Hepatosis is a group of liver diseases that occur with a violation of the metabolism of hepatocytes (liver cells), with the development of dystrophic changes on the background of weakly pronounced inflammatory changes or without them.
The main causes of hepatoses development
Hepatosis can be congenital (hereditary) or acquired character. Hereditary pigmented hepatoses include inherited genetically defeat of the liver - enzymopathies, which occur with a violation of the metabolism of bilirubin and periodically appears with jaundice. Changes in the structure of the liver in these types of hepatosis are small and roll in the form of pigment deposition.
- Congenital non-hemolytic jaundice of type I (Kryglerar Nayyar syndrome) - there are no enzymes that bind bilirubin in liver cells, therefore, it enters all organs at high concentrations, giving them toxic effects (the central nervous system, the heart, muscles are particularly seriously affected).)
- Congenital non-hemolytic jaundice of type II (Arias syndrome) - a small amount of the enzyme binding bilirubin is produced in the liver, therefore, the marked toxic lesions of the organs and in the first place - the central nervous system are noted.
- Gilbert's syndrome (non-hemolytic familial jaundice) - occurs in 5% of the subjects under examination for jaundice (90% of them are men), usually occurs with a periodic rise in unbound (indirect) bilirubin in the blood due to a violation of its penetration into liver cells, sometimes combined With hemolytic syndrome.
- Johnson syndrome and Rotor's syndrome are associated with a congenital defect in the excretory function of the liver cells. At the same time in the blood rises the content of bilirubin, and the allocation of bile acids is not disturbed. The disease first manifests itself at the age of 25 years, more often in men.
It is a group of liver lesions associated with the development of dystrophic changes in liver cells (hepatocytes), more often - without gross structural changes, but with prolonged course of the disease, disturbed protein and other types of metabolism in the liver, fibrosis may develop, and in severe cases, necrosis. They come in several types:
- Acute hepatosis (acute toxic dystrophy of the liver) is more often the result of severe toxic poisoning (alcohol and its surrogates, inedible fungi, medicinal products, arsenic, insecticides, etc.), as well as sepsis, viral hepatitis B.
- Chronic fatty hepatosis is usually associated with a disturbance of metabolism in the liver cells and the deposition of fat in them. Most often it is observed in people suffering from general obesity (metabolic syndrome), with unbalanced excessive nutrition, with diabetes, with alcoholism, poisoning, etc.
- Chronic cholestatic hepatosis - can develop with long-term treatment of certain drugs (aminazin, analogues of testosterone, progestogens and others) and is a consequence of bile formation and cholesterol disturbances in the liver cells.
Basic manifestations of hereditary pigmentary hepatoses.
With syndrome KryglerarNayyar jaundice develops immediately after birth and is observed throughout life. In this case infants are often observed cramps, increased muscle tone, lag in mental and physical development, often patients die in childhood due to damage to the nervous system.
The remaining types of hereditary pigmentary hepatoses are characterized by a benign course and do not lead to a shorter life expectancy. The workability is slightly disturbed only during the period of jaundice, which may first appear only in the adolescence or in an adult after stress, ARI and other diseases after taking alcohol. In some Gilbert syndrome, the first manifestation occurs only after acute viral hepatitis.
The general well-being of the patients is satisfactory, but sometimes they are concerned about the feeling of gravity and discomfort in the right hypochondrium (due to the attachment of a small inflammatory process in the biliary tract), nausea, bitter taste in the mouth, heartburn, loss of appetite. There may be flatulence, chest discomfort, fatigue, apathy, sleep disturbance. At the same time jaundice, sclera, skin, mucous membranes - the main symptom of the disease. It is moderate, the other may be accompanied by a slight itchy skin, in some cases there are small pigmented spots on the face and eyelids.
Gilbert's syndromes and Johnson's diabetes can exacerbate several times a year, but usually they end with health regeneration for 2-3 weeks.
Basic clinical manifestations of acquired hepatoses.
Acute fatty hepatosis (toxic dystrophy of the liver) manifests itself as marked digestive disorders, with rapid development of general intoxication and jaundice. Already at the beginning of the disease, the liver increases, it becomes painful, with the progression of the disease, a development of severe hepatic insufficiency, the appearance of hemorrhage (hemorrhage) on the skin can be observed. In severe cases, a possible lethal outcome from the hepatic coma, and in milder cases, the disease becomes chronic.
With chronic fatty liver disease, digestive disorders develop more slowly, accompanied by a general weakness, aching pain and a feeling of dislocation in the right hypochondrium. The liver is gradually enlarged and may be painful when palpated, but not dense (as, for example, with cirrhosis). With appropriate treatment and elimination of the effects of all negative factors possible recovery, but in severe cases possible development of chronic hepatitis and cirrhosis.
Cholestatic hepatosis can occur as an acute illness, but more often takes a prolonged chronic course. The most characteristic of this form of hepatosis is the appearance of jaundice with severe itching and low temperature due to cholestasis (stagnation of bile). At the same time, the stool is discolored, and the urine acquires a dark color. Often, due to the development of secondary cholangitis (inflammation of the bile ducts) and without proper treatment, there is a transition to chronic hepatitis
Survey on hepatoses.
- General blood test and general urine test,
- Determination of bilirubin in the blood and fasting test,
- Sample with phenobarbital and a sample with nicotinic acid,
- Feces analysis on sterkobilin,
- Blood enzymes: AST, ALT, GGTP, LF and others,
- Serum proteins and their fractions
- Prothrombin time
- Markers of hepatitis B, C, D,
- Bromosulfase test
- Ultrasound of the abdominal cavity
- Percutaneous puncture liver biopsy under the control of ultrasound (by indications),
- Genetic counseling (if necessary) and other research and counseling
Basic principles of treatment of hereditary hepatoses
Necessary constant compliance with the diet (table number 5 for Pevzner), adequate work and rest (to avoid stress and fatigue), with caution apply medicines, especially those that can adversely affect the work of the liver. It is desirable periodically to take hepatoprotectors courses.
Basic principles of treatment of acquired hepatoses
- In acute toxic hepatosis, treatment is needed in the hospital. At the same time, great attention is paid to combating hemorrhagic syndrome, generalized toxicosis, in severe cases, corticosteroids are added to the treatment, as well as treatment (or prophylaxis) of hepatic insufficiency
- In chronic hepatosis it is necessary to stop the effect of the factor that led to the onset of the disease. It is necessary to adhere strictly to the diet with restriction of fats of animal origin, but with high content of high-grade animal proteins. It is recommended to take hepatoprotectors - drugs that improve metabolic processes in the liver cells, protecting them from death.
- In cholestatic hepatosis, in addition to the diet, often prescribed corticosteroids, hepatoprotectors.