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The inhibition profile of methylene blue on nitrovasodilator induced cyclic guanosine monophosphate accumulation was quantitatively reproduced by extracellular generation of superoxide anion with xanthine (100 uM) and xanthine oxidase (5 mU)

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At the ultrastructural level, irregular and immature elastin fibers and fibers devoid of microfibrils were observed. These findings suggested that both elastic fiber abnormalities and reduced collagen content contribute to the clinical features seen in patients with clEDS. There were no consistent electron microscopy (EM) findings in the ten patients in our cohort who had EM performed

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Table I