haemophilia

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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.

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 ?
With 'consent' listed in its Terms of Reference, it is surely incumbent on this inquiry to investigate when and why haemophiliacs became involved in any medical research programmes used in the monitoring and treatment of infectious diseases.
Sue says that not only were haemophiliacs given contaminated blood products and not told about it, but they were also targeted like a 'group of guinea pigs.'.
Advising the inquiry on how to proceed, he called for investigation into claims by his clients of a coverup by NHS bosses and ministers, and allegations that some involved "profited" from the "exploitation of haemophiliacs".
"Documents have been uncovered that provide evidence government ministers gave their approval to the surveillance of haemophiliacs who had been infected with Hepatitis C and HIV.
"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.
Successive governments have offered settlements of up to pounds 60,000 to the haemophiliac patients involved in the controversy, but have so far refused to admit any fault on their part for using the contaminated products.
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".
The hearings were starting amid claims on BBC TV that Britain's doctors ignored warnings about using haemophiliacs to test out new blood products.
The probe will look at the deaths of 1,757 haemophiliacs after mistakes made in the early 1970s and early 1980s.
A HAEMOPHILIAC patient is considering legal action after being told he may be at risk of the human form of mad cow disease.
A no-fault payment was made by the Government in 1991 to haemophiliacs who had been infected with HIV.