Diagnosis and management of severe congenital protein C deficiency (SCPCD) : Communication from the SSC of the ISTH
(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.
(Less)
- author
- organization
- publishing date
- 2022-07
- 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
-
- pmid:35570324
- scopus:85132647530
- 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
- 2024-09-20 05:03:48
@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}}, }