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Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration

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Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration. / Busch, Albert; Hoffjan, Sabine; Bergmann, Frauke; Hartung, Birgit; Jung, Helena; Hanel, Daniela; Tzschach, Andeas; Kadar, Janos; von Kodolitsch, Yskert; Germer, Christoph-Thomas; Trobisch, Heiner; Strasser, Erwin; Wildenauer, René.

in: ORPHANET J RARE DIS, Jahrgang 11, Nr. 1, 03.08.2016, S. 111.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Busch, A, Hoffjan, S, Bergmann, F, Hartung, B, Jung, H, Hanel, D, Tzschach, A, Kadar, J, von Kodolitsch, Y, Germer, C-T, Trobisch, H, Strasser, E & Wildenauer, R 2016, 'Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration', ORPHANET J RARE DIS, Jg. 11, Nr. 1, S. 111. https://doi.org/10.1186/s13023-016-0491-2

APA

Busch, A., Hoffjan, S., Bergmann, F., Hartung, B., Jung, H., Hanel, D., Tzschach, A., Kadar, J., von Kodolitsch, Y., Germer, C-T., Trobisch, H., Strasser, E., & Wildenauer, R. (2016). Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration. ORPHANET J RARE DIS, 11(1), 111. https://doi.org/10.1186/s13023-016-0491-2

Vancouver

Busch A, Hoffjan S, Bergmann F, Hartung B, Jung H, Hanel D et al. Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration. ORPHANET J RARE DIS. 2016 Aug 3;11(1):111. https://doi.org/10.1186/s13023-016-0491-2

Bibtex

@article{b2b2142303ac4a808213c1c2dd5e0594,
title = "Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration",
abstract = "BACKGROUND: The vascular type represents a very rare, yet the clinically most fatal entity of Ehlers-Danlos syndrome (EDS). Patients are often admitted due to arterial bleedings and the friable tissue and the altered coagulation contribute to the challenge in treatment strategies. Until now there is little information about clotting characteristics that might influence hemostasis decisively and eventually worsen emergency situations.RESULTS: 22 vascular type EDS patients were studied for hemoglobin, platelet volume and count, Quick and activated partial thromboplastin time, fibrinogen, factor XIII, von Willebrand disease, vitamin D and platelet aggregation by modern standard laboratory methods. Results show a high prevalence of over 50 % for platelet aggregation disorders in vascular type EDS patients, especially for collagen and epinephrine induced tests, whereas the plasmatic cascade did not show any alterations. Additionally, more than half of the tested subjects showed low vitamin D serum levels, which might additionally affect vascular wall integrity.CONCLUSION: The presented data underline the importance of detailed laboratory screening methods in vascular type EDS patients in order to allow for targeted application of platelet-interacting substances that might be of decisive benefit in the emergency setting.",
keywords = "Adult, Aged, Blood Coagulation/physiology, Blood Coagulation Tests, Blood Platelets/physiology, Ehlers-Danlos Syndrome/blood, Factor XIII/metabolism, Female, Fibrinogen/metabolism, Humans, Male, Middle Aged, Vitamin D/blood, Young Adult, von Willebrand Factor/metabolism",
author = "Albert Busch and Sabine Hoffjan and Frauke Bergmann and Birgit Hartung and Helena Jung and Daniela Hanel and Andeas Tzschach and Janos Kadar and {von Kodolitsch}, Yskert and Christoph-Thomas Germer and Heiner Trobisch and Erwin Strasser and Ren{\'e} Wildenauer",
year = "2016",
month = aug,
day = "3",
doi = "10.1186/s13023-016-0491-2",
language = "English",
volume = "11",
pages = "111",
journal = "ORPHANET J RARE DIS",
issn = "1750-1172",
publisher = "BioMed Central Ltd.",
number = "1",

}

RIS

TY - JOUR

T1 - Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration

AU - Busch, Albert

AU - Hoffjan, Sabine

AU - Bergmann, Frauke

AU - Hartung, Birgit

AU - Jung, Helena

AU - Hanel, Daniela

AU - Tzschach, Andeas

AU - Kadar, Janos

AU - von Kodolitsch, Yskert

AU - Germer, Christoph-Thomas

AU - Trobisch, Heiner

AU - Strasser, Erwin

AU - Wildenauer, René

PY - 2016/8/3

Y1 - 2016/8/3

N2 - BACKGROUND: The vascular type represents a very rare, yet the clinically most fatal entity of Ehlers-Danlos syndrome (EDS). Patients are often admitted due to arterial bleedings and the friable tissue and the altered coagulation contribute to the challenge in treatment strategies. Until now there is little information about clotting characteristics that might influence hemostasis decisively and eventually worsen emergency situations.RESULTS: 22 vascular type EDS patients were studied for hemoglobin, platelet volume and count, Quick and activated partial thromboplastin time, fibrinogen, factor XIII, von Willebrand disease, vitamin D and platelet aggregation by modern standard laboratory methods. Results show a high prevalence of over 50 % for platelet aggregation disorders in vascular type EDS patients, especially for collagen and epinephrine induced tests, whereas the plasmatic cascade did not show any alterations. Additionally, more than half of the tested subjects showed low vitamin D serum levels, which might additionally affect vascular wall integrity.CONCLUSION: The presented data underline the importance of detailed laboratory screening methods in vascular type EDS patients in order to allow for targeted application of platelet-interacting substances that might be of decisive benefit in the emergency setting.

AB - BACKGROUND: The vascular type represents a very rare, yet the clinically most fatal entity of Ehlers-Danlos syndrome (EDS). Patients are often admitted due to arterial bleedings and the friable tissue and the altered coagulation contribute to the challenge in treatment strategies. Until now there is little information about clotting characteristics that might influence hemostasis decisively and eventually worsen emergency situations.RESULTS: 22 vascular type EDS patients were studied for hemoglobin, platelet volume and count, Quick and activated partial thromboplastin time, fibrinogen, factor XIII, von Willebrand disease, vitamin D and platelet aggregation by modern standard laboratory methods. Results show a high prevalence of over 50 % for platelet aggregation disorders in vascular type EDS patients, especially for collagen and epinephrine induced tests, whereas the plasmatic cascade did not show any alterations. Additionally, more than half of the tested subjects showed low vitamin D serum levels, which might additionally affect vascular wall integrity.CONCLUSION: The presented data underline the importance of detailed laboratory screening methods in vascular type EDS patients in order to allow for targeted application of platelet-interacting substances that might be of decisive benefit in the emergency setting.

KW - Adult

KW - Aged

KW - Blood Coagulation/physiology

KW - Blood Coagulation Tests

KW - Blood Platelets/physiology

KW - Ehlers-Danlos Syndrome/blood

KW - Factor XIII/metabolism

KW - Female

KW - Fibrinogen/metabolism

KW - Humans

KW - Male

KW - Middle Aged

KW - Vitamin D/blood

KW - Young Adult

KW - von Willebrand Factor/metabolism

U2 - 10.1186/s13023-016-0491-2

DO - 10.1186/s13023-016-0491-2

M3 - SCORING: Journal article

C2 - 27488172

VL - 11

SP - 111

JO - ORPHANET J RARE DIS

JF - ORPHANET J RARE DIS

SN - 1750-1172

IS - 1

ER -