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Related Experiment Videos

Nesidioblastosis in children.

J Knight, P J Garvin, R K Danis

    Archives of Surgery (Chicago, Ill. : 1960)
    |July 1, 1980
    PubMed
    Summary

    Severe hypoglycemia in infants, caused by nesidioblastosis, can lead to seizures. Subtotal pancreatectomy effectively treated this condition in three cases, preventing further neurological damage.

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    Area of Science:

    • Pediatric Endocrinology
    • Neonatology
    • Surgical Gastroenterology

    Background:

    • Nesidioblastosis is a rare cause of persistent hyperinsulinemic hypoglycemia in neonates.
    • Seizure disorder can be the initial presenting symptom of severe neonatal hypoglycemia.
    • Standard medical management often fails to control hypoglycemia in severe nesidioblastosis.

    Observation:

    • Three neonates presented with seizure disorders, later diagnosed with severe hypoglycemia due to nesidioblastosis.
    • Patients exhibited low fasting glucose and high serum insulin levels.
    • Initial treatments including frequent oral feedings and intravenous medications were ineffective.

    Findings:

    • All three patients underwent subtotal pancreatectomy within three weeks of diagnosis.
    • Two patients achieved euglycemia post-surgery with no central nervous system (CNS) damage.
    • One patient experienced residual fasting hypoglycemia and continued to have seizures and cognitive impairment.

    Implications:

    • Prompt diagnosis and aggressive treatment are crucial to prevent irreversible CNS damage in neonatal hypoglycemia.
    • Subtotal pancreatectomy is a safe and effective surgical option for nesidioblastosis.
    • Early surgical intervention can lead to favorable long-term outcomes, including euglycemia and preserved neurological function.

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