Diseases of the blood



hyperbilirubinemia - a pathological change in serum, which manifests itself in increasing the concentration of bilirubin, arising as a consequence of the enhanced breakdown of hemoglobin.

Causes of hyperbilirubinemia

hyperbilirubinemia syndrome develops most often in one of the two main pathogenic mechanisms.The first mechanism is triggered synthesis process of excess bilirubin, which is observed in acute single-step destruction of the bulk of erythrocyte blood cells.According to the second etiopathogenetic theory of hyperbilirubinemia, there are a number of pathological conditions associated with impaired metabolic transformation of bilirubin in the liver and elimination of its decay products.

thus increasing varying bilirubin fraction suggests etiopathogenetic factors of hyperbilirubinemia.

based etiopathogenetic classification of all forms of hyperbilirubinemia on the principle of determining the bilirubin fractions due to the increased rate of total serum biliru

bin.Thus, conjugation hyperbilirubinemia manifests the high concentration of the direct bilirubin fraction provoked a violation elimination of bilirubin from the body.For the development of this type of lesions in a patient must have any organic structure changes hepatobiliary system (presence of concrements in the lumen of the biliary tract, diffuse and nodular lesions of the liver parenchyma) continuously taking hormonal drugs group number.

In a situation where the body is enhanced erythrocyte hemolysis of blood cells, accompanied by an increase synthesizing indirect bilirubin, the conclusion set "unconjugated hyperbilirubinemia type."These changes often occur in practice neonatologists in hemolytic anemia neonatal period, and the adult patient population, this type of hyperbilirubinemia triggered toxic effects of toxic substances.

symptoms of hyperbilirubinemia

The most common manifestations of increased rates of bilirubin in the blood is damage to the skin in the form of discoloration on the skin lemon-yellow tint.However, primarily affects all the mucous membranes of the mouth, conjunctiva and eye proteins that take on a distinct yellow color.The above symptoms occur only with a significant increase in bilirubin.In a situation where a patient triggered hyperbilirubinemia liver disease and of the biliary system, in addition to the discoloration of the skin the patient noted marked changes in the skin itching, especially at night.

Increasing concentrations of bilirubin and products of its metabolic transformation has a toxic effect on the structure of the central nervous system, and therefore, almost 100% of hyperbilirubinemia accompanied by symptoms asthenovegetative symptom of fatigue, inability to perform usual physical activity, somnolence, and in severecases even varying degrees of impaired consciousness.

patients with hepatic option hyperbilirubinemia addition pathognomonic complaints testifying in favor of increasing the concentration of bilirubin, note the constant presence of the symptoms of the main background of liver disease - the severity and discomfort in the projection of right upper quadrant, feeling bitter taste in the mouth, persistent heartburn and nausea, and in the periodworsening uncontrollable vomiting.

subhepatic hyperbilirubinemia option also has the characteristic clinical features of a pronounced asthenoneurotic syndrome, characteristic changes of the chair, which changes not only the color but also the consistency in the direction of the liquid, with the presence of large amounts of fat.

All clinical and pathological variants of hyperbilirubinemia accompanied by a typical dark urine and feces lightening, which are more observed in the liver and subhepatic type hyperbilirubinemia.

hyperbilirubinemia in neonates

neonatal period is critical in relation to the development of symptoms of benign forms of hyperbilirubinemia, which provoked genetic enzimopaty.Due to the fact that the increase in the concentration of bilirubin in the newborn child is not accompanied by structural changes and functional disorders of the liver, as well as the child completely absent clinical manifestations of cholestasis and hemolysis, these changes are treated as "functional hyperbilirubinemia."

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

separate category hyperbilirubinemia is the so-called "normal variant", which is observed for all children in the neonatal period and does not cause pain and pathological changes in health status.Despite the favorable transient for all children in the neonatal period are subject to control parameters bilirubin, which currently applies a modern analyzer hyperbilirubinemia, allows you to quickly determine the level of the different fractions of bilirubin.Conducting screening children eliminates the possibility of developing severe hyperbilirubinemia, which in most cases provokes encephalopathic manifestations.

debut clinical manifestations hyperbilirubinemia occurs in the second or third day after birth, and self-leveled no more than a month.This form of hyperbilirubinemia is characterized by a gradual icteric staining of the skin, starting from the head and ending with the lower limbs.Symptoms of so-called kernicterus appear only in case of a critical increase in the concentration of bilirubin, resulting in the development of signs of lesions of the central nervous system structures.

alarming symptoms suggestive of progression of hyperbilirubinemia is nemotiviruemaya baby lethargy, decreased interest in feeding and sluggish act of sucking.Joining neck stiffness, opisthotonos, and favors the development of severe lesions of the brain membranes, which in the absence of immediate medical intervention is fatal.The symptoms of irreversible damage to the central nervous system of a newborn baby is a deep level of human consciousness as a lack of response to light, pain and temperature stimuli.

favorable for hyperbilirubinemia observed in the majority of healthy full-term babies, develops as a consequence of the imperfection of enzyme systems that do not allow to carry out fast elimination of the decay products of fetal hemoglobin.Currently practicing pediatricians noted another feature of the flow of hyperbilirubinemia in newborns, which consists in a progressive increase in the bilirubin after breastfeeding.However, despite this law, hyperbilirubinemia is not an indication for termination of breastfeeding.

In situations where high levels of bilirubin in the blood of a newborn baby are not accompanied by serious health disorder, the use of any therapeutic measures considered to be unfounded.A large concentration of bilirubin is well treated by ultraviolet baths assists in the elimination of bilirubin.Critical increase of bilirubin greater than 120 mmol / l, is an absolute indication for exchange transfusion.

Recent scientific observations noted a significant increase in the incidence of hyperbilirubinemia in infants with birth defects associated with the development cephalhaematoma.Increased bilirubin concentrations in this situation is caused by a massive destruction of red blood cells, which are composed of a hematoma.This form of hyperbilirubinemia is accompanied by a high concentration of indirect fraction and is subject to correction by means replace red blood cell transfusions.

treatment of hyperbilirubinemia

hyperbilirubinemia is classified as pathological conditions whose treatment should be etiologically valid, that is the key to recovery is to eliminate the underlying disease.

In situations where symptoms develop hyperbilirubinemia in humans for the first time, you need to have a complete medical examination and treatment in a hospital gastroenterological profile.Only benign hyperbilirubinemia does not require medical correction and in most cases leveled subject to the patient's main recommendations for the correction of eating behavior.In addition to the correction of nutrition in patients with hyperbilirubinemia due to pathology of the hepatobiliary system is necessary to eliminate the fact of the influence of physical and psycho-emotional overloads the body.

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

Due to the fact that the products of metabolic conversion of bilirubin have a toxic effect on all structures of the human body, especially the central nervous system, the priority must be to carry out adequate detoxication therapy using drugs group of antioxidants (Cystamine a daily dose of 0.2 g of tocopherol50 mg per day orally).In situations 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 conduct the parenteral disintoxication therapy in combination of insulin dose of 4 IU subcutaneously with 40 ml of 40% glucose solution intravenously.

ate diagnosed immunnovospalitelny version of hyperbilirubinemia, resulting from massive diffuse parenchymal liver damage, it is advisable to use a short course of glucocorticosteroid therapy (prednisolone at a daily dose of 30 mg orally for two weeks).Due to the fact that parenchymal hyperbilirubinemia in most cases accompanied by severe bleeding complications, all patients in this category is recommended prophylaxis Vikasol applied in a daily dose of 0.015 g orally or intramuscularly.

To facilitate the itching that often accompanies cholestatic jaundice option, use the outdoor use - rubbing alcohol and vinegar camphor baths.In order to reduce the concentration of bile acids in serum should be used drugs that target binding of bile acids in the small intestine (cholestyramine daily dose of 2 g).In addition, the method has a good effect of duodenal intubation and the use of choleretic drugs (Holosas a daily dose of 15 ml oral), provided there are no signs of mechanical type of jaundice.In a situation where the occurrence of elevated bilirubin concentration is observed as a result of the mechanical obstruction of the lumen of the bile ducts, the only treatment option is surgical removal of the defect (laparoscopic cholecystectomy).

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