Constitutional Nonhemolytic Hyperbilirubinemia with Findings Resembling Kernicterus

Jervis, George A.

doi:10.1001/archneurpsyc.1959.02340130075008

Cited by 24 publications

(5 citation statements)

References 38 publications

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“…However, bilirubin staining of the brain in a kernicteric pattern has been described in several Crigler-Najjar patients who survived the neonatal period without signs of central nervous system damage, only to develop signs and symptoms of such dam age later in life in connection with severe ill ness or trauma [20,21], Cases of kernicterus have also been described in adults with fulmi nant liver failure [22], These observations suggest that unconjugated hyperbilirubinemia is the main determinant of kernicterus, and that the age of the subject is of less, if any, importance.…”

Section: Discussionmentioning

confidence: 99%

Acute Entry of Bilirubin into Rat Brain Regions

Hansen

1995

Neonatology

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In kemicterus bilirubin appears at higher concentrations in the basal ganglia, the cerebellum, and the medulla. The mechnism for this distribution is unknown, but might involve differences in bilirubin entry among brain regions. We infused [³H]bilirubin (30 mg/kg over 5 min) intravenously into young Sprague-Dawley rats (n = 21, weight 144 ± 10 g, mean ± SD). Blood was sampled from the tail, and groups of rats were killed at 15, 30, 45, 60, and 75 min by intravenous pentobarbital. After in situ flushing of the brain vasculature, the brain was dissected into seven regions, each of which was weighed, dissolved in Soluene-350, and subsequently scintillation counted. Blood samples were analyzed for bilirubin (including specific activity), albumin, and blood gases. Brain bilirubin concentrations in each of the regions were calculated by comparing the radioactivity in that region with the specific activity of bilirubin. Using a curve-fitting program (EXPOFIT), the concentrations of bilirubin in each brain region at 5 min (i.e. the end of bilirubin infusion) could be estimated, and were as follows (nmol/g ± SD): cortex 6.4 ± 4.4; hippocampus 6.2 ± 3.3; striatum 6.3 ± 4.5; midbrain 7.3 ± 4.4; hypothalamus 6.3 ± 4.6; cerebellum 6.7 ± 4.1, and medulla 7.0 ± 4.0. There were no significant differences in the bilirubin concentrations between brain regions (F6 140 0.205). The mechanism for preferential localization of bilirubin to the basal ganglia probably does not involve the acute entry of bilirubin into brain.

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Section: Discussionmentioning

confidence: 99%

Acute Entry of Bilirubin into Rat Brain Regions

Hansen

1995

Neonatology

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“…Monitoring muscle bioenergetics with 31P-NMR is valuable in documenting therapeutic improvements in mitochondrial myop athies. Phosphorus nuclear magnetic resonance spectroscopy (31P-NMR) has been used to study energy metabolism in normal and diseased human muscles in vivo [1][2][3][4][5][6][7]9,12,[15][16][17][18]. The contribution of 3'P-NMR to the…”

Section: Discussionmentioning

confidence: 99%

Kernicterus in an adult

Waser

Kleihues

1986

Annals of Neurology

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A 43-year-old woman was initially seen because of icterus. Clinical investigations revealed severe hepatic damage probably due to non-A, non-B hepatitis. She was treated with extracorporeal charcoal-column perfusion but died two weeks later in a hepatic coma. At autopsy, the brain showed kernicterus with typical discoloration of the hippocampus, the subthalamic nuclei, and the cerebellar dentate nuclei. Kernicterus in an adult is very rare. In this case, extracorporeal charcoal-column perfusion treatment led repeatedly to severe depletion of fibrinogen, with extensive hemorrhages. Overload of the already reduced hepatic glucuronyl-transferase capacity resulted in high serum levels of unconjugated bilirubin, an apparent prerequisite for the development of bilirubin encephalopathy.

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“…Development of kernicterus, as observed for the child Da., occurs frequently [1,3,[13][14][15][16][17][18] but is no constant feature [19,20]. Most patients die in early childhood [1, 16, 17| although a few adults with this syndrome have been reported [20,21].…”

Section: Spectra (Da)mentioning

confidence: 99%