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[Persistent hyperinsulinemic hypoglycemia of infancy: clinical analysis of 12 cases]

Ai-Wen Wu1, Li Liu, Xiu-Zhen Li

  • 1Department of Endocrinology and Metabolism, Guangzhou Children's Hospital Affiliated to Guangzhou Medical College, Guangzhou 510120, China.

Zhongguo Dang Dai Er Ke Za Zhi = Chinese Journal of Contemporary Pediatrics
|October 24, 2009
PubMed

Insights

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) diagnosis involves blood glucose, insulin, and urinary ketone measurements. Diazoxide therapy shows effectiveness in some infants with this condition.

Area of Science:

  • Pediatric Endocrinology
  • Neonatal Medicine
  • Metabolic Disorders

Context:

  • Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a rare but serious condition.
  • Early diagnosis and effective management are crucial to prevent neurological damage.

Purpose:

  • To review the diagnostic methods and treatment outcomes for infants with persistent hyperinsulinemic hypoglycemia of infancy.
  • To evaluate the efficacy of diazoxide and surgical intervention in managing PHHI.

Summary:

  • Retrospective review of 12 infants with PHHI revealed common symptoms like convulsions and lethargy.
  • Laboratory findings confirmed persistent hypoglycemia and hyperinsulinism, with positive glucagon tests and absent urine ketones.
  • Diazoxide was effective in 4 of 7 treated infants, while one infant achieved normal blood glucose after subtotal pancreatectomy.

Impact:

  • Highlights the importance of specific laboratory tests (blood glucose, insulin, urinary ketones) for PHHI diagnosis.
  • Suggests diazoxide as a potential therapeutic option for PHHI, though not universally effective.
  • Underscores the long-term risk of psychomotor retardation in infants with PHHI, emphasizing the need for ongoing follow-up.
Abstract

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