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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.
Insights
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.
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.
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.
Abstract:
To assess the effect of chronic cholestasis and vitamin E deficiency on nervous system function, we did multimodality evoked potential testing of 17 children (mean age = 47 months) who had chronic liver disease. Evoked potential testing was repeated periodically in 11 patients 1 to 33 months after the initial study. Eight children had abnormal delays of the P100 peak of the visual evoked potential, and these children each had significantly higher total serum bile acid levels than did children who had normal visual evoked potentials (p = 0.002). Bilateral brainstem auditory evoked potential abnormalities consistent with conductive hearing losses were initially present in six patients. However, persistent conductive losses were found in four patients, all of whom had arteriohepatic dysplasia. Four children had mildly abnormal somatosensory evoked potentials that were due solely to a mild peripheral neuropathy. Biochemical measures of vitamin E status were not consistently associated with either normal or abnormal visual, brainstem auditory or somatosensory evoked potentials or a combination of evoked potential abnormalities, and an abnormality of one evoked potential type was not associated with an abnormality of any other. A similar lack of relationship between evoked potential results and plasma vitamin A measurement was noted. Following marked improvement in or resolution of cholestasis in four patients, the visual evoked potential became normal, but other evoked potentials did not change. Visual evoked potential improvement was greatest in two patients who underwent orthotopic liver transplantation. This is the first report that demonstrates frequent, potentially reversible visual system abnormalities that are associated with cholestasis and cannot be attributed solely to vitamin E and/or A deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)