TY - JOUR T1 - Hypoglycaemia in patients with type 1 SMA: an underdiagnosed problem? JF - Archives of Disease in Childhood JO - Arch Dis Child SP - 707 LP - 707 DO - 10.1136/archdischild-2019-318120 VL - 105 IS - 7 AU - Beatrice Berti AU - Roberta Onesimo AU - Daniela Leone AU - Concetta Palermo AU - Valentina Giorgio AU - Danilo Buonsenso AU - Marika Pane AU - Eugenio Mercuri Y1 - 2020/07/01 UR - http://adc.bmj.com/content/105/7/707.abstract N2 - 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. … ER -