haemophilia

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haemophilia

he·mo·phil·i·a

(hē'mō-fil'ē-ă)
An inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhages, spontaneous or traumatic, due to a defect in the blood coagulating mechanism.
Synonym(s): haemophilia.
[hemo- + G. philos, fond]

haemophilia

An X-linked recessive blood clotting disorder causing a life-long tendency to excessive bleeding. It cannot be passed from father to son because the father transmits only the Y chromosome to his sons. All the daughters of a haemophilic man are carriers of the gene but do not suffer the disease. There is a 50% chance that each of their sons will be a haemophiliac. Females can acquire the disease only if both X chromosomes carry the gene. Haemophilia A is due to the absence of Factor VIII, one of the coagulation factors. Haemophilia B (Christmas disease) is due to deficiency of Factor IX. Both feature bleeding, either spontaneous or on minor trauma, most commonly into the joints. This causes severe pain, swelling and muscle spasm. Repeated episodes lead to damage and severe joint disability. Tooth extraction or external injury are followed by prolonged bleeding. Spontaneous bleeding may occur into the bowel. Haemophilia is treated by repeated injections of Factor VIII or IX obtained from donated blood.

haemophilia

a rare human blood disorder in which BLOOD CLOTTING is deficient, resulting often in severe bleeding internally and externally. The condition is due to a lack of fibrin in the blood and is controlled by two closely linked genes on the X-CHROMOSOME that are responsible for the production of different clotting factors. Haemophilia A individuals lack antihaemophilic globulin (AHG) while haemophilia B individuals lack plasma thromboplastin. Males carrying the mutant ALLELE of either locus or (much more rarely) females homozygous for the recessive mutant alleles of either locus will be affected, although heterozygous females have normal blood. Haemophilia A is by far the most common form of the disease (about 80%) and can be treated by transfusions of AHG.

haemophilia

inherited coagulation cascade disorder, characterized by lifelong tendency to haemorrhage
  • haemophilia A; HA deficiency of factor VIII; X-linked recessive condition, expressed almost exclusively in males; characterized by delayed clotting (decreased formation of thromboplastin and reduced conversion of prothrombin)

  • haemophilia B; HB; Christmas disease see disease, Christmas

  • vascular haemophilia; von Willebrand's disease see disease, von Willebrand's

he·mo·phil·i·a

(hē'mō-fil'ē-ă)
Inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhages, spontaneous or traumatic, because of a defect in the blood-coagulating mechanism.
Synonym(s): haemophilia.
[hemo- + G. philos, fond]
References in periodicals archive ?
Carol, from Newcastle, has spent more than two decades campaigning for justice after losing her haemophiliac husband Peter Longstaff in 2005 to HIV and hepatitis C contracted from infected NHS blood products in the 1970s and 1980s.
Together we care" will be the mainstay of the managed care of haemophiliacs for the years to come, since this will be the basis for improving the quality of health assistance to all patients and, ultimately, their quality of life and life expectancy.
The investigation is examining the deaths of 1,757 haemophiliacs as a result of exposure to HIV and hepatitis C in what fertility expert Lord Winston dubbed "the worst treatment disaster in the NHS".
A no-fault payment was made by the Government in 1991 to haemophiliacs who had been infected with HIV.
The appeal for help from the haemophiliacs comes as it was revealed that over EUR289 million has been awarded in 1,488 claims from people affected by contaminated blood products under the Hepatitis C compensation scheme.
On Tuesday, Pete and partner Carol Grayson will join other haemophiliacs in a march to Downing Street, before handing in letters to Number 10.
But there have been warnings of a revolt among haemophiliacs if the American deal goes ahead.
Campaigners said about 365 haemophiliacs and up to 1200 other people were infected.
Papers leaked to a national newspaper show health officials were pre-occupied with controlling costs as the scandal of HIV - and hepatitis C - infected blood transfusions threatened the lives of haemophiliacs in the 1980s.
According to him, all male children of haemophiliacs are normal (they do not develop haemophilia) while all female children become carriers of the disease but do not suffer from it.
The Government agreed a compensation deal with haemophiliacs in 1991, but lawyers for Haydn and his wife have argued it was too little.