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Patients with hyperbilirubinemia generally have poor outcome, particularly those with liver-dysfunction led jaundice. Drug-induced liver injury, as Hy's law states, patients with bilirubin of >3 mg/dL have 10% mortality rate. [36] End stage liver disease models also include hyperbilirubinemia as a critical parameter in prognosis of cirrhosis.
Rotor syndrome (also known as Rotor type hyperbilirubinemia) [2] is a rare cause of mixed direct (conjugated) and indirect (unconjugated) hyperbilirubinemia, relatively benign, autosomal recessive [3] bilirubin disorder characterized by non-hemolytic jaundice due to the chronic elevation of predominantly conjugated bilirubin.
The prevalence of hereditary hyperbilirubinemia varies based on each disease. The most common being Gilbert syndrome which is found is 2-10% of the population. Other types of hereditary hyperbilirubinemia are less common and may even be exceedingly rare depending on the mutation. [1]
Gilbert syndrome has been reported to contribute to an accelerated onset of neonatal jaundice. The syndrome cannot cause severe indirect hyperbilirubinemia in neonates by itself, but it may have a summative effect on rising bilirubin when combined with other factors, [ 10 ] for example in the presence of increased red blood cell destruction due ...
Management consists of vigilant observation over days to detect progression and, if required, manage complications (e.g. hemorrhagic shock, unconjugated hyperbilirubinemia and jaundice from hemolyzed red blood cells). The subgaleal space is capable of holding up to 40% of a newborn baby's blood and can therefore result in acute shock and death.
Dubin–Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. [2]
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 ...
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.