Table 1.

Clinical characteristics of the patient reported

Patient characteristics
Current age	4 years
Age at presentation	8 months
Main presenting feature	Hyperinsulinemic Hypoglycemia
Family history	Emirati, first‐degree cousins
	No history of hypoglycemia or liver disease
Clinical features	Normal development
	Normal growth
	Hepatomegaly
Biochemical investigations	High liver transaminases
	Low albumin and total protein
	Hypoglycemia
	Positive urinary ketones
	High insulin level
	Positive glucagon challenge
Imaging	Hepatomegaly with a liver nodule
Genetic test result	Novel homozygous mutation (p.Ala288Val) at the MPI gene
	Both parents are carriers
Treatment	Diazoxide‐responsive