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Hyperinsulinism in syndromal disorders.

T Meissner¹, W Rabl, K Mohnike

¹Division of Metabolic and Endocrine Diseases, University Children's Hospital, Heidelberg, Germany.

Acta Paediatrica (Oslo, Norway : 1992)

|September 1, 2001

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Persistent hyperinsulinism in neonates can indicate syndromal disorders. Further research is needed to understand complex mechanisms beyond biochemical pathways in persistent hyperinsulinism.

Area of Science:

Pediatric Endocrinology
Genetics
Neonatology

Background:

Persistent hyperinsulinism (PH) is a significant cause of neonatal hypoglycemia.
Genetic and biochemical factors are well-established causes of PH.
Syndromal causes of PH are less understood.

Purpose of the Study:

To investigate the association between persistent hyperinsulinism and syndromal disorders in neonates.
To characterize the clinical presentation of neonates with PH and associated syndromal features.

Main Methods:

Analysis of a German database of 54 patients with neonatal persistent hyperinsulinism.
Clinical data review for patients with PH and additional symptoms suggestive of syndromal disorders.

Main Results:

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Five out of 54 patients (9.3%) with neonatal PH exhibited additional clinical symptoms indicating an underlying syndromal disorder.
Three patients presented with a novel clinical entity: severe psychomotor retardation, chronic pulmonary disease, hypothyroidism, and congenital heart defects.
One patient had congenital central hypoventilation syndrome, and another had Beckwith-Wiedemann syndrome with severe PH requiring subtotal pancreatectomy.

Conclusions:

Persistent hyperinsulinism in neonates can be associated with syndromal disorders involving multiple organs.
Complex pathophysiological mechanisms, beyond known biochemical pathways, contribute to persistent hyperinsulinism in syndromic cases.
Early recognition of syndromal features is crucial for comprehensive management of persistent hyperinsulinism.