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Long-term follow up of persistent hyperinsulinaemic hypoglycaemia of infancy
Insights
Diazoxide effectively treated most children with hyperinsulinemic hypoglycemia. However, 10 patients required surgery, and 11 experienced neurological issues, highlighting the need for vigilant monitoring and early intervention in pediatric hypoglycemia cases.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Clinical Research
Background:
- Hyperinsulinemic hypoglycemia is a serious condition in children.
- Accurate diagnosis relies on specific biochemical markers.
- Treatment options include medication and surgery.
Purpose of the Study:
- To evaluate the efficacy of diazoxide in treating pediatric hyperinsulinemic hypoglycemia.
- To assess outcomes in patients who did not respond to diazoxide and required surgery.
- To identify factors associated with neurological sequelae in treated children.
Main Methods:
- Retrospective analysis of 26 children diagnosed with hyperinsulinemic hypoglycemia between 1975 and 1995.
- Diagnosis confirmed by high insulin:glucose ratio and low fasting free fatty acid and 3-hydroxybutyrate levels.
- Treatment with diazoxide (max 20 mg/kg/day); surgical intervention for non-responders.
Main Results:
- Sixteen out of 26 children responded to diazoxide treatment.
- Ten children failed to respond and underwent pancreatic resection; six had neonatal onset.
- Eleven children developed neurological sequelae; head growth correlated with neurological outcome.
Conclusions:
- Diazoxide is an effective initial treatment for many children with hyperinsulinemic hypoglycemia.
- Surgical intervention is necessary for non-responders, with a significant proportion having neonatal onset.
- Neurological sequelae are common, and electroencephalogram abnormalities may indicate subclinical hypoglycemia.
Abstract:
Twenty six children with hypoglycaemia were diagnosed and followed between 1975 and 1995. Diagnosis was confirmed by a high insulin:glucose ratio, and low free fatty acid and 3-hydroxybutyrate on fasting. All patients were treated with diazoxide at a maximum dose of 20 mg/kg/day. Requirement of a higher dose was considered as a failure of medical treatment and an indication for surgery. Sixteen children Responded to diazoxide; 10 failed to respond and underwent pancreatic resection. Six of the latter group started with symptoms in the neonatal period. Eleven of the 26 children have neurological sequelae. Head growth and neurological outcome correlated well. Additionally, non-specific electroencephalogram abnormalities (slow waves) appear to be indicative of subclinical hypoglycaemia during follow up.