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Reducing the muscle free carnitine pool during conditions of high PDC flux limits the ability of CPT1 (the mediator enzyme bouncer at the door) to transport long-chain acyl-CoA into the mitochondrial matrix and thus the rate of fat oxidation

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Signs and symptoms Loose joints Joint pain Stretchy, velvety skin Fragile skin Abnormal scar formation Complications Aortic dissection Joint dislocations Scoliosis Chronic pain Early osteoarthritis Management Obstetrical anesthesia Difficult management, requires multidisciplinary effort High risk of preterm labor, uterine rupture, and hemorrhage Neuraxial anesthesia is relatively contraindicated, may need general anesthesia If vascular subtype recommendations are termination of pregnancy or Cesarean section before 32 weeks Keep in mind Avoid ambulatory surgery in EDS patients Be aware of typical EDS-related emergency-like situations such as difficult airway status, bleeding risks, and organ rupture in specific subtypes Suggested reading Malfait F, Francomano C, Byers P, et al