Benign Hyperbilirubinemia: Diagnosis and Treatment

Yellowing of the mucous membranes, eye sclera, and also the skin should alert any person. Everyone knows that such symptoms point to certain violations in the work of such an important organ as the liver. These ailments must necessarily be controlled by a doctor. He will put the correct diagnosis and prescribe the necessary treatment. When the level of bilirubin increases, as a rule, jaundice appears. Benign hyperbilirubinemia also has similar symptoms. In the article we will analyze in more detail, what kind of illness is it, 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 erythrocytes in hemoglobin, which decay due to involute changes in the cells of the liver, spleen, connective tissue and bone marrow.

Benign hyperbilirubinemia is an independent disease, it includes pigmentary hepatosis, simple family history, juvenile jaundice intermittent, nonhemolytic family jaundice, constitutional liver dysfunctions, retention jaundice and functional hyperbilirubinemia. The disease manifests itself as intermittent or chronic jaundice, obvious violations of the liver function and its structure without pronounced disorders. There are no obvious symptoms of cholestasis and elevated hemolysis.

Benign hyperbilirubinemia (ICD code 10: E 80 - common disorders of bilirubin and porphin exchange) also has the following codes E 80.4, E 80.5, E 80.6, E 80. Accordingly, Gilbert syndromes, Kriegler syndromes, and other disorders - Dubin-Johnson syndromes And Rotor syndromes, an unspecified disorder of bilirubin metabolism.

Causes

Benign hyperbilirubinemia in adults in most cases are diseases that are of a family nature, they are transmitted by a dominant type. This is confirmed by medical practice.

There is post-hepatitis hyperbilirubinemia - occurs as a consequence of viral acute hepatitis. Also, the cause of the disease can serve as a transferred infectious mononucleosis, after recovery in patients there may be symptoms of hyperbilirubinemia.

The cause of the disease is a failure in the exchange of bilirubin. Serum increases this substance, or there is a violation of the capture or transfer of it into the liver cells from the plasma.

Such a state is also possible in cases when there is a disruption of the binding processes of bilirubin and glucuronic acid, it can be explained by the permanent or temporary deficiency of an enzyme such as glucuronyltransferase.
These mechanisms of hyperbilirubinemia characterize the syndromes of Gilbert, Kriegler-Nayyar and post-hepatitis hyperbilirubinemia. With Rotor and Dabin-Johnson syndromes, serum bilirubin is increased due to a violation of pigment excretion into the bile ducts through the membranes of the hepatocyte.

The provoking factors

Benign hyperbilirubinemia, the diagnosis of which confirms the fact that most often it is detected in adolescence, can show its signs for many years and even a lifetime. In men, this ailment is detected much more often than in women.

Classical manifestation of the disease - yellowing of the sclera, icteric skin color can appear in some cases, not always. The manifestations of hyperbilirubinemia are often intermittent in nature, in rare cases are permanent, not passing.

Intensification of icterus can be triggered by the following factors:

• Marked physical or nervous overwork;
• Exacerbation of infections, damage to the biliary tract;
• Immunity of medicines;
• colds;
• Various surgical interventions;
• Reception of alcohol.

Symptoms of the disease

In addition, that sclera and skin are colored yellowish, the patients feel heaviness in the right hypochondrium. There are cases when disturbing dyspeptic phenomena - it's nausea, vomiting, stool, lack of appetite, increased gassing in the intestine.

Manifestations of hyperbilirubinemia can lead to the appearance of asthenovegetative disorders, which manifest themselves as depression, weakness, rapid fatigue.
On examination, the doctor first of all pays attention to the yellowed sclera and a dull yellow skin of the patient. In some cases, the skin does not turn yellow. The liver is palpable along the edges of the costal arch, or it may not be felt. There is some increase in the size of the body, the liver becomes soft, the patient experiences painful sensations when palpating. The spleen does not increase in size. Exceptions are cases where benign hyperbilirubinemia occurs as a consequence of hepatitis. Post-hepatitis hyperbilirubinemia may also occur after a previous infectious disease - mononucleosis.

Syndrome of benign hyperbilirubinemia

To benign hyperbilirubinemia in medical practice include seven congenital syndromes:

• Kriegler-Nayyar type 1 and type 2 syndromes;
• Dabin-Johnson Syndromes;
• Gilbert's syndromes;
• Rotor Syndromes;
• Illness of Byler (rare);
• Lucy-Driscoll Syndrome (rare);
• Family benign age cholestasis - benign hyperbilirubinemia (rare).

All these syndromes occur in connection with 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 an enormous role in the body, highly toxic bilirubin is processed into a low-toxic, diglucoronide-soluble compound (conjugated bilirubin). The free form of bilirubin easily penetrates into the elastic tissues, lingers in the mucous membranes, skin, on the walls of the blood vessels, and causes jaundice.

The Kriegler-Nayar Syndrome

American pediatricians V. Nayyar and J. Kriegler in 1952 revealed a new syndrome and described it in detail. It was called type 1 Kriegler-Nayyar syndrome. This congenital pathology has an autosomal-referential type of inheritance. The development of the syndrome occurs immediately in the first hours after the birth of the child. There are similar symptoms of the same frequency in both girls and boys.

The pathogenesis of the disease is due to the complete absence of such an enzyme as UDFGT (enzyme urndine-5-diphosphate-glucuronyltransferase). At 1 type of this syndrome UDFGT is absent completely, free bilirubin sharply increases, the indices reach 200 μmol / l and even more. After birth in the first day, the permeability of the hemato-encephalic barrier is high. In the brain (gray matter) there is a rapid accumulation of pigment, develops yellow nuclear jaundice. A trial with cordyamine in the diagnosis of benign hyperbilirubinemia, with phenobarbital - is negative.

Encephalopathy bilirubin 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, a fatal outcome is possible already in the first 24 hours. The liver does not change during autopsy.

Dabin-Johnson Syndrome

The syndrome of benign hyperbilirubinemia Dabin-Johnson was first described in 1954. Mostly this disease is common among the inhabitants of the Middle East. In men under the age of 25, it occurs in 0.2-1% of cases. Inheritance occurs in an autosomal dominant type. This syndrome has a pathogenesis, which is associated with disruption of the transport functions of bilirubin in the hepatocyte, and also because of the insolvency of the transport ATP-dependent membrane cell system. As a consequence, the intake of bilirubin in bile is disrupted, there is a reflux of bilirubin into the blood from the hepatocyte. This is confirmed by the peak concentration in the blood of the dye after two hours during the sampling using bromsulfalein.

Morphological characteristic feature is the liver of chocolate color, where the accumulation of coarse grainy pigment is high. Manifestations of the syndrome: persistent jaundice, occasional 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 contraceptive hormonal agents, as well as during pregnancy.

Diagnosis of the disease on the basis of bromsulfaleinovoy test, with cholecystography with delayed excretion in the bile contrast medium, in the absence of contrast in the gallbladder. Cordiamin in the diagnosis of benign hyperbilirubinemia in this case is not used.

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

Treatment of this syndrome is not developed. The duration of life does not affect the syndrome, but the quality of life in this pathology worsens.

Benign hyperbilirubinemia - Gilbert's syndrome

This hereditary disease occurs most often, we will tell more about it. The disease is transmitted from parents to children, is associated with a defect in the gene 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 unrelated blood (indirect) bilirubin leads to periodic manifestations of jaundice (mucous membranes, sclera, skin). At the same time, liver tests, the remaining parameters remain normal. In men, Gilbert's syndrome is 2-3 times more common than in women. For the first time can appear at the age of three to thirteen years. Often an ailment accompanies a person all his life.
Gilbert's syndrome includes fermentopathic benign hyperbilirubinemia (pigmentary hepatosis). They arise, as a rule, due to the indirect (unconjugated) fraction of bile pigments. This is due to genetic defects in the liver. The flow of benign - gross changes in the liver, expressed hemolysis does not occur.

Symptoms of Gilbert's Syndrome

Gilbert's syndrome has no pronounced symptoms, proceeds with minimal manifestations. Some physicians do not view the syndrome as a disease, but refer it to the physiological characteristics of the body.

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

• weakness;
• dizziness;
• Increased fatigue;
• Sleep disorders;
• insomnia.

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

• Absence or loss of appetite;
• A bitter eructation after eating;
• heartburn;
• Bitter taste in the mouth; Rarely vomiting, nausea;
• Feeling of heaviness, fullness of the stomach;
• Disorders of stool (constipation or diarrhea);
• Pain in the right hypochondrium of a dull aching character. Can arise with errors in the diet, after the abuse of acute, as well as fatty foods;
• There may be an increase in the liver.

Benign hyperbilirubinemia: treatment

If there are no concomitant diseases of the gastrointestinal tract, during the remission period, the doctor appoints a diet number 15. In acute periods, if there are concomitant diseases of the gallbladder, diet No. 5 is prescribed. Special hepatic therapy for patients is not required.

It is useful in these cases, vitamin therapy, the use of cholagogue funds. Do not need patients in special spa treatments.
Electrical or thermal procedures in the liver region will not only not be of use, but also have a harmful effect. The prognosis of the disease is quite favorable. Patients remain functional, but need to reduce nervous, as well as physical exertion.

Benign hyperbilirubinemia Gilbert also does not require special treatment. Patients should follow certain recommendations so as not to aggravate the manifestations of the disease.

Table number 5.

• Allowed for use: weak tea, compote, low-fat cottage cheese, wheat bread, soup on vegetable broths, low-fat beef, crumbly porridge, chicken, non-acid fruit.
• It is forbidden to eat: bacon, fresh baking, spinach, sorrel, fat meat, mustard, fatty fish, ice cream, pepper, alcohol, black coffee.
• Compliance with the regime - heavy physical exertion is completely excluded. The use of prescribed medications: anticonvulsants, antibiotics, anabolic steroids if necessary - analogues of sex hormones that are used to treat hormonal failures, as well as athletes - to improve athletic performance.
• Completely refrain from smoking, drinking alcohol.

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

• A group of barbiturates - antiepileptic drugs effectively reduce the level of bilirubin.
• Cholagogue means.
• Means that affect the function of the gallbladder, as well as its ducts. Prevent the development of cholelithiasis, cholecystitis.
• Hepatoprotectors (protective agents, protect liver cells from damage).
• Enterosorbents. Means, enhancing the excretion of bilirubin from the intestine.
• Digestive enzymes are prescribed for diarrheal disorders (vomiting, nausea, gas formation) - aid digestion.
• Phototherapy - exposure to light from blue lamps leads to destruction in tissues of fixed bilirubin. To prevent burns to the eyes, their protection is necessary.

When these recommendations are followed, patients have a normal level of bilirubin, the symptoms of the disease are manifested much less often.