haemophiliac$33400$ - translation to ολλανδικά
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haemophiliac$33400$ - translation to ολλανδικά

HUMAN GENETIC DISEASE THAT IMPAIRS THE BODY'S ABILITY TO MAKE BLOOD CLOTS, A PROCESS NEEDED TO STOP BLEEDING
Hemophilia; Hemophiliacs; Hæmophilia; Hemophilic; Haemophilic; Hemophiliac; Haemophila; Hemophelia; Haemophiliacs; Haemophiliac; Hemophilla; Antihemophilic globulin; Excessive bleeding disease; Non-stop bleeding disease; No blood coagulation disorder; Hemophilic arthropathy; Haemophillia; Gene therapy for haemophilia
  • An illustration of a woman with hemophilia
  • Haemophilia in European royalty
  • Commercially produced factor concentrates such as "Advate", a recombinant Factor VIII, come as a white powder in a vial which must be mixed with sterile water prior to [[intravenous]] injection.
  • contaminated blood products]].
  • [[X-linked recessive inheritance]]

haemophiliac      
adj. hemofilisch (erfelijke ziekte, het bloed klontert niet)

Βικιπαίδεια

Haemophilia

Haemophilia, or hemophilia (from Ancient Greek αἷμα (haîma) 'blood', and φιλία (philía) 'love of'), is a mostly inherited genetic disorder that impairs the body's ability to make blood clots, a process needed to stop bleeding. This results in people bleeding for a longer time after an injury, easy bruising, and an increased risk of bleeding inside joints or the brain. Those with a mild case of the disease may have symptoms only after an accident or during surgery. Bleeding into a joint can result in permanent damage while bleeding in the brain can result in long term headaches, seizures, or a decreased level of consciousness.

There are two main types of haemophilia: haemophilia A, which occurs due to low amounts of clotting factor VIII, and haemophilia B, which occurs due to low levels of clotting factor IX. They are typically inherited from one's parents through an X chromosome carrying a nonfunctional gene. Rarely a new mutation may occur during early development or haemophilia may develop later in life due to antibodies forming against a clotting factor. Other types include haemophilia C, which occurs due to low levels of factor XI, Von Willebrand disease, which occurs due to low levels of a substance called von Willebrand factor, and parahaemophilia, which occurs due to low levels of factor V. Haemophilia A, B, and C prevent the intrinsic pathway from functioning properly; this clotting pathway is necessary when there is damage to the endothelium of a blood vessel. Acquired haemophilia is associated with cancers, autoimmune disorders, and pregnancy. Diagnosis is by testing the blood for its ability to clot and its levels of clotting factors.

Prevention may occur by removing an egg, fertilizing it, and testing the embryo before transferring it to the uterus. Human embryos in research can be regarded as the technical object/process. Missing blood clotting factors are replaced to treat haemophilia. This may be done on a regular basis or during bleeding episodes. Replacement may take place at home or in hospital. The clotting factors are made either from human blood or by recombinant methods. Up to 20% of people develop antibodies to the clotting factors which makes treatment more difficult. The medication desmopressin may be used in those with mild haemophilia A. Studies of gene therapy are in early human trials.

Haemophilia A affects about 1 in 5,000–10,000, while haemophilia B affects about 1 in 40,000, males at birth. As haemophilia A and B are both X-linked recessive disorders, females are rarely severely affected. Some females with a nonfunctional gene on one of the X chromosomes may be mildly symptomatic. Haemophilia C occurs equally in both sexes and is mostly found in Ashkenazi Jews. In the 1800s haemophilia B was common within the royal families of Europe. The difference between haemophilia A and B was determined in 1952.