Hyperbilirubinemia is a pathological change in blood serum, which is manifested in an increase in the bilirubin concentration, which is the result of the increased breakdown of hemoglobin.

Causes of hyperbilirubinemia

The syndrome of hyperbilirubinemia develops most often in one of the two main pathogenetic mechanisms. The first mechanism is triggered by the process of excessive synthesis of bilirubin, which is observed in the acute instantaneous massive destruction of erythrocyte blood cells. According to the second etiopathogenetic theory of the development of hyperbilirubinemia, there are a number of pathological conditions of the organism, accompanied by a violation of metabolic transformations of bilirubin in the liver and elimination of its decay products.

Thus, the increase of one or the other bilirubin fraction allows one to assume the etiopathogenetic factors of the development of hyperbilirubinemia.

The basis of etiopathogenetic classification of all forms of hyperbilirubinemia is based on the principle of determining the bilirubin fraction, which increases the total serum bilirubin. Thus, conjugation hyperbilirubinemia is manifested by an increased concentration of the direct bilirubin fraction, provoked by a violation of bilirubin elimination from the body. To develop this type of pathological changes, it is necessary for the patient to have any organic changes in the structure of the hepato-biliary system (the presence of concrements in the lumen of the bile ducts, diffuse and nodal lesions of the liver parenchyma), and the prolonged intake of drugs from the hormone group.

In a situation in which the body undergoes enhanced hemolysis of erythrocyte blood cells, accompanied by an increased synthesis of the indirect fraction of bilirubin, the conclusion is "hyperbilirubinemia of the unconjugated type." These changes are most often observed in the practice of neonatologists in hemolytic anemia of the neonatal period, and in the adult category of patients this type of hyperbilirubinemia is provoked by the toxic effect of toxic substances.

Symptoms of hyperbilirubinemia

The most common manifestations of an increase in the bilirubin level in the blood is the damage to the skin as a change in the color of the skin to a lemon-yellow hue. However, all mucous membranes of the oral cavity, conjunctiva and ocular proteins are primarily affected, which acquire a pronounced yellow color. The above symptoms occur only with a significant increase in bilirubin. In a situation where the patient's hyperbilirubinemia is provoked by the pathology of the liver and the organs of the biliary system, in addition to changing the color of the skin, the patient notes the marked itching of the altered skin, especially at night.

An increase in the concentration of bilirubin and the products of its metabolic transformation has a toxic effect on the structures of the central nervous system, in connection with which, in almost 100% of cases, hyperbilirubinemia is accompanied by symptoms of the asthenovegetative symptom complex in the form of increased fatigue, the inability to perform habitual physical activity, drowsiness, and in severe cases even various degrees of impaired consciousness.

Patients with a hepatic variant of hyperbilirubinemia in addition to pathognomonic complaints testifying to the increase of bilirubin concentration note the constant presence of symptoms of the main background liver disease - heaviness and discomfort in the projection of the right hypochondrium, a bitter taste in the oral cavity, constant heartburn and nausea, and during an exacerbation indomitable vomiting.

The subhepatic version of hyperbilirubinemia also has characteristic clinical features in the form of a pronounced astheno-neurotic syndrome, a characteristic change in stool that changes not only the color but also the consistency towards the liquid, with the presence of a large amount of fat.

All clinical and pathomorphological variants of hyperbilirubinemia are accompanied by typical darkening of urine and clarification of stool, which are more observed in hepatic and subhepatic type of hyperbilirubinemia.

Hyperbilirubinemia in newborns

The neonatal period is critical for the development of signs of benign forms of hyperbilirubinemia, which are provoked by genetic enzymopathies. Due to the fact that an increase in bilirubin concentration in a newborn child is not accompanied by structural changes and functional disorders of the liver, as well as the child completely lacks clinical manifestations of cholestasis and hemolysis, these changes are interpreted as "functional hyperbilirubinemia".

ICD-10 classifies all hereditary forms of functional hyperbilirubinemia into several variants.

A separate category of hyperbilirubinemia is the so-called "physiological variant", which is observed in all children in the period of newborn and does not cause painful sensations and pathological changes in the state of health. Despite the favorable transitory course, all children who are in the period of newborns are subject to control of bilirubin indices, for which a modern analyzer of hyperbilirubinemia is currently used, which makes it possible to determine the level of various bilirubin fractions in the shortest possible time. Screening of children allows to exclude the possibility of developing a severe degree of hyperbilirubinemia, which in most cases provokes the emergence of encephalopathic manifestations.

The debut of the clinical manifestations of hyperbilirubinemia falls on the second-third day after the birth of the child, and self-leveling no more than a month. This form of hyperbilirubinemia is characterized by a gradual icteric staining of the skin, from the head to the lower limbs. Symptoms of the so-called nuclear jaundice appear only in the case of a critical increase in bilirubin concentration, which results in the development of signs of damage to the structures of the central nervous system.

An alarming symptom that indicates the progression of hyperbilirubinemia is the unmotivated lethargy and apathy of the child, a decrease in interest in feeding and a sluggish act of sucking. Attachment of stiff neck and opisthotonus stiffness testifies to the development of severe damage to the cerebral membranes, which leads to death in the absence of emergency medication. Symptoms of irreversible damage to the central nervous system of a newborn child is a deep degree of impairment of consciousness in the form of a lack of response to light, pain and temperature stimuli.

The favorable course of hyperbilirubinemia, observed in the majority of healthy full-term children, develops as a consequence of the imperfection of enzymatic systems that do not allow the rapid elimination of fetal hemoglobin degradation products. Currently, pediatric practitioners note another feature of the course of hyperbilirubinemia in newborns, consisting in a progressive increase in bilirubin after breastfeeding. However, despite this pattern, hyperbilirubinemia is not an indication for interrupting breastfeeding.

In a situation where high bilirubin levels in the blood of a newborn child are not accompanied by a serious health disorder, the use of any medical measures is considered unreasonable. A significant concentration of bilirubin can be treated with ultraviolet baths, which help to eliminate bilirubin. A critical increase in bilirubin content exceeding 120 μmol / L is an absolute indication for the use of a replacement blood transfusion.

Recent scientific observations indicate a significant increase in the incidence of hyperbilirubinemia in children with birth trauma, accompanied by the development of a cephalohematoma. The increased concentration of bilirubin in this situation develops as a result of massive destruction of red blood cells, which are part of the hematoma. This form of hyperbilirubinemia is accompanied by a high concentration of the indirect fraction and is subject to correction with the help of a replacement transfusion of erythrocyte mass.

Treatment of hyperbilirubinemia

Hyperbilirubinemia refers to the category of pathological conditions, the treatment of which must be etiologically justified, that is, the key to recovery is the elimination of the background disease.

In a situation where manifestations of hyperbilirubinemia develop in humans for the first time, it is necessary to conduct a full examination and treatment in a hospital of the gastroenterological profile. Only benign hyperbilirubinemia does not need a medical correction and in most cases is leveled if the patient complies with the basic recommendations for correcting eating behavior. In addition to the correction of nutrition for patients with hyperbilirubinemia, due to the pathology of the hepatobiliary system, it is necessary to exclude the fact of the influence of physical and psycho-emotional overloads of the organism.

Medicamental treatment of hyperbilirubinemia of any clinical form is divided into the following categories: etiopathogenetic, symptomatic and preventive.

Due to the fact that the products of the metabolic transformation of bilirubin have a toxic effect on all structures of the human body, especially the central nervous system, it is first necessary to conduct adequate disintoxication therapy with the use of antioxidant drugs (Cystamine in a daily dose of 0.2 g, Tocopherol 50 mg per day orally). In a situation where the patient has a critical concentration of bilirubin and there are signs of toxic brain damage in the form of signs of encephalopathy, it is necessary to carry out parenteral detoxification therapy in the form of a combination of Insulin at a dose of 4 U subcutaneously with 40 ml of 40% Glucose intravenously.

If the immune-inflammatory variant of hyperbilirubinemia is diagnosed as a result of massive diffuse damage of the hepatic parenchyma, it is advisable to use a short course of glucocorticosteroid therapy (Prednisolone in a daily dose of 30 mg orally for two weeks). Due to the fact that parenchymal hyperbilirubinemia in most cases is accompanied by severe hemorrhagic complications, all patients of this category are recommended to use for prophylactic purposes Vikasol in a daily dose of 0.015 g orally or intramuscularly.

To relieve the itching that often accompanies the cholestatic variant of jaundice, external agents are used - wiping with camphor alcohol and acetic baths. To reduce the concentration of bile acids in the blood serum should be used drugs, whose effect is directed to the binding of bile acids in the small intestine (Cholestyramine in a daily dose of 2 g). In addition, the method of duodenal sounding and the use of choleretic preparations (Holosas in a daily dose of 15 ml orally) also have a good effect, provided there are no signs of a mechanical type of jaundice. In a situation where the emergence of an increased concentration of bilirubin is observed as a result of mechanical obturation of the lumen of the biliary tract, the only option is surgical removal of the defect (cholecystectomy by laparoscopic access).