Research ExplorerPublicationsPregnancy in Upshaw-Schulman syndrome

Pregnancy in Upshaw-Schulman syndrome

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Pregnancy in Upshaw-Schulman syndrome. / Kentouche, K; Voigt, A; Schleussner, E; Schneppenheim, R; Budde, U; Beck, J F; Stefańska-Windyga, E; Windyga, J.

In: HAMOSTASEOLOGIE, Vol. 33, No. 2, 29.05.2013, p. 144-8.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Kentouche, K, Voigt, A, Schleussner, E, Schneppenheim, R, Budde, U, Beck, JF, Stefańska-Windyga, E & Windyga, J 2013, 'Pregnancy in Upshaw-Schulman syndrome', HAMOSTASEOLOGIE, vol. 33, no. 2, pp. 144-8. https://doi.org/10.5482/HAMO-13-04-0025

APA

Kentouche, K., Voigt, A., Schleussner, E., Schneppenheim, R., Budde, U., Beck, J. F., Stefańska-Windyga, E., & Windyga, J. (2013). Pregnancy in Upshaw-Schulman syndrome. HAMOSTASEOLOGIE, 33(2), 144-8. https://doi.org/10.5482/HAMO-13-04-0025

Vancouver

Kentouche K, Voigt A, Schleussner E, Schneppenheim R, Budde U, Beck JF et al. Pregnancy in Upshaw-Schulman syndrome. HAMOSTASEOLOGIE. 2013 May 29;33(2):144-8. https://doi.org/10.5482/HAMO-13-04-0025

Bibtex

@article{969a7139a5a947fea02b0b1a12a98cfe,
title = "Pregnancy in Upshaw-Schulman syndrome",
abstract = "The Upshaw Schulman syndrome (MIM #274150) is a hereditary deficiency of the von Willebrand factor cleaving protease (ADAMTS13) due to homozygous or compound heterozygous mutations in the ADAMTS13 gene. Patients are prone to bouts of thrombotic thrombocytopenic purpura. However, disease manifestation needs a second trigger event. Pregnancy is a known risk factor for TTP. Patients with USS may manifest during pregnancy and the postpartum period or relapse with a TTP bout. Before plasma therapy mortality for both the mother and the fetus was high, but even nowadays when plasma is delivered, therapy is challenging, still bearing a high risk for miscarriage or long term sequelae for the mother. In this report on pregnancies in three mothers with USS, plasma therapy was increased in frequency and amount given with regard to platelet count or ADAMTS13 activity, thus leading to a successful outcome.",
keywords = "Adolescent, Adult, Female, Humans, Plasma Exchange, Pregnancy, Pregnancy Complications, Hematologic, Pregnancy Outcome, Purpura, Thrombotic Thrombocytopenic, Treatment Outcome, Young Adult",
author = "K Kentouche and A Voigt and E Schleussner and R Schneppenheim and U Budde and Beck, {J F} and E Stefa{\'n}ska-Windyga and J Windyga",
year = "2013",
month = may,
day = "29",
doi = "10.5482/HAMO-13-04-0025",
language = "English",
volume = "33",
pages = "144--8",
journal = "HAMOSTASEOLOGIE",
issn = "0720-9355",
publisher = "Schattauer",
number = "2",

}

RIS

TY - JOUR

T1 - Pregnancy in Upshaw-Schulman syndrome

AU - Kentouche, K

AU - Voigt, A

AU - Schleussner, E

AU - Schneppenheim, R

AU - Budde, U

AU - Beck, J F

AU - Stefańska-Windyga, E

AU - Windyga, J

PY - 2013/5/29

Y1 - 2013/5/29

N2 - The Upshaw Schulman syndrome (MIM #274150) is a hereditary deficiency of the von Willebrand factor cleaving protease (ADAMTS13) due to homozygous or compound heterozygous mutations in the ADAMTS13 gene. Patients are prone to bouts of thrombotic thrombocytopenic purpura. However, disease manifestation needs a second trigger event. Pregnancy is a known risk factor for TTP. Patients with USS may manifest during pregnancy and the postpartum period or relapse with a TTP bout. Before plasma therapy mortality for both the mother and the fetus was high, but even nowadays when plasma is delivered, therapy is challenging, still bearing a high risk for miscarriage or long term sequelae for the mother. In this report on pregnancies in three mothers with USS, plasma therapy was increased in frequency and amount given with regard to platelet count or ADAMTS13 activity, thus leading to a successful outcome.

AB - The Upshaw Schulman syndrome (MIM #274150) is a hereditary deficiency of the von Willebrand factor cleaving protease (ADAMTS13) due to homozygous or compound heterozygous mutations in the ADAMTS13 gene. Patients are prone to bouts of thrombotic thrombocytopenic purpura. However, disease manifestation needs a second trigger event. Pregnancy is a known risk factor for TTP. Patients with USS may manifest during pregnancy and the postpartum period or relapse with a TTP bout. Before plasma therapy mortality for both the mother and the fetus was high, but even nowadays when plasma is delivered, therapy is challenging, still bearing a high risk for miscarriage or long term sequelae for the mother. In this report on pregnancies in three mothers with USS, plasma therapy was increased in frequency and amount given with regard to platelet count or ADAMTS13 activity, thus leading to a successful outcome.

KW - Adolescent

KW - Adult

KW - Female

KW - Humans

KW - Plasma Exchange

KW - Pregnancy

KW - Pregnancy Complications, Hematologic

KW - Pregnancy Outcome

KW - Purpura, Thrombotic Thrombocytopenic

KW - Treatment Outcome

KW - Young Adult

U2 - 10.5482/HAMO-13-04-0025

DO - 10.5482/HAMO-13-04-0025

M3 - SCORING: Journal article

C2 - 23715104

VL - 33

SP - 144

EP - 148

JO - HAMOSTASEOLOGIE

JF - HAMOSTASEOLOGIE

SN - 0720-9355

IS - 2

ER -