Article
A case of purpura fulminans is caused by homozygous delta8857 mutation (protein C-nagoya) and successfully treated with activated protein C concentrate.
British journal of haematology - 1 Sept 2000
Nakayama T, Matsushita T, Hidano H, Suzuki C, Hamaguchi M, Kojima T, Saito H
Abstract excerpt
We report a Japanese patient who developed purpura fulminans and disseminated intravascular coagulation (DIC) shortly after birth. The patient was diagnosed to be homozygous for protein C deficiency and was treated with an activated protein C (APC) concentrate. Intravenous infusions of APC markedly improved the necrotic skin lesions and the anticoagulation by APC enabled successful DIC control. The identified...
Topics
- Disseminated Intravascular Coagulation
- Female
- Humans
- IgA Vasculitis
- Infant, Newborn
- Male
- Mutation
- Pedigree
- Polymerase Chain Reaction
- Protein C
- Protein C Deficiency