Arch Pathol Lab Med. 1993 Oct;117(10):989-92.

Type IV Ehlers-Danlos syndrome with aspirin sensitivity. A family study.

Grenko RT, Burns SL, Golden EA, Byers PH, Bovill EG.

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Department of Pathology, University of Vermont, College of Medicine, Medical Center Hospital of Vermont, Burlington 05405.

Abstract

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.

PMID:: 8215841; [PubMed - indexed for MEDLINE]

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Chemical compound information

Aspirin

MW: 180.15 g/mol

MF: C₉H₈O₄

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