Syndrome Gilbert (disease Gilbert ) is a benign hepatic dysfunction, which consists in clearing it of indirect bilirubin , which is formed during the breakdown of hemoglobin.
Gilbert ‘s syndrome is a hereditary constitutional feature, so this pathology is not considered by many authors to be a disease.
Such an anomaly is found in 3-10% of the population, especially it is often diagnosed among the inhabitants of Africa. It is known that men are 3-7 times more likely to suffer from this disease.
The main characteristics of the described syndrome include the periodic increase in blood bilirubin and the associated jaundice.
Gilbert ‘s syndrome is a hereditary disease caused by a defect in a gene located in the second chromosome that is responsible for the production of the hepatic enzyme glucoronyl transferase. Indirect bilirubin binds to the liver through this enzyme. Its excess leads to hyperbilirubinemia (increased bilirubin content in the blood) and, as a result, to jaundice.
The factors that cause the aggravation of Gilbert’s syndrome (jaundice) are highlighted:
- infectious and viral diseases;
- violation of the diet;
- lack of sleep;
- excessive exercise;
- taking certain medications ( rifampicin , chloramphenicol, anabolic drugs, sulfonamides, hormones, ampicillin, caffeine, paracetamol, and others);
- alcohol consumption;
- surgical intervention.
In a third of patients, the pathology does not manifest itself. Elevated levels of bilirubin in the blood are observed from birth, but it is difficult to diagnose this diagnosis in infants due to physiological jaundice of newborns. As a rule, Gilbert’s syndrome is determined in young men aged 20-30 years during the examination for another reason.
The main symptom of Gilbert’s syndrome is icteric (jaundice) of the sclera and, sometimes, the skin. Jaundice in most cases is periodic and has a mild severity.
About 30% of patients during the period of exacerbation note the following symptoms:
- pain in the right hypochondrium;
- metallic taste in the mouth;
- loss of appetite;
- nausea and vomiting (especially when eating sweet foods);
- flatulence ;
- feeling of full belly;
- constipation or diarrhea.
The signs characteristic of many diseases are not excluded:
- general weakness and malaise;
- chronic fatigue;
- difficulty concentrating;
- chills (no fever);
- muscle pain.
Some patients complain of emotional disturbances:
- depression ;
- propensity for antisocial behavior;
- unreasonable fear and panic;
Emotional lability is most likely not associated with elevated bilirubin, but with self-suggestion (constant testing, visits to various clinics and doctors).
Various laboratory tests help confirm or refute Gilbert’s syndrome :
- complete blood count – reticulocytosis in the blood (elevated levels of immature red blood cells) and mild anemia – 100-110 g / l.
- urinalysis – no abnormalities. The presence in the urine of urobilinogen and bilirubin indicates the pathology of the liver.
- biochemical analysis of blood – blood sugar is normal or slightly reduced, blood proteins are within normal limits, alkaline phosphatase, AST , ALT are normal, and the thymol test is negative.
- blood bilirubin – in general, the total bilirubin content is 8.5-20.5 mmol / l. In Gilbert’s syndrome, there is an increase in total bilirubin due to indirect.
- blood clotting – prothrombin index and prothrombin time – within normal limits.
- viral hepatitis markers are absent.
- Ultrasound of the liver.
Perhaps some increase in the size of the liver during an exacerbation. Gilbert ‘s syndrome is often combined with cholangitis, gallstones, chronic pancreatitis.
It is also recommended to investigate the functions of the thyroid gland and conduct a blood test for serum iron, transferrin, total iron binding capacity (OZHSS).
In addition, special tests are conducted to confirm the diagnosis:
- Trial with fasting. Fasting within 48 hours or caloric restriction of
food (up to 400 kcal per day) leads to a sharp increase (by 2-3 times) in free
bilirubin. Unbound bilirubin is determined on an empty stomach on the
first day of the test and after two days. An increase in indirect
bilirubin by 50-100% indicates a positive test.
- Test with phenobarbital.
Acceptance of phenobarbital in a dose of 3 mg / kg / day for 5 days helps to reduce the level of unbound bilirubin.
- Test with nicotinic acid.
An intravenous injection of nicotinic acid in a dose of 50 mg leads to an increase in the amount of unbound bilirubin in the blood by a factor of 2-3 over the course of three hours.
- Trial with rifampicin . The administration of 900 mg
of rifampicin causes an increase in indirect bilirubin.
Also confirm the diagnosis allows percutaneous liver puncture. Histological examination of punctate shows no signs of chronic hepatitis and cirrhosis of the liver .
Another additional, but expensive, study is molecular genetic analysis (blood from a vein), which is used to determine defective DNA involved in the development of Gilbert’s syndrome.
Specific treatment of Gilbert’s syndrome does not exist. The gastroenterologist (in his absence – the therapist) monitors the condition and treatment of patients.
Diet for syndrome
Lifetime is recommended, and during periods of exacerbation – especially careful, adherence to a diet.
- sweet food;
- bakery products;
- fat creams;
In the diet should prevail vegetables and fruits, from cereals preference is given to oatmeal and buckwheat. Low-fat cottage cheese is allowed, up to 1 egg per day, not sharp hard cheeses, dry or condensed milk, a small amount of sour cream. Meat, fish and poultry should be low-fat varieties, the use of spicy foods or food with preservatives is contraindicated. Alcohol, especially strong, should be discarded.
Shows copious drinking. It is advisable to replace black tea and coffee with green tea and unsweetened sour berry juice (cranberries, lingonberries, cherries).
Meal – at least 4-5 times a day, in moderate portions. Fasting, like overeating, can lead to exacerbation of Gilbert’s syndrome.
In addition, patients with this syndrome should avoid exposure to the sun. It is important to inform physicians about the presence of this pathology so that the attending physician can select the appropriate treatment for any other reason.
During periods of exacerbation, an appointment is scheduled:
- hepatoprotectors ( Essentiale forte, Kars, silymarin, heptral);
- vitamins (B6);
- enzymes (festal, mezim);
Also shown are short courses of phenobarbital, which binds indirect bilirubin.
For the restoration of intestinal peristalsis and with severe nausea or vomiting using metoclopromide ( cerculated ), domperidone.
The prognosis for Gilbert’s syndrome is favorable. If you follow the diet and rules of conduct, the life expectancy of these patients does not differ from the life expectancy of healthy people. Moreover, maintaining a healthy lifestyle contributes to its increase.
Complications in the form of chronic hepatitis and cirrhosis of the liver are possible with alcohol abuse, excessive passion for “heavy” food, which is quite possible in healthy people.