Haemophilia
Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Top Contributors - Chelsea Reinhart, Alyssa Elliott, Tarina van der Stockt, Kim Jackson, Mandy Roscher, Robin Tacchetti, Elaine Lonnemann, Tony Lowe, WikiSysop, Lucinda hampton, Jess Bell, Aminat Abolade and Stacy Schiurring
Definition/Description[edit | edit source]
Hemophilia is a congenital X-linked bleeding disorder in which blood does not properly clot. It is categorized as a disorder of hemostasis. Hemostasis, the stopping of bleeding after a blood vessel is injured, normally is divided into two separate processes—primary and secondary hemostasis. In hemophilia, secondary hemostasis (which essentially results in the formation of a fibrin clot) is defected. Thus, deficient clotting is due to an abnormality of the functional plasma-clotting proteins, factors VIII and IX, that are involved in this process. [1]
There are two primary types of this disease—hemophilia A and hemophilia B. Hemophilia A (or classic hemophilia) affects approximately 80% of all cases. Hemophilia B (or Christmas disease) constitutes for approximately 15% of all cases. The difference between the two types is which clotting factor is deficient. [2]
Injuries and surgeries can cause bleeding episodes in individuals with hemophilia. Most also experience episodes of spontaneous bleeding. In regards to bleeding, they do not bleed any faster than the average person, but they do bleed for a longer period of time.
Severity of the disease is determined by blood tests. These tests determine the percentage of clotting factor present in blood plasma; concentrations from 50%-150% is normal. The severity of hemophilia can be described as mild, moderate, and severe.
-Mild hemophilia:
-25% of cases
-Spontaneous hemorrhages are rare
-Deep muscle and joint bleeding are uncommon
-Injury, trauma, surgery, and dental injury trigger bleeding
-these symptoms are addressed the same as with individuals with severe hemophilia
-Moderate hemophilia:
-15% of cases
-Spontaneous hemorrhages are usually not an issue
-Minor trauma can result in major bleeding incidences
-Severe hemophilia:
-60% of cases
-Spontaneous bleeding
-Slight trauma may precipitate bleeding into deep muscle and joints[2]
The Diagnostic section on this page includes a table displaying severity and the levels of factor VIII or IX for each in the blood.
Prevalence[edit | edit source]
Hemophilia, the most common inherited blood clotting disorder associated with clotting factor deficiencies, affects 1 in 5,000 male births. It is estimated that approximately 20,000 males in the United States, and approximately 400,000 people in the world have hemophilia. All racial and ethnic groups are affected by this disease. [2]
Characteristics/Clinical Presentation[edit | edit source]
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Associated Co-morbidities[edit | edit source]
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Medications[edit | edit source]
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Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
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Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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References[edit | edit source]
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- ↑ Goodman CC, Snyder TEK. Differential Diagnosis for Physical Therapists: Screening for Referral. 5th ed. St. Louis: Elsevier Inc, 2013.
- ↑ 2.0 2.1 2.2 Peterson C, Goodman CC, The Hematologic System. In: Goodman CC, Fuller KS editors. Pathology: Implications for the Physical Therapist. 3rd ed. St. Louis: Elsevier Inc, 2009. P678-741.
