Hyperbilirubinemia functional

- a group of diseases and syndromes characterized by icteric staining of the skin and mucous membranes, hyperbilirubinemia with normal other parameters of liver function and (in the main forms) the absence of morphological changes in the liver, benign course. These include hyperbilirubinemia post-hepatitis and hyperbilirubinemia functional congenital.

Hyperbilirubinemia functional congenital - groups of hereditarily transmitted (genetically determined) nonhemolytic Hyperbilirubinemia. Diseases are caused by a violation of the processes of capturing free bilirubin from the blood by hepatocytes, binding it to glucuronic acid to form bilirubin-glucuronide (bound bilirubin) and then isolating it with bile.

In all cases, hyperbilirubinemia and jaundice are found from early childhood, in most cases (except syndrome Crigler - Najjar) - insignificant, may be intermittent in nature (intensified under the influence of errors in diet, alcohol intake, intercurrent illnesses, physical fatigue and other reasons). There are often indistinctly expressed dyspeptic phenomena, mild asthenia, weakness, rapid fatigue. The liver is usually not enlarged, soft, painless, functional liver samples (except for Hyperbilirubinemia) are not changed. Radioisotope hepatology does not reveal any changes. The spleen is not enlarged. Osmotic resistance of erythrocytes, the duration of their life - are normal. Puncture biopsy in all forms (except Dubin-Johnson syndrome) does not reveal any changes.

Gilbert's syndrome (Gilbert's disease, Gilbert - Lerbulle symptom congenital familial cholehemia, hyperbilirubinemia, congenital, simple family cholehemia, idiopathic hyperbilirubinemia, constitutional hyperbilirubinemia, idiopathic unconjugated hyperbilirubinemia) is characterized by mild intermittent increase in the content of unbound (indirect) bilirubin in the blood, a decrease in the degree of Hyperbilirubinemia under the influence Phenobarbital, absence of other functional or morphological changes in the liver and autosomal dominant (?) Type of inheritance.

Crigler - Najjar syndrome (congenital negemolitiches-kai jaundice type 1, congenital non-hemolytic jaundice with icteric staining nerve nuclei, idiopathic hyperbilirubinemia) is characterized by extremely high blood free (indirect) bilirubin due to the absence of hepatocytes glyukuroniltransferazy, transforming unconjugated bilirubin in a related, The toxic effect of bilirubin on the basal and stem nuclei of the brain with the resulting incidence of incidence, often leading patients to death in childhood, and autosomal recessive type of inheritance.

Dubina -Dzhonsona syndrome (jaundice Dubina -Dzhonsona, non-hemolytic jaundice with the constitutional lipohromnym hepatosis) is characterized by a moderate increase in blood levels of bilirubin - direct (bilirubin-glucuronide) due to violation of the mechanisms of its transport from the microsomes of hepatocytes into the bile, distorted test results from the delay bromsulfaleina secondary increase in its content in the blood within 90 minutes after intravenous administration, nekontrastirovaniem gallbladder during oral holetsistografii, black liver due to accumulation in hepatocytes lipohromnogo brown pigment, increased urinary excretion of coproporphyrin I and an autosomal recessive mode of inheritance.

Rotor syndrome (hyperbilirubinemia idiopathic type of rotor) is characterized by a moderate increase in blood bilirubin (bilirubin-glucuronide), delayed allocation liver intravenously bromsulfaleina, increased allocation of kidneys coproporphyrin (preferably 1 isomer), the absence of other functional and morphological changes in the liver, autosomal recessive Type of inheritance.

The course with all forms (except for Kriegler-Nayyar syndrome) is benign, the prognosis is favorable; Work ability, as a rule, is preserved. With Krigler-Nayyar syndrome, patients usually die in early childhood.

Treatment. Patients in special treatment, as a rule, do not need, only during the period of jaundice strengthening appoint a sparing diet number 5, vitamin therapy, cholagogue. It is forbidden to drink alcoholic beverages, spicy or fatty foods, physical overloads are not recommended.