heamophilia sumit

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In the whole history of hemophilia, the Jews were first to recognize it. he enacted a law that if a woman had two sons and that die from circumcision then her third child will not be required to circumcised. This shows that they recognize that women carried hemophilia gene and passed it down to their sons.

The hemophilia disease became royal disease because of Queen Victoria was a carrier and passed the carrier status down to many of her daughter. In those day many of the emperor had been infected due to marry with each other families.

Early 1900s- Fresh blood could be transfused after a sufficient amount of lost. Blood storage was not practice at that time. Life expectancy for people with hemophilia was 13 years although some lived longer.

1950s - Plasma became available although it did not contain enough of the needed factor.

1965 - Cryoprecipitate “Cryo” improves treatment a bit. Basically “Cryo” was what settled to the bottom of a bag of plasma. It contained more of the needed factor. It was then frozen and infused, a long hospital procedure

1970s - Factor concentrates become available.

1980s - Factor concentrates infect 80% of the people with hemophilia in the U.S with HIV, many have since passed away.

1985 - First viral inactivated factor products become available.

1992 - First non plasma derived factor becomes available using recombinant DNA technology.

1995 - “Prophy” becomes the standard of treatment in the U.S. Instead of waiting for prolonged bleeding to occur, factor is taken regularly to prevent bleeding and increase quality of life.

Hemophilia A :- Classic hemophilia,

Factor VIII deficiency .

Hemophilia B :- Christmas disease,

Factor IX deficiency.

.Excessive bleeding:- Blood will come

continuously till the ending of blood in your body.

.Easy bruising:- symptoms range from

increased bleeding after trauma, injury, or surgery to sudden bleeding with no apparent cause.

If you have inherited hemophilia, you’re born with the condition. It caused by a defect in one of the gene that how the blood make blood clotting factor VIII or IX. These gene are located on X chromosome.

Chromosomes come in pairs. Female have two X chromosome and male have one X and one Y chromosome. Only X chromosome responsible for the blood clotting factors.

A male who has the abnormal gene on X chromosome will have hemophilia and female have must be both of the X chromosome has abnormal gene for infection so in female its rare.

A female is a “carrier” of hemophilia if she has abnormal gene on her one X chromosome. Even though she does’nt have condition she will pass her gene to children.

1 per 5,000 male births

1 per 10,000 population.

85% F-VIII deficiency

10-15% F-IX deficiency

HEMOPHILIA A:B =7:1

By family history

Its diagnosed by taking sample and measuring the level of factor activity in the blood.

Treatment with replacement therapy

Desmopressin

Antifibrinolytic

Treatment of a specific bleeding site.

Protect toddlers with helmets and knee pads when riding a bicycle.

Seat belt, higher chair safety belt, stroller belt.

Furniture with sharp edges.

Keep harmful and dangerous item from small children.

Doctors who specialized in blood disorder.

Social works:- Financial issues, transportation, and mental health

Special dentist

Researchers are trying to develop ways to correct the defective gene’s that cause hemophilia.

Such as gene therapy hasn't yet develop to the point that its an accepted treatment.

Researchers continue to test gene therapy for hemophilia in clinical trails.

THE END