Kernicterus

Kernicterus

ICDO =
OMIM =
MedlinePlus = 003243
eMedicineSubj = ped
eMedicineTopic = 1247
MeshID = D007647

Kernicterus 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 often polycythemic, meaning they have too many red blood cells. When they break down the cells, one of the byproducts is bilirubin, which circulates in the blood and causes jaundice. Alternately, Rh incompatibility between mother and fetus may cause hemolysis of fetal red blood cells, thereby releasing unconjugated bilirubin into the fetal blood. Since the fetal blood 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 of Crigler-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 the basal ganglia, causing irreversible damage. Depending on the level of exposure, the effects range from unnoticeable to severe brain damage.

Some medications, such as the antibiotic co-trimoxazole (a combination of trimethoprim/sulfamethoxazole) may induce this disorder in the baby either when taken by the mother or given directly to the baby. Due to displacement of bilirubin from binding sites on serum albumin. The bilirubin is then free to pass into the Central Nervous System, because the baby's blood-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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  • kernicterus — ▪ pathology       severe brain damage caused by an abnormal concentration of the bile pigment bilirubin in brain tissues at or shortly after birth. Kernicterus may occur because of Rh blood group (Rh blood group system) incompatibility between… …   Universalium

  • Kernicterus — Disorder due to jaundice in a newborn baby with a high blood level of the pigment bilirubin that is deposited in the brain resulting in damage. Some babies are at high risk. A baby who has bruises at birth is more likely to develop jaundice. A… …   Medical dictionary

  • Kernicterus — El kernicterus, ictericia nuclear o encefalopatía neonatal bilirrubínica es una complicación neurológica grave causada por la elevación de los niveles normales de bilirrubina en la sangre del neonato. Se debe a la acción directa de la bilirrubina …   Wikipedia Español

  • kernicterus — noun Damage to the brain centres of infants caused by increased levels of unconjugated indirect bilirubin which is free (not bound to albumin) …   Wiktionary

  • kernicterus — ker·nic·ter·us …   English syllables

  • kernicterus — n. staining and subsequent damage of the brain by bile pigment (bilirubin), which may occur in severe cases of haemolytic disease of the newborn. Immature brain cells in the basal ganglia are affected, and as brain development proceeds a pattern… …   The new mediacal dictionary

  • kernicterus — /kəˈnɪktərəs/ (say kuh niktuhruhs) noun a severe form of jaundice in the newborn, usually causing death or permanent brain damage. {German, from Kern core (see kern2) + Icterus icterus} …  

  • kernicterus — noun an abnormal accumulation of bile pigment in the brain and other nerve tissue; causes yellow staining and tissue damage • Hypernyms: ↑jaundice, ↑icterus …   Useful english dictionary

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