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Kernicterus is rare in adults but is prevalent in newborns with underdeveloped blood-brain barriers and lower albumin binding capacities, which otherwise buffers excess bilirubin in adults. To date, only 4 such cases have been published, with 3 of them associated with CN-2, the milder form of the syndrome.
Neonatal jaundice is a yellowish discoloration of the white part of the eyes and skin in a newborn baby due to high bilirubin levels. [1] Other symptoms may include excess sleepiness or poor feeding. [1] Complications may include seizures, cerebral palsy, or kernicterus. [1] In most of cases there is no specific underlying physiologic disorder. [2]
Depending on the type of hereditary hyperbilirubinemia, symptoms can be worsened when an additional cause of increased red blood cell turnover occurs, as these patients have a decreased ability to process bilirubin. Elevated levels of unconjugated bilirubin is neurotoxic and can cause damage to the brain, called bilirubin encephalopathy which ...
Kernicterus is a bilirubin-induced brain dysfunction. [1] The term was coined in 1904 by Christian Georg Schmorl.Bilirubin is a naturally occurring substance in the body of humans and many other animals, but it is neurotoxic when its concentration in the blood is too high, a condition known as hyperbilirubinemia.
Neonatal cholestasis refers to elevated levels of conjugated bilirubin identified in newborn infants within the first few months of life. [1] Conjugated hyperbilirubinemia is clinically defined as >20% of total serum bilirubin or conjugated bilirubin concentration greater than 1.0 mg/dL regardless of total serum bilirubin concentration. [2]
Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. [3] [6] Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme metabolism, liver dysfunction, or biliary-tract obstruction. [7]
When the total serum bilirubin increases over 95th percentile for age during the first week of life for high risk babies, it is known as hyperbilirubinemia of the newborn (neonatal jaundice) and requires light therapy to reduce the amount of bilirubin in the blood. Pathological jaundice in newborns should be suspected when the serum bilirubin ...
Newborn infant with Rhesus disease, a type of hemolytic disease of the newborn, suffering from hydrops fetalis (edema caused by heart failure). The infant did not survive. [4] Signs of hemolytic disease of the newborn include a positive direct Coombs test (also called direct agglutination test), elevated cord bilirubin levels, and hemolytic anemia.