- Kernicterus
ICDO =
OMIM =
MedlinePlus = 003243
eMedicineSubj = ped
eMedicineTopic = 1247
MeshID = D007647Kernicterus is damage to the brain centers of infants caused by increased levels of unconjugated-indirect
bilirubin which is free (not bound to albumin). This may be due to several underlying pathologic processes. Newborn babies are oftenpolycythemic , meaning they have too manyred blood cell s. When they break down the cells, one of the byproducts isbilirubin , which circulates in the blood and causes jaundice. Alternately, Rh incompatibility between mother and fetus may causehemolysis of fetal red blood cells, thereby releasing unconjugated bilirubin into the fetal blood. Since the fetalblood brain barrier is not fully formed, some of this released bilirubin enters the brain and interferes with normal neuronal development.Kernicterus may also be found in infants as a symptom ofCrigler-Najjar syndrome type I, a hereditary hyperbilirubinemia that is fatal within 18 months of life.In adults and older children, jaundice is harmless in and of itself. However, the tissues protecting the brain (the
blood-brain barrier ) are immature in newborns. Bilirubin penetrates the brain and is deposited in thebasal ganglia , causing irreversible damage. Depending on the level of exposure, the effects range from unnoticeable to severebrain damage .Some medications, such as the antibiotic
co-trimoxazole (a combination oftrimethoprim /sulfamethoxazole ) may induce this disorder in the baby either when taken by the mother or given directly to the baby. Due to displacement ofbilirubin from binding sites onserum albumin . Thebilirubin is then free to pass into theCentral Nervous System , because the baby'sblood-brain barrier is not fully developed.The word origantes from the German "kern", nucleus, and the Greek "ikterus", jaundice. [cite web
url = http://dissertations.ub.rug.nl/FILES/faculties/medicine/2006/a.m.hafkamp/01_1.pdf
title = Oral treatment of unconjugated hyperbilirubinemia
accessdate = 2008-09-06
author = Anja M. Hafkamp
year = 2006
format = PDF
work = PhD thesis
publisher = Department of Pediatrics; Center for Liver, Digestive and Metabolic Diseases; University Medical Center Groningen
pages = p. 21]References
External links
* [http://bilitool.org BiliTool - Hyperbilirubinemia Risk Assessment for Newborns]
* [http://www.cdc.gov/ncbddd/dd/kernichome.htm CDC’s National Center on Birth Defects and Developmental Disabilities]
* [http://www.pickonline.org/ PICK - Parents of Infants and Children with Kernicterus]
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