Diagnosis and management of neonatal purpura fulminans

V E Price; D L Ledingham; A Krümpel; A K Chan

doi:10.1016/j.siny.2011.07.009

Diagnosis and management of neonatal purpura fulminans

Semin Fetal Neonatal Med. 2011 Dec;16(6):318-22. doi: 10.1016/j.siny.2011.07.009. Epub 2011 Aug 11.

Authors

V E Price¹, D L Ledingham, A Krümpel, A K Chan

Affiliation

¹ Division of Pediatric Hematology/Oncology, Department of Pediatrics, IWK Health Centre, Dalhousie University, Halifax, Canada. [email protected]

PMID: 21839696
DOI: 10.1016/j.siny.2011.07.009

Abstract

Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated. The clinical presentation is that of acute disseminated intravascular coagulation and hemorrhagic skin necrosis. The management includes an acute phase of replacement therapy with fresh frozen plasma or protein C concentrate and a maintenance therapy that includes anticoagulation with warfarin or low molecular weight heparin. This review focuses on the management of severe protein C deficiency.

Publication types

Review

MeSH terms

Disease Management
Humans
Infant, Newborn
Protein C Deficiency / diagnosis*
Protein C Deficiency / therapy*
Protein S Deficiency / diagnosis*
Protein S Deficiency / therapy*
Purpura Fulminans / diagnosis*
Purpura Fulminans / therapy*