Key result
Intravenous APC concentrate resolves necrotic skin lesions and controls DIC in neonatal protein C deficiency.
Why the study?
Purpura fulminans caused by homozygous protein C deficiency due to the Delta8857 mutation is a fatal thrombotic disease requiring effective therapeutic and diagnostic approaches.
Does activated protein C concentrate improve necrotic skin lesions and control DIC in a newborn with homozygous protein C deficiency?
Case Report (n=1)
Does activated protein C concentrate improve necrotic skin lesions and control DIC in a newborn with homozygous protein C deficiency?
Activated protein C concentrate is an effective treatment for purpura fulminans and DIC in patients with homozygous protein C deficiency.
May support APC in neonatal homozygous protein C deficiency; hypothesis-generating pending controlled data.
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 mutation (Delta8857) results in impaired intracellular transport and protein maturation and would be the cause of the complete protein C deficiency. This is the seventh case of the mutation that has been exclusively reported in Japan, but is the first report of a homozygous case. Our findings propose new therapeutic and diagnostic tools for the management of this fatal thrombotic disease.
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Nakayama et al. (2000) conducted a case report in Purpura fulminans and disseminated intravascular coagulation (DIC) (n=1). Activated protein C (APC) concentrate was evaluated on Improvement of necrotic skin lesions and DIC control. Intravenous activated protein C concentrate successfully treated necrotic skin lesions and controlled DIC in a neonate with homozygous protein C deficiency caused by the Delta8857 mutation.
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