Document Detail


Clinical (video) findings and cerebrospinal fluid neurotransmitters in 2 children with severe chronic bilirubin encephalopathy, including a former preterm infant without marked hyperbilirubinemia VIDEO.
MedLine Citation:
PMID:  16264013     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Chronic bilirubin encephalopathy, characterized clinically by extrapyramidal movement abnormalities, vertical gaze abnormalities, and hearing loss, results from neuronal injury after marked hyperbilirubinemia in term and preterm infants. In premature infants, bilirubin staining of specific brain structures has been described at autopsy after only moderate hyperbilirubinemia, but classic chronic bilirubin encephalopathy without marked hyperbilirubinemia has been reported only rarely. We report a case of a 7-year-old, former 29-weeks' gestation, gravely ill premature infant with a peak bilirubin level of 13.3 mg/dL in the neonatal period. We compare this case with a 12-year-old, former term infant with a peak bilirubin level of 49.4 mg/dL on day 10 of life. Both children have dystonia, athetosis, upward gaze palsy, and sensorineural hearing loss, with MRIs showing characteristic abnormal signal in the globus pallidus. We add previously unreported cerebrospinal fluid neurotransmitter levels that show a mild decrease in the dopamine metabolite homovanillic acid in the former premature infant only.
Authors:
Stephanie L Merhar; Donald L Gilbert
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatrics     Volume:  116     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2005 Nov 
Date Detail:
Created Date:  2005-11-02     Completed Date:  2005-12-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1226-30     Citation Subset:  AIM; IM    
Affiliation:
University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
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MeSH Terms
Descriptor/Qualifier:
Child
Developmental Disabilities / etiology
Female
Follow-Up Studies
Globus Pallidus / pathology
Homovanillic Acid / cerebrospinal fluid
Humans
Hydroxyindoleacetic Acid / cerebrospinal fluid
Hyperbilirubinemia / cerebrospinal fluid
Infant, Newborn
Infant, Premature
Infant, Premature, Diseases / cerebrospinal fluid*
Kernicterus / cerebrospinal fluid*,  complications,  pathology
Magnetic Resonance Imaging
Neurotransmitter Agents / cerebrospinal fluid*
Tyrosine / analogs & derivatives,  cerebrospinal fluid
Chemical
Reg. No./Substance:
0/Neurotransmitter Agents; 306-08-1/Homovanillic Acid; 54-16-0/Hydroxyindoleacetic Acid; 55520-40-6/Tyrosine; 7636-26-2/3-methoxytyrosine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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