Abstract
Children with low skeletal muscle mass caused by neuromuscular disorders (NMDs) or cerebral palsy (CP) have altered body composition and many are wheelchair dependent. Feeding and swallowing issues are frequently reported, and they are at increased risk of malnutrition and nutrient deficiencies. Poor nutrition, physical inactivity or use of glucocorticoids also increase the risk of osteoporosis in the patient group. The aim of the study was to conduct a vitamin-, nutritional, and bone health screening in children with low skeletal muscle mass due to CP and NMDs followed in our clinic. In an observational study, a total of 66 patients < 18 years were included with: spinal muscular atrophy (11), Duchenne muscular dystrophy (DMD) (23), Merosin-deficient-muscular dystrophy (5), other myopathies (3), Charcot-Marie-Tooth neuropathy (2), and CP (22). The majority of participants were moderately or severely affected by their disease, 46/66 were wheelchair dependent. The vitamin-, nutrition and bone health were assessed by dual X-ray absorptiometry scan, blood samples (vitamins, minerals, hormones, lipids), anthropometric measurements, and a three-day diet registration. The prevalence of severe underweight (BMI z-score <-3) and underweight (BMI z-score <-2) were 13% (n=9) and 6% (n=4). Furthermore, 12% (n=8) were obese (BMI z-score > 3), and 15% (n=10) were overweight (BMI z-score 2-3). The majority of overweight patients had DMD (13/18). Decreased bone mineral density (BMD) was found in 29 patients (44%) (BMD z-score <-2). Moreover, vitamin D insufficiency (vitamin D <50 nmol/L) and deficiency (vitamin D <25 nmol/L) were found in 18% (N=12) and 4.5% (N=3), respectively. Otherwise, calcium, phosphate and parathyroid hormone were in the normal range in most of the patients. Total-cholesterol and LDL-cholesterol were elevated in 8 patients (n=12%). These findings suggest that children with low skeletal muscle mass should be systemically and thoroughly assessed for malnutrition and overweight, to offer early nutritional support. As DMD boys are regularly assessed for decreased BMD, these findings suggest a need for expanding the assessment of BMD and vitamins in other NMDs and CP patients.
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