Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH

Adrian Minford, Leonardo R. Brandão, Maha Othman, Christoph Male, Rezan Abdul-Kadir, Paul Monagle, Andrew D. Mumford, Dorothy Adcock, Björn Dahlbäck, Predrag Miljic, Maria T. DeSancho, Jun Teruya

Research output: Contribution to journalArticlepeer-review

Abstract

Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women’s Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard.

Original languageEnglish
Pages (from-to)1735-1743
Number of pages9
JournalJournal of Thrombosis and Haemostasis
Volume20
Issue number7
DOIs
Publication statusPublished - 2022 Jul

Subject classification (UKÄ)

  • Obstetrics, Gynecology and Reproductive Medicine

Free keywords

  • children
  • disseminated intravascular coagulation
  • neonates
  • purpura fulminans
  • thrombosis

Fingerprint

Dive into the research topics of 'Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH'. Together they form a unique fingerprint.

Cite this