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Related Experiment Videos

Evoked potential abnormalities in children with chronic cholestasis.

H A Cynamon1, K Norcross, J N Isenberg

  • 1Department of Pediatrics, University of Texas Medical Branch, Galveston 77550.

Hepatology (Baltimore, Md.)
|November 1, 1988
PubMed
Summary

Chronic liver disease in children can cause visual system problems, linked to high bile acid levels. These visual evoked potential abnormalities may reverse with cholestasis improvement, even after liver transplants.

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Area of Science:

  • Pediatric Neurology
  • Hepatology
  • Neurophysiology

Background:

  • Chronic cholestasis in children can impact nervous system function.
  • Vitamin E and A deficiencies are potential contributors to neurological issues in liver disease.

Purpose of the Study:

  • To evaluate the effects of chronic cholestasis and vitamin E deficiency on nervous system function in children.
  • To investigate the relationship between cholestasis, vitamin status, and evoked potential abnormalities.

Main Methods:

  • Multimodality evoked potential testing (visual, brainstem auditory, somatosensory) in 17 children with chronic liver disease.
  • Periodic retesting in 11 patients over 1-33 months.
  • Correlation of evoked potential findings with serum bile acid levels and vitamin E/A status.

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Main Results:

  • Eight children showed abnormal visual evoked potentials (P100 peak delay), correlated with higher serum bile acid levels.
  • Six patients initially had auditory evoked potential abnormalities; four with arteriohepatic dysplasia had persistent hearing loss.
  • Somatosensory evoked potentials were mildly abnormal in four children due to peripheral neuropathy.
  • Vitamin E and A levels did not consistently correlate with evoked potential abnormalities.
  • Visual evoked potentials normalized in four patients following cholestasis improvement, particularly after liver transplantation.

Conclusions:

  • Frequent, potentially reversible visual system abnormalities are associated with cholestasis in children.
  • These visual abnormalities are not solely attributable to vitamin E and/or A deficiency.
  • Evoked potential testing is a valuable tool for assessing neurological function in pediatric liver disease.