haemophilia

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

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 ?
This is what it's like to care for a hemophiliac, or be a hemophiliac: treading the fragile-as-glass balance between the desire to explore everything that life has to offer and yet keep within certain bounds to avoid fatal consequences.
Echavez said four patients died in government hospitals in Manila and in Davao, while one hemophiliac patient from Bohol died while on the way to Cebu to seek treatment.
"I want to know who is assuming responsibility for the deaths of hemophiliacs," said Fatima Shamdeen, the mother of a young patient.
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.
Although the operation took longer than expected because the hemophiliac patient bled more than usual, both he and his brother are progressing well, the officials said.
In 1995 the National Academy of Sciences' Institute of Medicine found that the federal government had failed to regulate the plasma industry properly or inform patients of risks from blood products that infected the 10,000 hemophiliacs. After years of lobbying by the hemophilia community, last year Congress unanimously passed-and Clinton signed-the Ricky Ray Hemophilia Relief Fund Act, to set up a $750 million fund to compensate those infected with contaminated blood because of government neglect.
Individual cases brought by infected hemophiliacs have not fared well.
The way the good news about hemophiliacs is expressed in an HIV-obsessed research culture is by lengthening the "latency period," a statistical fudge-factor that is different in every risk group and can be adjusted as necessary to explain why so many HIV-positive people are not sick.
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.
Unheated blood-clotting agents were used in Japan to treat hemophiliacs, newborns and traffic accident victims from the 1970s until the late 1980s when safer heated products were brought to the market.
The ministry has repeatedly said there were no such minutes or any other record of the discussions of the panel, which decided in 1984 to allow the continued use of such products, from which hemophiliacs contracted HIV, the AIDS-causing virus.