Persistent hyperinsulinemic hypoglycemia of infancy: long-term follow-up after surgical treatment

Aim The aim of this study was to investigate the long-term outcome of six children with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) after pancreatectomy who have been followed since 1990 at the Riyadh Military Hospital, Riyadh, Kingdom of Saudi Arabia. Patients and methods Data from six patients were retrospectively analyzed from chart review. PHHI was diagnosed on the basis of having high-insulin levels and low-glucose levels and a high insulin-to-glucose ratio. Lactate, pyruvate, ammonia, and urinary organic acid levels were recorded and tandem mass spectrometry screening was performed. The patients were assessed radiologically by MRI of the brain and by ultrasound examination of the abdomen. Patients who failed medical therapy underwent near-total pancreatectomy. Results Hypoglycemic convulsion was the most common presenting complaint. None of the patients responded to medical treatment and thus underwent surgery. Two patients still had hypoglycemic episodes after surgery, requiring medical treatment. However, they underwent subsequent surgery because of unresponsiveness to medical therapy. Three patients developed diabetes. None of them had malabsorption, and two patients later developed epilepsy. Conclusion The earlier the age at diagnosis, the better the neurological outcome. Delayed surgery might be associated with higher risk of development of diabetes. Near-total (90–95%) pancreatectomy is the treatment of choice for PHHI not responsive to medica