Why the study?
Severe congenital protein C deficiency is rare and there is substantial variation in its diagnosis and management.
This SSC communication provides standardized recommendations for the diagnosis and management of the rare condition of severe congenital protein C deficiency.
Substantial variation persists in SCPCD management; review extends expert guidance while leaving optimal strategies open to prospective trials.
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.
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Minford et al. (2022) conducted a review in Severe congenital protein C deficiency (SCPCD). Diagnosis and management recommendations was evaluated. The SSC of the ISTH provides a comprehensive review and set of recommendations for the diagnosis, acute management, and long-term management of severe congenital protein C deficiency.
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