Hemophilia
Answer. It is a hereditary disorder of blood coagulation, characterized by excessive hemorrhage due to prolonged bleeding time.
It is the X-linked genetic disorder of coagulation.
“Comprehensive Overview Of Hemophilia Symptoms”
Hemophilia Types
Following are the types of hemophilia, i.e.
- Hemophilia A or Classic Hemophilia: Deficiency of factor VIII or antihemophilic factor is the cause of hemophilia A. It is recessive X-linked.
Females are always carriers and males are sufferers. - Hemophilia B or Christmas Disease: A deficiency of factor IX is the cause of hemophilia B.
“Understanding The Genetic Causes Of Hemophilia“
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Hemophilia Clinical Features
- The most common manifestation is hemorrhage in the joint.
- Because of repeated hemorrhage, the knee joint becomes nonfunctional hemarthroses take place.
- Bleeding in muscles takes place.
“The Role Of Clotting Factors In Hemophilia Treatment”
- Bleeding from GIT occurs in the form of esophageal varices.
- Bleeding from the genitourinary tract occurs in the form of hematuria.
- A simple cut or injury may lead to profuse bleeding.
- The first symptom is seen in the form of large bruises and hematomas on the hips which regress as the child learns to walk.
Hemophilia laboratory diagnosis
Bleeding time is normal but coagulation time is prolonged.
Hemophilia Treatment
Hemophilia A
The main aim is to raise the factor VIII level, which can arrest bleeding.
- Replacement therapy: Varies of replacement therapy are available like plasma, cryoprecipitate, and factor VIII concentrates.
- Give 30% amount of factor VIII, because it is very expensive.
- Any major operation or tooth extraction requires a 100% concentration of factor VIII.
“Step-By-Step Guide To Managing Hemophilia”
- Hypovolemia, allergic reaction, and development of factor VIII antibodies are complications of factor VIII concentration.
- To avoid this complication, cryoprecipitate from animal origin should be given in between human cryoprecipitate.
Hemophilia B
Fresh frozen plasma or Factor IX concentrates should be given to the patient.
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