocities.com/HotSprings/5917/whatis.htm

2007-02-12 00:20:18 · answer #7 · answered by sushobhan 6 · 0 0

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