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Cardiovascular Cardiac findings in Ehlers-Danlos syndrome include: Heart valve defects (e.g., mitral valve prolapse) Cystic medial degeneration: can lead to aortic dilation, aneurysms, or dissection Aneurysms/dissections of the iliac, splenic, or renal arteries Berry aneurysms: rupture leads to subarachnoid hemorrhage Musculoskeletal Joint hypermobility with a tendency towards dislocation Skeletal abnormalities (e.g., scoliosis, pectus deformity) Skin Tendency to bruise easily Skin hyperextensibility Frequent skin lacerations (e.g., with minor scratches) Poor skin healing (e.g., following surgery ) Atrophic scars Eyes Myopia Retinal detachment and tears Other Hernias Organ rupture (e.g., gravid uterus) causing pain and (potentially life-threatening) internal bleeding, especially seen with vascular EDS Cervical insufficiency, uterine prolapse Dental abnormalities: hypodontia, delayed eruption, molluscoid pseudotumors The classic presentation of EDS involves hyperextensible skin, joint hypermobility, and a tendency to bleed easily

204 SteinbrennerH.SiesH

200 mg/kg/d disulfiram in 0.2 mL olive oil

Glutathione is a naturally occurring antioxidant the body already makes to defend cells from damage