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Persistent hyperinsulinemic hypoglycemia presenting with a rare complication: West syndrome

M Orhun Camurdan1, Peyami Cinaz, Ayşe Serdaroğlu

  • 1Department of' Pediatric Endocrinology, Gazi University, Ankara, Turkey.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|November 6, 2004
PubMed

Insights

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) can lead to West syndrome, a rare neurological condition. This case is the first report of West syndrome in normoammoniemic PHHI, highlighting the need for close neurological monitoring.

Area of Science:

  • Pediatric Endocrinology
  • Neonatal Neurology
  • Metabolic Disorders

Background:

  • Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) involves abnormal insulin secretion from pancreatic beta-cells.
  • Hypoglycemia, a common symptom of PHHI, can manifest as West syndrome, though this association is rare.
  • Previous reports linked West syndrome to the hyperammoniemic form of PHHI.

Observation:

  • A 6-month-old infant diagnosed with PHHI presented with infantile spasms (jerky movements).
  • Electroencephalogram (EEG) confirmed the characteristic hypsarrhythmia pattern, indicative of West syndrome.
  • This patient had the normoammoniemic form of PHHI.

Findings:

  • This case represents the first documented instance of West syndrome occurring in a patient with normoammoniemic PHHI.
  • The findings suggest a potential, albeit rare, neurological complication of PHHI even without hyperammonemia.
  • The study underscores the importance of recognizing West syndrome as a possible outcome in PHHI.

Implications:

  • Highlights the critical need for vigilant neurological follow-up in infants diagnosed with PHHI.
  • Emphasizes the potential risks associated with inadequately managed PHHI, including severe neurological sequelae.
  • Suggests that early detection and management of PHHI may help prevent neurological complications like West syndrome.
Abstract

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