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Hyperinsulinism in the Neonate

Katherine Lord1, Diva D De León1

  • 1The Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.

Clinics in Perinatology
|February 7, 2018
PubMed

Insights

Hyperinsulinism (HI) in infants causes persistent hypoglycemia, risking neurologic damage. Early treatment is vital, and advanced PET scans help distinguish focal from diffuse forms for effective surgical management.

Area of Science:

  • Pediatric Endocrinology
  • Neonatal Medicine
  • Molecular Genetics

Background:

  • Persistent hypoglycemia in infants is primarily caused by hyperinsulinism (HI).
  • Neurologic damage risk necessitates prompt recognition and treatment of HI, regardless of transient or permanent forms.
  • Congenital HI, often diazoxide unresponsive, frequently results from inactivating mutations in the β-cell ATP-sensitive potassium (KATP) channel (KATP-HI).

Purpose of the Study:

  • To highlight the critical distinction between diffuse and focal forms of KATP-HI.
  • To emphasize the importance of accurate diagnosis for guiding surgical intervention.
  • To introduce the role of 18-fluoro-L-3,4-dihydroxyphenylalanine PET scans in managing HI.

Main Methods:

  • Review of hyperinsulinism (HI) pathophysiology and clinical presentation.
  • Discussion of diagnostic challenges in differentiating focal and diffuse KATP-HI.
  • Evaluation of the impact of 18-fluoro-L-3,4-dihydroxyphenylalanine PET imaging on surgical planning.

Main Results:

  • KATP-HI presents in both diffuse and focal patterns.
  • Accurate differentiation between focal and diffuse HI dictates surgical outcomes: curative for focal, palliative for diffuse.
  • 18-fluoro-L-3,4-dihydroxyphenylalanine PET scanning enables precise localization of focal lesions.

Conclusions:

  • Effective management of infant hyperinsulinism hinges on distinguishing between focal and diffuse disease.
  • 18-fluoro-L-3,4-dihydroxyphenylalanine PET scanning is a transformative tool for localizing focal KATP-HI.
  • Early and accurate diagnosis improves outcomes and reduces the risk of neurologic sequelae in infants with HI.

Keywords:
HyperinsulinismHypoglycemiaInsulinNeonatePancreasPancreatectomy

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