Haemophilia

HAEMOPHILIA

In the human body, each cell contains 23 duads of chromosomes,

one of each pair inherited through the egg from the mformer(a), and the

other inherited through the spermatozoan of the father. Of these

chromosomes, those that determine sex be X and Y. Females guide XX

and males have XY. In addition to the information on sex, the X

chromosomes carry determinants for a number of other features of

the body including the levels of grammatical constituent octonary and factor IX.1 If

the genetic information determining the factor VIII and IX level is

defective, haemophilia results. When this happens, the protein

factors needed for normal blood turn be effected. In males,

the single X chromosome that is effected cannot neutralize for the

lack, and hence will show the defect. In females, however, only one

of the dickens chromosomes will be abnormal. (unless she is unlucky

enough to inherit haemophilia from both sides of the family, which

is rare.)2 The other chromosome is likely to be normal and she can

therefore compensate for this defect.

There are two types of haemophilia, haemophilia A and B.

Haemophilia A is a hereditary disoblige in which shed blood is due to

deficiency of the coagulation factor VIII (VIII:C)3.



In most of the

cases, this coagulant protein is reduced but in a rare amount of

cases, this protein is present by immunochemical assay but defective.4

Haemophilia A is the most common severe release disorder and

approximately 1 in 10,000 males is effected. The most common types

of inclineing are into the joints and muscles. Haemophilia is severe

if the factor VIII:C levels are less that 1 %, they are moderate if

the levels are 1-5% and they are mild if they levels become 5+%.5

(2)

Those with mild haemophilia bleed only in response to major trauma

or...

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