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(D68.1) Hereditary factor xi deficiency
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734 830 in individuals diagnosis hereditary factor xi deficiency confirmed
26 707 deaths with diagnosis hereditary factor xi deficiency
4% mortality rate associated with the disease hereditary factor xi deficiency
Diagnosis hereditary factor xi deficiency is diagnosed Women are 9.01% more likely than Men
334 299
Men receive the diagnosis hereditary factor xi deficiency
11 162 (3.3 %)Died from this diagnosis.
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400 531
Women receive the diagnosis hereditary factor xi deficiency
15 545 (3.9 %)Died from this diagnosis.
Risk Group for the Disease hereditary factor xi deficiency - Men aged 75-79 and Women aged 80-84
In Men diagnosis is most often set at age 0-95+
in in men, the disease manifests at any agein in women, the disease manifests at any age
In Women diagnosis is most often set at age 0-95+
Disease Features hereditary factor xi deficiency
Absence or low individual and public risk
Hereditary factor xi deficiency - what does this mean
Hereditary factor xi deficiency is an inherited disorder caused by a mutation in the gene that is responsible for producing factor xi, a clotting factor in the blood. this mutation causes a decrease in the production of factor xi, resulting in an increased risk of bleeding.
What happens during the disease - hereditary factor xi deficiency
Hereditary factor xi deficiency is caused by mutations in the f11 gene, which encodes for factor xi, a clotting factor involved in the intrinsic pathway of coagulation. these mutations lead to a decreased production of factor xi, resulting in a prolonged bleeding time and an increased risk of bleeding episodes.
Clinical Pattern
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How does a doctor diagnose
- Complete blood count
- Coagulation studies
- Factor XI antigen and activity levels
- Thrombin time
- Activated partial thromboplastin time
- Fibrinogen level
- Genetic testing
Treatment and Medical Assistance
Main Goal: To reduce the risk of bleeding episodes in patients with Hereditary Factor XI Deficiency
- Administer Factor XI concentrate replacement therapy.
- Monitor patient's Factor XI levels.
- Educate patient and family about the disease and its management.
- Monitor patient's response to treatment.
- Provide lifestyle advice to reduce risk of bleeding episodes.
- Administer desmopressin to reduce risk of bleeding episodes.
- Administer antifibrinolytics to reduce risk of bleeding episodes.
- Administer antifibrinolytics and desmopressin in combination to reduce risk of bleeding episodes.
- Monitor patient's response to treatment.
- Provide patient with preventive measures to reduce risk of bleeding episodes.
- Identify and manage any underlying medical conditions that may contribute to bleeding episodes.
14 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Hereditary factor xi deficiency - Prevention
Hereditary factor xi deficiency can be prevented by avoiding contact with people who have the condition, as it is an inherited disorder. genetic counseling and carrier testing may also be recommended for those at risk of passing the condition to their children.