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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
Summary
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.