Related Experiment Videos
Neurologic outcomes of 90 neonates and infants with persistent hyperinsulinemic hypoglycemia
F Menni1, P de Lonlay, C Sevin
1Fédération de Pédiatrie, Diabète-Métabolisme-Neurologie, Centre Hospitalier Universitaire Necker-Enfants Malades, Paris, France.
Insights
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) poses significant neurologic risks, particularly in neonates. Early diagnosis and treatment are crucial to mitigate severe outcomes like mental retardation and epilepsy.
Area of Science:
- Pediatric Endocrinology
- Neonatal Neurology
- Metabolic Disorders
Background:
- Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a rare but serious condition characterized by unregulated insulin secretion leading to severe hypoglycemia.
- Neurologic complications, including developmental delays and epilepsy, are significant concerns for affected infants.
- Understanding risk factors and treatment outcomes is essential for improving patient management.
Purpose of the Study:
- To investigate the long-term neurologic outcomes in infants and neonates diagnosed with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).
- To identify risk factors associated with adverse neurologic development in PHHI patients.
- To compare neurologic outcomes based on treatment modality (medical vs. surgical) and disease presentation (neonatal vs. infancy-onset).
Main Methods:
- A retrospective study analyzing the neurologic development of 90 patients with PHHI.
- Patients were categorized by age of onset (neonatal vs. infancy) and treatment (medical vs. surgical).
- Surgical interventions included pancreatectomy for focal adenomatous hyperplasia or diffuse hyperinsulinism.
Main Results:
- Severe psychomotor retardation was observed in 7 patients, predominantly those with neonatal-onset PHHI (6/7).
- Epilepsy was present in 16 patients, with neonatal onset identified as a primary risk factor for severe retardation and epilepsy.
- Medically treated patients generally experienced less severe outcomes compared to surgically treated individuals, irrespective of focal or diffuse disease forms.
Conclusions:
- Neonatal-onset PHHI remains a critical condition with a high risk of severe mental retardation and epilepsy.
- Prompt recognition and management are vital to prevent severe neurologic sequelae in infants with PHHI.
- Further research into optimizing treatment strategies for PHHI is warranted to improve neurodevelopmental outcomes.
Objective:
To evaluate the neurologic outcomes of neonates and infants suffering from persistent hyperinsulinemic hypoglycemia of infancy (PHHI).
Methods:
The neurologic development of 90 PHHI patients was studied retrospectively. Sixty-three patients were treated surgically and 27 were treated medically. Fifty-four patients were neonates, of whom 8 were treated medically and 46 were operated on (19 for a focal adenomatous hyperplasia and 27 for diffuse hyperinsulinism). Thirty-six patients had infancy-onset hyperinsulinism, of whom 19 were treated medically and 17 underwent pancreatectomy (10 patients for a focal adenomatous hyperplasia and 7 for diffuse hyperinsulinism).
Results:
Severe psychomotor retardation was found in 7 patients, 6 with neonatal-onset PHHI. Intermediate psychomotor disability existed in 12 patients; epilepsy existed in 16. Neonatal-onset was the main risk factor for severe retardation or epilepsy. Medically treated patients were less severely affected than those treated by surgery, and there was no difference between the diffuse and focal forms of hyperinsulinism.
Conclusion:
Neonatal hyperinsulinemic hypoglycemia is still a severe disease with an important risk to rapidly develop severe mental retardation and epilepsy.