Type IV Ehlers-Danlos syndrome with aspirin sensitivity. A family study.
The purpose of this study was to investigate a family with type IV Ehlers-Danlos syndrome (EDS-IV) and platelet sensitivity to aspirin, an association that, to our knowledge, has not been reported previously. Type IV Ehlers-Danlos syndrome is a rare disorder of type III collagen metabolism characterized by bruising easily and death at an early age from exsanguination by rupture of a major viscus or artery. Previous studies have suggested an association between various types of Ehlers-Danlos syndrome and abnormal platelet function. In this family, EDS-IV is inherited in an autosomal dominant pattern. The transmission of the platelet functional disorder from the proband to only one of two children affected with EDS-IV in this family suggests that there is a genetic component to this condition that is independent of EDS-IV.
Grenko
R T
RT
Department of Pathology, University of Vermont, College of Medicine, Medical Center Hospital of Vermont, Burlington 05405.
Burns
S L
SL
Golden
E A
EA
Byers
P H
PH
Bovill
E G
EG
D000328:Adult; D001241:Aspirin / Q000009:adverse effects*; D001777:Blood Coagulation / Q000187:drug effects*; D001792:Blood Platelets / Q000502:physiology; D002648:Child; D003094:Collagen / Q000032:analysis; D004535:Ehlers-Danlos Syndrome / Q000097:blood* / Q000235:genetics*; D005260:Female; D005340:Fibrinogen / Q000032:analysis; D006801:Humans; D008297:Male; D010375:Pedigree; D010974:Platelet Aggregation
D005340:Fibrinogen; D003094:Collagen; D001241:Aspirin