The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience.

Abstract excerpt

PURPOSE: Ehlers-Danlos syndrome type IV (EDS-IV) results from abnormal procollagen III synthesis and leads to arterial, intestinal, and uterine rupture. The purpose of this study was to review the spectrum, management, and clinical outcome of EDS-IV patients. METHODS: We retrospectively reviewed the clinical data of 31 patients (15 male and 16 female) with a clinical diagnosis of EDS-IV treated over a 30-year...

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