haemophilia Flashcards

1
Q

haemophilia A

A

deficiency factor VIII

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2
Q

haemophilia B

A

deficiency factor IX

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3
Q

inheritance

A

x-linked recessive

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4
Q

features

A

spontaneous bleeding
haematomas
haemarthrosis
muscle haematoma

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5
Q

diagnosis is based on

A

bleeding socres
coagulation factor assays
genetic testing

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6
Q

management

A

IV recombinant factor infusions
desmopressin
antibfibrinolytics e.g. tranexamic acid

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7
Q

why despomressin

A

it stimlates release of VWF

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