DISEASES

Disease-gene associations mined from literature

Human genes for kernicterus

Kernicterus [DOID:2382]

Kernicterus or chronic bilirubin encephalopathy is damage to the brain centers of infants caused by increased levels of unconjugated bilirubin. This may be due to several underlying pathologic processes. Newborn babies are often polycythemic. When they break down the erythrocytes, one of the byproducts is bilirubin, which circulates in the blood and causes jaundice. Alternatively, 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 resulting in a decreased ability in the liver to conjugate bilirubin that is fatal within 18 months of life. Other inherited genetic disorders that can contribute to the development of hyperbilirubinemia are Gilbert's syndrome and G6PD deficiency, especially if they are present concurrently.

Synonyms:  kernicterus,  DOID:2382,  Kernicterus (morphologic abnormality),  Kernicterus of newborn (disorder),  Kernicterus of newborn NOS ...