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

Minford, Adrian ; Brandão, Leonardo R. ; Othman, Maha ; Male, Christoph ; Abdul-Kadir, Rezan ; Monagle, Paul ; Mumford, Andrew D. ; Adcock, Dorothy ; Dahlbäck, Björn LU and Miljic, Predrag , et al. (2022) In Journal of Thrombosis and Haemostasis 20(7). p.1735-1743
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... (More)

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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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
children, disseminated intravascular coagulation, neonates, purpura fulminans, thrombosis
in
Journal of Thrombosis and Haemostasis
volume
20
issue
7
pages
9 pages
publisher
Wiley-Blackwell
external identifiers
  • scopus:85132647530
  • pmid:35570324
ISSN
1538-7933
DOI
10.1111/jth.15732
language
English
LU publication?
yes
id
d635d250-21dd-423e-a16c-bfd523857b60
date added to LUP
2022-10-06 09:10:24
date last changed
2024-06-14 22:38:35
@article{d635d250-21dd-423e-a16c-bfd523857b60,
  abstract     = {{<p>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.</p>}},
  author       = {{Minford, Adrian and Brandão, Leonardo R. and Othman, Maha and Male, Christoph and Abdul-Kadir, Rezan and Monagle, Paul and Mumford, Andrew D. and Adcock, Dorothy and Dahlbäck, Björn and Miljic, Predrag and DeSancho, Maria T. and Teruya, Jun}},
  issn         = {{1538-7933}},
  keywords     = {{children; disseminated intravascular coagulation; neonates; purpura fulminans; thrombosis}},
  language     = {{eng}},
  number       = {{7}},
  pages        = {{1735--1743}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of Thrombosis and Haemostasis}},
  title        = {{Diagnosis and management of severe congenital protein C deficiency (SCPCD) : Communication from the SSC of the ISTH}},
  url          = {{http://dx.doi.org/10.1111/jth.15732}},
  doi          = {{10.1111/jth.15732}},
  volume       = {{20}},
  year         = {{2022}},
}