Background:

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) affects between 0.5% and 5% of pediatric patients. This condition is caused by a dysfunction of pancreatic β-cells, leading to tumors and hyperinsulinism, which result in persistent hypoglycemia. These episodes are often resistant to conventional treatment and require interventions to reduce insulin production and increase glucose levels, thereby restoring metabolic balance.

Clinical cases:

We present three cases of PHHI. The first is a four-day-old Rarámuri patient with persistent hypoglycemia, diagnosed after a pancreatectomy and biopsy. The second case is a three-day-old Rarámuri patient with persistent hypoglycemia and a pancreatic lesion detected by scintigraphy, with PHHI confirmed after pancreatectomy. The third case is a three-day-old female patient with hypoactivity and persistent hypoglycemia, who was also diagnosed with PHHI after a pancreatectomy.

Conclusions:

While clinical presentation and biochemical findings raise high diagnostic suspicion, histopathological confirmation is essential. Early diagnosis is key to preventing long-term sequelae. PHHI should be considered in the differential diagnosis of hyperinsulinemic hypoglycemia, such as prematurity, intrauterine growth restriction, low birth weight, maternal diabetes, tyrosinemia type 1, or Beckwith-Wiedemann syndrome. These cases highlight the complexity of PHHI and its unusual presentation in the Rarámuri ethnic group, underscoring the importance of culturally sensitive medical care.