2017
DOI: 10.1016/j.clnu.2016.10.020
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Abstract: SummaryBackground & aimsDifferent neuromuscular functional domains in types I and II Spinal Muscular Atrophy (SMAI and SMAII) could lead to differences in body composition (BC) and resting energy expenditure (REE). Their identification could provide the key to defining appropriate strategies in clinical dietary management, but data comparing SMAI and SMAII in terms of BC and REE are not yet available. We measured total and regional fat (FM), lean (LBM), mineral (BMC) masses, body water (total, intra- and extra… Show more

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Cited by 38 publications
(51 citation statements)
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“…FM was increased in both SMAI and SMAII, showing increasing values with age. Previous studies by our group and others have shown that FM is increased and FFM decreased in SMA patients compared with healthy peers, and that FFM and lean body mass are significantly lower in SMAI than SMAII . Some authors have also reported that adiposity is increased in nonambulatory, high‐functioning SMAII compared with low‐functioning, and ambulatory patients .…”
Section: Discussionmentioning
confidence: 55%
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“…FM was increased in both SMAI and SMAII, showing increasing values with age. Previous studies by our group and others have shown that FM is increased and FFM decreased in SMA patients compared with healthy peers, and that FFM and lean body mass are significantly lower in SMAI than SMAII . Some authors have also reported that adiposity is increased in nonambulatory, high‐functioning SMAII compared with low‐functioning, and ambulatory patients .…”
Section: Discussionmentioning
confidence: 55%
“…Despite advances in clinical and pharmacological management of SMA patients, knowledge of their nutritional status and optimal T A B L E 1 Anthropometric measurements, body composition, and motor function scores of study subjects 6,14,15 and that FFM and lean body mass are significantly lower in SMAI than SMAII. 6 Some authors have also reported that adiposity is increased in nonambulatory, high-functioning SMAII compared with low-functioning, and ambulatory patients. 16 Due to this altered BC and the lack of SMA-specific growth charts, regular assessment of BC by DEXA has been included in the updated Standard of Care Recommendations to ensure proper nutritional management.…”
Section: Discussionmentioning
confidence: 99%
“…Spinal muscular atrophy (SMA) is a rare and severe neuromuscular disease associated with feeding disorders [1], changes in energy metabolism and impairment of nutritional status [2]. The recent advent of new drugs and clinical trials for SMA are changing the natural history of the disease [1], so now more than ever is important to focus on the correct assessment of nutritional status in SMA patients.…”
Section: Introductionmentioning
confidence: 99%
“…Patients with SMA usually display higher fat mass (FM) and lower fat-free mass (FFM), as measured by dual X-ray energy absorptiometry (DXA), with respect to the respective reference values for sex and age; indirect calorimetry shows a reduced resting energy expenditure (REE), than that derived from predictive formulas (i.e., Harris and Benedict, and Schofield) [ 3 ].…”
Section: Introductionmentioning
confidence: 99%