Factor XIII fibrin stabilising factor

Clinical

Characteristically produces delayed post-operative bleeding (6-24h later). Neonatal umbilical stump bleeding more common than with other deficiencies. High risk of cerebral haemorrhage.

Diagnosis

APTT and PT both normal so will be missed in a bleeding investigation unless specifically looked for by screening test (clot formed with thrombin and stability in acetic acid is measured).

Treatment

Only very low levels required for haemostasis; t,2 is very long. Severe defi-35g ciency should be treated with once-monthly prophylaxis with factor XIII concentrate.

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