Hemophilia Glossary
A B E G H I L M P R S T
Arthritis: Inflammation of a joint, usually accompanied by pain and swelling.
Arthroscope: A surgical instrument that has a lens and a lighting system through which the structures inside the joint are transmitted to a miniature television camera attached to the end of the arthroscope.
Arthroscopic surgery: A procedure to visualize, diagnose and treat joint problems. The name comes from the Greek word arthron, which means joint, and skopein, which means to look at. In arthroscopic surgery, an orthopaedic surgeon uses an arthroscope, to see the inside of a joint. After making an incision about the size of a buttonhole in the patient’s skin, a sterile salt water solution is injected to distend the joint. The arthroscope, an instrument the size of a pencil or smaller, is then inserted into the joint.
Arthroscopic synovectomy: Arthroscopic surgery to remove joint lining tissue (synovium) that is chronically inflamed.
Arthroscopy: The surgical technique of inserting a tiny instrument, an arthroscope, into a joint. The arthroscope projects an image of the joint onto a television screen.
Return to the top
Beta Radiation: Low energy radiation that penetrates only a small distance into human tussues. Radioactive phosphorus is one of the substances that naturally releases beta radiation, thus making it well suited for use in radionuclide synovectomies.
Elamax cream: A mixture of topical anesthetic drugs that are applied to the skin to help reduce the discomfort of an injection. EMLA cream is typically used for radionuclide synovectomies.
Gamma Radiation: Compared to Beta radiation, a more powerful form of energy that is released from some radioactive substances. Gamma radiation penetrates through human tissue, and is used for taking radiographs (X-ray pictures) and it is used for treating some malignant tumors (cancers).
Hemophilia: A hereditary disorder characterized by delayed clotting of blood, thus causing excessive bleeding.
Hemostasis: The stopping of bleeding.
Inhibitor: By definition, an inhibitor is any substance that interferes with a chemical reaction or other biological activity. In hemophilia, this refers to an antibody directed against the specific clotting factor, which is being replaced. This occurs in approximately 10 percent of patients with hemophilia. The presence of an inhibitor complicates medical management, as these inhibitors inactivate the clotting factor, which is being administered. There has varying severity and there are a number of medical strategies for dealing with inhibitors.
Local anesthesia: An injection of medication into an area which inhibits the local sensory nerves and thereby "deadens" pain sensation. Due to its rapid onset of effect, we commonly use Lidocaine® to anesthetize the skin for a radionuclide injection. Due to its long lasting effect, we typically use Marcaine® within a joint following a radionuclide or arthroscopic synovectomy.
Mild Hemophilia: Patients with hemophilia have varying severities of clotting factor deficiency. If the person's deficient clotting factor is five to 20 percent of the normal, the condition is generally termed "mild hemophilia." These patients rarely develop spontaneous joint bleeds and therefore, it is uncommon for them to develop significant joint disease.
Moderate Hemophilia: Patients with hemophilia have varying severities of clotting factor deficiency. If the person's deficient clotting factor is one to five percent of the normal, the condition is generally termed "moderate hemophilia." These patients may develop spontaneous joint bleeds and occasionally develop joint disease secondary to this.
Return to the top
Phosphorus 32 (P32): The radioactive form of the element phosphorus. In hemophilia, this substance is used to radiate abnormally thickened (diseased) joint lining. One of the properties of P32 that makes it well suited for this purpose is that P32 primarily releases beta radiation.
Prophylaxis: By definition, a preventative treatment. In reference to hemophilia, this refers to the strategy of giving repeated doses of clotting factor concentrates on a schedule designed to prevent blood levels from becoming low as the clotting factors are consumed. The principal is to recurrently dose the patient so that the deficient clotting factor level never reaches a critically low value. The goal is to prevent bleeding.
Primary prophylaxis: A treatment strategy involving the regularly scheduled use of clotting factor concentrates. By definition, "primary prophylaxis" is initiated at a young age before frequent bleeding has begun.
Radioactive: Pertaining to release of alpha, beta, or gamma radiation (electromagnetic energy) from elements such as uranium.
Radionuclide: Pharmaceutical substance with a radioactive element, such as phosphorus32, as a component.
Radionuclide Synovectomy: A procedure used in unique joint diseases, including hemophilia. This involves the injection of a radionuclide into a joint that has a chronically inflamed synovium (joint lining). The radiation which is released eliminates or reduces the diseased joint lining with which it come in contact. Synonyms include: isotopic synovectomy, synoviorthesis.
R.I.C.E.: Acronym for Rest, Ice, Compress and Elevate. These general principals are often used for joint injury, joint swelling and joint inflammation.
Secondary prophylaxis: A treatment strategy involving the regularly scheduled use of clotting factor concentrates. By definition, “secondary prophylaxis” is initiated in response to recurrent bleeding that has not responded well to “demand therapy” (treatment is response to clinically apparent bleeds.)
Severe Hemophilia: Patients with hemophilia have varying severities of clotting factor deficiency. If the person's deficiency clotting factor is less than one percent of the normal, the condition is generally termed "severe hemophilia." These patients frequently have spontaneous joint bleeds and are prone to develop joint inflammation and arthritis.
Surgical synovectomy: Removal of diseased joint lining can be done through several techniques. Surgically, a synovectomy can be performed through an open incision, or arthroscopically, utilizing multiple small incisions. Generally, open synovectomies are associated with greater pain scarring and stiffness, when compared to arthroscopic synovectomy.
Synovitis: Inflammation of the joint lining; eventually leads to surface cartilage destruction and joint stiffness.
Synovium: Joints are composed of the smooth cartilage surface at the end of two bones where they come together. Surrounding these surfaces is an envelope of mobile soft tissue called the joint capsule. The cells, which line the inner portion of this capsule, are the synovium. Amongst other things, these cells secrete lubricating material for the joint.
Topical anesthesia: A technique of applying substances to the skin to "deaden" the area. Topical anesthetics currently available have a limited depth of penetration and a variable clinical effect of relieving pain from an injection through the treated area of skin.
Thrombophilia: A group of conditions where there is increased tendency for excessive blood clotting.
Thrombosis: The formation, presence or development of a blood clot.
A B E G H I L M P R S T
Return to the top