Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration.
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.
['Busch|Albert|A|', 'Hoffjan|Sabine|S|', 'Bergmann|Frauke|F|', 'Hartung|Birgit|B|', 'Jung|Helena|H|', 'Hanel|Daniela|D|', 'Tzschach|Andeas|A|', 'Kadar|Janos|J|', 'von Kodolitsch|Yskert|Y|', 'Germer|Christoph-Thomas|CT|', 'Trobisch|Heiner|H|', 'Strasser|Erwin|E|', 'Wildenauer|René|R|']
D000328:Adult D000368:Aged D001777:Blood Coagulation D001780:Blood Coagulation Tests D001792:Blood Platelets D004535:Ehlers-Danlos Syndrome D005176:Factor XIII D005260:Female D005340:Fibrinogen D006801:Humans D008297:Male D008875:Middle Aged D014807:Vitamin D D055815:Young Adult D014841:von Willebrand Factor
Vascular type Ehlers-Danlos syndrome Platelet dysfunction Vitamin D EDS Bleeding disorder