Neurologic outcomes of 90 neonates and infants with persistent hyperinsulinemic hypoglycemia
Abstract
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.
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Early recognition and treatment of congenital hyperinsulinism are crucial for preventing developmental disability.
“Early recognition and treatment are crucial for preventing these sequelae.8–10”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [8]
The first few months of life are the most vulnerable period for developmental disability.
“Third, the first few months of life are the most vulnerable period for developmental disability, which occurs in ~25%–50% of children with congenital hyperinsulinism.”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [8]
Developmental disability occurs in approximately 25%-50% of children with congenital hyperinsulinism.
“Third, the first few months of life are the most vulnerable period for developmental disability, which occurs in ~25%–50% of children with congenital hyperinsulinism.”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [8]
Severe and prolonged hypoglycemia can cause permanent brain injury.
“Whereas recovery from brief periods of hypoglycemia is usually complete, severe and prolonged hypoglycemia can cause permanent brain injury.8–10,16”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [8]
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- Authors
- Menni, F., de Lonlay, P., Sevin, C., Touati, G., Peigné, C., Barbier, V., Nihoul-Fékété, C., Saudubray, J.-M., & Robert, J.-J.
- Affiliations
- From the Fédération de Pédiatrie, Diabète-Métabolisme–Neurologie and
- Clinique Chirurgicale Infantile, Centre Hospitalier Universitaire Necker–Enfants Malades, Paris, France.
- Venue
- PEDIATRICS
- Publisher
- American Academy of Pediatrics (AAP)
- Published
- 2001-03-01
- Volume
- 107
- Issue
- 3
- Pages
- 476-479
- ISSN
- 0031-4005 (ISSN-L)
- 1098-4275
- OpenAlex
- W2076523697
- Cited by
- 347
- References
- 22
- Citations by year
- 2026: 5 · 2025: 4 · 2024: 18 · 2023: 11 · 2022: 12 · 2021: 22 · 2020: 13 · 2019: 11 · 2018: 19 · 2017: 17 · 2016: 16 · 2015: 11 · 2014: 16 · 2013: 19 · 2012: 10
- FWCI
- 10.56
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- PEDIATRICS (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Keywords
- congenital hyperinsulinism, neurological outcome, psychomotor retardation, epilepsy, pancreatic resection, medical treatment, diffuse hyperinsulinism, risk factors
- MeSH terms
- Child, Developmental Disabilities, Child, Preschool, Diazoxide, Epilepsy, Humans, Hyperinsulinism, Hypoglycemia, Hypoglycemic Agents, Infant, Infant, Newborn, Neurologic Examination, Pancreatectomy, Psychomotor Disorders, Retrospective Studies, Age of Onset
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