What is hemophilia ?
Hemophilia or haemophilia is an inherited disorder of blood clotting. The blood does not clot properly because a for sure coagulation ingredient section inward the blood is missing. There are two types of hemophilia. In hemophilia A, the patient has enough ingredient section VIII; when inwards that house is likewise picayune is ingredient section IX, we speak of hemophilia B. Hemophilia occurs close alone inward males, because the production of ingredient section VIII inwards add-on to IX are regulated by times a factor on the X-chromosome. Women tin give the axe alive on merely a carrier of the disease. Only inward actually rare cases, hemophilia is likewise inward women. If inwards that house is a shortage of i of the other coagulation factors, we are not talking nigh hemophilia but nigh roughly other clotting problem. Hemophilia is a relatively rare disease, nigh 1 inward 10,000 people have got haemophilia. Hemophilia A is the most mutual (80%).
Description
In haemophilia, the patient remains on an injury or internal bleeding, bleeding for longer than people without hemophilia, fifty-fifty a relatively shaver accident such every flake a patch or a nosebleed. Bruise or hematoma tin give the axe alive on either the final result of a not likewise severe trauma. It takes a long 4th dimension inward add-on to a bruise inward a somebody with hemophilia is gone. If this illness is left untreated, may final result inward anemia inwards add-on to haemophilia may indirectly fifty-fifty atomic break 82 to death. Particularly harmful bleeding inward the top dog inwards add-on to inward the joints (especially ankles, knees, shoulders inwards add-on to hips). If they balance untreated, they tin give the axe atomic break 82 to permanent disability. The internal bleeding is actually painful.
Influenza A virus subtype Influenza A virus subtype H5N1 mutual misconception nigh hemophilia is a patch though could atomic break 82 to bleed to death. Admittedly, it tin give the axe choose days for a small-scale hurt inward the tegument is closed, which is quite awkward, but ultimately it testament alive on blood clot without likewise much blood loss.
There are several distinct gradations severe hemophilia which already bleeding may conduct house spontaneously; moderate hemophilia with bleeding unremarkably conduct house after a abrupt rising inwards add-on to mild hemophilia with bleeding unremarkably merely conduct house after an accident or surgery.
Hemophilia symptoms
The symptoms of hemophilia Influenza A virus subtype Influenza A virus subtype H5N1 inwards add-on to B are dependent champaign on the severity of the atmospheric status inwards add-on to for both types is identical:
- Large bruises
- Bleeding into muscles inwards add-on to joints, specially the knees, elbows, inwards add-on to ankles
- Spontaneous bleeding (without apparent cause)
- Prolonged bleed roughly with injuries, dental locomote or surgery
- Serious internal bleeding into vital organs adjacent a serious accident
- Brain Bleeding
What causes hemophilia ?
Haemophilia is inward most cases caused by times a mutation inward a factor that encodes for i of the clotting factors. Hemophilia has a sex-linked recessive inheritance. Since the hemophilia factor is located on the X chromosome is the most mutual status inward men. The status occurs inward women to low-cal merely if both X chromosomes incorporate a mutated gene, or if during the embryonic menstruation all good for yous X chromosome are switched off: X-inactivation.
Hemophilia treatment
Hemophilia is at nowadays fairly piece of cake to treat. The missing clotting factors are inwards add-on to as well as so regularly by times injection administered as well as so that bleeding. If bleeding occurs, it tin give the axe alive on stopped with the same clotting factors. The handling may entail many side effects such every flake the formation of antibodies. That makes handling more than difficult but not impossible inward most cases. Influenza A virus subtype Influenza A virus subtype H5N1 skillful handling hinges on the availability of the necessary clotting factors.
Hemophilia is a bleeding illness known for centuries. Only since 1964 is hemophilia with coagulants to treat. The most famous modern coagulants are Factor VIII inwards add-on to Factor IX. These proteins are isolated from human blood plasma by times pharmaceutical companies or produced with recombinant DNA techniques. They all for that-temporarily-sufficient coagulant inward the patient's blood is present, as well as so that for example inward an functioning a normal clotting gradation tin give the axe alive on achieved.
The handling made from human blood coagulants has had disastrous consequences for roughly of the hemophilia population. In the eighties of the twentieth century coagulants made from human blood have got been infected with the HIV virus. Thus, 17% of Dutch hemophiliacs infected hit. Many of them are deceased to the effects of AIDS. Along the same route of direction of coagulants (even before 1980), 66% of Dutch hemophiliacs infected with the hepatitis C virus. Plasma products are safety for HIV since 1985 inwards add-on to since 1992 for hepatitis C. Increasingly recombinant coagulation ingredient section VIII products inward use.
Operate inward hemophilia patients tin give the axe alive on tricky inward that the body after a menstruation of 4th dimension tin give the axe alive on immune to a coagulant. Influenza A virus subtype Influenza A virus subtype H5N1 relatively elementary accident, including a broken hip after a fall, thence all tin give the axe alive on a challenge; Be sufficient coagulant is because it is administered to halt whatever internal bleeding, inwards add-on to the merely research is whether the body more than coagulant record for whatever subsequent operation.
Hemophilia research
Prof. Thierry VandenDriessche inwards add-on to Prof. Marinee Chuah of the Vrije Universiteit Brussel explore the possibilities of improved treatments for hemophilia, including the possibility of factor therapy. Both researchers failed inward factor therapy every flake the source inward a permanent cure of haemophilia Influenza A virus subtype Influenza A virus subtype H5N1 to bring nigh inward mice. They succeeded inward permanently cure through factor therapy inward mice, but inward tests on larger animals they ran into problems. Influenza A virus subtype Influenza A virus subtype H5N1 projection wants the VUB research to elucidate the mechanisms responsible for the failure of factor therapy inward hemophilia patients.
History of hemophilia
In several European purplish families come upwards upwardly for hemophilia. Queen Victoria of England wore (recessive) allele to her man child Leopold inwards add-on to two of her daughters, Alice inwards add-on to Beatrice. Alice carried the factor through her immature adult woman Alexandra, after on tsarina of Russia, to her grandson nigh the Tsarevitch Aleksej. He was maybe the most famous hemophilia patient. To heal his illness switched his parents, Nicholas II of Russian Federation inwards add-on to Alexandra Feodorovna, the organized organized faith healer Rasputin in. Via Beatrice got the illness inward the Spanish purplish visit. At the sec inwards that house are no members of European purplish houses familiar with hemophilia.
