haemophilia

(redirected from Hemophiliacs)
Also found in: Dictionary, Thesaurus, Encyclopedia.

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 ?
I want to know who is assuming responsibility for the deaths of hemophiliacs," said Fatima Shamdeen, the mother of a young patient.
Without any push from the Administration, the hemophiliacs will get only a fraction of their due.
Members of the hemophiliac community have until October 15 to join a class action settlement offered by four major drug companies to people who were infected with HIV after using tainted blood-clotting products.
What is helping hemophiliacs to avoid immune deficiencies is not harmful and ineffective antiviral drugs, but new purified blood products that do not contain foreign proteins.
Perhaps the situation can be clarified by looking more closely at the events which led up to the infection of so many hemophiliacs with HIV-1 virus.
As often happens with infant hemophiliacs, Ryan White bled profusely aftr circumcision.
Taiwanese hemophiliacs were unaware of this strategy and the defendants' risky manufacturing practices until 2003 when the New York Times published an article summarizing internal documents filed into evidence in US hemophiliacs' cases.
They are known to have caused HIV, notably among hemophiliacs, and hepatitis, and the ministry has been looking into the extent of the damage.
Matsumura is charged with professional negligence that resulted in the deaths of hemophiliacs from AIDS.
The blood plasma concentrates upon which they relied afforded them a freedom which had been denied earlier generations of hemophiliacs and seemed in many ways a blessing.
Because hemophiliacs lack a clotting factor to make bleeding stop, any surgery poses grave risks--and costs.
In light of the importance of the correlation argument, it is astonishing that no controlled studies have been done for three of the major risk groups: transfusion recipients, hemophiliacs, and drug abusers.