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There has been increasing evidence that hyperglycaemia, hypoglycaemia and other metabolic abnormalities may occur in the severe end of the spectrum of type 1 spinal muscular atrophy (SMA). An increase in alpha cell number in pancreatic islet composition has been described in SMA knockout mice and in patients with type 1 SMA.1
Recent care recommendations suggest that prolonged fasting should be avoided in order to prevent the risk of hypoglycaemia.2 Type 1 children are often underweight, have a reduction of muscle mass and are therefore more likely to develop hypoglycaemia in the setting of a catabolic state.3 While there is a strong agreement on the need to avoid fasting, there is less consensus regarding the appropriate timing, with many experts recommending less than 6 hours. …
BB and RO are joint first authors.
Contributors BB, RO, MP and EM contributed to the conception and design of the study. BB, RO, DL, CP, VG, DB, MP and EM contributed to the acquisition and analysis of data. BB, RO, DL, CP, MP and EM contributed to drafting the text and preparing the table.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval The study is part of a larger natural history study on SMA approved by the ethics committee of our institution and fully informed consent from the parents of participants was obtained before the study.
Provenance and peer review Not commissioned; externally peer reviewed.
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