haemophilia

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Related to Hemophiliacs: classical hemophilia, bleeder's disease, hemophilic

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 ?
The tragedy of HIV infection among hemophiliacs has been blamed on negligence by the state, doctors and pharmaceutical companies.
Since the mid-1980s, hemophiliacs have accused the companies of putting their profits over safety by knowingly selling HIV-infected clotting products made from donated blood.
Abe was not indicted over other infections, but 23 of 48 hemophiliacs treated at Teikyo University Hospital tested HIV positive in September 1984.
The anguish suffered by hemophiliacs was plain to see at the conference, where a number of mothers stood up to vent their anger at the ministry's general manager, Dr.
In April, 18-year-old hemophiliac Ryan White dies; he had received an HIV-tainted blood transfusion five years earlier.
Many patient records are now unavailable, and because an AIDS test was not developed until later in the epidemic, it is difficult to pinpoint when foreign hemophiliacs were infected with HIV -- before Cutter began selling its safer medicine or afterward, the Times reported.
In March 1996, drugmakers settled with the hemophiliacs in the Tokyo and Osaka district courts, agreeing to pay 45 million yen per victim.
Surgeons at the University of Tokyo Hospital on Thursday morning completed a marathon operation to transplant part of a liver into a hemophiliac man who contracted HIV and hepatitis C from contaminated blood products.
For example, five years before Duesberg and Rasnick's assertion that pneumonia and candidiasis are "almost the only two" AIDS-defining conditions seen in hemophiliacs, a European hemophiliac cohort found that of 37 diagnosed with AIDS, 6 had toxoplasmosis, 3 had wasting syndrome, 3 had dementia, 2 had MAC, 1 had CMV and 1 had lymphoma as their AIDS-diagnosing illness.
Plaintiffs allege these companies failed to screen out blood obtained from high risk donors (prisoners, intravenous drug users and male homosexuals) who were at high risk of infection with transmissible viruses including Hepatitis and HIV and whose plasma byproducts should not have been used for treatment of hemophiliacs.
It is not an easy choice," said David Shrager, the Philadelphia attorney who is lead counsel for the hemophiliacs.
5) Mounting evidence indicates that what is called "AIDS" in hemophiliacs is caused not by HIV but by the (curable) effect of foreign proteins from treatment with unpurified Factor 8, the blood coagulant that saves hemophiliacs from an early death due to prolonged internal bleeding.