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Topic: Haemophilia B


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  The challenge arising from the cost of haemophilia care: an audit of haemophilia treatment at Auckland Hospital
Number of people with haemophilia seen at Auckland Haemophilia Centre There are 125 people with haemophilia A (47 severe, 18 moderate, 60 mild) and 43 with haemophilia B (19 severe, 17 moderate, 7 mild) registered at Auckland Haemophilia Centre.
One case with haemophilia B was on prophylaxis at an appropriate dose (recommended dose is 25 iu/kg twice a week, ie, 2600 iu/kg/year).
Haemophilia treatment is, however, relatively unique in that the extremely high costs are incurred primarily by a relatively small patient pool for the prevention of pain and joint destruction rather than for disease cure.
www.nzma.org.nz /journal/116-1180/561   (3443 words)

  
  Haemophilia - MSN Encarta
Haemophilia, hereditary blood disease characterized by the inability of blood to clot, or coagulate, leading to excessive bleeding, even from minor injuries.
The most common form, haemophilia A, is observed in 80 per cent of haemophiliacs and is caused by a lack of factor VIII; in the second most common, haemophilia B (Christmas disease), factor IX is missing.
A famous case of the transmission of haemophilia involved Queen Victoria, whose daughters carried the disease to the Spanish and Russian royal houses.
uk.encarta.msn.com /encyclopedia_761570021/Haemophilia.html   (447 words)

  
 Haemophilia B
Hemophilia B is similar to Hemophilia A. Both are abnormality of blood coagulation.
Hemophilia B is caused by the deficiency of Factor IX.
It has to be diagnosed accurately because clinically it is indistinguishable from factor VIII deficiency - Hemophilia A. Factor IX is one of a group of six proteins synthesized in the liver that require vitamin K for biologic activity.
www.manbir-online.com /diseases/haemophilia.3.htm   (70 words)

  
 Haemophilia A and B
Haemophilia A and B are two diseases characterised by slow and inefficient formation of blood clots leading to prolonged bleeding from minor wounds and spontaneous internal haemorrhage.
Haemophilia A is the commoner of the two diseases, affecting one in 5000 males, whereas haemophilia B affects approximately one in 40 000 males.
Haemophilia A is one of the better-known inherited disorders because Queen Victoria was a carrier and transmitted the disease to several European royal families.
genome.wellcome.ac.uk /doc_WTD020862.html   (562 words)

  
 Discussion Forum - Bit of help needed for a project   (Site not responding. Last check: 2007-10-15)
Haemophilia is characterised as "severe" when the activity of the affected clotting factor is less than 1% of normal.
Haemophilia B is inherited in the same way as haemophilia A, but it is five times less common.
The incidence of inhibitors complicating treatment of haemophilia A and B is approximately 10 to 30% in haemophilia A and 3 to 5% in haemophilia B patients.
www.wfh.org /publicForum/topic.asp?TOPIC_ID=31   (570 words)

  
 NHS Direct - Health encyclopaedia -Haemophilia
If you have haemophilia, your blood will not clot normally and you may bleed for longer than normal, or you may bleed internally, particularly into joints such as your knees, elbows and ankles.
Haemophilia A is also known as classical haemophilia and is the cause of about 80% of cases.
Haemophilia B is also known as Christmas disease and is the cause of about 20% of cases.
www.nhsdirect.nhs.uk /articles/article.aspx?articleId=183   (289 words)

  
 The Haemophilia Society - HAEMOPHILIA: GENERAL INFORMATION
Haemophilia B (Christmas Disease) is caused by a deficiency in factor IX (factor 9).
Haemophilia A and B are classed as mild, moderate or severe depending on the level of clotting factor in the individuals blood e.g.
Haemophilia A and B are treated by replacing the missing clotting factor.
www.haemophilia.org.uk /publications/haemophilia_general.htm   (3620 words)

  
 Haemophilia | DoctorNDTV: Health Information on Haemophilia
Haemophilia is a genetic (inherited) bleeding disorder caused by the deficiency of substances called clotting factors that are necessary to control bleeding in the body.
Generally, haemophilia A and B are passed from mother to son through one of the mother's genes.
Haemophilia is primarily treated by replacing the absent or abnormal clotting factors to prevent severe blood loss and complications from bleeding.
www.doctorndtv.com /topicsh/Haemophilia.asp   (972 words)

  
 Background © Haemophilia Alliance
There are several types of haemophilia, of which the most common is haemophilia A (factor VIII deficiency) with an incidence of between 1:5,000 and 1:10,000 males.
Haemophilia B (factor IX deficiency) is less common, with an incidence of between 1:35,000 and 1:50,000 males.
Although the delivery of haemophilia care is expensive, the lives of patients and their families can be transformed by high quality care.
www.haemophiliaalliance.org.uk /docs/background.htm   (495 words)

  
 Breathofair - Haemophilia - About Haemophilia
Haemophilia is an inherited bleeding disorder that affects mostly males.
Haemophilia is a lifelong inherited genetic condition, which affects females as carriers and males who inherit the condition.
Haemophilia A is caused by a deficiency of factor VIII, and Haemophilia B is caused by a deficiency of factor IX.
www.breathofair.co.uk /haemophilia/about.asp   (616 words)

  
 Haemophilia and Thrombophilia
The appropriate treatment for haemophilia depends on the severity of the condition, and the patient’s circumstances.
Haemophilia A is treated with factor VIII concentrate and haemophilia B with factor IX.
Factor IX remains in the circulation for longer than factor VIII so in haemophilia A, infusions are typically given three times a week, and in haemophilia B normally twice a week.
www.baxterhealthcare.co.uk /therapies/sub/haem_thromb.html   (678 words)

  
 Statements of Principles — Haemophilia — diagnostic protocol   (Site not responding. Last check: 2007-10-15)
The diagnosis of haemophilia can be accepted by the Repatriation Commission when it is confirmed by a general medical practitioner or specialist as the final diagnosis or on advice by a Departmental Medical Officer.
Haemophilia B, also known as Christmas disease, is indistinguishable clinically from haemophilia A, but accurate laboratory diagnosis is critical as treatment requires the administration of a different plasma fraction.
Haemophilia C involves the inherited deficiency of factor XI, and is especially common in Ashkenazi Jews.
www.dva.gov.au /pensions/statemnt/p010dp.htm   (746 words)

  
 Novo Nordisk Australasia - Haemophilia
Haemophilia is an inherited (congenital) bleeding disorder that occurs frequently in males.
In the case of haemophilia A, there is a problem with factor VIII and in haemophilia B it is factor IX.
Acquired haemophilia is not inherited, and cannot be passed on in the family.
www.novonordisk.com.au /documents/article_page/document/Haemophilia.asp   (244 words)

  
 Haemophilia   (Site not responding. Last check: 2007-10-15)
Haemophilia belongs to a group of inheritable blood disorders that includes haemophilia A, haemophilia B (Christmas disease) and Von Willebrand disease.
In haemophilia, the blood's ability to clot is severely reduced because an essential clotting factor is partly or completely missing.
Haemophilia A is a deficiency of an essential clotting factor called factor VIII, which is normally produced in the liver.
www.intellimeds.com /conditions/haemophilia.aspx   (665 words)

  
 Genes and You - Genetic Disorders - Haemophilia
Haemophilia is an inherited and lifelong blood disorder where an essential blood clotting factor is either partly or completely missing.
There are two types of Haemophilia, Haemophilia A in which factor VIII is affected and the rarer Haemophilia B, also known as Christmas Disease in which factor IX is affected.
A man with Haemophilia does not pass the faulty gene onto his sons because they receive a copy of his Y chromosome (their X chromosome comes from the mother).
www.gig.org.uk /genesandyou_haemophilia.htm   (718 words)

  
 Haemophilia B - Wikipedia, the free encyclopedia
Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene.
It is the second most common form of haemophilia, rarer than haemophilia A.
It is inherited X-linked recessive, which explains why - as in haemophilia A - only males are generally affected.
en.wikipedia.org /wiki/Haemophilia_B   (211 words)

  
 BloodGivers.com --- Blood Disorders - Haemophilia
Haemophilia A is a type of this disorder where Clotting Factor VIII is missing.
Haemophilia B also called Christmas Disease is the type caused by an abnormality in the clotting protein Factor IX.
There also is a 50% chance the carrier will pass the Haemophilia gene on to her female offspring, meaning that there is a 50% chance each of her daughters will also be carriers.
www.bloodgivers.com /haemophilia.asp   (876 words)

  
 The Basics   (Site not responding. Last check: 2007-10-15)
When a person with haemophilia is injured, he (or, very occasionally, she) does not bleed harder or faster than normal, but will have prolonged bleeding because his blood cannot make a firm clot.
People with haemophilia are vulnerable to episodes of uncontrolled internal bleeding, usually into a joint or a muscle.
The most common type of haemophilia is factor VIII deficiency, or haemophilia A. Recent studies indicate that around 87% of haemophilia cases globally are Haemophilia A*.
www.haemophilia.org.nz /overview.html   (251 words)

  
 Evidence for gene transfer and expression of factor IX in haemophilia B patients treated with an AAV vector - Nature ...
Haemophilia B is the bleeding diathesis resulting from mutations in the gene encoding F.IX (F9), a proenzyme required for generation of a fibrin clot.
The clinical severity of haemophilia B correlates closely with circulating levels of F.IX: individuals with less than 1% of normal activity are severely affected, whereas those with levels 1−5% of normal generally have a more moderate course.
The difference in factor levels seen among patients A, B and C may be accounted for by biologic variation, but another factor that may be important is the presence or absence of circulating F.IX antigen (Table 1).
www.nature.com /ng/journal/v24/n3/full/ng0300_257.html   (3773 words)

  
 HAEMOPHILIA, VON WILLEBRAND SYNDROME and other coagulation defects: Contact a Family - for families with disabled ...
Haemophilia occurs in about 1 in 10,000 of the population in the UK, with haemophilia A (six thousand patients) being five times as frequent as haemophilia B (one thousand two hundred and fifty patients).
These are generally milder than haemophilia and are usually autosomal recessive, only being fully expressed in the homozygous (having two identical forms of the gene, one inherited from each parent) or doubly heterozygous (having two different forms of a particular gene, one inherited from each parent) state.
Haemophilia A and B are sex (X) linked and it is usually possible to determine the causative genetic abnormality but new spontaneous mutations are not uncommon.
www.cafamily.org.uk /Direct/h18.html   (1296 words)

  
 Haemophilia B
Haemophilia B (or Christmas disease) is caused by a deficiency of a protein in the blood; Factor IX.
Moderate haemophilia is found in people with factor levels of 2-5%, they may experience spontaneous bleeds into joints and muscles.
The major problem in severe Haemophilia B is painful bleeding into joints, mainly the knees, ankles and elbows.
www.health.qld.gov.au /haemophilia/haemb.asp   (211 words)

  
 Haemophilia B (Factor IX Deficiency) - Patient UK
Haemophilia B is also known as Christmas disease after the patient in whom it was classically described.
Haemophilia A is due to a deficiency of clotting factor VIII whilst haemophilia B is due to a deficiency of clotting factor IX.
Haemophilia A is about 5 times as common as haemophilia B. The latter has a prevalence of around 1 in 25,000 to 30,000 males.
www.patient.co.uk /showdoc/40024634   (2478 words)

  
 Children First for Health - Teens - Health - Conditions - Haemophilia   (Site not responding. Last check: 2007-10-15)
Haemophilia is when a clotting factor (something that makes blood go thick and harden) is either partly or completely missing.
Most people who have haemophilia inherited it, but about a third of people are the first in their family who have it.
Haemophilia can usually be treated by replacing the missing clotting factor – that’s the factor VIII (factor 8) or factor IX (factor 9).
www.childrenfirst.nhs.uk /teens/health/conditions/h/haemophilia.html   (472 words)

  
 HAEMOPHILIA
Haemophilia is a rare inherited bleeding disorder in which the blood does not clot normally.
Babies born with haemophilia are missing or have a low level of a protein needed for normal blood clotting or blood coagulation.
Haemophilia A and B are classified as mild, moderate, or severe, depending on the amount of clotting factor VIII (8) or IX (9) in the blood.
www.medic8.com /healthguide/articles/haemophilia.html   (4004 words)

  
 © The Centre for Genetics Education   (Site not responding. Last check: 2007-10-15)
Haemophilia is a genetic condition that causes people to keep on bleeding for a long time unless treated.
Females with a faulty clotting factor gene on their X chromosome would not usually be affected with haemophilia A or B but would be carriers of the mutation and could pass it onto their children: they are genetic carriers of haemophilia.
If a mother is a carrier of a mutation in her factor VIII or factor IX genes on the X chromosome, she has a 1 in 2 chance of passing the faulty gene on to her son.
www.genetics.com.au /factsheet/38.htm   (890 words)

  
 Bayer Biological Products EU - About Haemophilia
Haemophilia B - caused by deficient or defective clotting factor IX.
Haemophilia is an X-linked (or sex linked) inherited disorder that can be passed along by females who carry a recessive gene for the disease.
Haemophilia, a potentially life-threatening disease, is categorized as mild, moderate, or severe, depending on the amount of bleeding that results from minor injuries.
www.biological.com /abouthaemophilia.cfm   (334 words)

  
 North West Group of the Haemophilia Society   (Site not responding. Last check: 2007-10-15)
Haemophilia is a blood condition in which an essential clotting factor is either partly or completely missing.
Treatment for haemophilia is usually by replacement of the missing clotting factor.
In severe haemophilia this is by injecting it on a regular basis (called prophylaxis) to help prevent bleeding.
www.haemophilianw.co.uk /haemophilia.htm   (288 words)

  
 Learn about Haemophilia
The defect lies in the clotting mechanism of the blood and is characterised by a life long tendency to excessive internal or external haemorrhage.
Haemophilia ‘B’ also known as Christmas disease is due to the deficiency of factor IX.
Both Haemophilia "A" and Haemophilia "B" are treated by replacing the missing clotting factor, the commonest source is human blood plasma.
fatimid.org /haemophilia.htm   (457 words)

  
 Families first for health: Dear Doc - haemophilia   (Site not responding. Last check: 2007-10-15)
Haemophilia is an inherited condition affecting boys and men in which a vital protein involved in blood clotting is missing.
About a third of children with haemophilia have no previous family history though and for these children, the haemophilia gene has ‘mutated’ or changed either in their own genetic make-up, or that of their mother.
In severe haemophilia, the clotting factor is given in the form of regular injections (prophylaxis) which help prevent bleeding.
www.childrenfirst.nhs.uk /families/health/dear_doc/haemophilia.html   (509 words)

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