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 ?
Cartilage damage in the haemophilic joints: pathophysiology, diagnosis and management.
The most common cause of bleeding in haemophilic patients in a dental office is procedure-induced trauma.
Between 1996 and Oct 2001 there were 17 cases of adverse thrombotic events reported in more than 480,000 standard doses of rVIIa in haemophilic patients (27).
Two known haemophilic females attained menarche and came with uncontrolled menstrual bleeding, which got corrected with calculated factor replacement.
Tenders are invited for Medicine for Anti Haemophilic Factor
Joint distraction results in clinical and structural improvement of haemophilic ankle arthropathy: a series of three cases," Haemophilia, vol.
Force during the maximu isometric voluntary contraction of the quadriceps femoris in haemophilic patients.
This study was proposed to evaluate the rate of sustained response and tolerance to combined therapy with the association of interferon alpha and ribavirin for chronic hepatitis C infection in haemophilic patients at Hospital Brigadeiro.
He told the tribunal that a Dublin hospital had not told him that his haemophilic son had HIV until two years after he was tested.
Tenders are invited for Supply Of Haemophilic Drugs To Tamilnadu Medical Services Corporation Limited For The Year 2016-2017.
Tenders are invited for Inj Anti Haemophilic Factor Ix 600 I.