Haemophilia

Haemophilia A-
Aetiology_it is an Xlinked genetic disorder of coagulation,affecting males.It result frm reduction in factor VIII -antihaemophilic factor.
Classifiction_severe_less than 1%of normal activity.
Moderate-1-5% of normal activity.
Mild_more than 5%of N activity.
Clinical features_
it is characterise by excessive bleeding,unusual the bady is 6 mönths old.
Hemarthoses_bleeding into joint.In acute stage,affected joint is swollen,hot,tender n movement severly restricted.
Repeated haemarthosis result in deformity,crippling n disuse atrophy of muscle around the joint.
Bleeding into muscle_muscle haematoma is common in psoas n calf muscles.Psoas haematoma cnmpress the femoral nerve resulting in sensory disturbance over thigh n quadrucep weakness.Calf haematomas result in cotraction n shortening of achilles tendö.
Other bleeding manifestation_
bleeding frm wound,bleeding frm socket after dental extraction,easy bruising,intracranial haemorrhage n haematuria.
Investigations_
bleeding time,prothrombin time n platelet count r normal.
Activated partial thromboplstin time is typically prolonged
specific factor VIII assay can confirm the diagnosis.
Antenatal diagnosis_
chorionic villous sampling at 8-9 wks of gastetion,sexing the foetus n using informative fact VIII probe.
Management_
local treatment-
wound n mucous membrane bleeding-local pressure applied digitally with pressure bandage or sutures.Topical haemostatic like adrenaline n thrombin.
Immobilasation of wound by bandage,splinting etc.
Haematomas n haemarthosis_
in acute stage,elevation of affected part n immobilisation by splinting n bandage should b used.Pain is relieved by analgesics like acetoaminophene.Aspirin is contraindicated..Physiotherapy,
Replacement therapy_
agent currently used for replacement therapy is cryoprecipitate n factor VIII concentrate iv.
Indication_1 ealy tretment of spontaneously bleedin episode.
2 established severe or prolonged wound n tissue bleeding. 3 control of bleeding during n after surgery n trauma.
Calculation of dose_
dose of factor VIII=desire factor lever in% multply by wt in kg  multiply by0.5
comlication of therapy_hepatitis,HIV infection