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

Beitrag in einer Fachzeitschrift


Details zur Publikation

Autorinnen und Autoren: Busch A, Hoffjan S, Bergmann F, Hartung B, Jung H, Hanel D, Tzschach A, Kadar J, Von Kodolitsch Y, Germer CT, Trobisch H, Strasser E, Wildenauer R
Zeitschrift: Orphanet Journal of Rare Diseases
Jahr der Veröffentlichung: 2016
Band: 11
Heftnummer: 1
ISSN: 1750-1172


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.


FAU-Autorinnen und Autoren / FAU-Herausgeberinnen und Herausgeber

Strasser, Erwin Prof. Dr.
Medizinische Fakultät


Zusätzliche Organisationseinheit(en)
Transfusionsmedizinische und Hämostaseologische Abteilung in der Chirurgischen Klinik


Einrichtungen weiterer Autorinnen und Autoren

amedes Medizinische Dienstleistungen GmbH
Ruhr-Universität Bochum (RUB)
SYNLAB Holding Deutschland GmbH
Technische Universität Dresden
Universitätsklinikum Hamburg-Eppendorf (UKE)
Universitätsklinikum Köln
Universitätsklinikum Würzburg


Zitierweisen

APA:
Busch, A., Hoffjan, S., Bergmann, F., Hartung, B., Jung, H., Hanel, D.,... Wildenauer, R. (2016). Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration. Orphanet Journal of Rare Diseases, 11(1). https://dx.doi.org/10.1186/s13023-016-0491-2

MLA:
Busch, Albert, et al. "Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration." Orphanet Journal of Rare Diseases 11.1 (2016).

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Zuletzt aktualisiert 2018-14-11 um 09:23