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Did you know? Hemophilia B has an indissoluble bond with the British royal family!
Hemophilia B, also known as Christmas disease, is a blood clotting disease caused by a deficiency of clotting factor IX. It manifests as easy bruising and bleeding, especially more common in men. The history of this disease dates back to 1952, when the medical community first recognized it as a distinct disease.
People with hemophilia B experience frequent bleeding episodes throughout their lives, necessitating regular factor replacement therapy to control their symptoms.
The prevalence of hemophilia B is approximately 1 in 40,000 people, and approximately 15% of people with hemophilia have the disease. However, many female carriers often have no obvious symptoms, but 10% to 25% of women may experience mild symptoms of hemophilia, and occasionally even moderate or severe symptoms.
Symptoms and complications
The main symptoms of hemophilia B include easy bruising, blood in the urine, nose bleeds and joint bleeding. Due to the fear of bleeding, patients often neglect oral hygiene, which makes them more susceptible to periodontal disease and caries problems. The most obvious sign of mild hemophilia in the mouth is bleeding gums during the loss of primary teeth or prolonged bleeding after surgery or tooth extraction.
In severe cases of hemophilia, patients may experience spontaneous bleeding from the oral tissues, including the soft palate, tongue, buccal mucosa, lips, and gums, and may even produce ecchymoses.
Genetics and Pathophysiology
Hemophilia B is caused by a deficiency of clotting factor IX, the gene for which is located on the X chromosome, which explains why males are more often affected. When the F9 gene mutates, it causes hemophilia B. An estimated one-third of patients have no family history of the disease, due to random genetic variations that occur during reproduction.
Diagnosis and differential diagnosis
Hemophilia B is usually diagnosed through methods such as coagulation screening tests and clotting factor analysis. The differential diagnosis related to this disease includes hemophilia A, factor XI deficiency, and von Willebrand disease.
Treatment and Dental Considerations
Currently, the treatment for hemophilia B is mainly through intravenous injection of factor IX. With the advancement of medical technology, Etranacogene dezaparvovec (Hemgenix) was approved by the U.S. Food and Drug Administration in 2022, becoming the first gene therapy that can be used to treat hemophilia B.
During treatment, surgical risks must be carefully considered to avoid excessive bleeding or bruising.
Connection to the British Royal Family
Hemophilia B is known as the "royal disease" because of its prevalence in several European royal families. Queen Victoria was a carrier of hemophilia B and passed it on to other royal families such as Russia, Spain and Germany. The history of this disease is not only a part of medical research, but it is also intertwined with the family stories of famous people, making hemophilia B part of history.
Conclusion
Hemophilia B is not just a blood disease, but also a mirror of history and society, reflecting the pursuit and challenges of health in different generations. Do these stories behind the scenes make you start thinking about the profound impact of health and genetics?
