Persistent neonatal hypoglycemia due to hyperinsulinism: medical aspects

Area of Science:

• Pediatric Endocrinology
• Neonatal Medicine
• Surgical Pathology

Background:

• Persistent hypoglycemia in neonates presents a significant clinical challenge.
• Elevated or inappropriate insulin levels are key indicators in affected infants.
• Intractable cases often require aggressive therapeutic interventions.

Purpose of the Study:

• To evaluate the clinical course and treatment outcomes of persistent hypoglycemia in a cohort of infants.
• To investigate the pathological findings in the pancreas of affected neonates.
• To assess the long-term neurodevelopmental outcomes following various treatment modalities.

Main Methods:

• Retrospective analysis of nine infants diagnosed with persistent hypoglycemia.
• Detailed review of clinical data, including blood glucose and insulin levels.
• Documentation of treatment strategies: medical management (e.g., diazoxide) and surgical intervention (pancreatectomy).
• Pathological examination of pancreatic tissue for nesidioblastosis or endocrine-cell dysplasia.
• Long-term follow-up for psychomotor development and head growth.

Main Results:

• Seven infants exhibited hyperinsulinism, with high or inappropriately normal insulin levels during hypoglycemia.
• Six patients underwent pancreatectomy due to intractable hypoglycemia resistant to medical therapy.
• Three patients achieved spontaneous remission, one as early as 4 months of age.
• Pathologic pancreatic changes consistent with nesidioblastosis/endocrine-cell dysplasia were observed in surgically treated and biopsied patients.
• Among six patients followed for ≥24 months, four demonstrated normal psychomotor development, despite early growth disturbances in three.

Conclusions:

• Persistent infantile hypoglycemia, often linked to nesidioblastosis, necessitates tailored treatment approaches.
• Pancreatectomy can be an effective treatment for severe, intractable cases.
• While challenging, many infants, particularly those treated surgically, can achieve favorable neurodevelopmental outcomes.
• Early recognition and intervention are crucial for managing this condition.