Table 2.

Case illustration.

Patient #14 carrying a GCK mutation had diazoxide-responsive CHI. A nasogastric tube had been inserted at birth to support feeding and manage hypoglycemia. Although glycemic stability was achieved, the patient continued to experience increasing problems with vomiting and oral feed intolerance, in spite of antireflux medication and variations in milk formulations, thereby requiring persistent nasogastric tube feeding as a reliable source of nutritional intake. Due to the reliance on nasogastric tube feeding, a gastrostomy tube was inserted at the age of 157 days. The patient required regular speech and language therapy and dietetic input and at the age of 2.5 years gradually improved oral intake, although occasionally reliant on gastrostomy feeding. This case illustrates the practical difficulty with weaning nasogastric tube feeding in some children with CHI complicated by FPs.