Rotor syndrome
Introduction
Introduction to Rotor Syndrome Rotor syndrome (RS) is a hereditary combination of bilirubin elevation II, first reported by Rotor in 1948, originally considered to be a subtype of DJS, but by organic anion clearance test and urinary coproporphyrin isomer analysis, It is confirmed that RS is an independent disease, less common than DJS, and is also autosomal recessive. Rotor syndrome is a rare childhood or adolescent jaundice. The onset and clinical symptoms of RS are similar to those of DJS, with a good prognosis and no treatment. However, in the laboratory examination, the following four aspects are obviously different from DJS: 1BSP retention test is significantly increased in 45min, often up to 20%-40%, and there is no re-elevation curve in 90-120min. 2 The appearance of the liver does not appear dark brown, and there is no specific pigment particle deposition in the liver cells. The total excretion of coproporphyrin in 324h urine increased most significantly, but the distribution of coproporphyrin isomers was as usual. 4 oral gallbladder angiography is well developed. basic knowledge The proportion of illness: 0.002% Susceptible population: almost all found in people under the age of 20 Mode of infection: non-infectious Complications: abdominal pain
Cause
Cause of Rotor syndrome
Due to the congenital defects of hepatocyte uptake of free biliary red and excretion combined with bilirubin, the combined blood bilirubin increased, and the greenish (ICG) excretion test was reduced.
Gallbladder angiography is well developed, a few are not developed, liver biopsy is normal, and there are no pigment particles in liver cells.
The so-called binding of bilirubin is the result of normal red blood cell metabolism. The life span of red blood cells in normal people is about 100-120 days, and the red blood cells of the human body are 1% aging every day. These dead red blood cells are cleared and decomposed by phagocytic cells to form biliverdin, which is quickly restored to no. Combined with bilirubin (unconjugated bilirubin), it is converted to bilirubin. The liver cells run it and excrete it into the capillary tube, which becomes one of the main components of bile.
Prevention
Rotor syndrome prevention
The prognosis of Rotor syndrome is good, liver function is normal, liver enzymes are not elevated, and there is no liver enlargement. Unlike many other liver diseases, patients may have itching. Rotor patients have a normal life expectancy and their condition will not worsen. However, jaundice may occur due to infection, pregnancy, taking oral contraceptives, drinking alcohol (alcohol), and the like. So you should avoid the above.
Complication
Complication of Rotor syndrome Complications, abdominal pain
Loss of appetite, abdominal pain.
Symptom
Symptoms of Rotor Syndrome Common Symptoms Jaundice Bilirubin Increases Abdominal Pain Fatigue Loss of Appetite
This syndrome is almost always seen in the incidence of children under the age of 20, there is no difference between men and women, mainly as jaundice, generally no other symptoms, sometimes fatigue, loss of appetite, abdominal pain. The liver is normal or slightly enlarged. The average serum bilirubin is 6 mg (4-20 mg)%, of which the direct reaction part accounts for more than 50%. The 45-minute bromosulfonate retention rate can be as high as 50-60%. The 131I-bromosulfonate liver time scan showed delayed liver uptake dye, bilirubin urine but normal urinary biliary excretion, other liver functions were normal, and oral gallbladder angiography was normal. There was no abnormality in liver biopsy and no pigmentation in hepatocytes, so it was different from Dubin-Johnson syndrome. The prognosis is good.
Examine
Examination of Rotor syndrome
Direct bilirubin/indirect bilirubin ratio check.
Diagnosis
Diagnosis and diagnosis of Rotor syndrome
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
No need to identify.
