Key result
FFP controls severe infant protein C deficiency symptoms but causes hyperproteinemia, while warfarin dosing varies.
Why the study?
The molecular abnormality and effectiveness of warfarin therapy in severe homozygous protein C deficiency were not well characterized.
Population
An infant with severe homozygous protein C deficiency presenting with purpura fulminans and severe bilateral vitreous hemorrhages
Comparison
Fresh frozen plasma infusions followed by warfarin therapy with gradual withdrawal of plasma
Design
Case report
Follow-up
>8 months
Authors
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Reducing fresh frozen plasma frequency risks hemolysis and renal failure; this Level 5 case leaves optimal long-term dosing open.
Case Report (n=1)
Warfarin therapy can be used to manage severe homozygous protein C deficiency in infants, allowing withdrawal of fresh frozen plasma, though dosing requires careful monitoring due to high variability.
Peters et al. (1988) conducted a case report in Severe homozygous protein C deficiency (n=1). Fresh frozen plasma and Warfarin was evaluated on Clinical response and complications. In an infant with severe homozygous protein C deficiency, fresh frozen plasma controlled symptoms but caused hyperproteinemia, while warfarin therapy required highly variable dosing.
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