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Medical guidance: Li Qingshan and Gao Guanlun, chief physician of the Department of Hematology, Guangzhou Red Cross Hospital
Medical guidance: Li Qingshan and Gao Guanlun, chief physician of the Department of Hematology, Guangzhou Red Cross HospitalApril 17 World Hemophilia Day is approaching.
Epidemiology
EpidemiologyThere are no ethnic or regional differences in the incidence of hemophilia.
Clinical manifestations and classification
Clinical manifestations and classificationThe clinical manifestations of hemophilia A and hemophilia B are the same, mainly manifested as joints, muscles and deep tissue bleeding, but also bleeding from the gastrointestinal tract, urinary tract, central nervous system, and bleeding after tooth extraction.
Hemophilia can be divided into mild, intermediate and severe types according to the activity levels of coagulation factors in patients.
Laboratory examination
Laboratory examination1.
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Treatment principles
Treatment principlesPatients with hemophilia should receive diagnosis, treatment and follow-up by the comprehensive care team at the hemophilia diagnosis and treatment center.
Family therapy allows patients to inject coagulation factors immediately to achieve the most ideal early treatment.
Patients with hemophilia should avoid intramuscular injections and trauma.
Alternative treatment of hemophilia A: that is, what is lacking to make up for, the first choice is genetically recombined FⅧ preparations or virus-inactivated blood-derived FⅧ preparations, and cryoprecipitate or fresh frozen plasma can be used only when the above conditions are not available.
According to the frequency of replacement treatment, it can be divided into on-demand treatment and preventive treatment (regular replacement treatment).
prevention
prevention①Because hemophilia is a recessive genetic disease, for couples of childbearing age who already know they are carriers or patients, genetic testing can be performed through prenatal diagnosis to see if the fetus is healthy.
②In infants and young children, if the child has bleeding after the needle is removed during the blood test, and the skin has bruises after being slightly bumped inadvertently, be vigilant.
③Strengthen education and publicize the nature of the disease, prevention knowledge and precautions to patients, family members, schools and units, so that they can correctly understand and treat the disease and work closely with medical staff to avoid trauma and strenuous activities, and encourage appropriate physical activity.