Metabolic assessment in children with neuromuscular disorders shows risk of liver enlargement, steatosis and fibrosis
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Metabolic assessment in children with neuromuscular disorders shows risk of liver enlargement, steatosis and fibrosis. / Naume, Marie Mostue; Jørgensen, Marianne Hørby; Høi-Hansen, Christina Engel; Born, Alfred Peter; Vissing, John; Borgwardt, Lise; Stærk, Dorte Marianne Rohde; Ørngreen, Mette Cathrine.
In: Acta Paediatrica, International Journal of Paediatrics, Vol. 112, No. 4, 2023, p. 846-853.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Metabolic assessment in children with neuromuscular disorders shows risk of liver enlargement, steatosis and fibrosis
AU - Naume, Marie Mostue
AU - Jørgensen, Marianne Hørby
AU - Høi-Hansen, Christina Engel
AU - Born, Alfred Peter
AU - Vissing, John
AU - Borgwardt, Lise
AU - Stærk, Dorte Marianne Rohde
AU - Ørngreen, Mette Cathrine
N1 - Publisher Copyright: © 2022 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.
PY - 2023
Y1 - 2023
N2 - Aim: The aim of this study was to conduct a metabolic and nutritional assessment of children with neuromuscular disorders, including the investigation of the liver and bone mineral density. Methods: In this observational study, we included 44 children with neuromuscular disorders. The nutritional status, bone health and liver were assessed by ultrasound, transient elastography, dual X-ray absorptiometry scan, blood samples, anthropometric measurements and 3-day diet registration. Results: Liver involvement was found in 31.0%: liver enlargement in 7.1%, steatosis in 4.8%, fibrosis in 14.3% and liver enlargement together with steatosis or fibrosis was found in 4.8%. These changes were found in 9/23 patients with Duchenne muscular dystrophy, 4/9 patients with spinal muscular atrophy type II and 0/12 patients with other neuromuscular diagnoses. Low bone mineral density was found in 44.0% of the patients, though the majority used daily vitamin D and calcium supplements. Vitamin D insufficiency or deficiency was found in 22.6%. Conclusion: The metabolic assessment in children with neuromuscular disorders shows an increased risk of liver enlargement, steatosis and fibrosis. Possible causes are obesity, decreased mobility, low skeletal muscle mass and for a subgroup the use of glucocorticoids. The findings suggest that monitoring liver function should be part of the nutritional assessment in patients with neuromuscular disorders.
AB - Aim: The aim of this study was to conduct a metabolic and nutritional assessment of children with neuromuscular disorders, including the investigation of the liver and bone mineral density. Methods: In this observational study, we included 44 children with neuromuscular disorders. The nutritional status, bone health and liver were assessed by ultrasound, transient elastography, dual X-ray absorptiometry scan, blood samples, anthropometric measurements and 3-day diet registration. Results: Liver involvement was found in 31.0%: liver enlargement in 7.1%, steatosis in 4.8%, fibrosis in 14.3% and liver enlargement together with steatosis or fibrosis was found in 4.8%. These changes were found in 9/23 patients with Duchenne muscular dystrophy, 4/9 patients with spinal muscular atrophy type II and 0/12 patients with other neuromuscular diagnoses. Low bone mineral density was found in 44.0% of the patients, though the majority used daily vitamin D and calcium supplements. Vitamin D insufficiency or deficiency was found in 22.6%. Conclusion: The metabolic assessment in children with neuromuscular disorders shows an increased risk of liver enlargement, steatosis and fibrosis. Possible causes are obesity, decreased mobility, low skeletal muscle mass and for a subgroup the use of glucocorticoids. The findings suggest that monitoring liver function should be part of the nutritional assessment in patients with neuromuscular disorders.
KW - bone mineral density
KW - fibrosis
KW - liver involvement
KW - neuromuscular disorders
KW - overweight
KW - steatosis
U2 - 10.1111/apa.16649
DO - 10.1111/apa.16649
M3 - Journal article
C2 - 36579362
AN - SCOPUS:85146329685
VL - 112
SP - 846
EP - 853
JO - Acta Paediatrica
JF - Acta Paediatrica
SN - 0803-5253
IS - 4
ER -
ID: 335679194