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. 2022 May 9;7(9):e155201. doi: 10.1172/jci.insight.155201

Figure 1. Lower lean mass in FRDA adults correlates with increased FRDA clinical disease severity.

Figure 1

(A) Body composition z scores in healthy adults (n = 24) and adults with FRDA (n = 24). Two-tailed t tests between the 2 groups revealed that while there was no difference between the fat mass index (FMI) z scores in adults with and without FRDA, lean BMI (LBMI) was lower in adults with FRDA (–1.31 ± 1.21) compared with those without (–0.67 ± 0.87) (difference of –0.64; 95% CI, –1.25 to –0.02; P = 0.04). The same was true for appendicular lean mass index (ALMI) z scores, which were –1.61 ± 1.38 for individuals with FRDA and –0.5 ± 1.02 for those without (difference of –1.11; 95% CI, –1.82 to –0.41, P = 0.003), and for ALMI adjusted for fat mass, which were –2.41 ± 1.69 for adults with FRDA and –0.55 ± 1.47 for adults without FRDA (difference of –1.86; 95% CI, –2.78 to –0.95; P = 0.0002). (B) Body composition z scores in pediatric participants with FRDA (n = 10). One-tailed t tests comparing z scores from children with FRDA to 0, the expected mean z score based on the reference population, revealed no difference between the height and BMI z scores of children with FRDA and a population of healthy children with an average z score of 0. FMI was higher in children with FRDA (0.56 ± 0.73, 95% CI, 0.04 to 1.08; P = 0.04) while LBMI was lower than expected in children with FRDA (–2.45 ± 1.65, 95% CI, –3.63 to –1.27; P = 0.001). The box plots depict the minimum and maximum values (whiskers), the upper and lower quartiles, and the median. The length of the box represents the interquartile range. (C) Pearson’s correlation between fat-adjusted ALMI z score and modified Friedreich’s Ataxia Rating Scale (mFARS) scores, a marker of clinical severity, in adults with FRDA. R = –0.5; 95% CI, –0.76 to –0.11; P = 0.02.