Rotor syndrome (RS) is a type II hereditary conjugated hyperbilirubinemia, first reported by Rotor in 1948. It was initially considered to be a subtype of DJS, but confirmed to be an independent disease through organic anion clearance tests and analysis of coproporphyrin isomers in urine, which is less common than DJS and also an autosomal recessive inheritance. Rotor syndrome is a rare childhood or adolescent jaundice.
The onset and clinical symptoms of RS are very similar to those of DJS, with a good prognosis and no need for treatment. However, in laboratory tests, the following four aspects are obviously different from DJS:
1. The BSP retention test is significantly elevated at 45 minutes, often reaching 20%-40%, and there is no further rise curve at 90-120 minutes.
2. The liver does not appear dark brown, and there are no specific pigment granules deposited in liver cells.
3. The total excretion of coproporphyrin in 24-hour urine is significantly increased, but the distribution of coproporphyrin isotypes is as normal as in healthy people.
4. Oral gallbladder imaging shows good contrast.
1. What are the causes of Rotor syndrome
The etiology of Rotor syndrome is complex and comprehensive, and the specific causes are as follows.
1. Due to the congenital defect in the liver cells' uptake of free bilirubin and excretion of conjugated bilirubin, the level of conjugated bilirubin in the blood is increased, and the indocyanine green (ICG) excretion test shows a decrease.
2. Gallbladder imaging often shows good contrast, while a few do not show contrast. Liver biopsy is normal, and there are no pigment granules in liver cells.
What is conjugated bilirubin? It is the result of normal red blood cell metabolism. The lifespan of red blood cells in normal people is about 100-120 days, and 1% of the red blood cells in the human body die and age every day. These dead red blood cells are cleared and decomposed by phagocytes, forming biliverdin, which is then quickly reduced to unconjugated bilirubin and then converted to conjugated bilirubin. Liver cells transport and excrete it into the bile canals, becoming one of the main components of bile.
4. How to prevent Rotor syndrome
The prognosis of Rotor syndrome is good, liver function is normal, liver enzymes have not increased, and there is no liver enlargement. Unlike many other liver diseases, patients may have an itchy sensation. The lifespan of Rotor patients is also normal, and the condition will not worsen. However, jaundice may occur due to infection, pregnancy, taking oral contraceptives, drinking alcohol (alcohol), and so on. Therefore, it is advisable to avoid the above-mentioned factors.
6. Dietary taboos for patients with Rotor syndrome
Patients with Rotor syndrome should have meals at regular intervals and in appropriate amounts, and all three meals should be balanced nutritionally. Eat slowly while chewing, as saliva secretion during chewing also has the function of neutralizing stomach acid. Do not have a preference for food, and do not let children eat and play while eating, or read books and watch TV while eating.
Prevent overeating and undereating, overeating when encountering favorite foods, and not eating or eating very little when encountering dishes that are not to one's taste, which makes the burden on the gastrointestinal tract fluctuate. Do not emphasize high nutrition unilaterally, and achieve scientific dietary matching, eating more high-protein, low-fat, and easily digestible foods. Do not eat刺激性大的食物 for a long time, and do not overeat cold drinks such as ice cream.
7. Conventional methods for treating Rotor syndrome in Western medicine
There is no specific medicine for Rotor syndrome, and some people may use phenobarbital to promote the transport and excretion of bilirubin. Rotor syndrome will not evolve into liver cancer or liver cirrhosis.
The prognosis of Rotor syndrome is good, liver function is normal, liver enzymes have not increased, and there is no liver enlargement. Unlike many other liver diseases, patients may have an itchy sensation. The lifespan of Rotor patients is also normal, and the condition will not worsen. However, jaundice may occur due to infection, pregnancy, taking oral contraceptives, drinking alcohol (alcohol), and so on.