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Hemodynamic Precision in the Neonatal Intensive Care Unit using Targeted Neonatal Echocardiography

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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

Summary

This summary is machine-generated.

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.

Keywords:

Hyperinsulinism Hypoglycemia Insulin Neonate Pancreas Pancreatectomy

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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 (K_ATP) channel (K_ATP-HI).

Purpose of the Study:

To highlight the critical distinction between diffuse and focal forms of K_ATP-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 K_ATP-HI.
Evaluation of the impact of 18-fluoro-L-3,4-dihydroxyphenylalanine PET imaging on surgical planning.

Main Results:

K_ATP-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 K_ATP-HI.
Early and accurate diagnosis improves outcomes and reduces the risk of neurologic sequelae in infants with HI.