Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II. / Orngreen, Mette Cathrine; Andersen, Annarita G.; Eisum, Anne-Sofie; Hald, Emma J.; Raaschou-Pedersen, Daniel E.; Lokken, Nicoline; Hoi-Hansen, Christina E.; Vissing, John; Born, Alfred P.; van Hall, Gerrit.

In: Acta Paediatrica, Vol. 110, No. 12, 2021, p. 3367-3375.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Orngreen, MC, Andersen, AG, Eisum, A-S, Hald, EJ, Raaschou-Pedersen, DE, Lokken, N, Hoi-Hansen, CE, Vissing, J, Born, AP & van Hall, G 2021, 'Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II', Acta Paediatrica, vol. 110, no. 12, pp. 3367-3375. https://doi.org/10.1111/apa.16074

APA

Orngreen, M. C., Andersen, A. G., Eisum, A-S., Hald, E. J., Raaschou-Pedersen, D. E., Lokken, N., Hoi-Hansen, C. E., Vissing, J., Born, A. P., & van Hall, G. (2021). Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II. Acta Paediatrica, 110(12), 3367-3375. https://doi.org/10.1111/apa.16074

Vancouver

Orngreen MC, Andersen AG, Eisum A-S, Hald EJ, Raaschou-Pedersen DE, Lokken N et al. Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II. Acta Paediatrica. 2021;110(12):3367-3375. https://doi.org/10.1111/apa.16074

Author

Orngreen, Mette Cathrine ; Andersen, Annarita G. ; Eisum, Anne-Sofie ; Hald, Emma J. ; Raaschou-Pedersen, Daniel E. ; Lokken, Nicoline ; Hoi-Hansen, Christina E. ; Vissing, John ; Born, Alfred P. ; van Hall, Gerrit. / Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II. In: Acta Paediatrica. 2021 ; Vol. 110, No. 12. pp. 3367-3375.

Bibtex

@article{584bf0934b7d48dabdb52c7dcc578ba8,
title = "Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II",
abstract = "Aim This study explored hypoglycaemia and metabolic crises, including hyperketosis, in patients with spinal muscular atrophy (SMA). Methods The study comprised four adolescents aged 15-17 and six adults aged 19-37 with SMA type II and eight adult controls aged 21-41, who were recruited by the Rigshospitalet, Denmark, from May 1st to October 30th 2017. We used stable isotope technique and indirect calorimetry to investigate fat and glucose metabolism during a 24-h fast or until hypoglycaemia occurred. Results All patients with SMA II developed moderate to severe hyperketosis and 60% had symptoms of hypoglycaemia or blood glucose levels below 3 mmol/L. None of the controls developed hyperketosis or hypoglycaemia. Plasma bicarbonate decreased, in line with increased ketone bodies, indicating the start of metabolic acidosis in patients with SMA II. Increased fat production and utilisation were seen in healthy controls during the fasting period, but were absent in patients with SMA II, indicating blunted fat oxidation. Conclusion Low skeletal muscle mass was the best explanation for why patients with SMA II had an increased risk of hypoglycaemia, hyperketosis, metabolic acidosis and disturbed fat and glucose metabolism during fasting. These risks have implications for children facing surgery and those with severe illnesses.",
keywords = "fasting, fatty acid oxidation, hyperketosis, hypoglycaemia, spinal muscular atrophy, KETOTIC HYPOGLYCEMIA, GLUCOSE",
author = "Orngreen, {Mette Cathrine} and Andersen, {Annarita G.} and Anne-Sofie Eisum and Hald, {Emma J.} and Raaschou-Pedersen, {Daniel E.} and Nicoline Lokken and Hoi-Hansen, {Christina E.} and John Vissing and Born, {Alfred P.} and {van Hall}, Gerrit",
year = "2021",
doi = "10.1111/apa.16074",
language = "English",
volume = "110",
pages = "3367--3375",
journal = "Acta Paediatrica",
issn = "0803-5253",
publisher = "Wiley-Blackwell",
number = "12",

}

RIS

TY - JOUR

T1 - Prolonged fasting-induced hyperketosis, hypoglycaemia and impaired fat oxidation in child and adult patients with spinal muscular atrophy type II

AU - Orngreen, Mette Cathrine

AU - Andersen, Annarita G.

AU - Eisum, Anne-Sofie

AU - Hald, Emma J.

AU - Raaschou-Pedersen, Daniel E.

AU - Lokken, Nicoline

AU - Hoi-Hansen, Christina E.

AU - Vissing, John

AU - Born, Alfred P.

AU - van Hall, Gerrit

PY - 2021

Y1 - 2021

N2 - Aim This study explored hypoglycaemia and metabolic crises, including hyperketosis, in patients with spinal muscular atrophy (SMA). Methods The study comprised four adolescents aged 15-17 and six adults aged 19-37 with SMA type II and eight adult controls aged 21-41, who were recruited by the Rigshospitalet, Denmark, from May 1st to October 30th 2017. We used stable isotope technique and indirect calorimetry to investigate fat and glucose metabolism during a 24-h fast or until hypoglycaemia occurred. Results All patients with SMA II developed moderate to severe hyperketosis and 60% had symptoms of hypoglycaemia or blood glucose levels below 3 mmol/L. None of the controls developed hyperketosis or hypoglycaemia. Plasma bicarbonate decreased, in line with increased ketone bodies, indicating the start of metabolic acidosis in patients with SMA II. Increased fat production and utilisation were seen in healthy controls during the fasting period, but were absent in patients with SMA II, indicating blunted fat oxidation. Conclusion Low skeletal muscle mass was the best explanation for why patients with SMA II had an increased risk of hypoglycaemia, hyperketosis, metabolic acidosis and disturbed fat and glucose metabolism during fasting. These risks have implications for children facing surgery and those with severe illnesses.

AB - Aim This study explored hypoglycaemia and metabolic crises, including hyperketosis, in patients with spinal muscular atrophy (SMA). Methods The study comprised four adolescents aged 15-17 and six adults aged 19-37 with SMA type II and eight adult controls aged 21-41, who were recruited by the Rigshospitalet, Denmark, from May 1st to October 30th 2017. We used stable isotope technique and indirect calorimetry to investigate fat and glucose metabolism during a 24-h fast or until hypoglycaemia occurred. Results All patients with SMA II developed moderate to severe hyperketosis and 60% had symptoms of hypoglycaemia or blood glucose levels below 3 mmol/L. None of the controls developed hyperketosis or hypoglycaemia. Plasma bicarbonate decreased, in line with increased ketone bodies, indicating the start of metabolic acidosis in patients with SMA II. Increased fat production and utilisation were seen in healthy controls during the fasting period, but were absent in patients with SMA II, indicating blunted fat oxidation. Conclusion Low skeletal muscle mass was the best explanation for why patients with SMA II had an increased risk of hypoglycaemia, hyperketosis, metabolic acidosis and disturbed fat and glucose metabolism during fasting. These risks have implications for children facing surgery and those with severe illnesses.

KW - fasting

KW - fatty acid oxidation

KW - hyperketosis

KW - hypoglycaemia

KW - spinal muscular atrophy

KW - KETOTIC HYPOGLYCEMIA

KW - GLUCOSE

U2 - 10.1111/apa.16074

DO - 10.1111/apa.16074

M3 - Journal article

C2 - 34407566

VL - 110

SP - 3367

EP - 3375

JO - Acta Paediatrica

JF - Acta Paediatrica

SN - 0803-5253

IS - 12

ER -

ID: 286635220