Key result
Restrictive respiratory involvement was suggested in 9.8% of FSHD patients overall, occurring in 25.0% of those with FSHD2 compared to 7.5% with FSHD1 (P=0.17).
Population
61 genetically confirmed facioscapulohumeral muscular dystrophy (FSHD) participants (53 FSHD1 and 8 FSHD2)
Design
Cross-sectional
Authors
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Supports respiratory assessment in severe FSHD; hypothesis-generating for subtype differences.
Cross-Sectional (n=61)
Absolute Event Rate: 25% vs 7.5%
p-value: p=0.17
Restrictive respiratory involvement occurs in nearly 10% of FSHD patients and is associated with more severe disease and proximal lower extremity weakness.
Scully et al. (2014) conducted a cross-sectional in Facioscapulohumeral muscular dystrophy (FSHD) (n=61). FSHD2 vs. FSHD1 was evaluated on Restrictive respiratory involvement (p=0.17). Restrictive respiratory involvement was suggested in 9.8% of FSHD patients overall, occurring in 25.0% of those with FSHD2 compared to 7.5% with FSHD1 (P=0.17).
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