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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.
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]
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]
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.
The level of total bilirubin is often further increased if the blood sample is taken after fasting for two days, [40] and a fast can, therefore, be useful diagnostically. A further conceptual step that is rarely necessary or appropriate is to give a low dose of phenobarbital: [41] the bilirubin will decrease substantially.
[2] [3] The two main pigments of bile are bilirubin, which is orange-yellow, and its oxidised form biliverdin, which is green. When mixed, they are responsible for the brown color of feces. [4] About 400 to 800 milliliters (14 to 27 U.S. fluid ounces) of bile is produced per day in adult human beings. [5]
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Shorter life span of fetal red blood cells, [22] being approximately 80 to 90 days in a full term infant, [24] compared to 100 to 120 days in adults. Relatively low conversion of bilirubin to urobilinogen by the intestinal flora, resulting in relatively high absorption of bilirubin back into the circulation. [22]