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Conjugated bilirubin, being water-soluble, is excreted through urine. Hence, dark urine tested bilirubin positive signifies conjugated hyperbilirubinemia. [3] A peripheral blood smear showing signs of haemolysis. Red blood cells are normally in a biconcave shape (round cells in this picture).
Bilirubin (BR) (from the Latin for "red bile") is a red-orange compound that occurs in the normal catabolic pathway that breaks down heme in vertebrates.This catabolism is a necessary process in the body's clearance of waste products that arise from the destruction of aged or abnormal red blood cells. [3]
Bilirubin is conjugated with glucuronic acid in the liver by the enzyme glucuronyltransferase, making it soluble in water. Much of it goes into the bile and thus out into the small intestine. Although 20% of the secreted bilirubinoid bile is reabsorbed by the small intestine, [2] conjugated
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 conjugated hyperbilirubinemia is a result of defective endogenous and exogenous transfer of anionic conjugates from hepatocytes into bile. [5] Impaired biliary excretion of bilirubin glucuronides is due to a mutation in the canalicular multiple drug-resistance protein 2 (MRP2). A darkly pigmented liver is due to polymerized epinephrine ...
Pathological jaundice in newborns should be suspected when the serum bilirubin level rises by more than 5 mg/dL per day, serum bilirubin more than the physiological range, clinical jaundice more than 2 weeks, and conjugated bilirubin (dark urine staining clothes). Haemolytic jaundice is the commonest cause of pathological jaundice.
Choluria (or bilirubinuria) is a symptom defining an abnormal darkness of the urine, mainly due to a high level of conjugated bilirubin. [1] [2] Choluria is a common symptom of liver diseases, such as hepatitis and cirrhosis. It can be described as dark or brown urine, often referred to as the color of Coca-Cola.
Dubin-Johnson syndrome is caused by mutations in ABCC2/MRP2 which usually transports conjugated bilirubin out of the liver cell. Rotor syndrome is clinically similar to Dubin-Johnson syndrome but can cause a mixed hyperbilirubinemia, elevating both unconjugated and conjugated bilirubin levels in the blood.