Chronic bilirubin encephalopathy: diagnosis and outcome
Section snippets
Diagnosis of chronic bilirubin encephalopathy (kernicterus)
The classic form of chronic bilirubin encephalopathy is also called kernicterus, originally a pathological term referring to the yellow staining (-icterus) of the deep nuclei of the brain (kern-, relating to the basal ganglia). The terms acute and chronic bilirubin encephalopathy are used to describe the clinical symptoms associated with the neuropathology. Common usage of the term kernicterus has expanded to include clinical bilirubin encephalopathy, and modern testing provides objective
Classification of kernicterus
Current means of diagnosing and treating kernicterus necessitate new clinical definitions. Recently, we have suggested that classical kernicterus be differentiated from subtle kernicterus, also known as bilirubin-induced neurological dysfunction (BIND), and further subdivided by a number of criteria including location and severity.26
Prediction of outcome
There are no data to determine whether history, exam, and laboratory tests predict the severity of outcome. Certainly, higher and longer duration of excessive hyperbilirubinemia and acute bilirubin encephalopathy, and more abnormal ABR and MRI abnormalities, predict a poorer outcome in the author's experience. However, the author has occasionally been surprised at children whose outcome are much better than expected, although the difference in outcome is usually about one category of severity,
Conclusion
Chronic bilirubin encephalopathy, also known as kernicterus, can be diagnosed following semi-objective criteria based on history, physical examination and laboratory findings. I find it useful to categorize kernicterus as mild, moderate, severe, and as classical, auditory predominant, motor predominant, and subtle. Common terminology and criteria for establishing the neurological sequelae of neonatal bilirubin toxicity and the diagnosis of kernicterus will be useful for evaluating the variety
Acknowledgement
The author would like to thank Dr Michael J. Painter for reviewing this manuscript and making many useful suggestions.
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