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[Congenital hyperinsulinism in 15 infants, 1981-1999; experiences and new insights]

S C Kuijpers1, C Noordam, C Boelen

  • 1Afd. Metabole & Endocriene Ziekten, Universitair Medisch Centrum St Radboud, Universitair Kinderziekenhuis, Postbus 9101, 6500 HB Nijmegen.

Nederlands Tijdschrift Voor Geneeskunde
|February 18, 2004
PubMed

Insights

Congenital hyperinsulinism (CHI) in infants presents with varied symptoms and requires high glucose administration. Surgery for CHI is effective but can lead to exocrine pancreas dysfunction.

Area of Science:

  • Pediatrics
  • Endocrinology
  • Neonatology

Context:

  • Congenital hyperinsulinism (CHI), also known as persistent hyperinsulinemic hypoglycemia, is a rare but serious condition in infants.
  • Early diagnosis and appropriate management are crucial for preventing neurological damage due to hypoglycemia.

Purpose:

  • To retrospectively analyze the diagnostic methods and treatment outcomes for infants with congenital hyperinsulinism (CHI).
  • To evaluate the effectiveness of diazoxide treatment and subtotal pancreatectomy in managing CHI.
  • To identify potential complications associated with CHI treatment, including neurological and pancreatic dysfunction.

Summary:

  • This study reviewed 15 infants diagnosed with CHI between 1981 and 1999, noting diverse presentations including macrosomia and neuroglycopenic symptoms.
  • Infants required significantly elevated glucose levels (16.9 mg/kg/min) to maintain normoglycemia.
  • Five infants responded to diazoxide, while 10 underwent subtotal pancreatectomy, resulting in normoglycemia in 4, but with 3 developing exocrine pancreas dysfunction. Disturbed neuropsychological development was noted in 5 infants.

Impact:

  • The findings highlight the challenges in diagnosing and managing CHI, emphasizing the need for tailored treatment strategies.
  • Subtotal pancreatectomy is a viable option for refractory cases, but potential long-term complications like exocrine dysfunction must be monitored.
  • This study contributes to understanding the long-term outcomes and management of congenital hyperinsulinism in infants.
Abstract

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