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Prevalence of pruritus and xanthoma among survivors by age. Pruritus, as measured by CSS, and the burden of xanthomas improve over time in ALGS survivors with native livers. CSS scores the physical manifestations of pruritus as follows: 0 = none, 1 = rubbing or mild scratching when undistracted, 2 = active scratching without evident skin abrasions, 3 = abrasion evident, 4 = cutaneous mutilation, hemorrhage, and scarring evident.
