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

doi:10.1111/jth.15732

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

Clinical Chemistry, Malmö

Research output: Contribution to journal › Article › peer-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 language	English
Pages (from-to)	1735-1743
Number of pages	9
Journal	Journal of Thrombosis and Haemostasis
Volume	20
Issue number	7
DOIs	https://doi.org/10.1111/jth.15732
Publication status	Published - 2022 Jul

Subject classification (UKÄ)

Gynaecology, Obstetrics and Reproductive Medicine

Free keywords

children
disseminated intravascular coagulation
neonates
purpura fulminans
thrombosis

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10.1111/jth.15732Licence: CC BY-NC

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Minford, A., Brandão, L. R., Othman, M., Male, C., Abdul-Kadir, R., Monagle, P., Mumford, A. D., Adcock, D., Dahlbäck, B., Miljic, P., DeSancho, M. T., & Teruya, J. (2022). Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH. Journal of Thrombosis and Haemostasis, 20(7), 1735-1743. https://doi.org/10.1111/jth.15732

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

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{\textquoteright}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.",

keywords = "children, disseminated intravascular coagulation, neonates, purpura fulminans, thrombosis",

author = "Adrian Minford and Brand{\~a}o, {Leonardo R.} and Maha Othman and Christoph Male and Rezan Abdul-Kadir and Paul Monagle and Mumford, {Andrew D.} and Dorothy Adcock and Bj{\"o}rn Dahlb{\"a}ck and Predrag Miljic and DeSancho, {Maria T.} and Jun Teruya",

year = "2022",

month = jul,

doi = "10.1111/jth.15732",

language = "English",

volume = "20",

pages = "1735--1743",

journal = "Journal of Thrombosis and Haemostasis",

issn = "1538-7933",

publisher = "Elsevier",

number = "7",

}

Minford, A, Brandão, LR, Othman, M, Male, C, Abdul-Kadir, R, Monagle, P, Mumford, AD, Adcock, D, Dahlbäck, B, Miljic, P, DeSancho, MT & Teruya, J 2022, 'Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH', Journal of Thrombosis and Haemostasis, vol. 20, no. 7, pp. 1735-1743. https://doi.org/10.1111/jth.15732

TY - JOUR

T1 - Diagnosis and management of severe congenital protein C deficiency (SCPCD)

T2 - Communication from the SSC of the ISTH

AU - Minford, Adrian

AU - Brandão, Leonardo R.

AU - Othman, Maha

AU - Male, Christoph

AU - Abdul-Kadir, Rezan

AU - Monagle, Paul

AU - Mumford, Andrew D.

AU - Adcock, Dorothy

AU - Dahlbäck, Björn

AU - Miljic, Predrag

AU - DeSancho, Maria T.

AU - Teruya, Jun

PY - 2022/7

Y1 - 2022/7

N2 - 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.

AB - 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.

KW - children

KW - disseminated intravascular coagulation

KW - neonates

KW - purpura fulminans

KW - thrombosis

UR - https://doi.org/10.1016/j.jtha.2023.01.019

U2 - 10.1111/jth.15732

DO - 10.1111/jth.15732

M3 - Article

C2 - 35570324

AN - SCOPUS:85132647530

SN - 1538-7933

VL - 20

SP - 1735

EP - 1743

JO - Journal of Thrombosis and Haemostasis

JF - Journal of Thrombosis and Haemostasis

IS - 7

ER -

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

Abstract

Subject classification (UKÄ)

Free keywords

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