What is the morbidity associated with NPF in protein C deficiency?

Updated: Jan 04, 2019
  • Author: Shamudheen Rafiyath, MD; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

NPF is a life-threatening condition that occurs in newborns with homozygous or compound heterozygous protein C deficiency, usually during the first several days of life. Affected neonates present with diffuse ecchymoses (see image below). Skin biopsy demonstrates extensive thrombosis of cutaneous venous and arterial channels, much as is observed in WISN. [45, 46] Laboratory testing reveals severe deficiency (< 1% of normal) of immunologic protein C levels. [47] Expeditious treatment with an exogenous source of protein C, (discussed in Treatment, Medical Care, is paramount.

A patient with neonatal purpura fulminans. A patient with neonatal purpura fulminans.

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