what is the type of jaundice in Rotor s syndrome?
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Rotor syndrome
jaundice appearing in childhood due to impaired biliary excretion; most of the plasma bilirubin is conjugated, liver function tests are usually normal, and there is no hepatic pigmentation.
A rare idiopathic form of hyperbilirubinaemia affecting both sexes, with onset shortly after birth or in childhood. It is characterised by nonhaemolytic jaundice, attacks of intermittent epigastric discomfort and occasionally abdominal pain, and fever. Pathological findings include low-grade pigment deposition, dissociation of liver cells, occasional necrotic foci, and fibrin precipitation. Aetiology unknown. Possibly, autosomal recessive inheritance. It is due to a defect in the excretion of unconjugated bilirubin into the biliary craniculi with the bilirubin being absorbed into the blood and excreted in the urine. Primarily reported in patients from the Philippines.
It is jaundice without evidence of liver damage, biliary obstruction, or hemolysis.
Rotor's syndrome is an autosomal recessive condition characterised by a chronic, predominantly conjugated hyperbilirubinaemia. The mechanism is similar to that seen in the Dubin-Johnson syndrome, with defective excretion of organic anions into bile.
The liver is a normal colour on biopsy. A rare idiopathic form of hyperbilirubinaemia affecting both sexes, with onset shortly after birth or in childhood. It is characterised by nonhaemolytic jaundice, attacks of intermittent epigastric discomfort and occasionally abdominal pain, and fever. Pathological findings include low-grade pigment deposition, dissociation of liver cells, occasional necrotic foci, and fibrin precipitation. Aetiology unknown. Possibly, autosomal recessive inheritance. It is due to a defect in the excretion of unconjugated bilirubin into the biliary craniculi with the bilirubin being absorbed into the blood and excreted in the urine. Primarily reported in patients from the Philippines.
Rotor’s syndrome is similar to the Dubin-Johnson syndrome, except that the gall bladder is usually visualised on an oral cholecystogram and there is no secondary appearance of the dye during the performance of bromsulphaphtalien. May be the same condition as hepatic storage disease reported in Japan and France.
The prognosis is excellent.
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