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Metabolic studies in a child with a pancreatic insulinoma
Insights
A child
Area of Science:
- Pediatric Endocrinology
- Pediatric Neurology
- Surgical Oncology
Background:
- Epilepsy management in children can be challenging, particularly when seizures become refractory to standard anticonvulsant medications.
- Hypoglycemia, or low blood sugar, can present with neurological symptoms, including seizures, and requires thorough investigation.
- Islet cell tumors, such as insulinomas, are rare in children but can cause significant metabolic disturbances.
Observation:
- An 8-year-old boy with a history of convulsive disorder experienced a relapse of seizures after a period of remission.
- The patient exhibited fasting and postprandial hypoglycemia with hyperinsulinemia, suggesting an underlying endocrine issue.
- Preoperative imaging revealed a pancreatic mass, later confirmed as a benign insulinoma.
Findings:
- Surgical extirpation of the intrapancreatic insulinoma resolved the hypoglycemia and hyperinsulinemia.
- Somatostatin infusion transiently improved glucose levels and decreased insulin concentrations, aiding in preoperative management.
- The patient's seizure disorder was linked to the previously undiagnosed insulinoma.
Implications:
- Investigating islet cell tumors is crucial in pediatric patients with refractory seizure disorders.
- Early diagnosis and surgical removal of insulinomas can effectively manage associated hypoglycemia and neurological symptoms.
- Somatostatin serves as a potential therapeutic agent for stabilizing glucose and insulin levels in patients with insulinomas prior to surgery.
Abstract:
An 8-year-old boy with a convulsive disorder for 3 1/2 years remined seizure free for 20 months while being treated with phenytoin (diphenylhydantoin) sodium, and then he had a relapse. He first demonstrated hypoglycemia when he fasted prior to being placed on a ketogenic diet. An oral glucose tolerance test indicated fasting and postglucose hypoglycemia and substantial hyperinsulinemia. Somatostatin infusion resulted in a modest increase in plasma glucose levels and a decrease in serum insulin concentrations. A discrete pancreatic mass was demonstrated preoperatively by celiac angiography that on surgical extirpation, proved to be a benign intrapancreatic insulinoma. Evaluation for islet cell tumors is of importance in children with seizure disorders unresponsive to anticonvulsant medication. Furthermore, somatostatin may be useful preoperatively in maintaining normal blood glucose concentrations in patients with islet cell adenomas.