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- W2080920210 abstract "Hereditary inclusion body myopathy (HIBM) or GNE myopathy is caused by a defect in the biosynthetic pathway for sialic acid. Muscle weakness begins in adulthood in the distal legs and progresses over decades, although the quadriceps remain relatively strong. The objective of this study was to characterize the pattern and extent of weakness, evaluate functional limitations and establish the relationship between strength and function in adults with HIBM. Ambulatory subjects enrolled in a Phase 2 study of extended release sialic acid were evaluated prior to randomization. Assessments included hand-held dynamometry (HHD), manual muscle testing, a six-minute walk test (6MWT), gait speed, sit-to-stand, stair climb and weighted arm lift tests. 47 subjects, 29 females and 18 males, with a mean age of 40 years (18–64) were evaluated. Most subjects utilized orthoses (62%) or an assistive device (51%) for walking. Intraclass correlation coefficients for repeat HHD and functional tests ranged from 0.814 to 0.987. Mean individual hip flexor, hip adductor and knee flexor force was ⩽20% of predicted normal and 81% of subjects lacked full anti-gravity dorsiflexion power bilaterally. Knee extensors were the strongest muscle group at 58% of predicted bilaterally. The mean 6MWT distance was 278 m, 39% of normal. Knee extensor strength was not a strong predictor of 6MWT distance (r = 0.60). A composite measure of lower extremity (LE) strength without knee extensors showed a strong positive relationship to 6MWT distance (r = 0.92) and sit-to-stand test repetitions (r = 0.82). Composite LE strength of" @default.
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- W2080920210 date "2013-10-01" @default.
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- W2080920210 title "P.3.2 Characterization of strength and function in adults with inclusion body myopathy (HIBM)/GNE myopathy" @default.
- W2080920210 doi "https://doi.org/10.1016/j.nmd.2013.06.427" @default.
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