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[Persistent hyperinsulinemic hypoglycemia of infancy: case report]
D F Lazarine1, M M Pahl, D Damiani
1Universidade de São Paulo (USP), SP, Brazil.
Jornal De Pediatria
|December 4, 2003
Summary
This case report details Persistent Hyperinsulinemic Hypoglycemia in twins, a rare condition causing seizures. Early diagnosis and treatment are crucial to prevent neurological damage in infants.
Area of Science:
- Pediatric Endocrinology
- Neonatology
- Genetics
Background:
- Persistent Hyperinsulinemic Hypoglycemia (PHHI) is a rare cause of infant hypoglycemia.
- Consanguinity in parents can increase the risk of genetic metabolic disorders.
- Hypoglycemia in infants, especially twins, requires thorough etiological investigation.
Purpose of the Study:
- To report a unique case of Persistent Hyperinsulinemic Hypoglycemia in identical twins.
- To highlight the importance of early diagnosis and management of PHHI.
- To emphasize the association between consanguinity and PHHI.
Main Methods:
- Case report of identical twins presenting with seizures due to hypoglycemia.
- Comprehensive laboratory investigations including hormone levels and stimulation tests.
- Histopathological examination of pancreatic tissue.
Main Results:
- Confirmed persistent hypoglycemia with hyperinsulinism in both twins.
- Glucagon stimulation test validated the insulin/glucose imbalance.
- Pancreatic histopathology confirmed Nesidioblastosis (now termed PHHI).
Conclusions:
- PHHI, though rare, should be suspected in infantile hypoglycemia, particularly in twins and offspring of consanguineous parents.
- Prompt initiation of appropriate therapy is vital to prevent long-term neurological sequelae.
- This case underscores the need for heightened clinical suspicion in non-neonatal hypoglycemia presentations.
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