Hemophilia
Question. Write short note on hemophilia.
Answer.
Hemophilia:-
Hemophilia is a potentially fatal inherited bleeding disorder characterized by profuse hemorrhage due to deficiency of clotting factors.
hemophilia
Types
- Hemophilia A or classic hemophilia: In this factor VIII deficiency is present.
- Hemophilia B or Christmas disease: In this factor IX deficiency is present.
- von Willebrand’s disease: In this condition, factor VIII deficiency is present along with defective platelet function.
“Risk factors for developing hemophilia”
Clinical Features
- The easy bruising mostly characterizes disease and prolongs bleeding, particularly accidental, surgical and dental trauma.
- Spontaneous bleeding into subcutaneous tissue or internal organs leads to recurrent soft tissue hematoma formation.
- Gastric hemorrhage may occur in case of gastric ulcer.
Joint problems occur due to degenerative changes in joint structures, osteoporosis, and muscle atrophy. - Severe hemorrhage from gingival tissue after dental extraction.
- Slight trauma may lead to hematoma formation in the tongue, lips, and palate.
- The hemophilic patient has high caries index and severe periodontal disease.
hemophilia A
“Role of blood tests in hemophilia diagnosis”
Treatment
- Immediate transfusion of factor VIII or IX concentrate is the primary treatment.
- Patient need a transfusion in every 12 hours until the bleeding stops.
- Analgesic and corticosteroids reduce joint pain and swelling.
- In mild hemophilia, use of IV desmopressin is done.
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