Functional hyperbilirubinemia. What is hyperbilirubinemia: symptoms, causes, treatment and types Hyperbilirubinemia in children

You need to protect your health and take care of your body. If this is not done, then life can turn into continuous visits to hospitals and doctors.

Diseases of the liver and gall bladder always cause many problems and also threaten the most serious consequences.

Blood serum tests often reveal increased levels of bilirubin, the pigment of bile.

This disease is called hyperbilirubinemia. Bilirubin is produced from hemoglobin in red blood cells of the bone marrow, liver and other internal organs.

In the first week of life, many newborns develop hyperbilirubinemia, which causes great concern for doctors and parents.

Many factors lead to increased bilirubin in the blood, among which are particularly genetic polymorphisms, hereditary defects, and racial characteristics. Let's look at this disease in more detail.

What it is

With hyperbilirubinemia, a characteristic symptom is an increase in bilirubin in the blood. This often leads to diseases of the internal organs. For example, cholelithiasis may develop or the gallbladder may become inflamed. As a result of the accumulation of bilirubin in the blood, the tissues become yellow and jaundice develops.

Process description

Bilirubin is mostly formed from hemoglobin and other hemoproteins. The microsomal enzyme heme oxygenase promotes the release of biliverdin from hemoglobin. Under the influence of biliverdin reductase, it is converted into bilirubin.

This is what the normal process of formation and elimination of a substance looks like:

Unconjugated bilirubin is called bilirubin that has not come into contact with glucuronic acid. Due to its connection with albumin, it is transported in the blood and does not dissolve in it.

In the liver, it binds to cytosolic proteins and, under the influence of UDP-glucuronyltransferase, goes through the stage of glucuronidation. As a result, bilirubin mono- and diglucuronides, abbreviated as BMG and BDG, are formed. These water-soluble compounds are excreted in the bile. Unconjugated bilirubin can enter the intestine in small quantities along with bile. There, the conjugates disintegrate and indirect bilirubin is metabolized.

The metabolites mesabilinogen and stercobilinogen are oxidized by oxygen. As a result, pigments are formed: mesobilin and stercobilin, yellow-brown in color. They are what makes the stool yellow.

Urobilinogen is also absorbed into the large intestine and kidneys. Urobilinogen, which is oxidized under the influence of atmospheric oxygen to urobilin, is partially excreted in the urine.

Types of disease

  1. Gilbert's syndrome is a hereditary pathology in men. With this disease, bilirubin metabolism is disrupted due to a lack of the enzyme transglucuronyltransferase and jaundice develops. It often occurs during physical and emotional stress, but at other times a person feels absolutely healthy.
  2. Conjugated or functional hyperbilirubinemia in men and women it is a hereditary disease that manifests itself during overload and overstrain of the body. In this disease, conjugated bilirubin accumulates in the blood, causing jaundice. The disease does not progress to hepatitis or cirrhosis.
  3. Crigler-Najjar syndrome is a non-hemolytic unconjugated bilirubinemia, which is also called kernicterus. The disease is accompanied by brain damage. The prognosis is favorable when the disease appears after 5 years, but its development from birth will be unfavorable.

Forms of hyperbilirubinemia:

Prevalence and significance

The most common type of this disease is Gilbert's syndrome. Most often it is found among the peoples of Africa. This disease was common among many famous people. For example, it is known that Napoleon suffered from this disease.

Risk factors

  • carrying out intensive hemolysis;
  • entry into the digestive tract or blood of toxic substances, that is, poisons;
  • long-term use of medications;
  • pregnancy.

Consequences

In most cases it does not cause severe complications. Bilirubin does not usually accumulate in the skin, so itching does not occur. But indirect bilirubin in some conditions and in newborns can enter the brain tissue. In this case, it develops encephalopathy, which is called kernicterus, or neurological disorders appear.

Bilirubin encephalopathy develops most often due to a high level of indirect bilirubin in the blood.

Causes

  1. Destruction of a large number of red blood cells, which leads to an increase in bilirubin.
  2. Problems with removing bilirubin from the body.
  3. Hepatic metabolic disorders.

As a result of increased hemolysis in the liver, bilirubin appears in an unbound form. This often leads to hemolytic disease of the newborn or fetus, as well as hemolytic anemias. In addition, this type of disease develops due to mechanical damage to red blood cells, poisoning, and liver problems.

This disease can develop with malaria, but there is no less risk with pulmonary infarctions. In this case, free bilirubin accumulates in the blood and tissues. The excretion of urobilinogen in urine and feces is increased in hemolytic jaundice.

If there is a violation of the excretion of bilirubin from the body, the patient may develop hepatitis and cirrhosis of the liver. Sometimes this disease occurs due to long-term use of hormonal contraceptives.

When practicing bodybuilding, when patients take steroids, similar phenomena can also be observed.

What are the symptoms of the disease

A high level of bilirubin in the blood can lead to disruption of the central nervous system: lethargy, lethargy, and rapid fatigue. When bilirubin enters the brain, it has a toxic effect. Most often, newborn babies die. This pathology can be inherited.

In mild cases of the disease, the liver is painless and the size of this organ is normal. In this case, the patient remains fully able to work.

With benign congenital hyperbilirubinemia, there are no disturbances in the functioning of the liver or the whole body. The only symptom of the disease can be considered a jaundiced coloration of the skin and whites of the eyes. This is especially noticeable under stress and overwork. As the disease worsens, pain, nausea, weakness appear, and appetite decreases.

The suprahepatic form of hyperbilirubinemia is accompanied by the following symptoms:

  • brown coloration of stool and urine;
  • yellow tint to the skin, mucous membranes and eyes;
  • drowsiness and malaise;
  • pallor of the mucous membrane;
  • irritability;
  • deterioration of vision and speech.

The hepatic form of hyperibilirubinemia manifests itself in the following symptoms:

  • reddish tint of the skin and mucous membranes;
  • a feeling of bitterness in the mouth;
  • discomfort in the liver area;
  • itching of the skin;
  • fatigue.

The subhepatic form of hyperbilirubinemia is characterized by the following:

  • mild pain in the liver area;
  • fatty white feces;
  • bitter taste in the mouth;
  • hypovitaminosis;
  • yellowing of the skin and eye sclera.

Treatment

Which doctors should I contact?

First of all, you need to contact a general practitioner, who, if necessary, can refer the patient to more specialized specialists: a gastroenterologist and hepatologist.

Drugs

Choleretic drugs are used to treat hepatitis: Ursofalk, Chofitol, Gepabene, as well as vitamins. To reduce bilirubin levels, the drugs Zixorin and Phenobarbital are used, but they are prescribed very rarely.

Thermal and electrical physiotherapeutic procedures in the liver area cannot be performed for this disease. To exclude the acute form of the disease, it is worth reducing nervous and physical stress.

Newborns are treated using a modern method - phototherapy. Bilirubin can be removed from the body using fluorescent light. Newborns with mild forms of the disease are prescribed treatment with a fiber-optic blanket. It can also be done at home.

Sorbovit-K is also prescribed to ensure the normal functioning of the body. To reduce the level of bilirubin in the blood, various choleretic drugs may be recommended.

In the acute period of the disease, the main goal is to reduce the toxic effect of high concentrations of bilirubin. For this purpose, medications with antioxidant properties are prescribed, for example, ionol and tocopherol. To treat severe hyperbilirubinemia, glucose is injected into the blood and insulin is given subcutaneously.

Surgery

If there is a risk of developing kernicterus in newborns, a blood transfusion is performed. This operation is performed in an operating room or treatment room where there is a heated radiant heat source. The blood transfusion operation is performed by a doctor with the help of an assistant.

Treatment with traditional medicine

Folk remedies can be used to treat hyperbilirubinemia. This is allowed during the recovery stage. They are also often prescribed for congenital pathologies. Traditional medicine offers herbal teas and individual herbs that have a choleretic effect. Among them, mint, corn silk, and calendula can be especially noted, which are taken for about a month.

Diet

For benign hyperbilirubinemia, a special diet is prescribed, for example, №15 . But it can be used only in the absence of disturbances in the gastrointestinal tract. In the presence of gallbladder diseases, diet No. 5 No. 5 is prescribed.

Prevention

To prevent the disease, you should not drink alcohol, as well as spicy and fatty foods. It is important to avoid heavy physical activity and stressful situations.

Forecast

If the disease is detected in a timely manner, prongosis is generally favorable. In this case, the severity of the disease and the presence of concomitant pathologies are important.

Conclusion

  1. Increased bilirubin in the blood is a condition called hyperbilirubinemia.
  2. As a result, pathologies of internal organs may develop, for example, gallstone disease or brain damage.
  3. Hyperbilirubinemia in newborns can occur for various reasons, but requires prompt medical intervention, as it often leads to the death of children.
  4. The disease must be treated promptly in adults to avoid serious complications.
  5. Prevention of this disease and adherence to diet play a huge role.

Hepatologist, Gastroenterologist, Proctologist

Alexey has been practicing medicine since 1996. Provides therapy for all diseases of the liver, gall bladder and gastrointestinal tract as a whole. Among them: hepatitis, pancreatitis, duodenal ulcer, colitis.


Hyperbilirubinemia is a pathological condition when the level of bilirubin in the blood increases. The development of the disease is provoked by various diseases of the internal organs. These are liver failure and gallstone disease. If the process of hyperbilirubinemia is at the first stage of development, then specific therapy is not carried out. In some cases, the gallbladder begins to become inflamed.

Most doctors do not consider this condition to be an independent pathology. Despite the fact that it has its own ICD-10 code, it is classified as a symptom of a certain pathological process. However, one or another sign indicates the development of a deviation in the body.

Etiology

The causes of hyperbilirubinemia are as follows:

  • genetic predisposition;
  • alcohol or drugs;
  • infectious diseases;
  • erroneously incorrect blood transfusion when the group and Rh factor do not match;
  • autoimmune diseases;
  • chronic form;
  • inflammatory process of liver tissue;
  • leukemia;
  • neoplasms of various types;
  • hemorrhages in the abdominal cavity after injury;
  • steroid use;
  • the use of antipyretic and antibacterial agents.

The identified syndrome of developing hyperbilirubinemia adversely affects the nervous system. It can also cause poisoning of the body.

If the increase in bilirubin increases in parallel with diseases such as cirrhosis, this indicates that the patient’s condition is extremely serious. This can especially happen after cholecystectomy.

If this condition occurs during pregnancy, it means that bile stagnation has occurred due to estrogen. Although this rarely occurs, it is still necessary to consult a doctor who will prescribe an examination, thereby avoiding the development of serious pathologies.

Each form and variety of the disease has its own ICD-10 code.

Classification

This pathology has its own classification:

  • Functional disorders diagnosed in adults. ICD-10 code: E80 is a general code, but there is also an ICD-10 code for varieties (syndromes). For example: ICD-10 code ‒ E80.4 - , ICD-10 code ‒ E80.5 - Crigler syndrome, ICD-10 code ‒ E80.6 - Dubin-Johnson syndrome and Rotor syndrome. There is also a code according to ICD-10 - E80.7 - disorder of bilirubin metabolism, unspecified.
  • Hereditary hyperbilirubinemia - ICD-10 code - D55 - D58.
  • in newborns - ICD-10 code - P50 - P61.

More specifically, this pathology is divided into the following types:

  • Benign hyperbilirubinemia. In most cases it is chronic, very rarely it is intermittent. It is believed that this is hereditary hyperbilirubinemia. The name benign has a collective character. It refers to disturbances in metabolic processes that are associated with the level of bilirubin. The main symptom of this disease is yellow color of the skin and sclera of the eyes. This condition can occur after experiencing severe stress, after surgery or an infectious disease, or during pregnancy.
  • Unconjugated hyperbilirubinemia or direct hyperbilirubinemia. It is characterized by an increase in indirect bilirubin. Most often this happens with hemolysis. Such hyperbilirubinemia occurs in newborns who have been diagnosed, as well as in patients with a hereditary form of hemolytic anemia, when red blood cells have been damaged. In most cases, this occurs when the body is poisoned, during, as well as during acute or. A complication of such hepatitis is posthepatitis hyperbilirubinemia.
  • Conjugative developing hyperbilirubinemia is an increase in the level of direct bilirubin, in other words, physiological jaundice. It is mainly diagnosed in premature babies because their liver enzyme system is not yet developed. Such hyperbilirubinemia in premature babies shows the first symptoms only on the third day. It progresses over the course of a week and then subsides. In children born prematurely, it will pass in three weeks, and in young children - in two weeks.
  • Transient hyperbilirubinemia is diagnosed in 70% of newborns. It occurs due to a weak reduction of bile pigments. It fully manifests itself on the third day after birth. The color of urine and feces remains unchanged. In some children, this is manifested by constant drowsiness, poor appetite, and in rare cases, occasional vomiting. Symptoms appear within a week, sometimes more. In such a situation, a pediatrician will help and prescribe effective treatment. This form of pathology almost never causes complications.

Recently, it has been increasingly diagnosed.

Symptoms

In each case, hyperbilirubinemia manifests symptoms quite clearly. The most characteristic is the yellow color of the skin, sclera of the eyes, and mouth.

Additionally, the following symptoms appear:

  • feeling of pain in the right hypochondrium;
  • nausea and occasional vomiting;
  • , feeling of bitterness in the mouth;
  • general weakness, refusal to eat;
  • sudden mood swings;
  • drowsiness;
  • rare and short-term fainting;
  • neurological disorders;
  • urine becomes dark in color;
  • there is skin itching;
  • a sharp decrease in body weight.

If such a pathology occurs during pregnancy, then the clinical picture may be supplemented by symptoms of the following nature:

  • constant feeling of fatigue, drowsiness;
  • general malaise and weakness;
  • loss of appetite;
  • If there are chronic diseases, then their exacerbation is possible.

Patients diagnosed with mild functional hyperbilirubinemia do not experience any discomfort when palpating the liver, and the organ does not increase in size.

In the moderate and severe stages of a disease such as indirect developing hyperbilirubinemia, the liver is enlarged and painful. A variation of this form of pathological process is Gilbert's syndrome.

If the yellow color of the skin is the only clinical sign and additional symptoms of health problems are not established even through diagnosis, a preliminary diagnosis of benign hyperbilirubinemia or congenital developing hyperbilirubinemia is made.

Diagnostics

The initial examination is carried out by a general practitioner:

  • first he conducts a visual inspection;
  • collects the patient's medical history.

Further diagnostics may include the following examinations:

  • general clinical and detailed biochemical blood test;
  • general analysis of urine and feces;
  • Ultrasound of the abdominal cavity;
  • Ultrasound of the liver;
  • liver tests.

In this case, you may need to consult an infectious disease specialist, hematologist, immunologist, or nephrologist. Based on the results of the examination, pathology is identified and appropriate treatment is prescribed.

Treatment

If hyperbilirubinemia is diagnosed, treatment should only be comprehensive. If it turns out that the patient has a hematologist, consultation with a hematologist is necessary, especially if this pathology is diagnosed in a pregnant woman.

Drug therapy includes:

  • antibacterial and antiviral drugs;
  • taking immunomodulators;
  • anti-inflammatory drugs;
  • antioxidants (if necessary), for example, Enterosgel;
  • ursodeoxycholic acid preparations, for example Ursosan;
  • Phenobarbital to lower bilirubin levels;
  • phytotherapeutic treatment.

Prevention

As preventive measures, you must adhere to the following recommendations:

  • following a certain diet with limited harmful foods;
  • give up bad habits, especially drinking alcohol;
  • Do not overload the body with physical activity;
  • should be avoided .

If the pathology is diagnosed in a timely manner and effective treatment is started, then the prognosis will be favorable. But the prognosis still depends on the degree of development of the disease and accompanying pathological processes.

Is everything in the article correct from a medical point of view?

Answer only if you have proven medical knowledge

Hyperbilirubinemia - what is it? Hyperbilirubinemia is a disease in which the level of bilirubin in the blood increases. The disease often causes diseases of the internal organs (liver failure and cholelithiasis), and in some cases can even lead to serious inflammation of the gallbladder. However, specific treatment for mild stages of the disease is not required.

By the way, many experts do not consider hyperbilirubinemia to be an independent disease. They relate this concept rather to the symptoms of a disease, for example, similar to a headache or an increase in a person’s body temperature in response to the cause of the disease. Nevertheless, every symptom is closely related to a pathological change in the body. But with which one? The answer to this question can only be given by a specialist after a thorough examination of the patient’s condition and after the necessary examinations.

There is a group of diseases in which the skin is characterized by a yellowish tint, but the functioning of the liver and other internal organs remains within normal limits.

In such cases, any morphological changes in the liver are completely absent. When such syndromes occur, doctors talk about the presence of congenital (functional) or benign hyperbilirubinemia.

  • Benign hyperbilirubinemia often manifests itself as chronic jaundice. Rarely, it may have the character of intermittent jaundice. If we talk about the causes of such a disease, then this is often a hereditary (family) factor. The medical term “benign hyperbilirubinemia” is a concept of a certain collective nature, talking about various pathologies of metabolic processes associated with the production of bilirubin and an increase in its 1111 level. In people with history of such a disease, the defining signs of the pathology are icteric discoloration of the skin, eye sclera, as well as a high level of bilirubin. This kind of jaundice can appear against the background of severe mental stress, after surgery, or after an infection during pregnancy. It is immediately necessary to clarify that hyperbilirubinemia of this kind during pregnancy does not pose any danger to life and is not a recommendation for termination of pregnancy. Special treatment in such cases is not prescribed.
  • Unconjugated hyperbilirubinemia is a disease in which the content of so-called indirect bilirubin in the blood increases. This happens mainly during hemolysis, and is closely related to its intensification. This disease can often be observed in newborns diagnosed with hemolytic disease, with a hereditary form of hemolytic anemia, and also due to damage (mechanical impact) of red blood cells. This often happens when the body is poisoned with toxic substances and heavy metals. An increase in the level of indirect bilirubin in the blood is associated with glucuronidation processes (the main reaction of phase II metabolism of the lymphatic system). Occurs with liver cirrhosis, chronic and acute hepatitis.

  • If we are talking about an increased concentration of direct bilirubin, then this disease is called conjugative hyperbilirubinemia, which in principle is a concept such as physiological jaundice. The disease is more common in children born prematurely (premature), because the liver enzyme system in such children is still immature. Hyperbilirubinemia in newborns of this kind, as a rule, does not appear immediately, but only on the 3rd day, progresses for a week, and then gradually declines. At the age of two weeks in full-term infants and three weeks or a little more in premature infants, the conjugative form of jaundice completely disappears.
  • Transient hyperbilirubinemia is recorded in most newborns in the very first days of life (60-70%). In such babies, transient jaundice is observed, caused by the processes of hemolysis of red blood cells due to the immaturity of the functions of the internal organs, mainly due to a weak reduction of bile pigments. This form of the disease manifests itself on days 2-3, the color of the stool does not change, and the urine does not change color either. True, increased drowsiness, inactive sucking, and occasional vomiting may be observed. Signs of the disease persist for a week, sometimes a little more. Treatment in such cases is prescribed by a pediatrician. The prognosis of the disease is always favorable and there are generally no consequences. In rare cases, transient jaundice can develop into pathology. This occurs when there is a malfunction in the body against the background of an increase in the concentration of bilirubin, called unconjugated.

Reasons for increased bilirubin levels (direct and indirect):

  • anemia (congenital and acquired hemolytic anemia);
  • hereditary factor (the structure of red blood cells is impaired);
  • poisoning with drugs and alcohol (intoxication of the body);
  • infections of various origins (viruses, sepsis);
  • blood transfusion, when a patient is mistakenly given blood that is incompatible with his group and Rh factor;
  • diseases of an autoimmune nature (in patients, the native immune system considers native cells to be foreign and begins to reject them);
  • chronic form of lupus erythematosus;
  • inflammation of the liver tissue;
  • leukemia;
  • tumors;
  • hemorrhages in the peritoneum, for example, after severe trauma;
  • medications (anabolic steroids), which are often used when playing sports to improve their records;
  • medications with antibacterial and antipyretic effects.

Hyperbilirubinemia can have a significant adverse effect on the central nervous system and cause intoxication of the body.

The described causes (hepatitis, cirrhosis), as well as the rapid increase in the level of direct bilirubin in the blood, indicate that the patient’s condition is pathologically severe.

The reasons for high levels of direct bilirubin during pregnancy are stagnation of bile caused by the influence of estrogens. As a rule, this pathology is very rare and develops in the third trimester of pregnancy. If such a disease is detected in a woman during pregnancy, it is necessary to undergo all the necessary examinations to exclude the presence of serious disorders and other dangerous diseases. All therapeutic measures in this case should be aimed at combating the underlying disease.

Symptoms of the disease

Many biochemical blood parameters cannot be visually determined, but hyperbilirubinemia can be determined even with the naked eye, because the symptoms of the disease are too obvious.

Characteristic symptoms are a yellowish (lemon) color of the skin, oral mucosa, whites of the eyes, dark urine (reminiscent of beer), as well as mild but persistent itching of the entire body.

Jaundice discoloration tends to worsen under stress. Obvious jaundice appears only when the bilirubin level increases several times (2 times or more).

Patients may often experience other symptoms:

  • discomfort under the right rib;
  • nausea, vomiting (profuse or scanty, single, less often repeated);
  • heartburn, bitter taste in the mouth (increases after eating);
  • weakness, apathy, loss of appetite;
  • mood swings;
  • drowsiness (mainly during pregnancy);
  • stool becomes greasy, colorless, similar to white clay (sometimes it can become dark brown);
  • short-term loss of consciousness (rare);
  • disorders of the neurological system (difficulty speaking, sharp deterioration of vision);
  • sudden weight loss.

During pregnancy, a woman diagnosed with hyperbilirubinemia quickly gets tired, wants to sleep all the time, and becomes slightly lethargic.

Women in an “interesting situation” are advised to follow a diet consisting of gentle foods and not eat a lot of fatty, salty foods. Naturally, we are not talking about any physical activity, especially beyond measure, during pregnancy. Each woman herself should be responsible for her own and her baby’s health, not neglect these recommendations, and, if necessary, undergo treatment prescribed by a therapist.

In patients with a mild form of the disease, liver enlargement and pain on pressure are not observed. But in the moderate and severe stages, during palpation the doctor detects an enlargement of the organ. If, along with pain, there are numerous symptoms, then delaying treatment is extremely irresponsible.

Treatment

When a person is diagnosed with high bilirubin levels, he needs to visit a physician. After the doctor talks with the patient and conducts a thorough examination, he will give a referral for examination, including an ultrasound. Based on the test data, the doctor will be able to determine the causes of the disease and prescribe the necessary treatment.

If the patient suspects hemolytic anemia, he will need to contact a hematologist. Consultation with such a doctor during pregnancy is especially important, because not only the health of the expectant mother, but also the baby’s is at stake.

During pregnancy, treatment is prescribed only after a thorough examination and all necessary studies. Medicines are prescribed based on benefit to the mother and minimal risk to the fetus.

  1. If it turns out that the main cause of the disease is bacterial or viral in nature, then the therapist, as a rule, prescribes treatment with antiviral and antibacterial drugs. In addition, the patient will be advised to take medications that stimulate bile secretion.
  2. But to boost immunity, drugs called immunomodulators can be prescribed.
  3. Anti-inflammatory drugs should be included in complex treatment if there is an inflammatory process in the liver.
  4. You may need to take a course of antioxidants to cleanse your body (mainly your intestines) of toxins.
  5. In addition to drug therapy, phototherapy is prescribed, which consists of irradiation procedures with blue light from special lamps (such a stream of rays can destroy even persistent bilirubin).

Each patient, on the doctor’s recommendation, must rearrange their diet, namely, remove from the table all foods that do not benefit the body and aggravate the course of the disease.

Hyperbilirubinemia is a human condition that is accompanied by increased levels of bilirubin, or red blood cell breakdown products, in the blood.

There is free bilirubin, which constantly circulates in the blood only after the breakdown of red blood cells. It is very toxic, poisons the body, so its accumulation in the human body leads to the appearance of certain symptoms. There is also direct hyperbilirubinemia, in which bilirubin has already undergone transformation in the liver in order to subsequently be excreted through urine or feces from the body. This concept is also called conjugative hyperbilirubinemia.

Causes

With different forms of hyperbilirubinemia, the causes vary depending on the diagnosis.

  • with suprahepatic hyperbilirubinemia occurs:
    • anemia:
      • hemolytic, when red blood cells are destroyed;
      • pernicious, when there is a lack of vitamin B12, which leads to anemia;
      • hemoglobinopathy, when the structure of hemoglobin is disrupted.
  • hereditary, that is, those that are passed on to children from parents, disorders of the construction of blood cells, for example, this could be a defect in the cell wall of an erythrocyte:
    • intoxication or poisoning from medications, alcohol;
  • infections:
    • sepsis or general inflammation of the body, which occurs mainly when bacteria are transferred into the blood;
    • viruses;
  • improper blood transfusion;
  • autoimmune diseases, when the body’s immune system does not perceive its own cells, but mistakes them for strangers and begins to attack them:
    • autoimmune hemolytic anemia;
    • systemic lupus erythematosus, which is a chronic disease of connective tissue that takes part in the protective and support functions of medium-sized blood vessels. Rash and joint pain are typical, and the kidneys are also affected;
    • autoimmune hepatitis or liver inflammation;
  • leukemia or blood cancer;
  • volumetric hemorrhages, perhaps they are directed into the abdominal cavity due to abdominal trauma;
  • hepatic hyperibilirubinemia:
    • Hepatitis:
      • viral, which are caused by hepatitis A, B, C, D and E viruses;
      • medicinal, that is, provoked by anabolic steroids or some antibacterial drugs, perhaps even antipyretics;
    • liver tumors and abscesses;
  • Gilbert's hyperibilirubinemia, which is characterized by periodically occurring yellowness of the skin and whites of the eyes without any other complaints. Short-term pain in the right hypochondrium is also possible. Such hyperibilirubinemia occurs in a calm state and does not particularly affect a person’s way of life;
  • Dabin-Johnson hyperibilirubinemia syndrome is characterized by yellowness of the eye whites and skin, and possible skin itching. When the syndrome occurs, one feels weakness, increased fatigue, the desire to eat food decreases, a bitter taste is felt in the mouth, and sometimes pain occurs in the right hypochondrium;
  • Benign hyperbilirubinemia (Rotor syndrome) is characterized by periodic yellowness of the skin, also the whites of the eyes, and itching of the skin. When jaundice occurs, there is weakness and fatigue, decreased appetite, bitterness in the mouth, sometimes there is pain in the hypochondrium and darkening of the urine;
  • with subhepatic hyperbilirubinemia occurs:
    • blockage of the bile duct with a stone and its inflammation;
    • narrowing of the duct due to its damage;
    • pancreatic or bile duct cancer;
    • Functional hyperbilirubinemia in pregnant women is possible against the background of diseases present in the woman before pregnancy - this could be chronic hepatitis or various liver tumors, anemia, or it can develop due to newly emerging diseases.

Symptoms

Most patients are sick without any symptoms due to increased breakdown of blood cells. With the disease Hyperbilirubinemia, the symptoms are sufficiently pronounced to understand the presence of the disease.

  • The development of suprahepatic hyperbilirubinemia occurs if the amount of bilirubin in the blood increases:
    • the white of the eye, the membrane of the mouth, pharynx, and the skin become lemon-yellow in color. This only happens if bilirubin doubles or more, and the norm is 8.5-20.5 µmol/l);
    • the person feels weak and somewhat lethargic;
    • feces are dark brown in color;
    • apathy occurs, the person is in a bad mood;
    • against the background of the skin, when compared, the pallor of the mucous membrane of the mouth and pharynx is visible;
    • dark colored urine;
    • there are loss of consciousness or neurological disorders, sometimes there is decreased vision, drowsiness or even difficulty speaking;
  • hepatic hyperbilirubinemia is directly related to liver tissue damage;
    • the skin, mucous membrane of the mouth, pharynx, and the white of the eye are colored saffron-yellow, if the skin color is more red - this is called “red jaundice,” which occurs only when bilirubin is increased by two or more times;
    • as well as in the above-described variant of the disease, weakness and lethargy are observed;
    • there is a bitter taste in the mouth;
    • a slight itching is felt throughout the body;
    • there is pain in the right side and general discomfort;
    • vomiting occurs, often multiple times rather than once;
    • sometimes heartburn occurs;
    • urine color turns dark;
    • Over time, the skin acquires a greenish tint;
  • subhepatic hyperbilirubinemia is detected when the passage of bile is blocked or its passage into the intestine is difficult:
    • just as in the first described case, the white of the eye, the membrane of the mouth, pharynx, and also the skin becomes lemon-yellow in color. This only occurs if bilirubin doubles or more;
    • itchy skin on the body;
    • discomfort and pain in the right hypochondrium;
    • stool becomes lighter and may be white;
    • there is an increased amount of fat in the stool;
    • there is a bitterness in the mouth;
    • body weight decreases;
    • hypovitaminosis occurs.

Diagnostics

For diagnosis, an analysis of the disease history and complaints is carried out: the time of onset of pain on the right side, yellowness of the skin and whites of the eyes, darkening of the urine. Also questions that relate to linking the patient's symptoms with the occurrence of the disease. Questions are also asked about nutrition, taking medications and drinking alcohol.

A study of the life history is carried out - past diseases of the gastrointestinal tract and blood, hepatitis, cholelithiasis or operations.

Also, family history: questions about relatives’ illness with hyperbilirubinemia, alcoholism.

The skin is examined for the presence of yellowness, pain on palpation of the liver, spleen and pancreas.

Instrumental research is also required. This is an ultrasound examination of the abdominal organs to assess the condition of the kidneys, gallbladder, intestines, biliary tract, pancreas and liver. Such a study will help to find possible pockets of scar tissue in the liver, possible compression of the bile ducts by a tumor or stone.

A computed tomography scan of the abdominal organs is performed to clearly and accurately assess the condition of the liver, identify difficult-to-diagnose tumors, damage, and nodes in the liver tissue.

Esophagogastroduodenoscopy, in which the condition of the inner surface of the esophagus is diagnosed in order to identify the pathology of varicose veins, damage to the mucous membrane, duodenum and stomach through an endoscope.

A liver biopsy is also needed for a microscopic examination of liver tissue obtained using a thin needle under ultrasound guidance, which will help establish an accurate and correct diagnosis, while excluding a tumor process.

Elastography or examination of liver tissue is performed for diagnosis using a special machine to determine the degree of liver fibrosis, which is a process that can be cured.

Hyperbilirubinemia in newborns is diagnosed in the same way as in adults.

Treatment

Hyperbilirubinemia in children and adults can be treated once the cause that caused the disease is identified.

Antibacterial and antiviral drugs are prescribed that destroy the bacterial or viral causes of the disease. Also hepatoprotectors, that is, drugs that preserve the activity of liver cells. Choleretic drugs are also prescribed, which enhance bile excretion. Immunomodulators are medications that stimulate the immune system to fight hyperbilirubinemia. Anti-inflammatory drugs that relieve liver inflammation. Antioxidants are drugs that reduce and remove the damaging effects of toxic substances in the body. Drugs from the barbiturate group are also prescribed as agents that reduce the level of bilirubin in the blood. Enterosorbents to better stimulate the excretion of bilirubin from the intestine. It is also possible to use phototherapy. This is done by using blue lamps to break down the bilirubin that has accumulated in the tissues.

Diet No. 5 is indicated: meals 5 to 6 times a day, while spicy and fatty, fried and smoked foods, as well as salt are excluded from the diet, protein intake is limited to 40 grams per day with the development of hepatic encephalopathy.

Taking vitamin complexes in courses lasting from one to two months. Enzyme preparations that do not contain bile are also indicated for the diagnosis of hyperbilirubinemia. Physical activity is limited and psycho-emotional stress is reduced.

Any treatment of pregnant women using medicinal methods is associated with the risk of complications in the mother and fetus; treatment must be strictly monitored by a doctor. Treatment of hyperbilirubinemia in newborns should also be carried out under the supervision of a physician.

Complications

It is possible that after such a disease the development of hypovitaminosis or deficiency of vitamins and microelements in the body, the occurrence of liver failure, the development of inflammation of the gallbladder, and the occurrence of cholelithiasis.

Prevention

  • prevention of hyperbilirubinemia in pregnant women is timely registration at the antenatal clinic, timely visit to the doctor, examination;
  • for the prevention of any person, correct diagnosis and timely treatment of the disease or other diseases associated with hyperbilirubinemia that cause the development of the disease are necessary;
  • anemia: destruction of red blood cells, poor supply of vitamin B12 to the body, associated with stomach disease and leading to anemia, impaired structure of the oxygen carrier protein;
  • poisoning from drugs or alcohol;
  • infections: severe inflammation of the body caused by the spread of bacteria into the blood and viruses;
  • neoplasms and ulcers in the liver;
  • blockage of the bile duct with a stone;
  • inflammation of the bile duct;
  • a rational and balanced diet with the consumption of foods rich in fiber, avoidance of fried, smoked, hot or spicy foods;
  • quitting smoking and alcoholic beverages;
  • strict control over the intake of medications during various courses of treatment, the expiration date of medications, and dosage.

FUNCTIONAL

HYPERBILIRUBINEMIA

DEFINITION

Benign (functional) hyperbilirubinemia (pigmentary hepatosis) - diseases associated with hereditary disorders of bilirubin metabolism (enzymopathies), manifested by chronic or intermittent jaundice without pronounced primary changes in the structure and function of the liver and without obvious signs of hemolysis (except for primary hyperbilirubinemia) and cholestasis (except for the syndrome Bayler).

TO functional hyperbilirubinemias include:

1) Gilbert's syndrome;

2) Crigler-Najjar syndrome types 1 and 2;

3) Dubin-Johnson syndrome;

4) Rotor syndrome;

5) Meulengracht's syndrome;

6) Lucy-Driscoll syndrome;

7) Aagenes syndrome;

8) Byler's syndrome;

9) primary hyperbilirubinemia.

GILBERT'S SYNDROME

Gilbert's syndrome is a hereditary disease and is transmitted in an autosomal dominant manner. At the heart of Gilbert's syndrome. there is a genetic defect encoding uridine diphosphate glucuronyl transferase (UDPGT). However, a decrease in enzyme synthesis alone is not enough for the development s-ma AND.; the presence of other factors is also necessary, for example, latent hemolysis and impaired transport of bilirubin in the liver. Therefore, with Gilbert's syndrome, there is also a slight disturbance in the release of bromsulfalein and tolbutamide. It is also assumed that the transport function of proteins that deliver unconjugated bilirubin to the smooth endoplasmic reticulum (microsomes) of hepatocytes is impaired. Thus, with Gilbert's syndrome, there is a decrease in the uptake from the blood and conjugation of bilirubin and the development of unconjugated hyperbilirubinemia and the appearance of jaundice.

Epidemiology

In the population, the incidence of Gilbert's syndrome is 2-5%, (1-5%), 10 times more common in men than in women. It is usually detected in adolescence and young adulthood (usually at 11-30 years old) and continues throughout life.

The general condition of patients is usually satisfactory.

Main complaints

1. Transient jaundice: it can intensify after intercurrent infections or after fasting, it can be provoked by stressful situations, heavy physical activity. May be accompanied, especially during periods of exacerbation, by mild pain in the right hypochondrium,

2. Dyspeptic syndrome (nausea, bitterness in the mouth, loss of appetite, belching, stool disorders, constipation or diarrhea, flatulence).

3. Atenovegetative manifestations (depression - depressed mood, fatigue, poor sleep, discomfort in the heart, dizziness).

Along with jaundice, in some cases, xanthelasmas of the eyelids, facial pigmentation, and scattered pigment spots on the skin are observed. Liver enlargement is observed in 25% of patients. The liver protrudes from under the costal arch by 1-2 (sometimes up to 4 cm), the consistency is normal (soft), painless on palpation. An enlarged spleen is not typical, but is observed in 10% of patients.

In 9% of patients, the disease is asymptomatic and is detected accidentally during a medical examination.

Diagnostics

CBC without pathology, an increase in Hb to 160 g/l and a decrease in ESR. Slight reticulocytosis is observed in 15% of patients.

BAC: increased levels of indirect bilirubin. Free bilirubin (indirect unconjugated) is usually no more than 85-100 μmol/l, even during periods of exacerbations. In this case, there is no bilirubinuria, the number of urobilin bodies in feces and urine is not changed.

During exacerbations, a slight increase in aminotransferases and a decrease in albumin are possible. However, this is usually with long-term illness and development of chronic disease. hepatitis A.

Special diagnostic tests

1) fasting test is positive (increase in serum bilirubin level during fasting by 2 times per day)

2) test with phenobarbital (taking phenobarbital causes a decrease in bilirubin levels)

3) test with nicotinic acid (iv administration of nicotinic acid, which reduces the osmotic resistance of red blood cells and causes an increase in bilirubin levels).

Drug treatment should not be carried out. However, in some cases, agents are used that reduce the level of bilirubin in the blood serum due to the induction of enzymes that conjugate bilirubin (phenobarbital, zixorine).

DUBIN-JOHNSON SYNDROME

This is a chronic benign disease manifested by idiopathic intermittent jaundice with unidentified liver cell pigment and elevated levels of predominantly conjugated bilirubin, which is based on a congenital defect of impaired excretory function of hepatocytes (postmicrosomal hepatocellular jaundice).

Epidemiology

A very rare disease that occurs predominantly in young men, in some cases from birth. This syndrome is prevalent primarily in the Middle East among Iranian Jews.

Pathogenesis

The syndrome is based on a deterioration in the transport into bile of many organic anions not related to bile acids, which is caused by a defect in the ATP-dependent transport system of the tubules. Inherited in an autosomal recessive manner. Some authors suggest an autosomal dominant mode of inheritance.

The first clinical signs of the disease are detected between birth and 25 years. The main symptom is jaundice, often severe, often intermittent, less often chronic. Jaundice may be accompanied by fatigue, nausea, sometimes abdominal pain, and occasionally a crisis. Skin itching is not typical, but may rarely be mild. On examination, there is usually a distinct subictericity of the sclera. The liver and spleen are of normal size. Occasionally there is an enlargement of the liver by 1-2 cm.

Diagnostics

Laboratory data

IN blood bilirubin content increased to 20-50 µmol/l (rarely up to 80-90 µmol/l) due to its predominantly direct fraction. Some authors note an increase in alkaline phosphatase.

IN urine - bilirubinuria, dark-colored urine.

Other diagnostic methods

Bromsulfalein test and radioisotope hepatography reveal a sharp violation of the excretory function of the liver. However, after an initial decrease in the concentration of bromsulfalein in the serum, a repeated increase occurs, so that after 120 minutes the concentration exceeds the concentration at the 45th minute.

X-ray examination is characterized by the absence of contrast or later and weak filling of the bile ducts with contrast (oral cholecystography).

With scintigraphy, the excretion of lidophenine indicates the absence of changes in the liver, bile ducts and gall bladder.

Not developed. Patients usually remain functional and generally in good condition without treatment.

ROTOR SYNDROME

This is chronic familial non-hemolytic jaundice with unconjugated hyperbilirubinemia and normal liver histology, without unidentified pigment in the hepatocytes.

Epidemiology

Epidemiology has not been studied due to low prevalence. Boys and girls get sick with the same frequency.

Pathogenesis

The pathogenesis of Rotor syndrome is similar to Dubin-Johnson syndrome, but the defect in bilirubin excretion is less pronounced. The possibility of an autosomal recessive mode of inheritance is assumed.

Often severe chronic jaundice. Other subjective signs (fatigue, pain in the right hypochondrium, loss of appetite) are unclear. The liver is not enlarged, occasionally slightly enlarged.

Diagnostics

Laboratory data

IN blood bilirubin content is increased, mainly due to conjugated bilirubin.

IN urine bilirubinuria, darkening of urine, periodically increased secretion of urobilin.

The total level of coproporphins in urine is increased as in cholestasis, due to isomer I (65% of all).

Other diagnostic methods

After a load of bromsulfalein, an increased retention of the dye is observed after 45 minutes, and a secondary increase in concentration as in Dubin-Johnson syndrome does not occur.

At X-ray examination normal results, i.e. The gallbladder is contrasted during oral cholecystography.

When studying with lidophenine, the liver, gallbladder and bile ducts are not visualized.

No treatment has been developed. Patients usually maintain working capacity and general satisfactory condition. There are observations when various hereditary hyperbilirubinemias reveal Dubin-Johnson syndrome in some family members, and Rotor syndrome in others, and alternation of conjugated and unconjugated hyperbilirubinemia is noted in the same patient. Such observations indicate the conventionality of these syndromes and the complexity of the mechanism of inheritance.

The prognosis is favorable, the course is long-term, without significant disturbances in the general condition. Periodically, exacerbation is possible under the pressure of the same factors as Dabin-Johnson syndrome. Both of these syndromes can lead to the development of cholelithiasis.