Hypoinsulinemic hypoglycemia with hemihypertrophy in a Filipino infant: management in a resource-limited setting.

Persistent infantile hypoglycemia is a medical emergency associated with significant risk of neurological injury. Although most commonly attributed to congenital hyperinsulinism, disorders of impaired insulin signaling with hypoketotic hypoglycemia despite low or undetectable insulin occur in fewer than 1 in 1 000 000 infants worldwide, leading to misdiagnosis and ineffective therapy. We report a Filipino infant with recurrent fasting hypoglycemic seizures and hemihypertrophy. Biochemical evaluation showed absent ketones with low to mildly detectable insulin, suggesting dysregulated insulin signaling. In a resource-limited setting without access to diazoxide or advanced imaging, clinical and biochemical evaluation guided management. Genetic testing later confirmed a pathogenic AKT2 variant (c.49G>A; p.Glu17Lys). Euglycemia was achieved through individualized nutritional strategies including uncooked cornstarch and nocturnal enteral feeding. This case highlights clues to AKT2-related hypoinsulinemic hypoglycemia with hemihypertrophy (HIHGHH), demonstrates that low insulin does not exclude insulin-mediated hypoglycemia, and underscores the importance of careful clinical evaluation alongside molecular diagnosis to guide management and prevent neurologic sequelae.