What is hyperbilirubinemia and why is it dangerous?

Causes of hyperbilirubinemia

There are many reasons for the increase in bilirubin content, starting with its excessive formation (massive simultaneous destruction of a large number of red blood cells) and ending with disturbances in the removal of the substance itself from the body or the process of uptake from the blood plasma and metabolism in liver cells. A distinction is made between direct and indirect bilirubin and depending on what stage of metabolism the conversion process is at - these concepts show whether this substance is in close contact with blood serum proteins for transport, or whether the compound has already completed metabolism in liver cells.

An increase in indirect bilirubin is called unconjugated hyperbilirubinemia. This disease is associated with increased hemolysis, and it received its name as a result of the fact that bilirubin enters the liver in an unbound form. An increase in the level of unconjugated bilirubin is observed in the presence of hemolytic disease of the fetus or newborn, as well as in hereditary and acquired hemolytic anemia. Also, the manifestation of unconjugated hyperbilirubinemia is promoted by poisoning and mechanical damage to red blood cells, as well as some liver diseases.

An increase in the content of direct, conjugated bilirubin is called “conjugative hyperbilirubinemia.” The main cause of development is disturbances in the removal of bilirubin from the body. Present in various forms of hepatitis, cirrhosis of the liver and in cases of obstructions to the outflow of bile (gallbladder stones, etc.). Also, an increase in direct bilirubin is facilitated by long-term use of hormonal contraceptives or the use of steroids (prescribed for the treatment of diseases of the body, as well as in athletes involved in bodybuilding).

Causes

Benign hyperbilirubinemia in adults in most cases is a disease that is familial in nature and is transmitted in a dominant manner. This is confirmed by medical practice.

There is post-hepatitis hyperbilirubinemia - it occurs as a consequence of viral acute hepatitis. The disease can also be caused by infectious mononucleosis; after recovery, patients may experience symptoms of hyperbilirubinemia.

The cause of the disease is a failure in the metabolism of bilirubin. This substance increases in the serum, or there is a violation of its uptake or transfer into liver cells from the plasma.

A similar condition is also possible in cases where there is a disruption in the binding processes of bilirubin and glucuropic acid, which can be explained by a permanent or temporary deficiency of an enzyme such as glucuronyl trapsferase. The listed mechanisms of hyperbilirubinemia characterize Gilbert, Crigler-Najjar syndromes and posthepatitis hyperbilirubinemia. In Rotor and Dabin-Johnson syndromes, serum bilirubin increases due to impaired excretion of pigment into the bile canaliculi through hepatocyte membranes.

Symptoms of the disease

With an increased level of bilirubin in the blood, there is a threat of disturbances from the central nervous system, namely, the person becomes lethargic, lethargic, and easily overtired. A complication is also possible when bilirubin enters the nuclei of the brain stem and has a toxic effect on them. This is especially true for newborns. Crigler-Nayjar syndrome (congenital malignant hyperbilirubinemia of an unconjugated nature, which is inherited) usually leads to the death of sick children.

With a mild course of the disease, general weakness and fatigue are observed. In such cases, the liver is not enlarged in size and is painless, the treatment will be effective and the ability to work will be maintained.

Benign hyperbilirubinemia, as a rule, is congenital in nature as a result of a congenital disorder of the liver, which does not have a serious effect on the functioning of the body as a whole. A characteristic sign of the disease is a yellow (icteric) coloration of the skin and whites of the eyes, especially pronounced as a result of overwork and stress. Sometimes, during an exacerbation, pain in the right hypochondrium, general weakness, nausea and appetite disturbances appear.

What is bilirubin?

Red blood cells are formed in the bone marrow. During the growth process, hemoglobin accumulates in them, then each red blood cell loses its nucleus (it is squeezed out or destroyed) and enters the blood. The lifespan of these cells is approximately 120 days, after which they are destroyed “from old age”, releasing bilirubin.

Most red blood cells are broken down in the liver and spleen into two components: heme and globin. In this case, the complex compound heme loses iron, which enters the bone marrow for reuse. And the remaining protein part of the molecule is converted into a red-brown bile enzyme - bilirubin. It enters the gastrointestinal tract along with bile and is then excreted.

Functional hyperbilirubinemia (benign jaundice) occurs when there is an increased content of bile pigment in the blood. Free (direct, unbound) bilirubin is toxic, so large amounts of it are dangerous. The bound (indirect, unconjugated) pigment is neutralized in the liver cells and excreted in the urine and feces.

Forms of the disease

Indirect hyperbilirubinemia is an increase in the amount of bilirubin as a result of an increase in the rate of its formation. At the same time, the process of natural cell death proceeds with the same speed, i.e., the breakdown products of red blood cells are excreted more slowly than new bilirubin is formed. This orange pigment accumulates in the skin, on the sclera of the eyes and mucous membranes.

There are three mechanisms for increasing pigment concentration:

• suprahepatic (an increase in the amount of indirect bilirubin to a level that the liver cannot process);
• hepatic (disturbance in the functioning of liver cells);
• subhepatic (the outflow and excretion of bile is impaired).

The causes of the suprahepatic form of hyperbilirubinemia are:

• various types of anemia;
• hereditary disorders in the structure of red blood cells;
• poisoning from medications or alcohol;
• severe inflammation, viruses;
• infusion of blood of an incompatible group;
• pathologies in the functioning of the immune system;
• leukemia;
• very large blood loss.

The hepatic form of the disease manifests itself as a result of:

• viral or drug-induced hepatitis;
• tumors or purulent processes in the liver;
• liver cirrhosis;
• syndromes of Gilbert, Rotor, Dabin-Johnson.

The subhepatic type of disease is caused by:

• cholelithiasis (stones block the lumen of the duct);
• inflammation or narrowing of the duct;
• tumor of the pancreas or bile ducts.

Treatment of each form of hyperbilirubinemia depends on the “culprit” of its occurrence. As a rule, the process of increasing the breakdown of red blood cells occurs without any symptoms, and in mild forms does not require therapy. For example, conjugative hyperbilirubinemia (neonatal jaundice) occurs in most infants and usually resolves without drug treatment within a few days. But there are also deadly types of the disease.

Important! Only a specialist can distinguish temporary, harmless yellowing from the first signs of a serious disease.

Congenital hyperbilirubinemia

Idiopathic (occurring on its own) unconjugated hyperbilirubinemia (Gilbert's syndrome) is a congenital disorder in which the amount of unconjugated bilirubin in the blood increases slightly. Rotor and Dabin-Johnson syndromes are characterized by a moderate increase in bilirubin. No special treatment is required; only during periods of exacerbation, diet and giving up bad habits are recommended.

In Crigler-Nayjar syndrome (congenital non-hemolytic hyperbilirubinemia type 1), the level of free pigment increases quite strongly. This disease requires treatment, since the toxic effect of unconjugated bilirubin on the body can lead to death, especially in early childhood.

We advise you to find out what symptoms appear with a pancreatic cyst.

Read: what role do lactobacilli play in the intestines.

It is impossible to figure out the causes of yellowing on your own. The course of all forms of the disease may look the same. It is important to consult a doctor in time and find out an accurate diagnosis. In some cases, jaundice is deadly. Thus, hyperbilirubinemia in Crigler-Najjar syndrome in young children often leads to death.

Methods of treatment and therapy

If the study revealed benign hyperbilirubinemia, treatment consists of following a diet. During remission and if you do not have concomitant disorders in the gastrointestinal tract, diet No. 15 is prescribed; during exacerbations, as well as in the presence of gallbladder diseases, doctors recommend using diet No. 5 (prescribed for most acute and chronic liver diseases).

Special liver therapy is not needed for this form of the disease. As a rule, patients are prescribed a course of vitamin therapy and choleretic drugs. If there are disturbances in the synthesis or excretion of bilirubin, thermal and electrical procedures in the liver area are contraindicated. It is necessary to limit physical and nervous stress to avoid the acute form of the disease.

Hyperbilirubinemia in newborns is treated primarily with phototherapy, which uses ultraviolet fluorescent light to transform bilirubin and accelerate its elimination from the baby's body. Standard phototherapy is used in hospitals. But newborns who have jaundice and no other signs of illness can be treated at home using a fiber-optic blanket. This blanket reduces bilirubin content more slowly than a fluorescent lamp, so it is only applicable in mild forms of the disease.

Fluorescent light, which is used for phototherapy, is not dangerous if the treatment is carried out correctly. Children wear a blindfold over their eyes during the procedure to avoid damaging their visual organs (to prevent burns to the retina). The bandage is removed while feeding the baby. As a rule, the light irradiation procedure is carried out at short intervals of 2-4 hours until the bilirubin returns to normal. Children are not at all bothered by dark blindfolds, since before birth, in the womb, they were in complete darkness and this is a familiar environment for them.

Hyperbilirubinemia syndrome is a congenital form of the disease, so its treatment consists of maintaining the body and ensuring normal functioning. In congenital forms, exacerbations occur, during which it is necessary to undergo a course of treatment with the Sorbovit-K sorbent, and after a decrease in bilirubin levels, one should return to preventive measures, that is, taking vitamins, choleretic drugs and following a diet.

Noticeable yellowing of the skin, mucous membranes and eye sclera will alert anyone. After all, almost every person knows that such symptoms indicate disturbances in the liver. Such ailments can be caused by many factors, and their treatment is carried out exclusively under the supervision of a doctor. The yellowing of the skin itself occurs when the amount of bilirubin in the blood serum increases; doctors classify this condition as hyperbilirubinemia. Let's talk about what benign hyperbilirubinemia is, the symptoms, its treatment, and the causes in a little more detail.

Bilirubin is essentially a bile pigment that has a yellow-red color. This substance is produced from hemoglobin of erythrocytes, which disintegrate due to involutive changes inside the cells of the spleen, liver, bone marrow, as well as inside the connective tissues of organs.

What is benign hyperbilirubinemia?

This pathological condition is an independent disease, which makes itself felt by constant or periodic jaundice, while the structure and functions of the liver remain normal, and there is no increase in hemolysis and cholestasis.

Why does benign hyperbilirubinemia occur, what are the reasons for its appearance?

As practice shows, benign hyperbilirubinemia in most cases is familial in nature - transmitted by a dominant type.

Posthepatitis hyperbilirubinemia also occurs as an outcome of acute viral hepatitis. The causes of benign hyperbilirubinemia can be attributed to infectious mononucleosis, since sometimes a similar condition is observed after it.

With benign hyperbilirubinemia, the patient experiences disruptions in bilirubin metabolism. Thus, an increase in this substance in the serum may be explained by a violation of the uptake or transfer of free bilirubin from plasma into liver cells.

Also, a similar condition can occur when the processes of binding bilirubin with glucuropic acid are disrupted, which is explained by a temporary or permanent deficiency of the enzyme glucuronyl trapsferase. A similar mechanism of hyperbilirubinemia is characteristic of Crigler-Najjar, Gilbert syndromes, as well as posthepatitis hyperbilirubinemia.

Symptoms of benign hyperbilirubinemia

This condition in most cases is diagnosed in adolescence and can last for many years, including throughout life. Most often, this disease affects men. The classic manifestation of benign hyperbilirubinemia is yellowing of the sclera, and a corresponding coloration of the skin is observed only in isolated cases. This manifestation of the disease is usually intermittent and only in rare cases is it permanent.

The appearance or intensification of jaundice can be provoked by severe fatigue (nervous or physical), exacerbation of infectious lesions of the biliary tract, and drug intolerance. Yellowing can also be caused by colds, various surgical interventions, alcohol intake, etc.

In parallel with the staining of the sclera (skin) in characteristic yellowish tones, many patients experience a feeling of heaviness in the area of ​​the right hypochondrium. They may also be bothered by a number of dyspeptic symptoms, represented by nausea, vomiting, lack of appetite, stool disturbances and increased gas formation in the intestines.

Hyperbilirubinemia leads to the appearance of some asthenovegetative disorders, namely depression, fatigue and weakness.

During the examination, doctors first of all pay attention to dull yellow skin and pronounced yellowness of the sclera. Sometimes the skin color remains completely normal. The liver may be palpated at the edges of the costal arch or not be felt at all. Sometimes the organ slightly increases in size, while it has a soft consistency, and palpation itself is painless.

The spleen with benign hyperbilirubinemia remains not enlarged. Its size can change only in patients with the posthepatitis form of this disorder.

How is benign hyperbilirubinemia corrected, and what is its effective treatment?

During remission of benign hyperbilirubinemia of the liver, patients are usually prescribed diet No. 15, and during an exacerbation they are recommended to adhere to treatment table No. 5 (diet 5 according to Pevzner).

With such a health problem, patients are not indicated for any special treatment. They do not need special “liver” therapy. Taking multivitamins and choleretic drugs will be beneficial. As for sanatorium-resort treatment, it is not indicated. And carrying out thermal and electrical procedures on the liver area can be harmful.

In the case of a congenital form of the disease, as well as during exacerbations, doctors can prescribe the use of sorbents, for example, Sorbovit-K.

With a benign form of hyperbilirubinemia, the prognosis is completely favorable. With this disorder, patients remain able to work, but they are strongly advised to limit physical and nervous stress.

Folk treatment of benign hyperbilirubinemia with folk remedies

Choleretic compounds can be used as folk remedies for benign hyperbilirubinemia. It would be a good idea to discuss the advisability of their use with your doctor.

So, as a good choleretic agent, you can use such a common herb as knotweed (instructions, the use of the drug before using it should be studied personally from the official annotation included in the package!). You will need the roots of this plant. Clean them, dry them and chop them well. Pour a tablespoon of the resulting raw material into a glass of cool, pre-boiled water. Place the container with this product in a boiling water bath and leave for half an hour. Afterwards, infuse the medicine for fifteen minutes. Take the strained broth one tablespoon three or four times a day.

Corn silk also gives a remarkable effect (the properties they possess are very useful in this case). Brew a tablespoon of crushed raw materials with a glass of boiling water. Infuse the medicine for an hour, then strain and take a tablespoon at an interval of three hours.

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.

Benign hyperbilirubinemia: diagnosis and treatment

Yellowing of the mucous membranes, eye sclera, and skin should alert any person.
Everyone knows that such symptoms indicate certain disturbances in the functioning of such an important organ as the liver. These ailments must be monitored by a doctor. He will make the correct diagnosis and prescribe the necessary treatment. When bilirubin levels increase, jaundice usually appears. Benign hyperbilirubinemia also has similar symptoms.

In this article we will look in more detail at what kind of disease this is, what are its causes and methods of treatment.

Definition of benign hyperbilirubinemia

In its essence, bilirubin is a bile pigment; it has a characteristic red-yellow color. This substance is produced from red blood cells in hemoglobin, which break down due to involutive changes in the cells of the liver, spleen, connective tissues and bone marrow.

Benign hyperbilirubinemia is an independent disease that includes pigmentary hepatosis, familial simple cholemia, intermittent juvenile jaundice, non-hemolytic familial jaundice, constitutional dysfunction of the liver, retention jaundice and functional hyperbilirubinemia. The disease manifests itself as intermittent or chronic jaundice, obvious disturbances in liver function and its structure without pronounced disturbances. There are no obvious symptoms of cholestasis and increased hemolysis.

Benign hyperbilirubinemia (ICD 10 code: E 80 - general disorders of bilirubin and porphin metabolism) also has the following codes E 80.4, E 80.5, E 80.6, E 80. Coded accordingly: Gilbert syndrome, Crigler syndrome, other disorders - Dubin-Johnson syndrome and Rotor syndromes, an unspecified disorder of bilirubin metabolism.

Causes

Benign hyperbilirubinemia in adults in most cases is a disease that is familial in nature and is transmitted in a dominant manner. This is confirmed by medical practice.

There is post-hepatitis hyperbilirubinemia - it occurs as a consequence of viral acute hepatitis. The disease can also be caused by infectious mononucleosis; after recovery, patients may experience symptoms of hyperbilirubinemia.

The cause of the disease is a failure in the metabolism of bilirubin. This substance increases in the serum, or there is a violation of its uptake or transfer into liver cells from the plasma.

A similar condition is also possible in cases where there is a disruption in the binding processes of bilirubin and glucuropic acid, which can be explained by a permanent or temporary deficiency of an enzyme such as glucuronyl trapsferase.

The listed mechanisms of hyperbilirubinemia characterize Gilbert's, Crigler-Najjar syndromes and posthepatitis hyperbilirubinemia.

In Rotor and Dabin-Johnson syndromes, serum bilirubin increases due to impaired excretion of pigment into the bile canaliculi through hepatocyte membranes.

Benign hyperbilirubinemia, the diagnosis of which is confirmed by the fact that it is most often detected in adolescence, can manifest its symptoms for many years and even throughout life. In men, this disease is detected much more often than in women.

The classic manifestation of the disease is yellowing of the sclera; icteric coloration of the skin may appear in some cases, but not always. Manifestations of hyperbilirubinemia are often intermittent in nature, in rare cases they are constant and do not go away.

Increased jaundice can be caused by the following factors:

• severe physical or nervous fatigue;
• exacerbation of infections, damage to the biliary tract;
• drug resistance;
• colds;
• various surgical interventions;
• drinking alcohol.

Symptoms of the disease

In addition to the fact that the sclera and skin turn yellowish, patients feel heaviness in the right hypochondrium. There are cases when dyspepsia is a concern - nausea, vomiting, stool disturbances, lack of appetite, increased gas formation in the intestines.

Manifestations of hyperbilirubinemia can lead to the appearance of asthenovegetative disorders, which manifest themselves as depression, weakness, and rapid fatigue.

During the examination, the doctor first of all pays attention to the yellowed sclera and the dull yellow tint of the patient’s skin. In some cases, the skin does not turn yellow. The liver is palpated along the edges of the costal arch, or may not be felt.

There is a slight increase in the organ's size, the liver becomes soft, and the patient experiences pain during palpation. The spleen does not increase in size.

Exceptions are cases when benign hyperbilirubinemia occurs as a consequence of hepatitis. Posthepatitis hyperbilirubinemia can also occur after an infectious disease - mononucleosis.

Benign hyperbilirubinemia syndrome

Benign hyperbilirubinemia in medical practice includes seven congenital syndromes:

• Crigler-Najjar syndromes types 1 and 2;
• Dubin-Johnson syndromes;
• Gilbert's syndromes;
• Rotor syndromes;
• Byler's disease (rare);
• Lucy-Driscoll syndrome (rare);
• Familial benign age-related cholestasis – benign hyperbilirubinemia (rare).

All of these syndromes occur due to a violation of bilirubin metabolism if the level of unconjugated bilirubin in the blood is increased, which accumulates in the tissues.

Conjugation of bilirubin plays a huge role in the body; highly toxic bilirubin is processed into low-toxic bilirubin, into diglucoronide - a soluble compound (conjugated bilirubin).

The free form of bilirubin easily penetrates elastic tissues, lingers in mucous membranes, skin, and on the walls of blood vessels, causing jaundice.

Crigler-Najjar syndrome

American pediatricians V. Nayyar and J. Krigler identified a new syndrome back in 1952 and described it in detail. It was named Crigler-Najjar syndrome type 1. This congenital pathology has an autosomal recessive type of inheritance. The development of the syndrome occurs immediately in the first hours after the birth of the child. Similar symptoms occur equally often in both girls and boys.

The pathogenesis of the disease is due to the complete absence of an enzyme such as UDPGT (urndin-5-diphosphate glucuronyltransferase enzyme). In type 1 of this syndrome, UDFGT is completely absent, free bilirubin increases sharply, reaching 200 µmol/l or even more.

After birth, on the first day, the permeability of the blood-brain barrier is high. In the brain (gray matter), pigment rapidly accumulates, and yellow kernicterus develops.

A test with cordiamine in the diagnosis of benign hyperbilirubinemia, with phenobarbital is negative.

Bilirubin encephalopathy leads to the development of nystagmus, muscle hypertension, athetosis, opisthotonus, clonic and tonic convulsions. The prognosis of the disease is extremely unfavorable. In the absence of intensive treatment, death is possible within the first day. The liver does not change at autopsy.

Dubin-Johnson syndrome

Dubin-Johnson benign hyperbilirubinemia syndrome was first described back in 1954. This disease is predominantly common among residents of the Middle East. In men under the age of 25 it occurs in 0.2-1% of cases. Inheritance occurs according to an autosomal dominant pattern.

This syndrome has a pathogenesis that is associated with disturbances in the transport functions of bilirubin into and out of the hepatocyte due to the failure of the ATP-dependent cell membrane transport system. As a result, the flow of bilirubin into the bile is disrupted, and reflux of bilirubin into the blood from the hepatocyte occurs.

This is confirmed by the peak concentration in the blood of the dye after two hours when conducting tests using bromsulfalein.

The morphological characteristic feature is a chocolate-colored liver, where there is a high accumulation of coarse granular pigment.

Manifestations of the syndrome: constant jaundice, periodic skin itching, pain on the right side in the hypochondrium, asthenic symptoms, dyspepsia, enlarged spleen and liver. The disease can begin at any age.

There is a risk of occurrence after long-term use of hormonal contraceptives, as well as during pregnancy.

The disease is diagnosed on the basis of a bromsulfalein test, cholecystography with delayed excretion of a contrast agent into the bile, in the absence of contrast in the gallbladder. Cordiamin is not used in the diagnosis of benign hyperbilirubinemia in this case.

Total bilirubin does not exceed 100 µmol/l, free and bound bilirubin has a 50/50 ratio.

Treatment for this syndrome has not been developed. The syndrome does not affect life expectancy, but the quality of life with this pathology worsens.

Benign hyperbilirubinemia – Gilbert's syndrome

This hereditary disease is the most common, we will tell you more about it. The disease is transmitted from parents to children and is associated with a gene defect that is involved in the metabolism of bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) is nothing more than Gilbert's syndrome.

Bilirubin is one of the important bile pigments, an intermediate product of the breakdown of hemoglobin, which is involved in the transport of oxygen.

An increase in the level of bilirubin (by 80-100 µmol/l), a significant predominance of bilirubin not associated with blood proteins (indirect) leads to periodic manifestations of jaundice (mucous membranes, sclera, skin).

At the same time, liver tests and other indicators remain normal. In men, Gilbert's syndrome is 2-3 times more common than in women. It may first appear between the ages of three and thirteen years. Often the disease accompanies a person throughout his life.

Gilbert's syndrome includes enzymopathic benign hyperbilirubinemia (pigmentary hepatosis). They arise, as a rule, due to the indirect (unconjugated) fraction of bile pigments. This is due to genetic liver defects.

The course is benign - there are no gross changes in the liver or pronounced hemolysis.

Symptoms of Gilbert's syndrome

Gilbert's syndrome does not have pronounced symptoms and occurs with minimal symptoms. Some doctors do not consider the syndrome as a disease, but attribute it to the physiological characteristics of the body.

The only manifestation in most cases is moderate jaundice with staining of the mucous membranes, skin, and eye sclera. Other symptoms are either mild or not present at all. Minimal neurological symptoms are possible:

• weakness;
• dizziness;
• increased fatigue;
• sleep disorders;
• insomnia.

Even more rare symptoms of Gilbert's syndrome are digestive disorders (dyspepsia):

• lack or decreased appetite;
• bitter belching after eating;
• heartburn;
• bitter taste in the mouth; rarely vomiting, nausea;
• feeling of heaviness, fullness in the stomach;
• bowel problems (constipation or diarrhea);
• dull aching pain in the right hypochondrium. They can occur due to errors in diet, after abuse of spicy and fatty foods;
• liver enlargement may occur.

Benign hyperbilirubinemia: treatment

If there are no concomitant diseases of the gastrointestinal tract, during the period of remission the doctor prescribes diet No. 15. In acute periods, if there are concomitant diseases of the gallbladder, diet No. 5 is prescribed. No special liver therapy is required for patients.

Vitamin therapy and the use of choleretic agents are useful in these cases. Patients do not need special spa treatments.

Electrical or thermal procedures in the liver area will not only bring no benefit, but will also have a harmful effect. The prognosis of the disease is quite favorable.

Patients remain able to work, but it is necessary to reduce nervous and physical stress.

Benign Gilbert's hyperbilirubinemia also does not require special treatment. Patients should follow some recommendations to avoid exacerbation of the disease.

Table No. 5.

• Allowed for consumption: weak tea, compote, low-fat cottage cheese, wheat bread, vegetable broth soup, lean beef, crumbly porridge, chicken, non-acidic fruits.
• Prohibited consumption: lard, fresh baked goods, spinach, sorrel, fatty meat, mustard, fatty fish, ice cream, pepper, alcohol, black coffee.
• Compliance with the regime - heavy physical activity is completely excluded. The use of prescribed medications: anticonvulsants, antibiotics, anabolic steroids, if necessary - analogues of sex hormones, which are used to treat hormonal imbalances, as well as by athletes - to improve athletic performance.
• Completely stop smoking and drinking alcohol.

If symptoms of jaundice occur, your doctor may prescribe a number of medications.

• The group of barbiturates - antiepileptic drugs effectively reduce bilirubin levels.
• Choleretic agents.
• Drugs that affect the functions of the gallbladder and its ducts. Prevents the development of cholelithiasis and cholecystitis.
• Hepatoprotectors (protective agents that protect liver cells from damage).
• Enterosorbents. Drugs that enhance the excretion of bilirubin from the intestines.
• Digestive enzymes are prescribed for dyspeptic disorders (vomiting, nausea, gas formation) to aid digestion.
• Phototherapy - exposure to light from blue lamps leads to the destruction of fixed bilirubin in the tissues. To prevent eye burns, eye protection is necessary.

When following these recommendations, patients have normal bilirubin levels, and symptoms of the disease appear much less frequently.

Source: https://FB.ru/article/314886/dobrokachestvennaya-giperbilirubinemiya-diagnostika-i-lechenie

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.

Definition of benign hyperbilirubinemia

In its essence, bilirubin is a bile pigment; it has a characteristic red-yellow color. This substance is produced from red blood cells in hemoglobin, which break down due to involutive changes in the cells of the liver, spleen, connective tissues and bone marrow.

Benign hyperbilirubinemia is an independent disease that includes pigmentary hepatosis, familial simple cholemia, intermittent juvenile jaundice, non-hemolytic familial jaundice, constitutional dysfunction of the liver, retention jaundice and functional hyperbilirubinemia. The disease manifests itself as intermittent or chronic jaundice, obvious disturbances in liver function and its structure without pronounced disturbances. There are no obvious symptoms of cholestasis and increased hemolysis.

Benign hyperbilirubinemia (ICD 10 code: E 80 - general disorders of bilirubin and porphin metabolism) also has the following codes E 80.4, E 80.5, E 80.6, E 80. Coded accordingly: Gilbert syndrome, Crigler syndrome, other disorders - Dubin-Johnson syndrome and Rotor syndromes, an unspecified disorder of bilirubin metabolism.

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 infants 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.

Benign hyperbilirubinemia – Gilbert's syndrome

This hereditary disease is the most common, we will tell you more about it. The disease is transmitted from parents to children and is associated with a gene defect that is involved in the metabolism of bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) is nothing more than Gilbert's syndrome.

Bilirubin is one of the important bile pigments, an intermediate product of the breakdown of hemoglobin, which is involved in the transport of oxygen.

An increase in the level of bilirubin (by 80-100 µmol/l), a significant predominance of bilirubin not associated with blood proteins (indirect) leads to periodic manifestations of jaundice (mucous membranes, sclera, skin). At the same time, liver tests and other indicators remain normal. In men, Gilbert's syndrome is 2-3 times more common than in women. It may first appear between the ages of three and thirteen years. Often, the disease accompanies a person throughout his life. Gilbert's syndrome includes enzymopathic benign hyperbilirubinemia (pigmented hepatosis). They arise, as a rule, due to the indirect (unconjugated) fraction of bile pigments. This is due to genetic liver defects. The course is benign - there are no gross changes in the liver or pronounced hemolysis.

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.

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.

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.

Increased bilirubin during gestation

During pregnancy, hyperbilirubinemia develops as a result of:

• repeated vomiting;
• intrahepatic cholestasis (impaired bile synthesis);
• preeclampsia and eclampsia;
• fatty liver in acute form (a pathology found only in pregnant women).

As a rule, jaundice of pregnant women occurs towards the end of pregnancy and goes away a couple of weeks after birth. But any case of yellowing of the sclera, skin or change in the color of urine and feces in a pregnant woman should prompt an immediate visit to the doctor.

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 is understood as an increased level of bilirubin in the blood serum, a yellow-red substance that is one of the pigments of bile. Bilirubin is a derivative of red blood cell hemoglobin, which breaks down in the cells of the spleen, bone marrow and liver, as well as in the connective tissues of organs.

Hyperbilirubinemia - can cause hypovitaminosis, liver failure, cholelithiasis or provoke inflammation of the gallbladder.

How does hyperbilirubinemia manifest, treatment, what are its causes? Let's talk about this in more detail:

Main forms of pathological condition

There are three main forms of hyperbilirubinemia:

Suprahepatic - occurs due to increased formation of bilirubin in the blood.

Hepatic - develops due to damage to liver tissue.

Subhepatic - causes blockage of the ducts with difficulty passing bile into the intestines.

Why does hyperbilirubinemia occur? Causes of pathology

The main reasons for the development of this condition are usually called: intense hemolysis, long-term use of medications, exposure to toxic substances.

An increase in indirect bilirubin “unconjugated hyperbilirubinemia” is usually diagnosed in infants with hemolytic disease, as well as due to hereditary or later acquired hemolytic anemia. An increase in indirect bilirubin can also be caused by poisoning of the body with toxins, poisons, or mechanical damage to red blood cells. Some liver diseases may also be the cause.

An increase in direct bilirubin “conjugative hyperbilirubinemia” occurs due to impaired, ineffective removal of bilirubin from the body. This form is observed in hepatitis (its various forms), liver cirrhosis, cholelithiasis. An increase in the level of direct bilirubin is facilitated by long-term use of certain contraceptive hormonal drugs and frequent use of steroids (which is practiced, in particular, by athletes).

How is hyperbilirubinemia recognized? Symptoms

In the initial stages, with a mild form of the pathological condition, the normal functioning of the central nervous system may be disrupted, which is expressed in some lethargy, general weakness, and lethargy. A person gets tired quickly and is easily overtired.

In mild forms, liver enlargement is not observed; upon palpation it is painless. Mild forms can be effectively treated while maintaining the patient’s normal performance.

Characteristic symptoms include jaundice - a yellowish tint to the skin, mucous membranes, and whites of the eyes. Jaundice increases with overwork and stress.

When the disease worsens, which often happens after drinking alcohol or eating disorders, hyperbilirubinemia makes itself felt by pain in the right hypochondrium, weakness, nausea, and a feeling of bitterness in the mouth. Dark color of urine and stool may be observed.

How is hyperbilirubinemia corrected? Treatment

If you suspect the occurrence of this pathological condition, you should consult a doctor and undergo the necessary examination. If an elevated level of bilirubin in the blood serum is confirmed, the doctor will prescribe the necessary treatment. Its goal is to reduce bilirubin and improve the patient's condition.

If the main cause of hyperbilirubinemia is bacterial or viral in nature, antibacterial or antiviral drugs are prescribed.

To maintain the vital activity of liver cells, drugs are prescribed - hepatoprotectors.

In addition, choleretic agents are prescribed to enhance the secretion of bile.

To enhance immunity, immunomodulators are prescribed. These drugs help the immune system fight pathology. If there is liver inflammation, the doctor will prescribe an anti-inflammatory drug. Patients take medications from the barbiturate group, designed to reduce the level of bilirubin in the blood.

Also used in treatment: antioxidants - to eliminate and remove poisonous substances and toxins from the body. Enterosorbents are taken to activate the removal of bilirubin from the intestines.

In addition to medications, the patient may be prescribed phototherapy sessions. In this case, irradiation with light from blue lamps is carried out, which helps to destroy bilirubin accumulated in the tissues of the body.

The patient is recommended to review the diet, excluding spicy, salty, smoked foods. You should avoid fatty, fried foods. In case of development of hepatic encephalopathy, limit the consumption of protein foods to 40 g per day. Alcohol is strictly contraindicated.

Hyperbilirubinemia, which is hereditary, does not have any particular negative impact on health. The patient only needs to follow a gentle diet, do not drink alcohol, and avoid fatty, salty and spicy foods. Physical activity is contraindicated. Be healthy!

Hyperbilirubinemia is a disease characterized by an increase in the volume of bilirubin in the blood. Often, such a violation causes serious deviations in the functioning of internal organs. Experts believe that hyperbilirubinemia can lead to the development of cholecystitis or.

In the initial stages, the disease does not require specific treatment, but if the symptoms are ignored for a long time and the doctor’s recommendations are not followed, serious deviations in the functioning of the gastrointestinal tract can occur.

Hyperbilirubinemia is a disease that does not develop overnight. A person can treat individual symptoms for years without suspecting the presence of a serious deviation in the functioning of his body.

The following symptoms usually help diagnose this disease:

:

Causes of the disease

The causes of hyperbilirubinemia depend on the form of the disease. Typically, the adrenal type of such damage occurs against the background of active destruction of hemoglobin and red blood cells, which are processed in the kidneys. Because of this, the chemical composition of the blood changes, which leads to disturbances in the liver.

This condition may occur due to the following reasons:

:

With hepatic hyperbilirubinemia, the lesions become more extensive. The functioning of not only the liver, but also the entire gastrointestinal tract is disrupted. The person begins to fade before our eyes, he needs immediate medical assistance.

The following reasons can provoke hepatic hyperbilirubinemia:

:

Hyperbilirubinemia during pregnancy

Pregnancy is a difficult and important stage in the life of any woman. At this time, the immune system becomes as vulnerable as possible; it may not work at all. Because of this, pregnant women are often diagnosed with various viral and infectious diseases, and chronic ailments become aggravated. Hyperbilirubinemia was no exception. This disease occurs quite often during pregnancy.

Pregnant women are most often diagnosed with a transient, functional or conjugative form of hyperbilirubinemia. However, treating doctors classify this disease into two separate groups:

• Triggered by factors beyond the control of the pregnant woman: infectious, viral processes and many others.
• Arose due to changes associated with gestation.

The first type of hyperbilirubinemia occurs against the background of serious damage to the body. It is usually accompanied by severe pain in the right hypochondrium, increased body temperature, and jaundice. All this leads to serious complications.

For this reason, it requires immediate medical treatment. The second type is formed against the background of fatty degeneration, early or late toxicosis.

Hyperbilirubinemia in newborns

After birth, the body of a newborn child undergoes serious changes - in a short period of time it needs to completely adapt to a new way. Now the mother’s body does not protect him from the influence of the external environment.

Immediately after birth, newborns are diagnosed with exorbitant bilirubin levels, which begin to fall a few days later. This phenomenon is commonly called benign physiological jaundice.

If the child feels normal, his internal organs are functioning, then no treatment is required. In the event of an acute disorder, the specialist must prescribe complex drug treatment. The mechanism for the development of bilirubinemia in children is the rapid breakdown of blood cells. At the same time, enzymatic reactions slow down. Due to the low level of albumin in the blood, bilirubin begins to be freely released from the liver tissue.

Hyperbilirubinemia in newborns occurs due to a conflict of the Rh factor or incompatibility of blood groups between the child and the mother. In this case, the female body perceives the fetus as a foreign body. He may also perceive the child as a pathogenic creature. It is very important to consult a hematologist.

Diet food

Dietary nutrition is an integral part of any treatment of the liver or gastrointestinal tract. With hyperbilirubinemia, the liver cannot function normally and process all wastes and toxins. Because of this, it is very important to review your diet, which will also help reduce bilirubin levels and improve blood chemistry.

First of all, the patient must stop eating harmful foods. All dishes must be prepared without adding salt or spices.

It is strictly forbidden to consume baked goods and any flour products. You should also avoid dishes that have been prepared with baking soda or baking powder. Remember that people with hyperbilirubinemia need to eat often, but in small portions.

Alcoholic drinks and soda are completely prohibited. Remember to drink clean water, at least 2 liters per day. It is very important to constantly follow a diet.

The best way to prepare food is by baking or boiling. This way you will be able to retain the maximum amount of nutrients .

Treatment of hyperbilirubinemia

If a person is diagnosed with high levels of bilirubin in the blood, he needs to immediately visit a general practitioner. After assessing the results obtained, the specialist should send the patient for a more detailed study. It should include a thorough examination, history taking, and ultrasound examination of the abdominal cavity.

Only by assessing the condition of the internal organs will the doctor be able to determine the true cause of hyperbilirubinemia and determine the most appropriate and effective treatment.

Phototherapy
If the cause of this condition lies in hemolytic anemia, then you will definitely be sent to a hematologist. It is very important to visit this doctor during pregnancy, since hyperbilirubinemia can cause serious complications for both the mother and her unborn child.

Typically, complex treatment of hyperbilirubinemia should meet the following principles

:

• In addition to drug therapy, it is very important to conduct complex phototherapy. It involves irradiating the skin with blue lamps. With their help, it is possible to destroy part of the bilirubin even through the skin.
• It is necessary to take a course of immunomodulators, which will increase the body's protective functions.
• If hyperbilirubinemia is caused by a viral or bacterial infection of the body, then it is necessary to carry out complex therapy with antibacterial and antiviral drugs.
• To reduce the negative impact on the gastrointestinal tract, it is very important to take choleretic drugs.
• If there is an inflammatory process in the liver, then it is very important to take anti-inflammatory drugs.
• To cleanse the body of waste and toxins, you need to take a course of antioxidants.

Surgical and drug treatment of hyperbilirubinemia

Surgical treatment of hyperbilirubinemia (according to clinical protocols) is necessary when a stone is detected in the bile duct or a tumor of the pancreas or duodenum. Drainage (restoration of patency) is less often necessary when the bile ducts are narrowed.

If there is no effect of drugs for hemolytic anemia, the spleen is removed. All other forms of high bilirubin are treated with medications.

Adults

Treatment of an adult with high bilirubin takes place in a hospital (in the gastroenterology or infectious diseases department), less often at home. Can be assigned:

• for hemolysis of red blood cells - hormones (Prednisolone), cytostatics (Cyclosporine), immunoglobulin intravenously, droppers with solutions, folic acid;
• for viral hepatitis - antiviral (Zeffix, Ribavirin), hepatoprotectors (Essentiale, Heptral), facilitating the excretion of bile (Ursohol, Gepabene);
• for stagnation of bile - Allohol, Cholenzym, Ursofalk, choleretic teas with chamomile, calendula, yarrow;
• for hereditary diseases - Phenobarbital short course.

Newborns, children

The most effective method of reducing bilirubin in newborns is phototherapy; it is used in children with physiological jaundice as the main method of treatment. After irradiating the skin with blue light, the toxic pigment becomes soluble and is excreted from the body by the kidneys.

If the result is insufficient, an exchange blood transfusion is performed, and hardware methods of purification are performed - plasmapheresis, hemosorption. Phenobarbital may be prescribed.

Also recommended:

• applying to the breast at least 8-12 times without a night break;
• Give the baby water as often as possible between feedings;
• droppers with glucose solutions, saline, adding vitamins B, C;
• Smecta for the absorption of bilirubin in the intestines;
• Duphalac, Hilak forte to improve intestinal digestion.

Phototherapy

Traditional methods

With increased bilirubin due to stagnation of bile and Gilbert's syndrome, teas with choleretic herbs are indicated. For example, the following plants may be recommended:

• mint and birch leaves;
• chamomile, immortelle, calendula flowers;
• chicory root, dandelion, elecampane;
• herb St. John's wort, yarrow.

To prepare, crushed raw materials (a tablespoon) are poured into 300 ml of boiling water and brewed in a thermos for 8 hours (left overnight). Take 100 ml 3 times a day half an hour before meals. The course of treatment is 30 days, then a break is taken for 2 weeks and the medicinal plant is changed.

Diet

Regardless of the reason for the increase in bilirubin in the blood, it is important to create maximum relief for the liver and biliary system. To do this you need:

• frequent meals (5-6 times) in portions of no more than 300 g;
• exclude: alcohol, fatty foods, baked goods, fresh bread, broths, canned food, mushrooms, smoked meats, cakes, legumes, spices, lard, sour fruits and berries;
• use boiling, baking, stewing for cooking;
• include in the diet dried bread, cereal porridge, vegetable soups and side dishes, lean meat and fish, protein omelet, cottage cheese 2-5%, fresh fermented milk drinks;
• drink enough water; permitted drinks include weak tea, fruit juice, compote, and rosehip decoction.

Video

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