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• W1994348564 abstract "Congenital muscular dystrophy (CMD) is a group of heterogeneous disorders with progressive respiratory insufficiency. Recommendations include screening for respiratory insufficiency by spirometry and obtaining a polysomnogram if the vital capacity (VC) is <60% predicted (a predictor of sleep disordered breathing), or if there is a >20% decline between sitting and supine VC measures. Reports of pulmonary function, especially change in sitting to supine forced VC (FVC) % predicted, and its association with CMD subtypes and ambulatory status are limited. Therefore, in 49 individuals with CMD [Collagen VI-related myopathy (CVI-RM; n = 30) and LAMA2-related muscular dystrophy (LAMA2-MD; n = 19], we compared sitting with supine FVC % predicted, ambulatory and non-invasive mechanical ventilation (NIV) status. The CVI-RM cohort comprised ages 4–61 years with 18 (60%) males and 22 (73%) children (⩽18 years). The LAMA2-MD cohort comprised ages 5–54 years with 7 (37%) males and 18 (95%) children. We found a significant decrease in FVC % predicted from sitting to supine in subjects with CVI-RM only (p < 0.0001). 22 (73%) subjects with CVI-RM were ambulatory, and 5 (42%) of the CVI-RM subjects with FVC <60% were ambulatory. In contrast, 4 (21%) of LAMA2-MD subjects were ambulatory, and none of LAMA2-MD subjects with FVC <60% were ambulatory. %FVC sitting correlated moderately with ambulatory status (0.553, p = 0.002) and inversely with NIV status (−0.539, p = 0.002) in CVI-RM, but only showed a trend with ambulatory status in LAMA2-MD (0.443, p = 0.058). In conclusion, this is the first report of change in sitting to supine % FVC predicted in subjects with CMD to our knowledge. This study highlights the physiologic differences between CMD subtypes and suggests a need for a more tailored approach to screening. Additional studies are needed to evaluate the predictive power of postural changes in FVC for nocturnal hypoventilation and the need to initiate ventilator support. Congenital muscular dystrophy (CMD) is a group of heterogeneous disorders with progressive respiratory insufficiency. Recommendations include screening for respiratory insufficiency by spirometry and obtaining a polysomnogram if the vital capacity (VC) is <60% predicted (a predictor of sleep disordered breathing), or if there is a >20% decline between sitting and supine VC measures. Reports of pulmonary function, especially change in sitting to supine forced VC (FVC) % predicted, and its association with CMD subtypes and ambulatory status are limited. Therefore, in 49 individuals with CMD [Collagen VI-related myopathy (CVI-RM; n = 30) and LAMA2-related muscular dystrophy (LAMA2-MD; n = 19], we compared sitting with supine FVC % predicted, ambulatory and non-invasive mechanical ventilation (NIV) status. The CVI-RM cohort comprised ages 4–61 years with 18 (60%) males and 22 (73%) children (⩽18 years). The LAMA2-MD cohort comprised ages 5–54 years with 7 (37%) males and 18 (95%) children. We found a significant decrease in FVC % predicted from sitting to supine in subjects with CVI-RM only (p < 0.0001). 22 (73%) subjects with CVI-RM were ambulatory, and 5 (42%) of the CVI-RM subjects with FVC <60% were ambulatory. In contrast, 4 (21%) of LAMA2-MD subjects were ambulatory, and none of LAMA2-MD subjects with FVC <60% were ambulatory. %FVC sitting correlated moderately with ambulatory status (0.553, p = 0.002) and inversely with NIV status (−0.539, p = 0.002) in CVI-RM, but only showed a trend with ambulatory status in LAMA2-MD (0.443, p = 0.058). In conclusion, this is the first report of change in sitting to supine % FVC predicted in subjects with CMD to our knowledge. This study highlights the physiologic differences between CMD subtypes and suggests a need for a more tailored approach to screening. Additional studies are needed to evaluate the predictive power of postural changes in FVC for nocturnal hypoventilation and the need to initiate ventilator support." @default.
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• W1994348564 date "2013-10-01" @default.
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• W1994348564 title "P.1.3 Postural changes in forced vital capacity characterize congenital muscular dystrophy subtypes" @default.
• W1994348564 doi "https://doi.org/10.1016/j.nmd.2013.06.387" @default.
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