Fatty hepatosis code. Hepatosis during pregnancy: symptoms and treatment, effects on the fetus. Do-it-yourself options

Typical clinical and laboratory features of alcoholic fatty hepatosis:

• patients complain of a feeling of heaviness and distention, pain in the right hypochondrium and epigastrium; intolerance to fatty foods; general weakness, rapid fatigability, decreased performance, irritability; bloating; in 50% of patients, subjective manifestations are absent;
• the leading clinical sign is hepatomegaly; the liver is moderately enlarged, its consistency is tightly elastic or doughy, the edge is rounded; palpation may be moderately painful;
• liver function tests are slightly changed, in approximately 20-30% of patients, there is a moderate increase in the activity of aminotransferases (ALT, ASAT) and alkaline phosphatase in the blood serum, a slight increase in the blood levels of bilirubin and γ-glutamyl transpeptidase; possibly an increase in the level of triglycerides, free fatty acids, lipoproteins in the blood;
• Ultrasound of the liver reveals the following characteristic signs: enlargement of the liver, a uniform increase in echogenicity, indistinctness of the contour of the liver, uniformity of the structure (the structure is more delicate, consists of many small identical points, as if sprinkled with “semolina.” However, according to A.F. Bluger (1984 ), it is also possible to identify the acoustic heterogeneity of the liver due to the possible presence in its tissue of areas of compaction of various sizes and shapes;
• radioisotope hepatography reveals a violation of the secretory-excretory function of the liver;
• puncture biopsy of the liver is critical in the diagnosis of fatty hepatosis. The diagnosis is reliable when at least 50% of hepatocytes contain fat droplets, which push the nucleus and organelles of the hepatocyte to the periphery. These changes are most pronounced in the centrilobular zone;
• with abstinence from alcohol intake, fatty hepatosis undergoes a complete reverse development.

A special and rare form of fatty hepatosis in chronic alcoholism is Zieve syndrome. It is characterized by the fact that pronounced fatty degeneration of the liver is accompanied by hyperbilirubinemia, hypercholesterolemia, hypertriglyceridemia, hemolytic anemia. Hemolysis of erythrocytes is due to a decrease in the content of vitamin E in blood serum and erythrocytes, a powerful antioxidant factor. A decrease in antioxidant activity contributes to a sharp activation of free radical lipid oxidation and hemolysis of erythrocytes.

Clinically, Tsive's syndrome proceeds as acute alcoholic hepatitis with severe jaundice, pain in the liver, a significant increase in body temperature, and cholestasis syndrome.

A.F.Blyuger and I.N. Novitsky (1984) report a special form of alcoholic fatty hepatosis - "Massive obesityliver ". This form is characterized by severe hepatomegaly, bright hepatocellular failure, cholestasis. Even a lethal outcome is possible.

When making a diagnosis of alcoholic fatty degeneration of the liver, it should be remembered that fatty hepatosis also develops in obesity, diabetes mellitus, protein deficiency, and medicinal liver damage.

At the initial stage of development, fat accumulates in hepatocytes, which over time simply leads to degeneration of the liver cells.

If the disease is not diagnosed at an early stage and appropriate therapy is not carried out, then irreversible inflammatory changes occur in the parenchyma, which lead to the development of tissue necrosis. If fatty hepatosis is not treated, then it can develop into cirrhosis, which is no longer treatable. In the article, we will consider why the disease develops, methods of its treatment and classification according to ICD-10.

Causes of fatty hepatosis and its prevalence

The reasons for the development of the disease have not yet been precisely proven, but factors are known that can confidently provoke the onset of this ailment. These include:

• completeness;
• diabetes;
• violation of metabolic processes (lipid);
• minimal exercise with a nutritious daily diet high in fat.

Most cases of fatty hepatosis are registered by doctors in developed countries with a standard of living above average.

There are a number of other factors associated with hormonal disruption, such as insulin resistance and the presence of sugar in the blood. The hereditary factor cannot be omitted either, it also plays an important role. But still, the main reason is unhealthy diet, a sedentary lifestyle and excess weight. All the reasons have nothing to do with the intake of alcoholic beverages, therefore, fatty hepatosis is often called non-alcoholic. But if you add alcohol dependence to the above reasons, then fatty hepatosis will develop many times faster.

In medicine, it is very convenient to use the coding of diseases for their systematization. It is even easier to indicate the diagnosis on the sick leave with the help of a code. Codes for all diseases are represented in the International Classification of Diseases, Injuries and Various Health Problems. At this time, the option of the tenth revision is in effect.

All liver diseases according to the International classification of the tenth revision are encrypted under the codes K70-K77. And if we talk about fatty hepatosis, then according to ICD 10, it falls under the code K76.0 (fatty liver degeneration).

You can learn more about the symptoms, diagnosis and treatment of hepatosis from separate materials:

Treatment of fatty hepatosis

The treatment regimen for non-alcoholic hepatosis is to eliminate possible risk factors. If the patient is obese, then you need to try to optimize it. And start by reducing the total mass by at least 10%. Doctors recommend using minimal physical activity in parallel with dietary meals to achieve the goal. Limit the use of fats in the diet as much as possible. At the same time, it is worth remembering that drastic weight loss will not only not be beneficial, it can, on the contrary, damage, aggravating the course of the disease.

For this purpose, the attending physician may prescribe thiazolidinoids in combination with biguanides, but this line of drugs has not yet been fully studied, for example, for hepato toxicity. Metformin can help correct the process of metabolic disturbances in carbohydrate metabolism.

As a result, we can confidently say that with the normalization of the daily diet, a decrease in body fat mass and abandoning bad habits, the patient will feel an improvement. And only in this way it is possible to fight such a disease as non-alcoholic hepatosis.

LIVER DISEASES (K70-K77)

Included: medicinal:

• idiosyncratic (unpredictable) liver disease
• toxic (predictable) liver disease

If it is necessary to identify a toxic substance, an additional external cause code (class XX) is used.

Excluded:

• Budd-Chiari syndrome (I82.0)

Included:

• hepatic:
  • coma NOS
  • encephalopathy NOS
• hepatitis:
  • fulminant, not elsewhere classified, with hepatic impairment
  • malignant, not elsewhere classified, with hepatic impairment
• liver (cell) necrosis with liver failure
• yellow atrophy or liver dystrophy

Excluded:

• alcoholic hepatic impairment (K70.4)
• liver failure complicating:
  • abortion, ectopic or molar pregnancy (O00-O07, O08.8)
  • pregnancy, childbirth and the puerperium (O26.6)
• jaundice of fetus and newborn (P55-P59)
• viral hepatitis (B15-B19)
• in combination with toxic liver damage (K71.1)

Excludes: hepatitis (chronic):

• alcoholic (K70.1)
• medicinal (K71.-)
• granulomatous NEC (K75.3)
• reactive nonspecific (K75.2)
• viral (B15-B19)

Excluded:

• alcoholic liver fibrosis (K70.2)
• cardiac sclerosis of the liver (K76.1)
• cirrhosis of the liver):
  • alcoholic (K70.3)
  • congenital (P78.3)
• with toxic liver damage (K71.7)

Excluded:

• alcoholic liver disease (K70.-)
• amyloid degeneration of the liver (E85.-)
• cystic liver disease (congenital) (Q44.6)
• hepatic vein thrombosis (I82.0)
• hepatomegaly NOS (R16.0)
• portal vein thrombosis (I81)
• liver toxicity (K71.-)

In Russia, the International Classification of Diseases of the 10th revision (ICD-10) has been adopted as a single normative document to take into account the incidence, the reasons for the population's visits to medical institutions of all departments, and the causes of death.

ICD-10 was introduced into health care practice throughout the Russian Federation in 1999 by order of the Ministry of Health of Russia dated 05/27/97. No. 170

A new revision (ICD-11) is planned by WHO in 2017 2018.

As amended and supplemented by WHO

Processing and translation of changes © mkb-10.com

fatty hepatosis code by mkb

In the section Diseases, Medicines to the question Fatty hepatosis asked by the author Sergey senatorov the best answer is Serious .. The mother-in-law had

Chronic fatty hepatosis (fatty degeneration, fatty infiltration, liver steatosis, etc.) is characterized by fatty (sometimes with elements of proteinaceous) degeneration of hepatocytes and a chronic course. Etiology, pathogenesis: most often alcoholism, less often endogenous (with severe pancreatitis, enteritis) protein and vitamin deficiency, chronic intoxication with carbon tetrachloride, organophosphorus compounds, other toxic substances with hepatotropic action, bacterial toxins, various metabolic disorders in the body (hypovitaminosis , general obesity, diabetes mellitus, thyrotoxicosis, etc.). The pathogenesis of liver damage in these cases is mainly reduced to a violation of lipid metabolism in hepatocytes and the formation of lipoproteins. In the progression of dystrophic and necrobiotic changes, not only the direct action of the damaging factor on the hepatic cell is important, but also toxic-allergic processes.

Symptoms for. Possible malosymptomatic form, in which the clinic is masked by manifestations of the underlying disease (thyrotoxicosis, diabetes mellitus, etc.), toxic damage to other organs or concomitant diseases of the gastrointestinal tract. In other cases, there are pronounced dyspeptic symptoms, general weakness, dull pain in the right hypochondrium; sometimes mild jaundice. The liver is moderately enlarged, with a smooth surface, painful on palpation. Splenomegaly is uncommon. The content of aminotransferases in the blood serum is moderately or slightly increased, and the content of cholesterol and beta-lipoproteins is often also increased. The results of bromsulfalein and vafaverdin tests are characteristic: a delay in the excretion of these drugs by the liver is observed in most cases. Other laboratory tests are uncommon. The data of a puncture biopsy of the liver (fatty degeneration of hepatocytes) are of decisive importance in the diagnosis.

The course is relatively favorable: in many cases, especially with the exclusion of the action of the damaging agent and timely treatment, recovery is possible. However, hepatosis in some cases can transform into chronic hepatitis and cirrhosis. Differential diagnosis. The absence of splenomegaly makes it possible with a certain degree of confidence to differentiate chronic hepatosis with hepatitis and cirrhosis of the liver. With cirrhosis of the liver, there are usually hepatic stigmata (hepatic asterisks - teleangiectasia, bright red or crimson tongue, "pearl" nails, etc.), signs of portal hypertension, which does not happen with hepatosis. It should also be borne in mind hepatolenticular degeneration, hemochromatosis. Percutaneous liver biopsy is very important for the differential diagnosis of hepatosis with other liver lesions.

Treatment. It is necessary to strive to terminate the action of the etiological factor. The intake of alcoholic beverages is strictly prohibited. Prescribe diet number 5 with a high content of complete proteins of animal origin (dog / day) and lipotropic factors (cottage cheese, boiled cod, yeast, buckwheat, oatmeal, etc.). Limit the intake of fats, especially refractory fats, of animal origin. Lipotropic drugs are prescribed: choline chloride, lipoic acid, folic acid, vitamin B12, drugs containing extracts and liver hydrolysates (sirepar 5 ml intramuscularly daily, Essentiale, etc.).

phosphogliv in capsules is also suitable, liver cells are replaced by adipose tissue, the liver increases in size

this is the liver in layers of fat, I used "Esenziale Forte"

Fatty liver hepatosis

Description of the disease

Fatty liver hepatosis (hepatic steatosis, fatty liver disease, fatty liver infiltration) is a chronic liver disease characterized by fatty degeneration of the liver cells. It occurs quite often, develops under the influence of alcohol, toxic substances (medicines), with diabetes mellitus, anemia, lung diseases, severe pancreatitis and enteritis, malnutrition, obesity.

Causes

According to the mechanism of development, hepatosis occurs due to excessive intake of fats in the liver, liver overload with dietary fats and carbohydrates, or due to impaired excretion of fats from the liver. Violation of the excretion of fat from the liver occurs with a decrease in the amount of substances involved in the processing of fats (protein, lipotropic factors). The formation of phospholipids, beta-lipoproteins, lecithin from fats is impaired. And excess free fats are deposited in the liver cells.

Symptoms

Patients with hepatosis usually do not present complaints. The course of the disease is erased, slowly progressing. Over time, there are constant dull pain in the right hypochondrium, nausea, vomiting, stool disturbances. The patient is worried about weakness, headache, dizziness, fatigue during exercise. Very rarely, hepatosis with a pronounced clinical picture is observed: severe pain, weight loss, itching, bloating. On examination, an enlarged, slightly painful liver is found. The course of the disease is usually not severe, but sometimes fatty hepatosis can turn into chronic hepatitis or cirrhosis of the liver.

Diagnostics

With ultrasound of the abdominal cavity - an increase in the echogenicity of the liver, an increase in its size. In a biochemical study of blood, a slight increase in the activity of liver tests and changes in protein fractions.

Treatment

First of all, one should either exclude or minimize the effect of the factor that led to the deposition of fat in the liver. This is almost always possible in relation to alcohol, if we are not talking about the formation of addiction, when the help of a narcologist is required. Patients with diabetes mellitus and hyperlipidemia should be monitored jointly by an endocrinologist and a cardiologist, respectively. All patients require a low fat diet and adequate daily physical activity.

In obese patients, doctors usually find it necessary to reduce the patient's body weight. The effect of weight loss on the course of fatty hepatosis is ambiguous. A rapid weight loss naturally leads to an increase in the activity of inflammation and the progression of fibrosis. A decrease in weight nkg / year has a positive effect on the severity of steatosis, inflammation and the degree of liver fibrosis. The most effective weight loss is considered to be no more than 1.6 kg / week, which is achieved with a daily calorie intake of 25 calories / kg / day.

Fatty liver hepatosis in the ICD classification:

hello dear doctors. question from Tashkent. we are now in a very difficult condition, as my brother cannot be treated for hepatitis "A" for 4 months already. no medicine helps. today we visited a virologist, he said that this could be the beginning of liver cirrhosis. please help us. can't figure out what kind of hepatitis is this? why not be treated?

Which doctor should i contact if there is fatty liver hepatosis:

Good afternoon. I am 67 years old, height 158 ​​cm, weight 78 kg. We start to gain weight after the death of my husband. I do not abuse alcohol. I am moderately walking, what should I do? Analyzes are normal - and the ultrasound diagnosis: echo signs of fatty hepatosis, chronic cholecystitis, chronic pancreatitis. What to do?

Fatty liver degeneration (K76.0)

Version: MedElement Disease Handbook

general information

Short description

Fatty liver degeneration is a disease characterized by liver damage with changes similar to those in alcoholic liver disease (fatty degeneration of hepatocytes hepatocyte is the main liver cell: a large cell that performs various metabolic functions, including the synthesis and accumulation of various substances necessary for the body, neutralization of toxic substances and the formation of bile (Hepatocyte)

), however, with fatty liver degeneration, patients do not consume alcohol in quantities that can cause liver damage.

The definitions most commonly used in NAFLD are:

1. Non-alcoholic fatty liver disease (NAFL). The presence of fatty degeneration of the liver without signs of damage to hepatocytes hepatocyte - the main cell of the liver: a large cell that performs various metabolic functions, including the synthesis and accumulation of various substances necessary for the body, neutralization of toxic substances and the formation of bile (Hepatocyte)

in the form of balloon dystrophy or without signs of fibrosis. The risk of developing cirrhosis and liver failure is minimal.

2. Non-alcoholic steatohepatitis (NASH). The presence of hepatic steatosis and inflammation with damage to hepatocytes hepatocyte is the main liver cell: a large cell that performs various metabolic functions, including the synthesis and accumulation of various substances necessary for the body, neutralization of toxic substances and the formation of bile (Hepatocyte)

(balloon dystrophy) with or without signs of fibrosis. May progress to liver cirrhosis, liver failure, and (rarely) liver cancer.

3. Non-alcoholic cirrhosis of the liver (NASH Cirrhosis). Presence of signs of cirrhosis with current or previous histological evidence of steatosis or steatohepatitis.

4. Cryptogenic Cirrhosis - cirrhosis without obvious etiological reasons. Patients with cryptogenic cirrhosis usually have high risk factors associated with metabolic disorders such as obesity and metabolic syndrome. Increasingly, cryptogenic cirrhosis, when examined in detail, turns out to be an alcohol-related disease.

5. Assessment of NAFLD activity (NAS). The aggregate of points, calculated with a comprehensive assessment of the signs of steatosis, inflammation and balloon dystrophy. It is a useful tool for the semi-quantitative measurement of histological changes in liver tissue in patients with NAFLD in clinical trials.

K75.81 - Non-alcoholic steatohepatitis (NASH)

K74.0 - Fibrosis of the liver

K 74.6 - Other and unspecified cirrhosis of the liver. \

Classification

Types of fatty liver degeneration:

1. Macrovesicular type. The accumulation of fat in hepaticitis is local in nature and the nucleus of the hepatocyte is shifted away from the center. With fatty liver infiltration of the macrovesicular (coarse droplet) type, triglycerides, as a rule, act as accumulated lipids. In this case, the morphological criterion of fatty hepatosis is the content of triglycerides in the liver over 10% of dry weight.

2. Microvesicular type. The accumulation of fat occurs evenly and the core remains in place. With microvesicular (small droplet) fatty degeneration, lipids other than triglycerides (for example, free fatty acids) accumulate.

There are also focal and diffuse hepatic steatosis. Most often, diffuse steatosis occurs, which is of a zonal nature (the second and third zones of the lobule).

Etiology and pathogenesis

Primary non-alcoholic fatty disease is considered as one of the manifestations of metabolic syndrome.

Hyperinsulinism leads to the activation of the synthesis of free fatty acids and triglycerides, a decrease in the rate of beta-oxidation of fatty acids in the liver and the secretion of lipids into the bloodstream. As a result, fatty degeneration of hepatocytes develops hepatocyte - the main cell of the liver: a large cell that performs various metabolic functions, including the synthesis and accumulation of various substances necessary for the body, neutralization of toxic substances and the formation of bile (Hepatocyte)

The onset of inflammatory processes is predominantly centrilobular in nature and is associated with increased lipid peroxidation.

Increasing the absorption of toxins from the intestines is of certain importance.

A sharp decrease in body weight;

Chronic protein-energy malnutrition.

Inflammatory bowel disease;

Celiac disease Celiac disease is a chronic disease caused by a deficiency of enzymes involved in the digestion of gluten.

Small bowel diverticulosis;

Microbial contamination Contamination is the entry into a certain environment of any impurity that changes the properties of this environment.

Operations on the gastrointestinal tract.

Type II diabetes mellitus;

Triglyceridemia, etc.

Epidemiology

Sign of prevalence: Widespread

Sex ratio (m / f): 0.8

The estimated prevalence is between 1% and 25% of the general population in various countries. In developed countries, the average level is 2-9%. Many findings are accidentally discovered during liver biopsies performed for other indications.

Most often, the disease is detected in age, although no age (except for breastfed children) excludes the diagnosis.

The sex ratio is unknown, but the prevalence of the female sex is assumed.

Factors and risk groups

The high-risk group includes:

more than 30% of cases are associated with the development of hepatic steatosis Liver steatosis is the most common hepatosis, in which fat accumulates in the liver cells

and in 20-47% with non-alcoholic steatohepatosis.

2. Persons with type 2 diabetes mellitus or impaired glucose tolerance. In 60% of patients, these conditions are found in combination with fatty degeneration, in 15% - with non-alcoholic steatohepatitis. The severity of liver damage is related to the severity of the glucose metabolism disorder.

3. Persons with diagnosed hyperlipidemia, which is detected in 20-80% of patients with non-alcoholic steatohepatitis. A characteristic fact is the more frequent combination of non-alcoholic steatohepatitis with hypertriglyceridemia than with hypercholesterolemia.

4. Middle-aged women.

and non-controlling blood pressure. There is a higher prevalence of fatty degeneration of the liver in patients with essential hypertension without risk factors for the development of fatty degeneration of the liver. The prevalence of the disease is estimated to be almost 3 times higher than in the control groups, corresponding in age and sex and keeping blood pressure at the recommended level.

Malabsorption syndrome Malabsorption syndrome (malabsorption) is a combination of hypovitaminosis, anemia and hypoproteinemia due to malabsorption in the small intestine

(as a consequence of the imposition of ileojejunal ileojejunal - related to the ileum and jejunum.

anastomosis, extended resection of the small intestine, gastroplasty for obesity, etc.);

and some others.

Clinical picture

Clinical diagnostic criteria

Symptoms, course

Most patients with non-alcoholic fatty liver disease have no complaints.

Slight discomfort in the upper right quadrant of the abdomen (about 50%);

Pain in the upper right quadrant of the abdomen (30%);

Moderate hepatosplenomegaly Hepatosplenomegaly - simultaneous significant enlargement of the liver and spleen

Arterial hypertension AH (arterial hypertension, hypertension) is a persistent increase in blood pressure from 140/90 mm Hg. and higher.

Dyslipidemia Dyslipidemia is a violation of the metabolism of cholesterol and other lipids (fats), which consists in a change in their ratio in the blood

Impaired glucose tolerance.

The appearance of telangiectasias Telangiectasia is a local excessive expansion of capillaries and small vessels.

Palmar erythema Erythema - limited hyperemia (increased blood supply) of the skin

Ascites Ascites - accumulation of transudate in the abdominal cavity

Jaundice, gynecomastia Gynecomastia - breast enlargement in men

Signs of liver failure and other signs of fibrosis, cirrhosis, non-infectious hepatitis require coding in the appropriate subheadings.

The identified relationship with alcohol, medication, pregnancy and other etiological reasons also requires coding in other subheadings.

Diagnostics

Laboratory diagnostics

are detected in 50-90% of patients, however, the absence of these signs does not exclude the presence of non-alcoholic steatohepatitis (NASH).

The level of serum transaminases is slightly increased - 2-4 times.

The value of the AST / ALT ratio in NASH:

Less than 1 - observed in the initial stages of the disease (for comparison, in acute alcoholic hepatitis, this ratio is usually> 2);

Equal to 1 or more - may be an indicator of more pronounced liver fibrosis;

More than 2 is considered as an unfavorable prognostic sign.

2. In 30-60% of patients, an increase in the activity of alkaline phosphatase (as a rule, no more than twofold) and gamma-glutamyl transpeptidase (may be isolated, not associated with an increase in alkaline phosphatase) is detected. GGTP levels> 96.5 U / L increase the risk of fibrosis.

3. In 12-17% of cases, hyperbilirubinemia occurs within% of the norm.

In clinical practice, insulin resistance is assessed by the ratio of the levels of immunoreactive insulin and blood glucose. It should be remembered that this is a calculated indicator that is calculated by various methods. The indicator is influenced by the level of triglycerides in the blood and race.

7. Hypertriglyceridemia is observed in 20-80% of patients with NASH.

Many patients will have low HDL cholesterol as part of their metabolic syndrome.

As the disease progresses, cholesterol levels often decrease.

It should be borne in mind that a low positive antinuclear antibody titer is not uncommon in NASH, and less than 5% of patients may have a positive low antinuclear antibody titer.

are more characteristic of cirrhosis or severe fibrosis.

Unfortunately, this indicator is not specific; in case of its increase, it is necessary to exclude a number of oncological diseases (bladder, breast, etc.).

11. Complex biochemical tests (BioPredictive, France):

Steato test - allows you to identify the presence and degree of liver steatosis;

Nash test - allows you to detect NASH in patients with overweight, insulin resistance, hyperlipidemia, as well as patients with diabetes mellitus).

It is possible to use other tests if non-alcoholic fibrosis or hepatitis is suspected - Fibro-test and Acti-test.

Differential diagnosis

Complications

Fibrosis Fibrosis is the proliferation of fibrous connective tissue that occurs, for example, as a result of inflammation.

Liver cirrhosis Liver cirrhosis is a chronic progressive disease characterized by dystrophy and necrosis of the hepatic parenchyma, accompanied by its nodular regeneration, diffuse proliferation of connective tissue and deep restructuring of the liver architectonics.

In detail (it develops especially rapidly in patients with tyrosinemia. Tyrosinemia is an increased concentration of tyrosine in the blood. The disease leads to an increase in urinary excretion of tyrosine compounds, hepatosplenomegaly, nodular cirrhosis of the liver, multiple defects of renal tubular reabsorption and vitamin D in resistant rickets. Tyrosinemia and excretion occur in resistant rickets. a number of inherited (p) fermentopathies: fumarylacetoacetase deficiency (type I), tyrosine aminotransferase (type II), 4-hydroxyphenylpyruvate hydroxylase (type III)

Almost bypassing the stage of "pure" fibrosis);

Liver failure (rarely - in parallel with the rapid formation of cirrhosis).

Treatment

Forecast

Life expectancy in non-alcoholic fatty liver disease is no lower than that of healthy individuals.

Half of the patients develop progressive fibrosis, and 1/6 develop liver cirrhosis.

Hospitalization

Prophylaxis

1. Normalization of body weight.

2. Patients should be screened for hepatitis viruses. In the absence of viral hepatitis, they should be offered vaccination against hepatitis B and A.

/ Internal diseases / 3 chapter LIVER DISEASES AND BILISTRY SYSTEM-p

DISEASES OF THE LIVER AND BILISTRY SYSTEM

Dyskinesia of the biliary tract.

Fatty hepatosis (FG) - hepatic steatosis, chronic fatty degeneration of the liver - an independent chronic disease or syndrome caused by fatty degeneration of hepatocytes with intra- and / or extracellular fat deposition.

ICD10: K76.0 - Fatty liver disease, not elsewhere classified.

FG is a polyetiologic disease. It often occurs as a result of metabolic disorders caused by an unbalanced diet. Especially if there is a bad habit or there are circumstances in which the entire daily need for food is satisfied in almost 1 reception. In such cases, taking into account the limited possibilities of depositing carbohydrates and proteins in the liver and other organs, they turn into easily and infinitely deposited fat.

GH is often a secondary syndrome accompanying obesity, diabetes mellitus, endocrine diseases, primarily Cushing's disease, chronic alcoholism, intoxication, including drugs, chronic circulatory failure, metabolic X-syndrome, and many other diseases of internal organs.

As a result of excessive accumulation of fat in the liver tissue, the function of the organ as a dynamic depot of carbohydrates (glycogen) is first of all disrupted, which leads to destabilization of the mechanisms for maintaining a normal level of glucose in the blood. In addition, metabolic changes associated with prolonged exposure to etiological factors can cause toxic and even inflammatory damage to hepatocytes, the formation of steatohepatitis with a gradual transition to liver fibrosis. In many cases, the etiological factors that caused GH can contribute to the formation of homogeneous cholesterol stones in the gallbladder.

FG is characterized by complaints of general weakness, reduced ability to work, dull aching pains in the right hypochondrium, poor alcohol tolerance. Many have hypoglycemic states in the form of paroxysmal sharp weakness, sweating, feelings of "emptiness" in the abdomen, which quickly pass after eating food, even one candy. Most patients have a tendency to constipation.

The overwhelming majority of patients with GI have a habit of eating 1–2 times a day. Many have a history of drinking a large amount of beer, long-term drug therapy, work under conditions of toxic effects, various diseases of internal organs: diabetes mellitus, metabolic X-syndrome, chronic circulatory failure, etc.

An objective study usually pays attention to the overweight of the patient. Percussion determined liver sizes are increased. The anterior edge of the liver is rounded, compacted, weakly sensitive.

Symptoms of pathological changes in other organs detected during FG are usually related to diseases that led to the formation of fatty degeneration of the liver.

General analysis of blood and urine: no deviations.

Biochemical blood test: increased levels of cholesterol, triglycerides, increased activity of AST and ALT.

Ultrasound examination: enlargement of the liver with a diffuse or focal uneven increase in the echogenicity of the liver parenchyma, depletion of the tissue pattern with small vascular elements. There is no portal hypertension. As a rule, signs of pancreatic steatosis are simultaneously detected: an increase in the volume of the gland, diffusely increased echogenicity of its parenchyma in the absence of pathological expansion of the Wirsung duct. Concrements in the gallbladder, signs of diffuse, reticular or polypous cholesterosis of the gallbladder can be recorded.

Laparoscopic examination: the liver is enlarged, its surface is yellowish-brown.

Liver biopsy: diffuse or localized in different parts of the lobule fatty degeneration of liver cells, extrahepatic location of fatty drops. With a prolonged course of the disease, signs of steatohepatitis are revealed - cellular inflammatory infiltration with predominant localization in the center of the lobules. Sometimes infiltrates capture the entire lobule, spread to the portal tracts and the periportal zone, which indicates the likelihood of the formation of liver fibrosis.

It is carried out with alcoholic liver disease, chronic hepatitis.

Unlike LH, alcoholic liver disease is characterized by anamnestic information about long-term alcohol abuse. In biopsies of the liver of alcoholics, a large number of hepatocytes containing Mallory's little bodies - a condensed smooth endoplasmic reticulum - are detected. In their blood, a marker of long-term alcoholism is detected - transferrin does not contain sialic acids.

Chronic hepatitis differs from GH by deviations in the general and biochemical blood tests, indicating the presence of a chronic inflammatory process in the liver, violations of the protein-forming and liposynthetic functions of the organ. Markers of infection with hepatitis B, C, D, G viruses are detected. The results of a puncture biopsy of the liver allow to reliably distinguish between GH and chronic hepatitis.

General blood analysis.

Immunological analysis for the presence of markers of hepatitis B, C, D, G.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

A mandatory transition to a fractional diet - 5-6 meals a day with an even distribution of calorie intake and component composition (carbohydrates-proteins-fats) of food. The use of animal fats is limited. Dishes containing cottage cheese and vegetable fibers are recommended. With a tendency to constipation, steamed rye or wheat bran should be consumed 1-3 teaspoons 3-4 times a day with meals.

A daily intake of balanced multivitamin preparations such as "Troll", "Jungle", "Enomdan" and the like is mandatory.

The most effective remedy for the treatment of GH is Essentiale-forte, which contains essential phospholipids and vitamin E. Unlike Essentiale-Forte, Essentiale does not contain vitamin E, nor does Essentiale for parenteral administration. Essentiale-forte take 2 capsules 3 times a day with meals for 1-2 months.

Other lipotropic drugs can be used to treat GH:

Legalon - 1-2 tablets 3 times a day.

Lipofarm - 2 tablets 3 times a day.

Lipostabil - 1 capsule 3 times a day.

Lipoic acid - 1 tablet (0.025) 3 times a day.

The effectiveness of the treatment can be monitored using ultrasound, which reveals a tendency towards a decrease in the size of the liver, a decrease in the echogenicity of the organ parenchyma.

Usually auspicious. With the exclusion of harm, effective treatment, prophylactic intake of multivitamin preparations, complete recovery is possible.

TESTS FOR SELF-CONTROL

What circumstances can not lead to the formation of fatty hepatosis?

Eating 1-2 times a day.

Excessive consumption of foods containing animal fats.

Eating cottage cheese, plant products.

Professional and household intoxication.

What diseases can not to form fatty hepatosis.

Chronic circulatory failure.

What diseases and syndromes can not occur with prolonged exposure to the etiological factor that caused the formation of fatty hepatosis?

Anything can arise.

What are the clinical manifestations not typical for fatty hepatosis?

Overweight.

An increase in the size of the liver.

Dense, rounded, sensitive edge of the liver.

What abnormalities of the biochemical blood test are not typical for fatty hepatosis?

Increased content of cholesterol, triglycerides.

Increased activity of AST and ALT.

High levels of bilirubin.

What points of the plan for examining patients with fatty hepatosis can be excluded without compromising the quality of diagnosis.

Biochemical blood test: fasting sugar, total protein and its fractions, bilirubin, cholesterol, uric acid, AST, ALT, gamma-glutamyl transpeptidase, sialic acid-free transferrin.

Immunological analysis for the presence of markers of hepatitis B, C, D, G viruses.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

What ultrasound results are not typical for fatty hepatosis?

Increased liver volume.

High echogenicity of the liver parenchyma.

Signs of pancreatic lipomatosis.

Signs of gallstone disease.

Signs of portal hypertension.

What are the criteria do not allow to distinguish fatty degeneration of the liver in algogolny disease from fatty hepatosis?

The presence of sialic acid-free transferrin in the blood.

Biopsy specimens contain many cells containing Malory bodies.

The presence of fat droplets in intracellular vacuoles and outside hepatocytes.

All criteria allow.

None of the criteria allows you to do this.

The transition to a fractional diet with 5-6 times food intake during the day.

An even distribution of the calorie intake of the diet throughout the day.

The use of lipotropic (cottage cheese) and herbal products.

What drugs it does not follow to give to patients with fatty hepatosis?

What are the clinical manifestations not typical for fatty hepatosis?

Aching pains in the right hypochondrium.

Increased abdominal volume, ascites.

Constipation tendency.

Pigmented hepatosis - hereditary disorders of the metabolism and transport of bilirubin in hepatocytes, manifested by constant or recurrent jaundice in the absence of changes in the morphological structure of the liver.

In adults, the following variants of disorders in the metabolism of bilirubin in the liver are found:

Gilbert's syndrome is a syndrome of unconjugated hyperbilirubinemia.

Rotor syndrome is a syndrome of conjugated hyperbilirubinemia.

Dabin-Jones syndrome is a syndrome of conjugated hyperbilirubinemia with excessive deposition of melanin-like pigment in hepatocytes.

More often than others, unconjugated hyperbilirubinemia, Gilbert's syndrome, occurs in clinical practice.

Gilbert's syndrome (SG) is a genetically determined enzymopathy that causes impaired conjugation of bilirubin in the liver, which is manifested by an increase in the content of unconjugated bilirubin in the blood, jaundice, and accumulation of lipofuscin pigment in hepatocytes.

ICD10: E80.4 - Gilbert's syndrome.

The syndrome is associated with an autosomal dominant defect in the UGTA1A1 and GNT1 genes, which causes insufficient formation in hepatocytes of the enzyme glucuronyl transferase, which provides neutralization in the liver, including the conjugation of bilirubin with glucuronic acid. Men suffer from SJ 10 times more often than women. Acute viral hepatitis (“post-hepatitis” unconjugated hyperbilirubinemia) can be a triggering factor for GS.

In the pathogenesis of the disease, the main role is played by:

Disturbances in the transport function of proteins that deliver unconjugated bilirubin to the smooth endoplasmic reticulum - hepatocyte microsomes.

Inadequacy of the microsomal enzyme UDP-glucuronyltransferase, with the participation of which conjugation of bilirubin with glucuronic and other acids is carried out.

With SF, as well as with other forms of pigmentary hepatosis, the liver retains a histological structure identical to the normal one. However, in hepatocytes, an accumulation of a golden or brown pigment, lipofuscin, can be detected. As a rule, there are no signs of dystrophy, necrosis, fibrosis in the liver with SF, as with other pigmentary hepatosis.

In the gallbladder in patients with SJ, calculi consisting of bilirubin can form.

All patients with SJ complain of recurrent yellowness of the sclera and skin. There are usually no other complaints. Only in isolated cases do you experience rapid fatigue, a feeling of heaviness in the right hypochondrium. Jaundice occurs and grows under conditions of emotional and physical stress, with respiratory infections, after surgery, after drinking alcohol, during fasting or low-calorie (less than 1/3 of the norm) diet low in fat (vegetarianism), after taking certain medications (nicotinic acid , rifampicin). Patients with FS are often neurotic because they are worried about their jaundice.

The leading symptom of the disease is scleral icterus. Yellowness of the skin is present only in some patients. Characterized by a dull-icteric color of the skin, especially on the face. In some cases, there is a partial staining of the palms, feet, axillary regions, and the nasolabial triangle. In some cases, despite the increased level of bilirubin in the blood, the skin has a normal color - cholemia without jaundice. In some patients, pigmentation of the face occurs, scattered pigment spots appear on the skin of the trunk.

According to the description of Gilbert himself, in the typical course of the disease, a triad should be revealed: hepatic mask, xanthelasma of the eyelids, yellow skin color.

Some clinicians consider urticaria, hypersensitivity to cold, and goose bumps to be characteristic of this syndrome.

An objective study in 1/4 of patients can reveal a moderate increase in the liver. Palpation of the liver is soft, painless. With the formation of pigment stones in the gallbladder, clinical manifestations of cholelithiasis, chronic calculous cholecystitis are possible

Complete blood count: in one third of cases of SJ, hemoglobin content increased over 160 g / l, erythrocytosis, decreased ESR are detected (these changes are usually combined with increased gastric acidity).

General urine analysis: the color is normal, there is no bilirubin.

Biochemical blood test: isolated unconjugated hyperbilirubinemia, which only in rare cases exceeds the level of µmol / l, averaging about 35 µmol / l. All other biochemical parameters,

characterizing liver function, usually normal.

Instrumental methods (ultrasound, computed tomography, isotope scintigraphy) do not reveal any changes in the structure of the liver that are specific to the SF.

Ultrasound in the gallbladder often reveals calculi of the pigment structure. Puncture liver biopsy: no signs of necrosis, inflammation, activation of fibrosis processes. In the liver cells, the presence of a pigment, lipofuscin, is determined.

To detect Gilbert's syndrome, provocative tests with restriction of the energy value of food and with a load of nicotinic acid, which cause an increase in the level of unconjugated hyperbilirubinemia, help:

Examine serum bilirubin in the morning on an empty stomach. Then, within 2 days, the patient receives food with limited energy value - about 400 kcal / day. Re-examine the level of serum bilirubin. If it turns out to be more than the initial one by 50% or more, then the sample is considered positive.

The initial serum bilirubin content is recorded. Introduce intravenously 5 ml of a 1% solution of nicotinic acid. After 5 hours, a control study of bilirubin is carried out. If its level rises by more than 25%, the test is considered positive.

One of the most convincing diagnostic tests is a stress test with the appointment of a patient with phenobarbital or zixorin - inducers of transport proteins and hepatocyte glucuronyltransferase:

10 days after the start of oral administration of phenobarbital 0 times a day or zixorin 0.2 - 3 times a day after meals in people with Gilbert's syndrome, the level of unconjugated bilirubin significantly decreases or normalizes.

It is carried out primarily with hemolytic jaundice, mainly with hereditary microspherocytosis. Criteria such as the appearance of the first clinical symptoms (jaundice) of Gilbert's syndrome in adolescence are taken into account, while hemolytic jaundice appears much earlier, in childhood. Microspherocytosis is characterized by splenomegaly and moderate anemia, which is not the case with SF. Serum bilirubin levels are usually lower in FS than in hemolytic jaundice.

Unlike chronic hepatitis, which can also be predominantly unconjugated hyperbilirubinemia, Gilbert's syndrome does not show signs of carriage of hepatotropic viruses. Unlike hepatitis, there is no hepatomegaly laboratory data indicating the presence of an active inflammatory process in the liver. The analysis of liver biopsies does not reveal signs of inflammation, necrosis of liver cells, active fibrosis. In hepatocytes, the presence of a pigment, lipofuscin, is determined.

General blood analysis.

Biochemical blood test: bilirubin, cholesterol, AST, ALT, gamma-glutamyl transpeptidase.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

Provocative tests with restriction of the energy value of food or the intake of nicotinic acid.

Exercise tests with glucuronyl transferase inducers - phenobarbital or zixorin.

SD is not a reason for prescribing any specific treatment. Prophylactic complex vitamin therapy may be indicated. It should be remembered that such people need a complete, high-calorie diet with a sufficient amount of fat in the diet. They must stop drinking alcohol. In vocational guidance, the undesirability of emotional and physical overload is taken into account. Drugs that can induce jaundice (niacin) should be avoided. In the presence of concomitant gallstone disease, cholecystectomy using minimally invasive, laparoscopic surgery is an effective way of treating it.

In the classical course of the process, the prognosis is favorable.

Dabin-Johnson syndrome (SDS) is a genetically determined enzymopathy that causes disruption of bilirubin transport in the liver, which is manifested by an increase in the content of conjugated bilirubin in the blood, jaundice, and the accumulation of melanin-like pigment in hepatocytes.

ICD10: E80.6 - Other disorders of bilirubin metabolism.

Diabetes mellitus is an inherited disorder. Individuals with DMD have an autosomal recessive genetic defect that causes impairment of the transfer of organic anions, including the transport of conjugated bilirubin from hepatocytes to the bile ducts. In men, SDD occurs more often than in women.

As a result of a violation of the mechanism of directed transport of bilirubin from hepatocytes into the lumen of the bile ducts, part of the conjugated bilirubin returns to the blood. Postmicrosomal hepatocellular jaundice occurs with a moderate increase in direct bilirubin in the blood. Pathogenetically, SDS is identical to the Rotor syndrome, from which it differs in one feature - the accumulation in hepatocytes of a large amount of melanin-like pigment, which gives the liver a dark bluish-green, almost black color. In patients with diabetes mellitus, calculi from bilirubin salts may form in the gallbladder.

Complaints of recurrent jaundice of the sclera, skin, sometimes together with slight itching are characteristic. During the period of jaundice, many patients feel general weakness, physical and mental fatigue, loss of appetite, mild nausea, bitterness in the mouth, sometimes dull aching pain in the right hypochondrium. When jaundice occurs, the urine becomes dark in color.

Jaundice can be provoked by physical and psycho-emotional stress, fever caused by a respiratory viral infection, alcoholic excess, and the use of anabolic steroids.

Biliary cholelithiasis is usually asymptomatic, but sometimes it manifests itself as biliary colic, symptoms of calculous cholecystitis, and in some cases it can cause obstructive jaundice.

Among the objective manifestations, there is a moderate icterus of the sclera and skin, a slight increase in the volume of the liver. Palpation of the liver is not compacted, painless.

Complete blood count: no deviations.

General urine analysis: dark color, high content of bilirubin.

Biochemical blood test: an increase in the content of bilirubin due to the conjugated fraction.

Samples with a load of bromsulfalein, radioisotope hepatography reveal a pronounced violation of the excretory function of the liver.

Ultrasound: the liver is of normal structure. Intra- and extrahepatic bile ducts are not dilated. Portal hemodynamics is not impaired. In the gallbladder, dense, echo-positive calculi can be detected.

Laparoscopy: the liver surface is dark bluish green or black.

Puncture biopsy: the morphological structure of the liver is not changed. Melanin-like pigment is detected in hepatocytes.

It is performed with obstructive jaundice, from which SDD is distinguished by the absence of an increase in blood cholesterol levels, the activity of enzymes specific to cholestasis - alkaline phosphatase, gamma-glutamyl transpeptidase. An ultrasound scan with SDD does not show the expansion of the intra- and extrahepatic bile ducts - a specific sign of obstructive jaundice.

General blood analysis.

General urine analysis with determination of bilirubin, urobilin, hemosiderin.

Coprogram with the definition of stercobilin.

Biochemical blood test: bilirubin, cholesterol, alkaline phosphatase, AST, ALT, gamma-glutamyl transpeptidase.

Test with bromsulfalein to assess the excretory function of the liver.

Radioisotope hepatography to assess the excretory function of the liver.

Immunological analysis: markers of infection with hepatitis B, C, G viruses.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

No special treatment is required. Individuals with SDD should completely stop drinking alcohol. They should avoid any intoxication, limit the intake of medications as much as possible. They can be advised to take complex multivitamin preparations. In the presence of gallstone disease, especially if it proceeds with attacks of colic, cholecystectomy using minimally invasive surgery methods is indicated.

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Gilbert's Syndrome

ICD-10 code

E80.4. Gilbert's syndrome.

Gilbert's syndrome is pigmented hepatosis (simple familial cholemia, constitutional hyperbilirubinemia, idiopathic unconjugated hyperbilirubinemia, non-hemolytic familial jaundice) with an autosomal dominant mode of inheritance, characterized by a moderate intermittent increase in the content of unbound (indirect) bilirubin in the blood. The syndrome was first described by the French doctors A.N. Gilbert and P. Lereboullet in 1901.

This is the most common form of hepatitis pigmented hepatosis, which occurs in 2-5% of the population. Among Caucasians, the prevalence of the syndrome is 2-5%, among Mongoloids - 3%, among Negroids - 36%. The disease manifests itself in adolescence and proceeds almost throughout life. It is more common in males.

Etiology and pathogenesis

The syndrome is caused by a mutation in a gene UGT1A1, which encodes the enzyme uridine diphosphate glucuronyl transferase (UDPGT). The following links lie in the pathogenesis of the syndrome:

Violation of the capture of bilirubin by microsomes of the vascular pole of hepatocytes;

Disruption of bilirubin transport by glutathione-8-transferase, which delivers unconjugated bilirubin to hepatocyte microsomes;

Inadequacy of the microsomal enzyme UDFGT, with the help of which bilirubin is conjugated with glucuronic and other acids.

In Gilbert's syndrome, the activity of UDPGT decreases by only 10-30% compared to the norm, the main importance is attached to the violation of the uptake of bilirubin by hepatocytes, which is associated with an abnormality of membrane permeability and a defect in the protein of intracellular transport.

Bilirubin exchange consists of its transport in the blood plasma, capture by the liver, conjugation, biliary excretion (Fig. 6-1).

Every day, the human body produces about 250-300 mg of unconjugated bilirubin: 70-80% of this amount is the result of the daily breakdown of erythrocyte hemoglobin; 20-30% is formed from heme proteins in the bone marrow or liver. About 1% of circulating erythrocytes disintegrates in a healthy person per day.

Bilirubin, which is formed in the cells of the reticuloendothelium, is a toxic compound. It is called unconjugated, indirect, or free, unbound bilirubin (due to the specificity of the reaction in its determination), is water-insoluble. That is why it is present in blood plasma in the form of a compound with albumin. The albumin-bilirubin complex prevents bilirubin from entering the urine through the glomerular membrane.

With the blood flow, indirect bilirubin enters the liver, where this form of bilirubin is converted into a less toxic form - direct (bound, conjugated) bilirubin. Both fractions make up total bilirubin.

In the liver, unconjugated bilirubin is separated from albumin at the level of microvilli of hepato-

Rice. 6-1. Exchange and conjugation of bilirubin

cytes, its capture by intrahepatic protein. The conjugation of bilirubin with the formation of mono- and diglucuronides (conjugated bilirubin) is provided by UDFGT.

The release of bilirubin into bile is the final stage of pigment exchange and occurs through the cytoplasmic membranes of hepatocytes.

In bile, conjugated bilirubin forms a macromolecular complex with cholesterol, phospholipids, and bile salts. Then, with bile, it enters the duodenum and small intestine, where it is transformed into urobilinogen, part of which is absorbed through the intestinal wall, enters the portal vein and is transported with blood flow to the liver (intestinal-hepatic circulation), where it is completely destroyed.

The main amount of urobilinogen from the small intestine enters the large intestine, where, under the action of bacteria, it is converted into stercobilinogen and excreted in the feces. The amount of fecal stercobilinogen and stercobilin varies from 47 to 276 mg / day, depending on body weight and gender.

Less than 2% of bilirubin is excreted in the urine as urobilin.

Clinical picture

Mild jaundice, including icterus of the sclera, is the main symptom of the disease. In some cases, staining of the skin occurs (Fig. 6-2, a), especially the feet, palms, nasolabial triangle, armpits.

Rice. 6-2. Gilbert's syndrome: a - a patient - a participant in a beauty contest; b - ultrasound: no changes; c - a macropreparation of the liver with accumulation of lipofuscin

Patients should be examined in daylight. Under electric lighting, skin color is distorted and can be misinterpreted.

The yellowness of the skin and visible mucous membranes becomes clearly visible when the level of bilirubin in the blood serum reaches 43-50 μmol / L and higher.

Jaundice and hyperbilirubinemia are intermittent, so these symptoms are rarely permanent. Stress (for example, during exams or during high physical exertion caused by heavy lifting) contributes to the appearance of jaundice and increased icterus of the sclera. Various operations, colds, improper diet, fasting, drinking alcohol and some types of drugs contribute to the intensification of symptoms. Total bilirubin in Gilbert's syndrome ranges from 21 to 51 μmol / L and periodically rises to 85-140 μmol / L.

In half of the cases, dyspeptic complaints are observed: flatulence, stool disturbance, nausea, belching, lack of appetite. The onset of jaundice can be accompanied by discomfort in the liver and weakness.

The syndrome is associated with connective tissue dysplasia (especially often similar to the Marfan and Ehlers-Danlos syndromes).

Diagnostics

Diagnosing a disease involves testing.

Serum bilirubin test, which rises against the background of starvation. The patient receives food for 2 days, the energy value of which does not exceed 400 kcal / day. The level of bilirubin in blood serum is determined on an empty stomach and after 48 hours. The sample is positive if its rise is

50-100%.

Phenobarbital test- the level of bilirubin decreases while taking phenobarbital due to the induction of conjugated liver enzymes.

Nicotinic acid test- intravenous administration of the drug causes an increase in the level of bilirubin due to a decrease in the osmotic resistance of erythrocytes.

The result of a stool test for stercobilin is usually negative.

Liver tests, in particular the levels of the enzymes AST, ALT, ALP, etc., as a rule, are within normal limits or slightly increased. An increase in total protein and dysproteinemia may be observed; prothrombin time - within normal limits. There are no markers of hepatitis B, C, D viruses.

Molecular diagnostics include DNA analysis of the UDFGT gene.

With the help of ultrasound of the abdominal organs, the size and condition of the liver parenchyma are determined (Fig. 6-2, b); size, shape, wall thickness, possible calculi in the gallbladder and bile ducts.

If there are indications to exclude chronic hepatitis (CG), liver cirrhosis, a percutaneous puncture biopsy of the liver with a morphological assessment of the biopsy is performed.

Pathomorphology

Morphological changes in the liver are characterized by fatty degeneration of hepatocytes and the accumulation of yellowish-brown lipofuscin pigment in them, more often in the center of the lobules along the bile capillaries (Fig. 6-2, c).

Differential diagnosis

Differential diagnosis is carried out with all types of hyperbilirubinemia (Table 6-1), hemolytic anemias, congenital cirrhosis of the liver and hepatitis, atresia of the bile ducts or small intestine, etc.

Table 6-1. Differential diagnosis of hereditary hepatosis

Treatment

Patients, as a rule, do not need special treatment, since Gilbert's syndrome is not a disease, but an individual, genetically determined feature of the body. Observance of the regime of study, work, rest, nutrition is of fundamental importance.

Alcoholic beverages and fatty foods are highly undesirable, physical overload (professional sports), sun exposure, long breaks between meals, and fluid restriction are not recommended.

Components of therapy and prevention of exacerbations of Gilbert's syndrome:

Diet therapy;

Elimination of provoking factors (infections, physical and mental stress, the use of hepatotoxic drugs and alcohol);

Contraindication to insolation.

An episode of jaundice can resolve on its own, without the use of drugs.

If the bilirubin level reaches 50 μmol / l and is accompanied by poor health, it is possible to take phenobarbital in a short course (1.5-2.0 mg / kg, or 30-200 mg / day in 2 doses for 2-4 weeks). Phenobarbital (luminal *) is part of such drugs as corvalol *, barboval *, valocordin *, therefore, sometimes they prefer to use these drugs (20-30-40 drops 3 times a day for 1 week),

although the effect of such treatment is observed in only a small proportion of patients. The inducers of enzymes of the monoxidase system of hepatocytes, in addition to phenobarbital, include zixorin (flumecinol *), prescribed to adolescents at a dose of 0.4-0.6 g (4-6 capsules) once a week or 0.1 g 3 times a day in within 2-4 weeks. Under the influence of these drugs, the level of bilirubin in the blood decreases, dyspeptic symptoms disappear, but in the process of treatment, lethargy, drowsiness, and ataxia occur. In such cases, these drugs are prescribed in minimal doses before bedtime, which allows them to be taken for a long time.

Due to the fact that a significant part of patients develop cholecystitis and gallstone disease, it is recommended to take infusions of choleretic herbs, periodic tubing of sorbitol (xylitol), Karlovy Vary salt, etc. Hepatoprotectors are shown: ursodeoxycholic acid preparations (ursosan *, ursofalk *), phospholipids (Essentiale *), Silibinin (Carsil *), Milk Thistle Fruit Extract (Legalon 70 *), Field Artichoke Leaf Extract (Hofitol *), Liv 52 *; choleretics: cholagol *, cholenzyme *, allochol *, berberine *, cholosas *; vitamin therapy, especially B vitamins.

The elimination of conjugated bilirubin is possible with the help of enhanced diuresis, the use of activated carbon, which adsorbs bilirubin in the intestine.

Thermal physiotherapy for the liver area is contraindicated.

Through phototherapy, the destruction of bilirubin fixed in tissues is achieved, thereby releasing peripheral receptors that can bind new portions of bilirubin, preventing its penetration through the blood-brain barrier.

Prophylaxis

Prevention includes adherence to the regime of work, nutrition, rest. Avoid significant physical exertion, fluid restriction, fasting and hyperinsolation. The use of alcoholic beverages, hepatotoxic drugs is unacceptable.

Gilbert's syndrome is not a reason to refuse vaccinations.

Reorganization of chronic foci of infection and treatment of the existing pathology of the biliary tract are mandatory.

Forecast

The prognosis is favorable. Hyperbilirubinemia persists for life, but is not accompanied by progressive changes in the liver and increased mortality. With life insurance, such people are classified as a normal risk group. When treated with phenobarbital, the bilirubin level is reduced to normal values. The development of inflammation in the biliary tract, gallstone disease, psychosomatic disorders is possible.

Parents of children with this syndrome should consult a geneticist before planning their next pregnancy.

The same should be done if relatives of a married couple planning to have children are diagnosed with this syndrome.

FAT LIVER DEGENERATION

ICD-10 code

K76.0. Fatty degeneration of the liver.

Hepatosis (liver steatosis, non-alcoholic steatohepatitis) is a group of liver diseases, which are based on metabolic disorders in hepatocytes and the development of dystrophic changes in liver cells, while inflammatory phenomena are absent or mild.

In recent years, there has been a significant increase in the incidence of fatty liver degeneration, mainly associated with an increase in the prevalence of obesity. Among patients who underwent liver biopsy, approximately 7-9% of cases of hepatosis in Western countries and 1-2% in Japan are detected.

Etiology and pathogenesis

The causes of the disease are considered obesity, diabetes mellitus, dyslipidemia, rapid weight loss, lack of protein in the diet, congenital defects in β-oxidation of fatty acids, α-1-antitrypsin deficiency, exposure to liver toxic substances, including alcohol, etc. Hepatosis can be both an independent disease and a manifestation of other diseases.

Excessive fat accumulation in liver tissue (in hepatocytes and Ito cells) can result from first impact(Fig. 6-3, a, d) - saturated with lipids, simple carbohydrates and high calorie food:

Increasing the supply of free fatty acids to the liver;

Reducing the rate of β-oxidation of free fatty acids in liver mitochondria;

Increased synthesis of fatty acids in liver mitochondria;

Reducing the synthesis or secretion of very low density lipoproteins and the export of triglycerides in their composition.

The result of a violation of the diet is insulin resistance and fatty liver infiltration.

Second impact(see Fig. 6-3, d) implies a violation of the excretion of lipids from the liver, which occurs when the amount of substances involved in their processing (protein, lipotropic factors) decreases. The formation of phospholipids, β-lipoproteins, lecithin from fats is impaired. In the pathogenesis, tumor necrosis factor-α, endotoxin, immune factors are important. It is assumed that, regardless of the reasons for the development of steatosis, the basis of inflammatory-necrotic changes in the liver are universal mechanisms. Being highly reactive compounds, free fatty acids serve as a substrate for lipid peroxidation. The generated free radicals cause the destruction of lipid, protein components of membranes, liver receptors, etc., causing further changes in the liver.

Classification

Distinguish between pigmentary and fatty hepatosis. Most often, the term "hepatosis" means fatty hepatosis (steatosis), since pigmentary hepatosis occurs much less frequently and is considered separately (see "Rare syndromes"), with the exception of Gilbert's syndrome.

Clinical presentation and diagnosis

In the initial stages, symptoms are minimal. As a rule, the course of the disease is latent, only an increase in the activity of hepatic transaminases and hepatomegaly are noted. In many patients, liver dysfunctions are diagnosed by chance, during examination for other diseases. There is a minimal or moderately pronounced activity of inflammation in the liver, detected by biochemical studies of blood serum. However, without treatment, a transition to cirrhosis of the liver can be observed, the phenomena of liver failure gradually increase.

Fatty hepatosis is often concluded by doctors of ultrasound diagnostics on the basis of characteristic signs: a uniform increase in the liver, a diffuse increase in its echogenicity (sometimes pronounced) while maintaining its homogeneity, although with the progression of the process, a characteristic granularity of the parenchyma appears, indicating the onset of the development of steatohepatitis and hepatitis (Fig. 6-3, b).

Pathomorphology

According to morphological studies, steatohepatitis is an excessive accumulation of triglycerides in the liver, which is accompanied by damage to cell membranes and other organelles of hepatocytes, an inflammatory process, fibrosis up to liver cirrhosis (Fig. 6-3, c).

Rice. 6-3. Functions and diseases of the liver: a - participation of the liver in lipid metabolism; b - ultrasound: hepatomegaly and increased echogenicity of the liver; c - macropreparation: liver steatosis; d - staged formation of liver pathology

Treatment

Diet therapy is a permanent and safe method of treating fatty liver disease.

In order to normalize the oxidation of fatty acids in mitochondria, improve the transport of triglycerides from the liver, and reduce the processes of lipid peroxidation, drugs that improve lipid metabolism are prescribed - hepatoprotectors, vitamin B 12, folic acid, thioctic acid (lipoic acid *), etc.

Prophylaxis

Healthy lifestyles and healthy eating are the cornerstones of primary prevention (Figure 6-4). Adequate physical activity is recommended.

Rice. 6-4. Nutritional pyramid for fatty liver degeneration

Dispensary observation is described below (see "Prevention of chronic hepatitis").

Forecast

With the exclusion of causative factors and timely treatment, recovery is possible, however, hepatosis can transform into chronic hepatitis and cirrhosis (see Fig. 6-3, d).

CHRONIC HEPATITIS

ICD-10 code

K73. Chronic hepatitis.

Chronic hepatitis is a group of diseases accompanied by the development of a diffuse inflammatory process in the liver, lasting more than 6 months, confirmed by biochemical parameters, the results of morphological examination of the liver, as well as specific markers in the blood serum.

The prevalence of hCG has not been precisely established due to the large number of erased and asymptomatic forms, the lack of population studies. Chronic viral hepatitis (CVH) caused by persistence of hepatitis B viruses (29.2%), C (33.3%), chronic hepatitis B + C (16.7%), less often B + D (4.1 %), D + G (no more than 2%). In 16.7% of cases, hepatitis of unknown etiology is detected.

Classification

The modern classification of hepatitis is presented in table. 6-2. Taking into account the etiology, the following types of hepatitis are distinguished.

. Specific viral hepatitis. The main forms of such hepatitis are hepatitis A, B and C. Hepatitis D is less common in the world. Hepatitis E remains a major problem in developing countries. Other hepatitis viruses (G, TTV, etc.) have been described, but their clinical significance is not great.

. Nonspecific viral hepatitis are caused by a group of viruses that can infect both the liver and other organs. For example, the virus of infectious mononucleosis (Epstein-Barr virus) selectively affects the cells of the reticuloendothelial system (clinically manifested in the form of angina, hypersplenism, hepatitis, etc.). Adenovirus causes pharyngoconjunctival fever, acute pneumonia, hepatitis. Herpes simplex virus is an AIDS indicator infection.

Hepatitis - manifestation of an etiologically independent disease(with leptospirosis, pseudotuberculosis).

Hepatitis associated with the use of drugs - toxic-allergic and medicinal hepatitis. Alcoholic hepatitis is a combined lesion with acetaldehyde and some other factor.

. Nonspecific reactive hepatitis- the reaction of liver cells to the pathology of neighboring organs: pancreas, gallbladder, duodenum. Reactive hepatitis develops in patients with chronic pancreatitis, duodenal ulcer.

Among autoimmune forms of chronic hepatitis identified 3 types of diseases (see table. 6-2).

Row rare liver diseases may have clinical and histological features of chronic persistent hepatitis:

Primary biliary cirrhosis;

Wilson-Konovalov's disease;

Primary sclerosing cholangitis;

Lack of α-1-antitrypsin.

The stage of fibrosis is established on the basis of pathomorphological examination of liver biopsies (Table 6-3), roughly - according to ultrasound data (Table 6-4).

Table 6-2. Classification of Chronic Hepatitis (International Expert Group, Los Angeles, 1994)

* Established according to the results of histological examination of liver tissue and tentatively - according to the degree of ALT and AST activity (1.5-2 norms - minimum, 2-5 norms - low, 5-10 norms - moderate, above 10 norms - pronounced). ** Established on the basis of morphological examination of the liver and approximately - according to ultrasound data.

Table 6-3. Index of histological activity of hepatitis in points (Knodell R..J. Et al., 1994)

Note: 1-3 points - the minimum degree of activity of chronic hepatitis; 4-8 - chronic hepatitis of moderate severity; 9-12 points - moderate chronic hepatitis; 13-18 points - severe chronic hepatitis.

Table 6-4. Ultrasound criteria for the stages of liver fibrosis in chronic hepatitis in children

Mixed hepatitis established as the main diagnosis in the presence of simultaneous replication of 2 types of virus or more. With the replication of one and the integration of the other, the main hepatitis and concomitant hepatitis are established.

Chronic viral hepatitis

ICD-10 codes

B18. Chronic viral hepatitis.

818.0. Chronic viral hepatitis B with D-agent.

818.1. Chronic viral hepatitis B without D-agent.

818.2. Viral hepatitis C is chronic.

818.8. Other chronic viral hepatitis.

818.9. Unspecified chronic viral hepatitis. In more than 70% of cases, the cause of chronic hepatitis is hepatotropic viruses B, C and D. There are 350-400 million people infected with the hepatitis B virus in the world, and about 1 million people die annually from diseases associated with hepatitis B virus (HBV) infection. ... The prevalence of HBV infection in different countries ranges from 0.1 to 20%. The risk of acute HBV infection becoming chronic with age decreases: with perinatal infection it reaches 90%, with infection at the age of 1-5 years - 25-35%, and with infection of adults - less than 10%.

Etiology and pathogenesis

The mechanism of formation, diagnosis of hepatitis B and C are shown in Fig. 6-5. Viral hepatitis B (8 main genotypes - A-H) is found in blood and other biological fluids (semen, saliva, nasopharyngeal mucus), transmitted in four main ways:

Sexual;

Perinatal (from mother to child in the prenatal period and during childbirth);

Parenteral (through blood);

Horizontal (with close household contact or through infected common objects; mainly observed in early childhood).

In children, the main route of transmission of viral hepatitis B is perinatal. If a pregnant woman is a carrier of viral hepatitis B (and, in addition, is HBeAg-positive), the probability of infection of a newborn with the development of a carrier of the virus is 90%. As adults, 25% of these children die from chronic liver failure or liver cancer. Although HBsAg, HBeAg and HBV DNA are found in breast milk, the type of feeding does not affect the risk of HBV transmission. Other risk factors for contracting hepatitis B include:

Transfusion of blood and / or its components;

Injection of drugs, tattoos, piercings and other invasive procedures on the skin;

Unprotected penetrating sex, especially anal and vaginal intercourse;

Organ transplant;

Work in medical institutions;

Hemodialysis.

In regions with a low endemicity of HBV infection, adolescents and young people have the highest incidence. The most common routes of transmission of viral hepatitis B in these groups are sexual and parenteral (with unsafe drug injections, in particular, the repeated use of disposable syringes).

It is believed that chronic hepatitis B(CHB) is a disease that is primarily chronic or arising after an erased or subclinical form of acute infection.

CHB phases:

Initial, or immune tolerance;

Immune response (replicative), proceeding with pronounced clinical and laboratory activity;

Integrative;

Carriage of HBsAg.

The hepatitis B DNA virus (HBV DNA) itself does not cause cytolysis. Damage to hepatocytes is associated with immune responses in response to circulating viral and hepatic antigens. In the 2nd phase of viral replication, viral antigens are expressed: HBsAg (surface), HBcAg, (nuclear), HBeAg (Fig. 6-5, a), the immune response is more pronounced, which causes massive necrosis of the liver parenchyma and further mutation of the virus.

Replication of the hepatitis B virus is also possible outside the liver - in bone marrow cells, mononuclear cells, thyroid and salivary glands, which causes extrahepatic manifestations of the disease.

Transmission routes chronic hepatitis C(CHC) are similar to those with CHB. Unlike viral hepatitis B, the hepatitis C RNA virus has a direct hepatotoxic effect. As a result, the replication of the virus and its persistence in the body are associated with the activity and progression of hepatitis. It is interesting that viral hepatitis C is able to block apoptosis (programmed death) of cells affected by it in order to stay in the human body for a long time. Apoptosis is a normal process that rid the body of "worn out" or diseased cells. A protein encoded in the genome of hepatitis C virus, known as NS5A, blocks the opening of potassium channels in liver cells, protecting their "shelters" from natural death and thus staying in the human body for a long time. The life cycle of viral hepatitis C is shown in Fig. 6-5, b.

Rice. 6-5. Chronic hepatitis C and B: a - diagnosis of hepatitis C and B and the dynamics of serological markers of hepatitis B; b - life cycle of the hepatitis C virus

Causative agent chronic hepatitis D(HGO) is an RNA-containing particle, the outer shell of which is represented by HBsAg. In the center of the particle is the antigen of the hepatitis D virus. The delta virus is able to multiply in the liver cells only in the presence of hepatitis B virus, since its proteins are used to leave the cell of the delta virus particle. The disease proceeds simultaneously with viral hepatitis B in the form of a coinfection or superinfection.

Clinical picture

The clinical picture of chronic hepatitis is weak and nonspecific. Asymptomatic course is observed in 25% of patients. The formation of chronic hepatitis occurs more often in the outcome of acute hepatitis, proceeding in the form of atypical (erased, anicteric, subclinical) forms and extremely rarely - with manifest (icteric) forms of acute hepatitis. The acute phase of hepatitis and the appearance of clinical symptoms of the chronic form of the disease are separated by 5 years or more.

Clinical manifestations of hCG depend on the child's age at the time of infection, the severity of morphological

changes in the liver, phases of the infectious process (replication, integration), premorbid background. In children, unlike adults, cholestatic variant HCG is rare; in the presence of cholestasis, it is necessary to exclude congenital pathology of the intra or extrahepatic passages, α-1-antitrypsin deficiency, cystic fibrosis. The main syndromes of the disease are shown in table. 6-5.

Table 6-5. The main syndromes of chronic viral hepatitis

Extrahepatic manifestations associated with extrahepatic replication of the virus, more characteristic of CHC, can manifest as recurrent dermatitis, hemorrhagic vasculitis, glomerulonephritis, arthropathies, thyroiditis, Sjogren's syndrome, pancreatopathies. Extrahepatic manifestations often develop at puberty, girls are characterized by the development of endocrine disorders, and boys develop glomerulonephritis and other diseases.

Extrahepatic manifestations include vascular changes (Table 6-6; Fig. 6-6). In children, they are much less common, their presence obliges an extended study of liver function.

Table 6-6. Vascular extrahepatic manifestations in chronic hepatitis

Rice. 6-6. Vascular extrahepatic manifestations in chronic hepatitis: a - telangiectasia; b - capillary; c - palmar erythema

Diagnostics

Specific methods. With the help of enzyme-linked immunosorbent assay (ELISA), the main markers of hCG are detected, with the help of polymerase chain reaction (PCR) - DNA or RNA virus (Table 6-7; Fig. 6-5, a).

Table 6-7. Marker diagnostics of chronic hepatitis B and C

Serological markers viral hepatitis B is used to establish the diagnosis and stage of the disease.

Antigens were presented above (see Fig. 6-5, a). Antibodies to the surface antigen of the virus (anti-HBsAg) appear in the blood after 3-6 months and persist for many years or possibly for life. Their detection indicates either a previous infection or a previous vaccination.

Nuclear antigen (HBcAg) in the blood usually does not circulate, however, antibodies to it appear in the early stages of the disease, their titer quickly reaches a maximum, and then gradually decreases (but does not completely disappear). First, antibodies of the IgM class (anti-HBcAg IgM) appear, then IgG appears. Antigen E (HBeAg) appears in the blood for a short time at the onset of the disease, which is accompanied by the production of antibodies to it (anti-HBe).

Chronic CHB infection is characterized by the presence of HBsAg and anti-HBcAg IgG in the blood.

In CHC, in addition to viremia (HCV RNA), antibodies of the IgM and IgG classes are detected. Without exacerbation of RNA, HCV and anti-HCV IgM are not detected, but antibodies of the IgG class remain (see Tables 6-7).

TO non-specific methods include biochemical, immunological tests and instrumental studies.

Biochemical tests do not carry information about the etiology of the disease, but reflect the nature of liver damage and the state of its function. These include:

An increase in the level of liver enzymes: in chronic hepatitis, an increase in ALT is more pronounced than AST, which is associated with different localization of enzymes (ALT - in the cytoplasm, AST - in mitochondria), in cirrhosis, on the contrary, the activity of AST prevails over that of ALT; also characterized by an increase in enzymes such as lactate dehydrogenase, γ-glutamyl transpeptidase,

ALF;

Violation of fat and pigment metabolism: an increase in the direct fraction of bilirubin, the content of total cholesterol, β-lipoproteins, alkaline phosphatase activity, 5-nucleotidase;

Violation of the protein-synthetic function of the liver: a decrease in total protein, an increase in the thymol test, a decrease in sublimate test, a decrease in the level of prothrombin, persistent dysproteinemia due to an increase in globulin fractions, especially γ-globulins, and a decrease in albumin.

Biochemical syndromes reflecting liver dysfunctions are presented in Chapter 1 (see Table 1-8, changes in protein fractions - Fig. 1-16, b).

Immunological tests. Characterized by a decrease in the levels of T-suppressors, an increase in the levels of serum immunoglobulins.

Instrumental methods. Ultrasound of the liver is an obligatory research method for chronic hepatitis, since it allows visualizing the liver, determining its size, revealing liver cirrhosis and portal hypertension. Even with an asymptomatic course of the disease, this method can reveal an increase in the liver, a change in the echogenicity of the parenchyma. Reohepatography, puncture biopsy of the liver can be used.

Today liver biopsy is the gold standard for the diagnosis of liver diseases (Fig. 6-7, a). During the biopsy, a piece of liver with a diameter of about 1 mm is obtained using a special needle. The procedure is carried out under local or general anesthesia and under ultrasound control, since it is necessary to control the needle movement, which makes the manipulation safe.

The degree of activity of hCG is most often assessed using a semi-quantitative histological index of activity, also known as the Knodell system, determined in points (see Table 6-3). The histology of a biopsy (tissue sample) of the liver allows you to make a decision about the need and tactics of antiviral therapy.

Pathomorphology

Morphological examination of liver biopsies already in the first months of a child's life with primary chronic hepatitis reveals signs of inflammation that persist for many years, as well as progressive fibrosis with the formation of liver cirrhosis.

Rice. 6-7. Diagnostics of chronic hepatitis: a - biopsy technique; histological picture: b - CHB (staining with hematoxylineosin; χ 400); c - CHC (x 400).

CHB is characterized by necrosis (Fig. 6-7, b); a pathognomonic sign in CHC is vacuolization of hepatocyte nuclei, the so-called opaque-vitreous hepatocytes, as well as their stepwise necrosis (Fig. 6-7, c).

Differential diagnosis

Treatment

V replication phase (exacerbation) hospitalization in a specialized department, bed rest, strict diet therapy are shown.

Basic therapy includes appointment antiviral drugs. Indications for its appointment:

The presence of markers of active hepatitis replication;

ALT levels are more than 2-3 times higher than normal;

Absence of cholestasis and signs of liver cirrhosis with decompensation;

Absence of severe concomitant diseases in the stage of decompensation;

Absence of autoimmune diseases, immunodeficiency state, mixed hepatitis.

Interferon inducers are characterized by low toxicity and the absence of side effects, in contrast to interferon preparations, thanks to their use, it is possible to significantly increase the life expectancy in children and adults (Fig. 6-8).

Rice. 6-8. Chronic hepatitis (course and treatment): a - antiviral treatment of children and adults with chronic viral hepatitis B and C and the years of life gained; b - natural course of hepatitis B

Interferon preparations contraindicated in psychosis, epidemic syndrome, severe neutro- and thrombocytopenia, autoimmune diseases (AIH, thyroiditis, etc.), decompensated liver cirrhosis and kidney disease, heart disease in the stage of decompensation.

Interferon-a-2b (reaferon *, roferon *, neuroferon *) - lyophilisate for preparation of suspension for oral administration - is prescribed 30 minutes before meals, 1-2 ml of chilled boiled water are added to the contents of the bottle before use. The drug in injections is injected with CHB at a dose of 5 million IU / m 2, with CHC - 3 million IU / m 2 body surface area three times a week (1 time with an interval of 72 hours) s / c or i / m. The calculated dose of interferon is initially administered within 3 months. After this period, a control study is carried out (RNA or DNA of the virus, activity). If there is no clear positive dynamics of these indicators (the disappearance of RNA, virus DNA from the blood, a decrease in ALT), it is better to stop treatment according to this scheme or switch to combination therapy. But if there is a decrease in ALT activity, a drop in the concentration of RNA, DNA of the virus in the blood, treatment according to the chosen scheme is continued for another 3 months, followed by a control

laboratory research. With positive dynamics in CHC, treatment is continued for 3 months to consolidate the results of treatment. Thus, the course of treatment for CHB is 6 months, for CHC - 9-12 months.

In pediatric practice, Viferon is used (a combination of α-interferon with membrane stabilizers), which is produced in rectal suppositories. Doses for children: up to 3 years old - 1 million IU, over 3 years old - 2 million IU 2 times a day with an interval of 12 hours 3 times a week. In patients treated according to the protocol program using Viferon, the effectiveness of treatment is assessed according to the above principles. If in this category of patients during the control study 3 months after the start of therapy there is no positive effect, then Viferon can be replaced with Reaferon *, Roferon *.

The inductor of α-interferon meglumine acridone acetate (cycloferon *) is administered with chronic hepatitis at 6-10 mg / kg per day, 10 injections daily, then 3 times a week for 3 months as a complex therapy.

The antiviral drug tilorone (amiksin) is prescribed to children over 7 years old in tablets of 0.125 orally after meals, the first 2 days daily, then 125 mg every other day - 20 tablets, then 125 mg once a week for 10-20 weeks. The course of treatment for HCA is 2-3 weeks, for CHB - 3-4 weeks.

In CHB against the background of viral replication, the antiviral chemotherapy drug lamivudine (zeffix, epivir *) in oral solution and tablets is recommended. Dosed at 3 mg / kg per day for children from 3 months of age, but not more than 100 mg orally 1 time per day for a course of 9-12 months. Tablets of 100 mg 1 time per day are prescribed to adolescents (16 years of age and older) by mouth, regardless of food intake.

In general, interferon therapy is effective in 40% of patients with CHB and in 35% of patients with CHC, but in 10-30% of patients after the end of treatment, relapses of the disease are possible.

In severe chronic hepatitis C is prescribed glucocorticoids: prednisolone or methylprednisolone tablets 0.001; 0.0025 and 0.005 mg at 1-2 mg / kg per day in 2 divided doses without taking into account the daily rhythm. After achieving remission, the dose is reduced by 5-10 mg to a maintenance dose of 0.3-0.6 mg / kg per day: 10-15 mg / day of prednisolone or 8-12 mg / day of methylprednisolone.

Criteria for the effectiveness of treatment:

. biochemical - the most informative is the determination of the ALT level, and during treatment, the ALT activity should be determined throughout the course and for another 6 months after cancellation, and then every 3-6 months for 3 years;

Virological - determination of RNA, DNA of the virus using PCR;

Histological - the most informative for assessing the effectiveness of treatment, but in practice they are not always realizable, especially in pediatrics.

Biochemical remission at the end of treatment involves the normalization of enzyme levels immediately after the end of the course of therapy; complete remission- normalization of AST and ALT levels and the disappearance of RNA, DNA of the virus immediately after treatment; stable biochemical remission- maintaining the normal value of transaminases after 6 months or more after discontinuation of therapy; stable complete remission- maintenance of normal levels of AST and ALT and the absence of RNA, DNA of the virus 6 months after treatment.

If a stable complete remission is achieved, it is recommended to continue monitoring the patient for at least 2 years with a frequency of 1 every six months. In the remission phase (CVH integration phase), antiviral therapy is usually not carried out, the treatment consists of the organization of the diet, regimen, the inclusion of probiotics, enzymes, herbal remedies, laxatives according to indications to prevent gastrointestinal dysfunction and intestinal autointoxication.

Accompanying therapy is a symptomatic and pathogenetic treatment.

In order to stop cholestasis, ursodeoxycholic acid preparations (ursosan *, urdoksa *, ursofalk *) are used as monotherapy in the non-replicative phase of hepatitis, in the replicative phase - in combination with interferons up to 6-12 months at 10 mg / kg once a day before bedtime.

Hepatoprotectors with the ability to protect hepatocytes are prescribed in courses of up to 1.5-2 months. Repeated course - in 3-6 months according to indications.

Artichoke leaf extract (chophytol *) is a herbal remedy that has hepatoprotective and choleretic effects. Hofitol * is prescribed for children over 6 years old in 1-2 tablets or 1/4 tsp. solution for oral administration 3 times a day before meals, adolescents - 2-3 tablets or 0.5-1 tsp. solution 3 times a day, course - 10-20 days. Solution for intramuscular or intravenous slow administration - 100 mg (1 ampoule) for 8-15 days; average doses can be significantly increased, especially in inpatient treatment.

Hepatoprotector "Liv 52 *" is a complex of biologically active substances of plant origin; it is prescribed for children over 6 years old 1-2 tablets 2-3 times a day, for adolescents 2-3 tablets 2-3 times a day.

Ademetionine (Heptral *) is a hepatoprotector that has choleretic and cholekinetic, as well as some antidepressant effect. Children are prescribed with caution orally, intramuscularly, intravenously. With intensive care in

the first 2-3 weeks of treatment - 400-800 mg / day intravenously slowly or intramuscularly; the powder is dissolved only in a special supplied solvent (L-lysine solution). For maintenance therapy - 800-1600 mg / day orally between meals, without chewing, preferably in the morning.

Prophylaxis

The main preventive measures should be aimed at preventing infection with hepatitis viruses, therefore, early detection of patients with erased forms of the disease and their adequate treatment are required. Carriers of HBsAg require regular (at least once every 6 months) monitoring of biochemical and virological parameters in order to prevent the activation and replication of the virus.

Recombinant vaccines are used for vaccination against hepatitis B: "Biovac B *", "Engerix B *", "Euvax B *", "Shanvak-B *", etc. RD for newborns and children under 10 years of age - 10 μg (0, 5 ml of suspension), for children over 10 years old - 20 μg (1 ml of suspension).

Newborns born to mothers - carriers of hepatitis B, along with the vaccine, are recommended to administer immunoglobulin against hepatitis B, while the drugs should be injected in different places. In accordance with the rules existing in the Russian Federation, this category of children is vaccinated four times according to the scheme: 0 (on the birthday) -1- 2-12 months of age. Against hepatitis B, adolescents aged 11-13 years old must be vaccinated according to the same scheme.

Medical workers and people from risk groups for hepatitis B infection are widely vaccinated. Vaccination leads to a gradual decrease in the level of infection of the population of the Russian Federation with the hepatitis B virus.

A vaccine against hepatitis C has not yet been developed, and therefore the prevention of hepatitis C is based on the suppression of all the possibilities of parenteral (including transfusion) infection.

Dispensary observation is described below.

Forecast

The likelihood of complete recovery is negligible. With CHB, there is a long-term persistence of the pathogen virus, possibly a combination with an active pathological process. On average, after 30 years, 30% of patients with chronic active hepatitis B develop cirrhosis of the liver. Within 5 years, approximately every fourth patient with cirrhosis caused by hepatitis B develops liver function decompensation, another 5-10% of patients develop liver cancer (see Fig. 6-8). Without treatment, approximately 15% of patients with cirrhosis die within 5 years. In 1-1.5% of cases, cirrhosis is formed, and in the remaining 89%, long-term remission occurs with HBsAg carriage. With ΧΓD, the prognosis is unfavorable: in 20-25% of cases, the process flows into liver cirrhosis; release from the pathogen does not occur. CHC flows slowly, gently, without stopping viremia for many years, with a periodic increase in transaminase activity and with a pronounced tendency to fibrosis. As the process progresses, liver cirrhosis and hepatocellular carcinoma develop.

AUTOIMMUNE HEPATITIS

ICD-10 code

K75.4. Autoimmune hepatitis.

AIH is a progressive hepatocellular inflammation of the liver of unknown etiology, characterized by the presence of periportal hepatitis, frequent association with other autoimmune diseases, increased concentration of immunoglobulins (hypergammaglobulinemia), and the presence of autoantibodies in the blood.

Like other autoimmune diseases, AIH is more common in females, with an overall incidence of approximately 15-20 cases per 100,000 population. In childhood, the proportion of AIH among chronic hepatitis ranges from 1.2 to 8.6%, observed at the age of 6-10 years. The ratio of girls to boys is 3-7: 1.

Etiology and pathogenesis

The pathogenetic mechanism of development of AIH is based on a congenital defect in membrane HLA receptors. Patients have a defect in the function of T-suppressors linked by the HLA haplotype; as a result, uncontrolled synthesis of IgG antibodies by B-lymphocytes occurs, destroying the membranes of normal hepatocytes, and pathological immune reactions against their own hepatocytes develop. Often, not only the liver is involved in the process, but also large glands of external and internal secretion, including the pancreas, thyroid, salivary glands. Genetic predisposition (immunoreactivity to autoantigens) is considered as the main factor in the pathogenesis of AIH, which, however, is not sufficient in itself. It is believed that triggering agents (triggers) are needed to implement the process, among which viruses (Epstein-Barr, measles, hepatitis A and C) and some drugs (for example, interferon preparations) and unfavorable environmental factors are considered.

Rice. 6-9. AIH pathogenesis

The pathogenesis of AIH is shown in Fig. 6-9. The effector mechanism of hepatocyte damage is probably more related to the reaction of autoantibodies to hepato-specific antigens of hepatocytes than to direct T-cell cytotoxicity.

Classification

Currently, there are 3 types of AIH:

- type 1- the classic version, it accounts for 90% of all cases of the disease. Detect antibodies to anti-smooth muscle cells (Smooth Muscle Antibody- SMA) and nuclear antigens (liver-specific

squirrel - Antinuclear antibodies- ANA) in the title of more than 1:80 in adolescents and more than 1:20 in children;

-type 2- makes up about 3-4% of all cases of AIH, most of the patients are children from 2 to 14 years old. Detect antibodies to liver and kidney microsomes (Liver Kidney Microsomes- LKM-1);

-type 3- characterized by the presence of antibodies to soluble hepatic antigen (Soluble Liver Antigen- SLA) and hepato-pancreatic antigen (LP).

Some features of AIG, taking into account the types, are presented in table. 6-8.

Table 6-8.Classification and features of AIH types

Clinical picture

The disease in 50-65% of cases is characterized by the sudden onset of symptoms similar to those in viral hepatitis. In some cases, it begins gradually and is manifested by increased fatigue, anorexia and jaundice. Other symptoms include fever, arthralgia, vitiligo (pigmentation abnormalities resulting in the disappearance of the melanin pigment in certain areas of the skin), and nosebleeds. The liver protrudes from under the edge of the costal arch by 3-5 cm and becomes denser, there is splenomegaly, the abdomen is enlarged (Fig. 6-10, a). As a rule, extrahepatic signs of chronic liver pathology are detected: spider veins, telangiectasias, palmar erythema. Some patients have a cushingoid appearance: acne, hirsutism and pink striae on the thighs and abdomen; 67% are diagnosed with other autoimmune diseases: Hashimoto's thyroiditis, rheumatoid arthritis, etc.

Diagnostics

Diagnosis is based on the detection of syndromes of cytolysis, cholestasis, hypergammaglobulinemia, an increase in IgG concentration, hypoproteinemia, a sharp increase in ESR, confirmed by the detection of autoantibodies against hepatocytes.

Characteristic hypersplenism syndrome, its signs:

Splenomegaly;

Pancytopenia (a decrease in the number of all blood cells): anemia, leukopenia, neutropenia, lymphopenia, thrombocytopenia (with a sharp degree of severity, bleeding syndrome appears);

Compensatory hyperplasia of the bone marrow.

In diagnostics, instrumental research methods (scanning, liver biopsy, etc.) are of absolute importance.

Pathomorphology

Morphological changes in the liver with AIH are characteristic, but nonspecific. HCG, as a rule, turns into multilobular cirrhosis of the liver (Fig. 6-10, b); characterized by a high degree of activity: periportal

necrosis, port-portal or central-portal bridge necrosis, less often - portal or lobular hepatitis, mainly lymphocytic infiltration with a large number of plasma cells, the formation of rosettes (Fig. 6-10, c).

Rice. 6-10. AIH: a - a child with an outcome in liver cirrhosis; b - macropreparation: macronodular cirrhosis; c - micropreparation: histological picture (staining with hematoxylin-eosin; χ 400)

Differential diagnosis

Differential diagnosis is carried out with CHB, cholecystitis, Wilson-Konovalov disease, drug hepatitis, α-1-antitrypsin deficiency, etc.

A definite and probable AIH is distinguished. The first option is characterized by the presence of the above indicators, including an increase in autoantibody titers. In addition, there are no viral markers in the blood serum, damage to the bile ducts, copper deposition in liver tissue, there are no indications of blood transfusion and the use of hepatotoxic drugs.

The probable variant of AIH is justified when the existing symptoms make it possible to think about AIH, but are insufficient for making a diagnosis.

Treatment

The basis is immunosuppressive therapy. Prescribe prednisolone, azathioprine or their combinations, which make it possible to achieve clinical, biochemical and histological remission in 65% of patients within 3 years. Treatment is continued for at least 2 years until all criteria are in remission.

Prednisolone is prescribed at a dose of 2 mg / kg (the maximum dose is 60 mg / day) with a gradual decrease by 5-10 mg every 2 weeks under weekly monitoring of biochemical parameters. In the absence of normalization of the level of transaminases, azithioprine is additionally prescribed at an initial dose of 0.5 mg / kg (the maximum dose is 2 mg / kg).

A year after the onset of remission, it is advisable to cancel immunosuppressive therapy, but only after a control puncture biopsy of the liver. Morphological examination should indicate the absence or minimal activity of inflammatory changes.

With the ineffectiveness of glucocorticoid therapy, cyclosporine (sandimmum neoral *) is used for oral administration from the first year of life, which is released in a solution of 100 mg in 50 ml in a vial, capsules of 10, 25, 50 and 100 mg,

the drug is prescribed at a dose of 2-6 mg / kg per day (no more than 15 mg / m 2 per week). Cyclophosphamide (cyclophosphamide *) is prescribed intravenously drip at a dose of 10-12 mg / kg 1 time in 2 weeks, then in tablets of 0.05 g at 15 mg / kg 1 time in 3-4 weeks, course dose - no more 200 mg / kg.

Primary treatment resistance is observed in 5-14% of patients. They are primarily subject to consultation at liver transplant centers.

Prophylaxis

Primary prevention has not been developed, secondary prevention consists in early diagnosis, dispensary observation of patients (described below) and long-term immunosuppressive therapy.

Forecast

The disease without treatment continuously progresses and does not have spontaneous remission - cirrhosis of the liver is formed. In type 1 AIH, glucocorticoids are more effective and the prognosis is relatively favorable: in many cases, it is possible to achieve long-term clinical remission. In AIH type 2, the disease usually progresses rapidly to cirrhosis. Type 3 is not clinically well-defined and its course has not been studied.

With the ineffectiveness of immunosuppressive therapy, patients are shown liver transplantation, after which the 5-year survival rate is more than 90%.

Medicinal hepatitis

ICD-10 code

K71. Medicinal hepatitis.

Medicinal hepatitis - toxic liver damage, including idiosyncratic (unpredictable) and toxic (predictable) medicinal liver disease associated with the intake of hepatotoxic drugs and toxic substances.

Etiology and pathogenesis

The liver plays an important role in the metabolism of xenobiotics (foreign substances). A group of enzymes located in the endoplasmic reticulum of the liver, known as cytochrome P450, is the most important family of enzymes for metabolism in the liver. Cytochrome P450 assimilates about 90% of toxic and medicinal products.

Often, the liver becomes a target for their damaging effects. Direct and indirect types of liver damage are distinguished.

Direct type of liver damage depends on the dose of the drug and is due to the effect of the drug itself on the liver cells and its organelles. To drugs with obligate dose-dependent hepatotoxic action include paracetamol and antimetabolites, leading to necrosis of hepatocytes. Direct liver damage can also be caused by tetracycline, mercaptopurine, azathioprine, androgens, estrogens, etc.

Indirect type of liver damage independent of the dose of drugs, observed when taking nitrofurans, rifampicin, diazepam, meprobamate, etc. This type reflects the individual reaction of the child's body as a manifestation of hypersensitivity to drugs.

The liver is involved in the metabolism of various xenobiotics through biotransformation processes, which are divided into two phases.

. Phase one- oxidative reactions involving cytochromes P450. During this phase, active metabolites can be formed, some of which have hepatotoxic properties.

. Second phase, during which the previously formed metabolites are conjugated with glutathione, sulfate or glucuronide, as a result of which non-toxic hydrophilic compounds are formed, which are excreted from the liver into the blood or bile.

Medicinal, or drug-induced, hepatitis occupies a special place among the toxic liver lesions. Their formation occurs more often as a result of the uncontrolled use of drugs (Fig. 6-11, a). Almost any drug can cause liver damage and the development of hepatitis of varying severity.

Toxins can be roughly divided into household and industrial toxins. There are industrial poisons of organic nature (carbon tetrachloride, chlorinated naphthalene, trinitrotoluene, trichlorethylene, etc.), metals and metalloids (copper, beryllium, arsenic, phosphorus), insecticides (dichlorodiphenyltrichloroethane - DDT, carbofos, etc.).

Rice. 6-11. Drug hepatitis: a - the formation of drug hepatitis with hepatocyte necrosis; b - histological picture of medicinal hepatitis after treatment of acute leukemia (staining with hematoxylin-eosin; χ 400)

Particularly severe forms of damage to hepatocytes develop when poisoning with substances such as paracetamol, pale toadstool poison, white phosphorus, carbon tetrachloride, all industrial poisons.

Clinical picture

Typical forms of liver damage with hepatotoxic effects of drugs are presented in table.

6-9.

Table 6-9. Most common hepatotoxic drug effects

Drug reactions can be transient, chronic hepatitis is rarely observed. Liver function tests can return to normal within a few weeks (up to 2 months) after drug withdrawal, but with cholestatic hepatitis, this period can increase to 6 months. Jaundice always indicates more severe liver damage, possibly the development of acute liver failure.

Diagnostics

The basis for the diagnosis of medicinal liver lesions is a carefully collected history of the drugs used, prescribed or used as self-medication. Usually, the time interval between taking the drug and the onset of the disease is from 4 days to 8 weeks.

A biopsy may be indicated if there is a suspicion of previous liver pathology or in the absence of normalization of blood biochemical parameters (liver function tests) after drug withdrawal.

Pathomorphology

Discomplexation of the hepatic tracts, severe protein (granular and balloon) degeneration of hepatocytes, polymorphism of hepatocyte nuclei, dystrophic and necrobiotic changes in the nuclei of hepatocytes are observed (Fig. 6-11, b).

Differential diagnosis

The possibility of toxic effects of drugs should be taken into account in the differential diagnosis of liver failure, jaundice. It is necessary to exclude other causes: viral hepatitis, diseases of the bile ducts, etc. In rare cases, it is necessary to carry out differential diagnostics with congenital metabolic diseases that can cause liver damage, type I glycogenosis (Gierke's disease),

Type III (measles disease), type IV (Andersen's disease), type VI (Hers disease). These diseases are caused by excessive accumulation of glycogen in liver cells. Chronic liver lesions of drug origin should also be differentiated from lipidoses: Gaucher disease (based on the accumulation of nitrogen-containing cerebrosides in reticulohistiocytic cells) and Niemann-Pick disease (resulting from the accumulation of phospholipids in the cells of the reticuloendothelial system, mainly sphingomyelin). It is also necessary to exclude galactosemia and fructosemia.

Treatment

A prerequisite and main condition for treatment is a complete rejection of the use of a hepatotoxic drug.

A high-calorie (90-100 kcal / kg per day) diet rich in proteins (2 g / kg per day) and carbohydrates helps to restore the functional state of the liver. For therapeutic purposes, essential phospholipids are recommended, which have a membrane stabilizing and hepatoprotective effect, as well as inhibitors of lipid peroxidation processes. Thioctic acid is also prescribed.

lot (lipoic acid *, lipamide *), which reduces the toxic effect of drugs due to its antioxidant effect; children over 12 years old - flavonoid silibinin (carsil *) 5 mg / kg in 3 divided doses (do not chew pills, take after meals with plenty of water).

Forecast

The prognosis depends on how quickly the drug that caused liver damage is canceled. Usually, clinical manifestations and changes in biochemical parameters are normalized within a few days, rarely weeks.

The prognosis is always serious when a picture of chronic liver damage with hepatocellular failure is formed.

Prevention of chronic hepatitis

Primary prevention has not been developed, secondary prevention consists in early recognition and adequate treatment of children with acute viral hepatitis.

The widespread introduction of vaccination against hepatitis A and B will solve the problem of not only acute, but also chronic hepatitis.

CIRRHOSIS OF THE LIVER

ICD-10 codes

K71.7. Toxic liver damage with fibrosis and cirrhosis of the liver.

K74. Fibrosis and cirrhosis of the liver is cryptogenic. K74.3. Primary biliary cirrhosis. K74.4. Secondary cirrhosis of the liver. K74.5. Biliary cirrhosis, unspecified. K74.6. Other and unspecified cirrhosis of the liver. P78.3. Congenital cirrhosis.

Liver cirrhosis is a chronic progressive disease characterized by dystrophy and necrosis of the hepatic parenchyma, accompanied by its nodular regeneration, diffuse proliferation of connective tissue. It is a late stage of various diseases of the liver and other organs, in which the structure of the liver is disturbed, and the functions of the liver are not performed in full, as a result of which liver failure develops.

It is necessary to distinguish cirrhosis of the liver from its fibrosis. Fibrosis is a focal proliferation of connective tissue with various liver lesions: abscesses, infiltrates, granulomas, etc.

In economically developed countries, cirrhosis of the liver occurs in 1% of the population, is one of the 6 main causes of death in patients aged 35 to 60 years. Every year in the world, 40 million people die from viral cirrhosis of the liver and hepatocellular carcinoma, which develops against the background of the carriage of the hepatitis B virus. It is more often observed in males, the ratio with the female sex is 3: 1.

Biliary atresia is one of the common causes of biliary cirrhosis in infants, with an incidence of 1 in 10,000-30,000 newborns.

Etiology and pathogenesis

Many diseases of the liver and other organs, long-term use of drugs (see Fig. 6-11, a, 6-12, a), etc. lead to cirrhosis of the liver. In addition, other diseases are important in the formation of cirrhosis:

Primary biliary cirrhosis;

Hereditary metabolic disorders (hemochromatosis, hepatolenticular degeneration, galactosemia, α-1-antitrypsin deficiency, etc.);

Violation of venous outflow from the liver (Budd-Chiari syndrome, veno-occlusive disease, severe right ventricular heart failure), etc.

Biliary atresia are referred to developmental anomalies, which in most cases are associated with intrauterine hepatitis, often caused by one of the reoviruses. In some children, the occurrence of this malformation is due to unfavorable factors that acted at 4-8 weeks of intrauterine life. Usually, these children have malformations of other organs (more often the kidneys, heart, spine). Some children have an association with trisomies on the 13th and 18th pairs of chromosomes. Atresia is characterized by complete closure of the intra-, extrahepatic bile ducts in various forms. More often (in 70-80% of cases) there is an intrahepatic form of atresia.

One of the main signs and complications of liver cirrhosis is portal hypertension syndrome, which occurs due to an increase in pressure in the portal vein (a vein that brings blood from the abdominal organs to the liver) more than 5 mm Hg. As a result of increased pressure in the portal vein, blood cannot flow from the abdominal organs and blood stagnation occurs in these organs (Fig. 6-12, b).

The approximate cellular composition of the liver: 70-80% - hepatocytes, 15% - endothelial cells, 20-30% - Kupffer cells (macrophages), 5-8% - Ito cells (Fig. 6-13, a). Ito cells(synonyms: hepatic stellate cells, fat-storing cells, lipocytes) located in the perisinusoidal space of Disse play a key role in the pathogenesis of liver cirrhosis. As the main cells of the connective tissue in the liver, they form the extracellular matrix, normally accumulating lipids. When the liver is damaged, Ito cells begin to produce type I collagen and cytokines, acquiring fibroblast-like properties (Fig. 6-13, b). This process takes place with the participation of hepatocytes and Kupffer cells.

Rice. 6-12. Liver cirrhosis: a - etiological factors; b - the portal system of the liver and the mechanism of the formation of portal hypertension

The pathogenesis of liver cirrhosis is shown in Fig. 6-13, b, but in approximately 10-35% of patients, the etiology and pathogenesis of liver cirrhosis remain unknown.

1 Rice. 6-13. a - part of the hepatic lobule and its cellular composition; b - pathogenesis of liver cirrhosis

Changes in the liver with cirrhosis are usually diffuse, only with biliary cirrhosis they can be focal. The death of hepatocytes associated with inflammation and fibrosis leads to disruption of the normal liver architectonics: loss of normal hepatic vasculature with the development of portocaval shunts and the formation of regeneration nodes of preserved hepatocytes (Fig. 6-14, a), rather than normal hepatic lobules detected in autopsy material or in vivo using MRI (Fig. 6-14, b).

Rice. 6-14. Changes in the liver in cirrhosis: a - a macro-preparation of micronodular cirrhosis of the liver; b - MRI of the liver: the arrow indicates the regeneration node

Classification

Allocate extrahepatic biliary atresia (without or in combination with gallbladder atresia), intrahepatic bile duct atresia (without or in combination with extrahepatic biliary atresia), total atresia. The classification of liver cirrhosis is presented in table. 6-10.

Table 6-10. Classification of liver cirrhosis

Clinical picture

In primary biliary cirrhosis of the liver, which is manifested by inflammation of the bile ducts of the liver with impaired outflow of bile, jaundice, pruritus, fever and other symptoms are observed. Biliary cirrhosis associated with congenital atresia of the biliary tract forms quickly, leading to death in the absence of surgery for health reasons.

Alcoholic cirrhosis of the liver develops in persons who consume alcoholic beverages in excessively large doses for a long time; it is not considered in childhood hepatology.

Liver cirrhosis in older children develops slowly and may initially be asymptomatic. The signs indicated in table. 6-11, as a rule, develop gradually and are imperceptible for a child who has been suffering from a chronic disease of the liver or other organs for a long time, and for his parents.

Hepatomegaly is observed at the onset of the disease. Gradual destruction of hepatocytes, fibrosis as the underlying disease progresses lead to a decrease in the size of the liver. A decrease in the size of the liver is especially characteristic in cirrhosis caused by viral and autoimmune hepatitis.

Table 6-11. Signs of liver cirrhosis

Complications of liver cirrhosis are portal hypertension syndrome (Table 6-12), varicose veins of the lower extremities, bleeding from the dilated veins of the esophagus, hepatic coma.

Table 6-12. Diagnostics of the portal hypertension syndrome

Varicose veins- complication of liver cirrhosis, manifested by pain in the extremities, visible and significant increase in veins. Bleeding from dilated veins of the esophagus manifested by the discharge of blood from the mouth and / or blackening of the stool. Hepatic coma- brain damage that develops as a result of the accumulation of a large amount of toxic substances in the blood, as a rule, develops with decompensated cirrhosis; the main signs of the syndrome of hepatocellular failure are presented in table. 6-13.

Table 6-13. Signs of hepatocellular failure syndrome

Diagnostics

In biochemical analysis, initially the syndromes of cytolysis, cholestasis, inflammation are detected, and later - hepatodepressive syndrome (see Table 1-8).

Ultrasound describes micronodular (Fig. 6-15, a) or macronodular (Fig. 6-15, b) types of liver cirrhosis. Histological synonyms for these names:

Small-nodular cirrhosis is characterized by the formation of small nodules (about 1 mm in diameter);

Large-nodular cirrhosis - in areas of previous destruction of the hepatic architectonics, large fibrous scars are revealed.

Pathomorphology

A classic macropreparation of the liver, clearly representing biliary cirrhosis of the liver, is shown in Fig. 6-15, c.

During a child's life, only a biopsy can accurately indicate liver cirrhosis, which reveals severe dystrophic changes in hepatocytes, cholestasis, foci of proliferation of connective tissue (fibrous nodes), between which islets are located normal liver cells (Fig. 6-15, d).

Differential diagnosis

Treatment

The main principles for the treatment of liver cirrhosis are as follows.

Elimination of the causes leading to cirrhosis (etiotropic treatment): antiviral therapy (viral hepatitis), withdrawal symptoms (alcoholic cirrhosis), drug withdrawal (drug hepatitis).

Rice. 6-15. Liver cirrhosis according to ultrasound data: a - micronodular; b - macronodular: congenital atresia of the bile ducts with the formation of cirrhosis: c - macropreparation; d - micropreparation (staining with hematoxylin-eosin; χ 400)

Diet therapy.

Therapy of the developed complications of liver cirrhosis: symptomatic treatment of hepatic encephalopathy, portal hypertension syndrome, etc.

Pathogenetic: removal of excess iron and copper (hemochromatosis, Wilson-Konovalov disease), immunosuppressive therapy (AIH), treatment of cholestasis (primary biliary cirrhosis).

With an established diagnosis biliary atresia surgical treatment: choledochojejunostomy or protoenterostomy (Kasai's operation - creating a direct anastomosis between the decapsulated open surface of the liver in

area of ​​the gate and intestines), transplantation of a part of the liver. Treatment is supportive before surgery. Glucocorticoids are ineffective, as are other drugs. At the same time, vitamin K should be administered parenterally once a week, and courses of hepatoprotectors, vitamins E, D should be conducted periodically.

Treatment of complications of liver cirrhosis

Strict bed rest;

Hyponosodium diet: with minimal and moderate ascites - limiting the intake of table salt to 1.0-1.5 g / day; with tense ascites - up to 0.5-1.0 g / day;

Limiting fluid intake to 0.8-1.0 liters per day;

Diuretic therapy: aldosterone antagonists and natriuretics;

Therapeutic paracentesis (3-6 liters) with intravenous administration of albumin solution (at the rate of 6-8 g per 1 liter of ascitic fluid removed);

Ultrafiltration using a peritoneal-venous shunt, transjugular intrahepatic portosystemic shunt;

Liver transplant.

Diuretics Hydrochlorothiazide (hypothiazide *) in tablets and capsules is administered orally to children from 3 to 12 years old at 1-2 mg / kg per day in 1 dose. Hypokalemia can be avoided by taking medications containing potassium or eating foods rich in potassium (fruits, vegetables).

Spironolactone (veroshpiron *, aldactone *, veropilactone *) in tablets, capsules, initial daily dose - 1.33 mg / kg, maximum - 3 mg / kg in 2 divided doses, or 30-90 mg / m2, course - 2 weeks ... Contraindicated in infancy.

Furosemide (lasix *) in tablets of 40 mg and granules for suspension preparation, ampoules 1% - 2 ml. Newborns are prescribed 1-4 mg / kg per day 1-2 times, 1-2 mg / kg intravenously or intramuscularly 1-2 times a day, children - 1-3 mg / kg per day, adolescents - 20 -40 mg / day

Diuretics are prescribed in the morning. It is necessary to control the level of potassium in the blood serum, ECG.

The criterion for the effectiveness of the therapy is a positive water balance, amounting to 200-400 ml / day with a small volume of ascites and 500-800 ml / day - with edematous ascitic syndrome in older children. Paracentesis performed according to strict indications (with a large amount of liquid) with the simultaneous administration of albumin in an amount of 4-5 g IV. If drug therapy is ineffective, surgical treatment (shunting) is possible.

Hemostatic therapy (ε-aminocaproic acid, vicasol *, calcium gluconate, dicinone *, erythrocyte mass).

Restoration of the volume of circulating blood (albumin solution, plasma).

Pharmacological reduction of portal pressure (vasopressin, somatostatin, octreotide).

Mechanical tamponade of the esophagus (Sengstaken-Blackmore probe).

Endoscopic methods for stopping bleeding (sclerotherapy with ethanolamine, polidocanol, ligation of vein trunks).

Transjugular intrahepatic portosystemic shunt.

Prevention of stress gastrointestinal ulcers (blockers of H2-histamine receptors, PPI).

Prevention of hepatic encephalopathy (lactulose, siphon enemas).

Prevention of spontaneous bacterial peritonitis (antibiotics).

The main pharmacological agents for hemorrhagic syndrome

ε-Aminocaproic acid for intravenous administration and in granules for preparing a suspension for oral administration, the daily dose for children under 1 year old is 3 g; 2-6 years old - 3-6 g, 7-10 years old - 6-9 g.

Menadione sodium bisulfate (vicasol *) 1% solution is prescribed for children under 1 year old - 2-5 mg / day, 1-2 years old - 6 mg / day, 3-4 years old - 8 mg / day, 5-9 years old - 10 mg / day, 10-14 years old - 15 mg / day. The duration of treatment is 3-4 days, after a 4-day break, the course is repeated.

Etamsylate (dicinone *) is produced in tablets of 250 mg and in the form of a 12.5% ​​solution in ampoules of 2 mg (250 mg in an ampoule) for intramuscular and intravenous administration. For bleeding, children under 3 years old are injected with 0.5 ml, 4-7 years old - 0.75 ml, 8-12 years old - 1-1.5 ml and 13-15 years old - 2 ml. This dose is repeated every 4-6 hours for 3-5 days. In the future, treatment with dicinone * can be continued in tablets (daily dose - 10-15 mg / kg): children under 3 years old - 1/4 tablet each, 4-7 years old - 1/2 tablet, 8-12 years old - 1 tablet each and 13-15 years old - 1.5-2 tablets 3-4 times a day.

A remedy for strengthening the vascular wall - the flavonoid troxerutin, ascorbic acid + rutoside (ascorutin *).

To reduce portal pressure, desmopressin (minirin *) is used, an analogue of the natural hormone arginine-vasopressin, 100-200 mg per night.

Treatment malignant neoplasm of the liver carried out by specialists of the oncological center. Indications for splenectomy

Segmental extrahepatic portal hypertension.

Severe hypersplenism with hemorrhagic syndrome.

Lagging in the physical and sexual development of children with liver cirrhosis.

Giant splenomegaly with severe pain syndrome (heart attacks, perisplenitis).

Treatment spontaneous bacterial peritonitis carried out with cephalosporins of the III-IV generation.

A radical treatment for liver cirrhosis is liver transplantation.

Prophylaxis

The basis secondary prevention is a timely etiotropic and pathogenetic treatment of acute and chronic hepatitis.

Prevention of cirrhosis per se tertiary and quaternary, since they carry out treatment aimed at stabilizing the pathological process in the liver, preventing exacerbations, reducing the risk of developing and progression of complications. Children should be under dynamic supervision in specialized clinics and centers, and on an outpatient basis - under the supervision of a pediatrician and gastroenterologist. Immunoprophylaxis is carried out strictly individually.

Prevention of complications, for example, the first bleeding from varicose veins of the esophagus, is possible thanks to endoscopic examination at least 1 time in 2-3 years in order to dynamically observe their possible development. The condition of patients with the initial stage of esophageal varicose veins is monitored endoscopically once every 1-2 years. Prophylactic treatment is carried out with moderate and severe degrees.

Forecast

The prognosis of liver cirrhosis is unfavorable and, as a rule, uncertain and unpredictable, since it depends on the cause of cirrhosis, the patient's age, the stage of the disease, and the possibility of unforeseen fatal complications. By itself, cirrhosis of the liver is incurable (except for those cases when a liver transplant was made), however, correct treatment of cirrhosis allows for a long time (20 years or more) to compensate for the disease. Compliance with diet, traditional and alternative methods of treatment (Fig. 6-16), giving up bad habits significantly increase the patient's chances of compensating for the disease.

Rice. 6-16. Treatment options for patients with cirrhosis

Without surgical treatment, children with biliary atresia die in the 2-3rd year of life. The earlier the operation is performed, the better the prognosis. About 25-50% of early operated children survive 5 years or more when they receive a liver transplant. The outcome depends on the presence or absence of an inflammatory and sclerotic process in the liver.

LIVER FAILURE

ICD-10 codes

K72. Liver failure. K72.0. Acute and subacute liver failure. K72.1. Chronic liver failure. K72.9. Hepatic impairment, unspecified.

Liver failure is a complex of symptoms characterized by a violation of one or more functions of the liver, resulting from damage to its parenchyma (hepatocellular or hepatocellular failure syndrome). Portosystemic or hepatic encephalopathy is a symptom complex of central nervous system disorders that occurs in liver failure with profound impairment of numerous vital functions of the liver.

Mortality from liver failure is 50-80%. In acute liver failure, it is possible to develop hepatic encephalopathy, which is rare in acute liver diseases, but the mortality rate can reach 80-90%.

Etiology and pathogenesis

Acute liver failure occurs in severe forms of viral hepatitis A, B, C, D, E, G, poisoning with hepatotropic poisons (alcohol, some drugs, industrial toxins, mycotoxins and aflatoxins, carbon dioxide, etc.). Its causes may be herpes viruses, cytomegalovirus, infectious mononucleosis virus, simple and shingles, Coxsackie virus, measles causative agent; septicemia with liver abscesses. Acute liver failure is described in toxic hepatosis (Reye's syndrome, condition after the small intestine is disconnected), Wilson-Konovalov's disease, Budd-Chiari syndrome.

Budd-Chiari Syndrome(ICD-10 code - I82.0) develops as a result of progressive narrowing or closure of the hepatic veins. Due to thrombophlebitis of the umbilical vein and the Arancian duct, which flows into the mouth of the left hepatic vein, Budd-Chiari syndrome can begin in early childhood. As a result, stagnation develops in the liver with compression of the liver cells.

Reye's syndrome(ICD-10 code - G93.7) - acute encephalopathy with cerebral edema and fatty liver infiltration that occurs in previously healthy newborns, children and adolescents (more often at the age of 4-12 years), associated with a previous viral infection (for example, varicella smallpox or influenza type A) and taking medications containing acetylsalicylic acid.

Chronic liver failure is a consequence of the progression of chronic liver diseases (hepatitis, liver cirrhosis, malignant liver tumors, etc.). The main etiological factors are indicated in Fig. 6-17, a.

At the heart of pathogenesis liver failure there are two processes. First, severe dystrophy and widespread necrobiosis of hepatocytes lead to a significant decrease in liver function. Secondly, due to the numerous collaterals between the portal and vena cava, a significant part of the absorbed toxic products enters the systemic circulation bypassing the liver. Poisoning is caused by non-neutralized protein breakdown products, metabolic end products (ammonia, phenols).

Emergence hepatic encephalopathy in liver failure, it is associated with disorders of homeostasis, acid-base state and electrolyte composition of the blood (respiratory and metabolic alkalosis, hypokalemia, metabolic acidosis, hyponatremia, hypochloremia, azotemia). Cerebrotoxic substances enter the systemic circulation from the gastrointestinal tract and liver: amino acids and their decay products (ammonia, phenols, mercaptans); products of hydrolysis and oxidation of carbohydrates (lactic acid, pyruvic acid, acetone); products of impaired fat metabolism; false neurotransmitters (asparagine, glutamine), which have toxic effects on the central nervous system. The mechanism of damage to brain tissue is associated with dysfunction of astrocytes, which make up approximately 30% of brain cells. Astrocytes play a key role in regulating the permeability of the blood-brain barrier, in ensuring the transport of neurotransmitters to the neurons of the brain, as well as in the destruction of toxic substances (in particular, ammonia) (Fig. 6-17, b).

Rice. 6-17. Chronic liver failure and hepatic encephalopathy: a - etiology of liver failure; b - the mechanism of the formation of hepatic encephalopathy

Ammonia exchange. In healthy people, the liver converts ammonia to uric acid in the Krebs cycle. It is required in the reaction of the conversion of glutamate to glutamine, which is mediated by the enzyme glutamate synthetase. With chronic liver damage, the number of functioning hepatocytes decreases, creating the prerequisites for hyperammonemia. When portosystemic shunting occurs, ammonia, bypassing the liver, enters the systemic circulation - hyperammonemia occurs. Ammonia entering

into the brain, leads to disruption of the functioning of astrocytes, causing morphological changes in them. As a result, with liver failure, cerebral edema occurs, and intracranial pressure rises.

In conditions of cirrhosis of the liver and portosystemic shunting, the activity of glutamatesynthetase of skeletal muscles increases, where the process of destruction of ammonia begins. This explains the decrease in muscle mass in patients with liver cirrhosis, which, in turn, also contributes to hyperammonemia. The processes of metabolism and excretion of ammonia also occur in the kidneys.

Clinical picture

The clinical picture is manifested by disorders of consciousness and cognitive functions, drowsiness, monotonous speech, tremors, and discoordination of movements. Particularly important signs are a rapid decrease in the size of the liver, its softening and tenderness to palpation. Table 6-14 briefly summarize the clinical manifestations of the stages of liver failure and encephalopathy, the differences between acute and chronic liver failure - in table. 6-15.

Table 6-14. Classification of stages of liver failure and encephalopathy

Table 6-15. Differential diagnosis of acute and chronic liver failure

Hepatic coma is preceded by general excitement, which turns into depression of consciousness: stupor and stupor, then its complete loss occurs. Meningeal phenomena, pathological reflexes (grasping, sucking), motor restlessness, convulsions appear. Breathing becomes irregular, like Kussmaul or Cheyne-Stokes. Pulse is small, irregular. From the mouth and from

hepatic odor emanates from the skin (fetor hepatica), due to the release of methyl mercaptan; jaundice and hemorrhagic syndrome increase, ascites, hypoproteinemic edema increase (Fig. 6-18, a). The clinical manifestations of decompensated and terminal stages are clearly shown in Fig. 6-18, b-d. The term "malignant form" (the most severe form) denotes a qualitatively new clinical condition that occurs in patients with viral hepatitis B if they develop massive or submassive liver necrosis.

Rice. 6-18. Hepatic failure: a - clinical manifestations; a and b - decompensated stage; c - terminal stage ("floating eyeball"); d - hepatic coma

Over the next 2-3 days, a deep hepatic coma develops. Sometimes a coma occurs, bypassing the stage of excitement.

Diagnostics

Laboratory and instrumental studies are carried out.

A general blood test reveals anemia, leukocytosis, thrombocytopenia, increased ESR.

In a biochemical study, bilirubinemia, azotemia, hypoalbuminemia, hypocholesterolemia are diagnosed, the levels of ALT, AST, ALP increase, the levels of fibrinogen, potassium, sodium, prothrombin index decrease, metabolic acidosis is noted.

Ultrasound, CT scan of the liver reveals a change in the size and structure of the liver parenchyma.

Pathomorphology

Morphological changes in the liver concern all its tissue components: parenchyma, reticuloendothelium, connective tissue stroma, to a lesser extent - biliary tract.

Distinguish three variants of the acute form of the disease:

Acute cyclic form;

Cholestatic (pericholangiolytic) hepatitis;

Massive liver necrosis.

The severity of morphological changes depends on the severity and etiology of the disease (Fig. 6-19, a, b). At the height of the disease, alternative, exudative processes prevail, during the period of recovery - the processes of proliferation and regeneration.

Rice. 6-19. Liver necrosis, macro- and micropreparations: a - the etiology is unknown; b - adenoviral etiology; c - χ 250; d - χ 400 (staining with hematoxylin-eosin)

In cholestatic (pericholangiolytic) hepatitis, morphological changes concern mainly intrahepatic bile ducts (cholangiolitis and pericholangiolitis).

Liver necrosis is an extreme degree of changes in the liver, which can be massive, when almost the entire hepatic epithelium dies or a slight border of cells is preserved along the periphery of the lobules, or submassive, in which most hepatocytes undergo necrobiosis, mainly in the center of the lobules (Fig. 6-19 , c, d).

Differential diagnosis

For the purpose of differential diagnosis, it is necessary to exclude extrahepatic causes of the onset of symptoms from the central nervous system. Determine the level of ammonia in the blood upon admission to the hospital of a patient with liver cirrhosis and signs of CNS damage. It is necessary to establish the presence in the patient's history of such pathological conditions as metabolic disorders, gastrointestinal bleeding, infections, constipation.

When symptoms of hepatic encephalopathy occur, differential diagnosis is performed with diseases, which include the following.

Intracranial pathological conditions: subdural hematoma, intracranial bleeding,

stroke, brain tumor, brain abscess.

Infections: meningitis, encephalitis.

Metabolic encephalopathy, which developed against the background of hypoglycemia, electrolyte disturbances, uremia.

Hyperammonemia caused by congenital anomalies of the urinary tract.

Toxic encephalopathy caused by alcohol intake, acute intoxication, Wernicke's encephalopathy.

Toxic encephalopathy, which has arisen against the background of taking medications: sedatives and antipsychotics, antidepressants, salicylates.

Postconvulsive encephalopathy.

Treatment

Treatment consists in limiting the amount of protein in the diet, prescribing lactulose. Patients with hepatic encephalopathy are candidates for liver transplantation.

In the complex of therapeutic measures for liver failure, there are stages (Fig. 6-20), and also there are basic (standard) therapy and a number of more radical means aimed at cleansing the body of toxic products of metabolic disorders, as well as replacing (temporary or permanent) functions the affected liver.

Basic therapy acute liver failure is aimed at correcting the electrolyte, energy balance, acid-base state, vitamins and cofactors, disorders of the blood coagulation system, hemocirculation, elimination of hypoxia, prevention of complications, prevention of absorption of putrefactive decay products from the intestine. The basic therapy includes the use of glucocorticoids.

General principles of management of a patient with acute liver failure

Individual post of a nurse.

Monitor urinary output, blood glucose and vital signs every hour.

Rice. 6-20. Stages of hepatic encephalopathy treatment

Serum potassium control 2 times a day.

Blood test, determination of the content of creatinine, albumin, assessment of the coagulogram daily.

Prevention of bedsores.

General principles of management of a patient with chronic hepatic failure

Active monitoring of the patient's condition, taking into account the severity of symptoms of encephalopathy.

Weighing the patient daily.

Daily assessment of the balance of fluid consumed and excreted per day.

Daily determination of blood test, electrolyte content, creatinine.

Determination of the content of bilirubin, albumin, activity of AST, ALT, ALP 2 times a week.

Coagulogram, prothrombin content.

Assessment of the necessity and possibility of liver transplantation in the final stage of liver cirrhosis.

Hepatic encephalopathy treatment

Elimination of provoking factors.

Stopping gastrointestinal bleeding.

Suppression of the growth of proteolytic microflora in the colon and treatment of infectious diseases.

Normalization of electrolyte disorders.

Reducing the degree of hyperammonemia:

a) decrease in ammoniacal substrate:

Cleansing the gastrointestinal tract (siphon enemas, laxatives);

Decreased protein intake;

b) binding of ammonia in the blood:

Ornithine (hepa-merz *);

c) suppression of the formation of ammonia:

Broad-spectrum antibiotics;

Acidification of intestinal contents with lactulose. To reduce the ammonia content, enemas are recommended.

or using laxatives to empty your bowels at least 2 times a day. For this purpose, lactulose (normase *, duphalac *) is prescribed in syrup, 20-50 ml orally every hour until diarrhea appears, then 15-30 ml 3-4 times a day. For use in an enema, the preparation is diluted up to 300 ml in 500-700 ml of water.

Before the patient is discharged from the hospital, the dose of lactulose should be reduced to 20-30 ml at night with possible subsequent cancellation at the outpatient stage.

TO radical treatment methods include the following measures for the massive removal of toxic products from the patient's blood.

Guided hemodilution.

Plasmapheresis.

Substituted blood transfusion.

Temporary (or permanent) replacement of the patient's liver by extracorporeal connection of xeno liver (porcine), cross circulation.

Hetero- and orthotopic liver transplantation.

Prophylaxis

The best way to prevent liver failure is to prevent the risk of developing cirrhosis or hepatitis. This requires specific immunization, it is important to observe a healthy lifestyle, personal hygiene rules, and diet therapy.

The introduction of specific immunoglobulin in case of accidental transfusion of infected blood and at the birth of a child to a mother who is a carrier of HBsAg or a patient with hepatitis B will allow passive immunization. Active immunization - vaccination of a child in the first day after birth, unvaccinated children of any age, as well as persons from risk groups: professional (doctors, emergency workers, military, etc.), persons on programmed hemodialysis, etc. (revaccination every 7 years). Vaccination against viral hepatitis B protects against hepatitis D infection.

Forecast

By eliminating the cause of the liver failure, the manifestations of hepatic encephalopathy can be reduced. Chronic hepatic coma is fatal, but with acute hepatocellular failure, recovery is sometimes possible. With the development of hepatic encephalopathy, mortality can reach 80-90%.

The development of fatty hepatosis is based on the violation of metabolic processes in the human body. As a result of this liver disease, healthy organ tissue is replaced by fatty tissue. At the initial stage of development, fat accumulates in hepatocytes, which over time simply leads to degeneration of the liver cells.

If the disease is not diagnosed at an early stage and appropriate therapy is not carried out, then irreversible inflammatory changes occur in the parenchyma, which lead to the development of tissue necrosis. If fatty hepatosis is not treated, then it can develop into cirrhosis, which is no longer treatable. In the article, we will consider why the disease develops, methods of its treatment and classification according to ICD-10.

Causes of fatty hepatosis and its prevalence

The reasons for the development of the disease have not yet been precisely proven, but factors are known that can confidently provoke the onset of this ailment. These include:

• completeness;
• diabetes;
• violation of metabolic processes (lipid);
• minimal exercise with a nutritious daily diet high in fat.

Most cases of fatty hepatosis are registered by doctors in developed countries with a standard of living above average.

There are a number of other factors associated with hormonal disruption, such as insulin resistance and the presence of sugar in the blood. The hereditary factor cannot be omitted either, it also plays an important role. But still, the main reason is unhealthy diet, a sedentary lifestyle and excess weight. All the reasons have nothing to do with the intake of alcoholic beverages, therefore, fatty hepatosis is often called non-alcoholic. But if you add alcohol dependence to the above reasons, then fatty hepatosis will develop many times faster.

In medicine, it is very convenient to use the coding of diseases for their systematization. It is even easier to indicate the diagnosis on the sick leave with the help of a code. Codes for all diseases are represented in the International Classification of Diseases, Injuries and Various Health Problems. At this time, the option of the tenth revision is in effect.

All liver diseases according to the International classification of the tenth revision are encrypted under the codes K70-K77. And if we talk about fatty hepatosis, then according to ICD 10, it falls under the code K76.0 (fatty liver degeneration).

Treatment of fatty hepatosis

The treatment regimen for non-alcoholic hepatosis is to eliminate possible risk factors. If the patient is obese, then you need to try to optimize it. And start by reducing the total mass by at least 10%. Doctors recommend using minimal physical activity in parallel with dietary meals to achieve the goal. Limit the use of fats in the diet as much as possible. At the same time, it is worth remembering that drastic weight loss will not only not be beneficial, it can, on the contrary, damage, aggravating the course of the disease.

For this purpose, the attending physician may prescribe thiazolidinoids in combination with biguanides, but this line of drugs has not yet been fully studied, for example, for hepato toxicity. Metformin can help correct the process of metabolic disturbances in carbohydrate metabolism.

As a result, we can confidently say that with the normalization of the daily diet, a decrease in body fat mass and abandoning bad habits, the patient will feel an improvement. And only in this way it is possible to fight such a disease as non-alcoholic hepatosis.

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Hepatomegaly diffuse changes in the liver and pancreas

Hepatomegaly (ICD code - 10 R16, R16.2, R16.0) is a process of liver enlargement. Indicates multiple diseases. Signs of hepatomegaly are bright or mild. There is moderate hepatomegaly, severe hepatomegaly.

The reasons for the development of fatty and diffuse changes are different. It can be organ obesity or ordinary poisoning with poisons. Timely ultrasound examination, treatment and diet will help to get rid of pathology forever.

What is pathology

The liver is a filter of the human body. It is in this organ that the processes of decay of non-toxic and toxic elements take place, which are subsequently excreted in urine and feces. In medicine, there is no separate concept that diffuse changes are an independent pathology.

Enlargement of the liver, pancreas or spleen (ICD code - 10 R16, R16.2, R16.0) is a syndrome indicating that the state of the parenchyma and tissues of other organs is unsatisfactory.

Pathology is determined using ultrasound examination and palpation.

Reasons for diffuse changes in the parenchyma:

The above pathologies cause damage, swelling of the parenchyma.

Signs of diffuse changes

The diffuse change, which entails the growth and enlargement of the organ, is very well felt on palpation. Another specter of changes is painful sensations on palpation. Such symptoms suggest that immediate liver treatment should be carried out. But first of all, you need to find out for what reasons the organ enlargement syndrome has developed. When the symptoms are studied, ultrasound examinations of the liver, ultrasound of the pancreas, the doctor will be able to prescribe treatment.

Diffuse changes can develop at different ages. But there are factors that can provoke this condition.

The risk group includes people:

1. Alcohol abusers. Ethanol has a detrimental effect on the liver. It provokes the development of cirrhosis, fatty hepatosis and cancer.
2. Uncontrollably taking medications, drugs, dietary supplements, vitamins for a long time.
3. Weak immunity. Viral infections lead to liver changes.
4. Malnourished and overweight. Eating fatty, spicy, or salty food items causes an enlarged liver.

Symptoms of the pathological process directly depend on the pathology that provoked hepatomegaly.

What symptoms, in addition to an increase in the organ and pain sensations, can be observed:

• pain and colic in the right hypochondrium, especially when entering or if a person suddenly gets up from a chair or sofa;
• the skin becomes yellow, the sclera of the eyes acquire the same shade;
• skin rashes, itching;
• diarrhea and constipation;
• a feeling of heartburn, an unpleasant odor from the mouth;
• a feeling of nausea, often ending in vomiting;
• hepatic asterisks in certain areas of the skin (with the development of fatty hepatosis);
• a feeling of fluid accumulation in the abdomen.

Hepatomegaly can also develop against the background of extrahepatic pathologies. For example, with metabolic disorders. Impaired glycogen catabolism leads to the accumulation of the substance in the liver. As a consequence, there is a slow increase. In addition to the hepatic parenchyma, the kidneys, spleen, and pancreas increase in size. They provoke diffuse organ processes and cardiovascular pathologies.

With a weak contractility, a violation of blood flow develops. As a result, swelling and organ growth develops. Therefore, in order to determine the true causes, an ultrasound scan should be performed.

Enlargement of the liver and spleen

Moderate hepatomegaly and splenomegaly (ICD code - 10 R16, R16.2, R16.0) are two pathologies that occur, in most cases, simultaneously. Splenomegaly is an enlargement of the spleen.

It develops for the following reasons:

The liver and spleen suffer due to the fact that the functionality of the two organs is closely related. Moreover, the growth of the spleen occurs more often in children, in most cases in newborns. Violations in ultrasound diagnostics are determined.

Hepatomegaly in children

In newborns and children under 10 years of age, the development of moderate (age-related) hepatomegaly is most often observed. ICD code R16, R16.2, R16.0. That is, an increase in the liver by 10-20 mm is considered an acceptable norm. If a child under 10 years of age or newborns have a size that exceeds the permissible norm, while symptoms of liver damage are present, you should immediately visit a doctor.

What signs, besides an increase, may indicate developing pathologies in children:

• pain in the right side, even at rest;
• nausea and vomiting;
• yellowing of the sclera and skin;
• bad breath;
• sleepiness and fatigue.

Reasons for organ enlargement in children

The signs are as follows:

1. If inflammation is present due to congenital infections. Hepatomegaly develops against the background of rubella, toxoplasmosis, herpes, liver abscess, obstruction, intoxication, hepatitis A, B, C.
2. In case of metabolic disorders, when a pregnant woman does not eat properly.
3. If genetic disorders are present. These include excessive amounts of porphins in the body; hereditary enzyme defects; violation of protein metabolism, metabolic diseases of the connective tissue.
4. In the presence of a benign enlargement of the parenchyma, for example, with hepatitis, hypervitaminosis, blood poisoning.
5. With diagnosed congenital fibrosis, multicystosis, cirrhosis.
6. The reasons for the growth of the organ in newborns and children under 10 years of age are infiltrative lesions. This can occur with malignant neoplasms, leukemia, lymphoma, metastases, histiocytosis.

Another reason for diffuse changes in the liver of children under 10 years of age is the impaired outflow of blood and secretion that the gallbladder produces. It develops with blockage of the bile ducts, stenosis or thrombosis of blood vessels, heart failure, cirrhosis.

Sometimes children develop mild diffuse hepatomegaly as the body's response to an infection. But this condition is not a pathology. There is no need to treat him.

It is possible to correct the size of the liver and pancreas by eliminating the cause. Diet in childhood is also important. Symptoms of diffuse changes in children are the same as in adults. Children under 10 years of age become capricious, their appetite disappears, and stool disorders are observed.

Echoes, ultrasound examination allow you to accurately identify the degree of increase: unexpressed, moderate and pronounced.

Treatment in children

Age-related physiological moderate enlargement of the liver, pancreas in children does not need to be treated. In this case, it is enough to undergo an ultrasound examination.

Treatment is prescribed only if there is a pathological process that provoked a change in the size of the liver.

As mentioned above, a prerequisite not only for children, but also for adults is diet. All unhealthy foods are excluded. The diet is saturated with vegetables and fruits.

Treatment in adults

Treatment is based on the results of the tests performed, ultrasound studies and visual examination. An ultrasound will show how much the organ has increased. The main goal of therapy is to eliminate the cause of the enlargement of the liver.

Antiviral and hepatoprotective treatment of viral hepatitis leads to complete recovery. The parenchyma is being restored. Hepatomegaly is absent.

If cirrhosis is diagnosed, then, in most cases, it is not cured. Because there is a replacement of healthy cells with connective tissue. Unfortunately, this process is irreversible.

Each disease, accompanied by an enlargement of the liver or pancreas, requires individual specific treatment, which can only be prescribed based on the results of an ultrasound scan. Sometimes an ultrasound examination is not enough and an MRI scan is required. But basically all patients with hepatomegaly are prescribed hepatoprotective treatment. The drugs will help to quickly restore the affected cells.

The most common remedies for recovery are:

1. Hepabene.
2. FanDetox.
3. Liv 52.
4. Heptral.
5. Carsil.
6. Essentiale forte.
7. Oatsol.
8. Phosphogliv.
9. Ursofalk.

It is advisable to undergo an ultrasound examination throughout the year.

Excluded:

• Budd-Chiari syndrome (I82.0)

Included:

• hepatic:
  • coma NOS
  • encephalopathy NOS
• hepatitis:
  • fulminant, not elsewhere classified, with hepatic impairment
  • malignant, not elsewhere classified, with hepatic impairment
• liver (cell) necrosis with liver failure
• yellow atrophy or liver dystrophy

Excluded:

• alcoholic hepatic impairment (K70.4)
• liver failure complicating:
  • abortion, ectopic or molar pregnancy (O00-O07, O08.8)
• jaundice of fetus and newborn (P55-P59)
• viral hepatitis (B15-B19)
• in combination with toxic liver damage (K71.1)

Excludes: hepatitis (chronic):

• alcoholic (K70.1)
• medicinal (K71.-)
• granulomatous NEC (K75.3)
• reactive nonspecific (K75.2)
• viral (B15-B19)

Excluded:

• alcoholic liver fibrosis (K70.2)
• cardiac sclerosis of the liver (K76.1)
• cirrhosis of the liver):
  • alcoholic (K70.3)
  • congenital (P78.3)
• with toxic liver damage (K71.7)

Excluded:

• alcoholic liver disease (K70.-)
• amyloid degeneration of the liver (E85.-)
• cystic liver disease (congenital) (Q44.6)
• hepatic vein thrombosis (I82.0)
• hepatomegaly NOS (R16.0)
• portal vein thrombosis (I81)
• liver toxicity (K71.-)

In Russia, the International Classification of Diseases of the 10th revision (ICD-10) has been adopted as a single normative document to take into account the incidence, the reasons for the population's visits to medical institutions of all departments, and the causes of death.

ICD-10 was introduced into health care practice throughout the Russian Federation in 1999 by order of the Ministry of Health of Russia dated 05/27/97. No. 170

A new revision (ICD-11) is planned by WHO in 2017 2018.

As amended and supplemented by WHO

Processing and translation of changes © mkb-10.com

What is fatty hepatosis: ICD code 10

The development of fatty hepatosis is based on the violation of metabolic processes in the human body. As a result of this liver disease, healthy organ tissue is replaced by fatty tissue. At the initial stage of development, fat accumulates in hepatocytes, which over time simply leads to degeneration of the liver cells.

If the disease is not diagnosed at an early stage and appropriate therapy is not carried out, then irreversible inflammatory changes occur in the parenchyma, which lead to the development of tissue necrosis. If fatty hepatosis is not treated, then it can develop into cirrhosis, which is no longer treatable. In the article, we will consider why the disease develops, methods of its treatment and classification according to ICD-10.

Causes of fatty hepatosis and its prevalence

The reasons for the development of the disease have not yet been precisely proven, but factors are known that can confidently provoke the onset of this ailment. These include:

Most cases of fatty hepatosis are registered by doctors in developed countries with a standard of living above average.

There are a number of other factors associated with hormonal disruption, such as insulin resistance and the presence of sugar in the blood. The hereditary factor cannot be omitted either, it also plays an important role. But still, the main reason is unhealthy diet, a sedentary lifestyle and excess weight. All the reasons have nothing to do with the intake of alcoholic beverages, therefore, fatty hepatosis is often called non-alcoholic. But if you add alcohol dependence to the above reasons, then fatty hepatosis will develop many times faster.

In medicine, it is very convenient to use the coding of diseases for their systematization. It is even easier to indicate the diagnosis on the sick leave with the help of a code. Codes for all diseases are represented in the International Classification of Diseases, Injuries and Various Health Problems. At this time, the option of the tenth revision is in effect.

All liver diseases according to the International classification of the tenth revision are encrypted under the codes K70-K77. And if we talk about fatty hepatosis, then according to ICD 10, it falls under the code K76.0 (fatty liver degeneration).

You can learn more about the symptoms, diagnosis and treatment of hepatosis from separate materials:

Treatment of fatty hepatosis

The treatment regimen for non-alcoholic hepatosis is to eliminate possible risk factors. If the patient is obese, then you need to try to optimize it. And start by reducing the total mass by at least 10%. Doctors recommend using minimal physical activity in parallel with dietary meals to achieve the goal. Limit the use of fats in the diet as much as possible. At the same time, it is worth remembering that drastic weight loss will not only not be beneficial, it can, on the contrary, damage, aggravating the course of the disease.

For this purpose, the attending physician may prescribe thiazolidinoids in combination with biguanides, but this line of drugs has not yet been fully studied, for example, for hepato toxicity. Metformin can help correct the process of metabolic disturbances in carbohydrate metabolism.

As a result, we can confidently say that with the normalization of the daily diet, a decrease in body fat mass and abandoning bad habits, the patient will feel an improvement. And only in this way it is possible to fight such a disease as non-alcoholic hepatosis.

K70-K77 Diseases of the liver. V. 2016

International classification of diseases 10th revision (ICD-10)

K70-K77 Liver diseases

K70-K77 Liver diseases

Reye's syndrome (G93.7)

viral hepatitis (B15-B19)

K70 Alcoholic liver disease

K71 Toxic liver damage

Badda-Chiari syndrome (I82.0)

"Pure" cholestasis K71.1 Toxic liver damage with hepatic necrosis Liver failure (acute) (chronic) due to drugs K71.2 Toxic liver damage proceeding as acute hepatitis

yellow atrophy or liver dystrophy

liver failure complicating:

• abortion, extrauterine or molar pregnancy (O00-O07, O08.8)
• pregnancy, childbirth and the puerperium (O26.6)

jaundice of fetus and newborn (P55-P59)

viral hepatitis (B15-B19)

in combination with toxic liver damage (K71.1)

K74 Fibrosis and cirrhosis of liver

cardiac sclerosis of liver (K76.1)

cirrhosis of the liver:

• alcoholic (K70.3)
• congenital (P78.3)

with toxic liver damage (K71.7-) K74.0 Liver fibrosis

• acute or subacute
  • NOS (B17.9)
  • non-viral (K72.0)
• viral hepatitis (B15-B19)

toxic liver damage (K71.1)

cholangitis without liver abscess (K83.0)

pylephlebitis without liver abscess (K75.1) K75.1 Portal phlebitis Pylephlebitis Excluded: pylephlebitic liver abscess (K75.0)

amyloid liver degeneration (E85.-)

cystic liver disease (congenital) (Q44.6)

hepatic vein thrombosis (I82.0)

portal vein thrombosis (I81.-)

toxic liver damage (K71.-)

Focal nodular hyperplasia of the liver

Hepatoptosis K76.9 Liver disease, unspecified

Portal hypertension in schistosomiasis B65.- †)

Liver injury in syphilis (A52.7 †) K77.8 * Liver injury in other diseases classified elsewhere Liver granulema in:

• beryllium disease (J63.2 †)
• sarcoidosis (D86.8 †)

Notes. 1. This version corresponds to the 2016 version of the WHO (ICD-10 Version: 2016), some positions of which may differ from the version of ICD-10 approved by the Ministry of Health of Russia.

2. The translation into Russian of a number of medical terms in this article may differ from the translation in the ICD-10 approved by the Ministry of Health of Russia. All comments and clarifications on translation, design, etc. are accepted with gratitude by e-mail.

3. NOS - no additional clarifications.

4. NCDR - not elsewhere classified.

5. A cross † marks the main codes of the underlying disease, which must be used without fail.

6. An asterisk marks optional additional codes related to the manifestation of the disease in a separate organ or area of ​​the body, which is an independent clinical problem.

/ Internal diseases / 3 chapter LIVER DISEASES AND BILISTRY SYSTEM-p

DISEASES OF THE LIVER AND BILISTRY SYSTEM

Dyskinesia of the biliary tract.

Fatty hepatosis (FG) - hepatic steatosis, chronic fatty degeneration of the liver - an independent chronic disease or syndrome caused by fatty degeneration of hepatocytes with intra- and / or extracellular fat deposition.

ICD10: K76.0 - Fatty liver disease, not elsewhere classified.

FG is a polyetiologic disease. It often occurs as a result of metabolic disorders caused by an unbalanced diet. Especially if there is a bad habit or there are circumstances in which the entire daily need for food is satisfied in almost 1 reception. In such cases, taking into account the limited possibilities of depositing carbohydrates and proteins in the liver and other organs, they turn into easily and infinitely deposited fat.

GH is often a secondary syndrome accompanying obesity, diabetes mellitus, endocrine diseases, primarily Cushing's disease, chronic alcoholism, intoxication, including drugs, chronic circulatory failure, metabolic X-syndrome, and many other diseases of internal organs.

As a result of excessive accumulation of fat in the liver tissue, the function of the organ as a dynamic depot of carbohydrates (glycogen) is first of all disrupted, which leads to destabilization of the mechanisms for maintaining a normal level of glucose in the blood. In addition, metabolic changes associated with prolonged exposure to etiological factors can cause toxic and even inflammatory damage to hepatocytes, the formation of steatohepatitis with a gradual transition to liver fibrosis. In many cases, the etiological factors that caused GH can contribute to the formation of homogeneous cholesterol stones in the gallbladder.

FG is characterized by complaints of general weakness, reduced ability to work, dull aching pains in the right hypochondrium, poor alcohol tolerance. Many have hypoglycemic states in the form of paroxysmal sharp weakness, sweating, feelings of "emptiness" in the abdomen, which quickly pass after eating food, even one candy. Most patients have a tendency to constipation.

The overwhelming majority of patients with GI have a habit of eating 1–2 times a day. Many have a history of drinking a large amount of beer, long-term drug therapy, work under conditions of toxic effects, various diseases of internal organs: diabetes mellitus, metabolic X-syndrome, chronic circulatory failure, etc.

An objective study usually pays attention to the overweight of the patient. Percussion determined liver sizes are increased. The anterior edge of the liver is rounded, compacted, weakly sensitive.

Symptoms of pathological changes in other organs detected during FG are usually related to diseases that led to the formation of fatty degeneration of the liver.

General analysis of blood and urine: no deviations.

Biochemical blood test: increased levels of cholesterol, triglycerides, increased activity of AST and ALT.

Ultrasound examination: enlargement of the liver with a diffuse or focal uneven increase in the echogenicity of the liver parenchyma, depletion of the tissue pattern with small vascular elements. There is no portal hypertension. As a rule, signs of pancreatic steatosis are simultaneously detected: an increase in the volume of the gland, diffusely increased echogenicity of its parenchyma in the absence of pathological expansion of the Wirsung duct. Concrements in the gallbladder, signs of diffuse, reticular or polypous cholesterosis of the gallbladder can be recorded.

Laparoscopic examination: the liver is enlarged, its surface is yellowish-brown.

Liver biopsy: diffuse or localized in different parts of the lobule fatty degeneration of liver cells, extrahepatic location of fatty drops. With a prolonged course of the disease, signs of steatohepatitis are revealed - cellular inflammatory infiltration with predominant localization in the center of the lobules. Sometimes infiltrates capture the entire lobule, spread to the portal tracts and the periportal zone, which indicates the likelihood of the formation of liver fibrosis.

It is carried out with alcoholic liver disease, chronic hepatitis.

Unlike LH, alcoholic liver disease is characterized by anamnestic information about long-term alcohol abuse. In biopsies of the liver of alcoholics, a large number of hepatocytes containing Mallory's little bodies - a condensed smooth endoplasmic reticulum - are detected. In their blood, a marker of long-term alcoholism is detected - transferrin does not contain sialic acids.

Chronic hepatitis differs from GH by deviations in the general and biochemical blood tests, indicating the presence of a chronic inflammatory process in the liver, violations of the protein-forming and liposynthetic functions of the organ. Markers of infection with hepatitis B, C, D, G viruses are detected. The results of a puncture biopsy of the liver allow to reliably distinguish between GH and chronic hepatitis.

General blood analysis.

Immunological analysis for the presence of markers of hepatitis B, C, D, G.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

A mandatory transition to a fractional diet - 5-6 meals a day with an even distribution of calorie intake and component composition (carbohydrates-proteins-fats) of food. The use of animal fats is limited. Dishes containing cottage cheese and vegetable fibers are recommended. With a tendency to constipation, steamed rye or wheat bran should be consumed 1-3 teaspoons 3-4 times a day with meals.

A daily intake of balanced multivitamin preparations such as "Troll", "Jungle", "Enomdan" and the like is mandatory.

The most effective remedy for the treatment of GH is Essentiale-forte, which contains essential phospholipids and vitamin E. Unlike Essentiale-Forte, Essentiale does not contain vitamin E, nor does Essentiale for parenteral administration. Essentiale-forte take 2 capsules 3 times a day with meals for 1-2 months.

Other lipotropic drugs can be used to treat GH:

Legalon - 1-2 tablets 3 times a day.

Lipofarm - 2 tablets 3 times a day.

Lipostabil - 1 capsule 3 times a day.

Lipoic acid - 1 tablet (0.025) 3 times a day.

The effectiveness of the treatment can be monitored using ultrasound, which reveals a tendency towards a decrease in the size of the liver, a decrease in the echogenicity of the organ parenchyma.

Usually auspicious. With the exclusion of harm, effective treatment, prophylactic intake of multivitamin preparations, complete recovery is possible.

TESTS FOR SELF-CONTROL

What circumstances can not lead to the formation of fatty hepatosis?

Eating 1-2 times a day.

Excessive consumption of foods containing animal fats.

Eating cottage cheese, plant products.

Professional and household intoxication.

What diseases can not to form fatty hepatosis.

Chronic circulatory failure.

What diseases and syndromes can not occur with prolonged exposure to the etiological factor that caused the formation of fatty hepatosis?

Anything can arise.

What are the clinical manifestations not typical for fatty hepatosis?

Overweight.

An increase in the size of the liver.

Dense, rounded, sensitive edge of the liver.

What abnormalities of the biochemical blood test are not typical for fatty hepatosis?

Increased content of cholesterol, triglycerides.

Increased activity of AST and ALT.

High levels of bilirubin.

What points of the plan for examining patients with fatty hepatosis can be excluded without compromising the quality of diagnosis.

Biochemical blood test: fasting sugar, total protein and its fractions, bilirubin, cholesterol, uric acid, AST, ALT, gamma-glutamyl transpeptidase, sialic acid-free transferrin.

Immunological analysis for the presence of markers of hepatitis B, C, D, G viruses.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

What ultrasound results are not typical for fatty hepatosis?

Increased liver volume.

High echogenicity of the liver parenchyma.

Signs of pancreatic lipomatosis.

Signs of gallstone disease.

Signs of portal hypertension.

What are the criteria do not allow to distinguish fatty degeneration of the liver in algogolny disease from fatty hepatosis?

The presence of sialic acid-free transferrin in the blood.

Biopsy specimens contain many cells containing Malory bodies.

The presence of fat droplets in intracellular vacuoles and outside hepatocytes.

All criteria allow.

None of the criteria allows you to do this.

The transition to a fractional diet with 5-6 times food intake during the day.

An even distribution of the calorie intake of the diet throughout the day.

The use of lipotropic (cottage cheese) and herbal products.

What drugs it does not follow to give to patients with fatty hepatosis?

What are the clinical manifestations not typical for fatty hepatosis?

Aching pains in the right hypochondrium.

Increased abdominal volume, ascites.

Constipation tendency.

Pigmented hepatosis - hereditary disorders of the metabolism and transport of bilirubin in hepatocytes, manifested by constant or recurrent jaundice in the absence of changes in the morphological structure of the liver.

In adults, the following variants of disorders in the metabolism of bilirubin in the liver are found:

Gilbert's syndrome is a syndrome of unconjugated hyperbilirubinemia.

Rotor syndrome is a syndrome of conjugated hyperbilirubinemia.

Dabin-Jones syndrome is a syndrome of conjugated hyperbilirubinemia with excessive deposition of melanin-like pigment in hepatocytes.

More often than others, unconjugated hyperbilirubinemia, Gilbert's syndrome, occurs in clinical practice.

Gilbert's syndrome (SG) is a genetically determined enzymopathy that causes impaired conjugation of bilirubin in the liver, which is manifested by an increase in the content of unconjugated bilirubin in the blood, jaundice, and accumulation of lipofuscin pigment in hepatocytes.

ICD10: E80.4 - Gilbert's syndrome.

The syndrome is associated with an autosomal dominant defect in the UGTA1A1 and GNT1 genes, which causes insufficient formation in hepatocytes of the enzyme glucuronyl transferase, which provides neutralization in the liver, including the conjugation of bilirubin with glucuronic acid. Men suffer from SJ 10 times more often than women. Acute viral hepatitis (“post-hepatitis” unconjugated hyperbilirubinemia) can be a triggering factor for GS.

In the pathogenesis of the disease, the main role is played by:

Disturbances in the transport function of proteins that deliver unconjugated bilirubin to the smooth endoplasmic reticulum - hepatocyte microsomes.

Inadequacy of the microsomal enzyme UDP-glucuronyltransferase, with the participation of which conjugation of bilirubin with glucuronic and other acids is carried out.

With SF, as well as with other forms of pigmentary hepatosis, the liver retains a histological structure identical to the normal one. However, in hepatocytes, an accumulation of a golden or brown pigment, lipofuscin, can be detected. As a rule, there are no signs of dystrophy, necrosis, fibrosis in the liver with SF, as with other pigmentary hepatosis.

In the gallbladder in patients with SJ, calculi consisting of bilirubin can form.

All patients with SJ complain of recurrent yellowness of the sclera and skin. There are usually no other complaints. Only in isolated cases do you experience rapid fatigue, a feeling of heaviness in the right hypochondrium. Jaundice occurs and grows under conditions of emotional and physical stress, with respiratory infections, after surgery, after drinking alcohol, during fasting or low-calorie (less than 1/3 of the norm) diet low in fat (vegetarianism), after taking certain medications (nicotinic acid , rifampicin). Patients with FS are often neurotic because they are worried about their jaundice.

The leading symptom of the disease is scleral icterus. Yellowness of the skin is present only in some patients. Characterized by a dull-icteric color of the skin, especially on the face. In some cases, there is a partial staining of the palms, feet, axillary regions, and the nasolabial triangle. In some cases, despite the increased level of bilirubin in the blood, the skin has a normal color - cholemia without jaundice. In some patients, pigmentation of the face occurs, scattered pigment spots appear on the skin of the trunk.

According to the description of Gilbert himself, in the typical course of the disease, a triad should be revealed: hepatic mask, xanthelasma of the eyelids, yellow skin color.

Some clinicians consider urticaria, hypersensitivity to cold, and goose bumps to be characteristic of this syndrome.

An objective study in 1/4 of patients can reveal a moderate increase in the liver. Palpation of the liver is soft, painless. With the formation of pigment stones in the gallbladder, clinical manifestations of cholelithiasis, chronic calculous cholecystitis are possible

Complete blood count: in one third of cases of SJ, hemoglobin content increased over 160 g / l, erythrocytosis, decreased ESR are detected (these changes are usually combined with increased gastric acidity).

General urine analysis: the color is normal, there is no bilirubin.

Biochemical blood test: isolated unconjugated hyperbilirubinemia, which only in rare cases exceeds the level of µmol / l, averaging about 35 µmol / l. All other biochemical parameters,

characterizing liver function, usually normal.

Instrumental methods (ultrasound, computed tomography, isotope scintigraphy) do not reveal any changes in the structure of the liver that are specific to the SF.

Ultrasound in the gallbladder often reveals calculi of the pigment structure. Puncture liver biopsy: no signs of necrosis, inflammation, activation of fibrosis processes. In the liver cells, the presence of a pigment, lipofuscin, is determined.

To detect Gilbert's syndrome, provocative tests with restriction of the energy value of food and with a load of nicotinic acid, which cause an increase in the level of unconjugated hyperbilirubinemia, help:

Examine serum bilirubin in the morning on an empty stomach. Then, within 2 days, the patient receives food with limited energy value - about 400 kcal / day. Re-examine the level of serum bilirubin. If it turns out to be more than the initial one by 50% or more, then the sample is considered positive.

The initial serum bilirubin content is recorded. Introduce intravenously 5 ml of a 1% solution of nicotinic acid. After 5 hours, a control study of bilirubin is carried out. If its level rises by more than 25%, the test is considered positive.

One of the most convincing diagnostic tests is a stress test with the appointment of a patient with phenobarbital or zixorin - inducers of transport proteins and hepatocyte glucuronyltransferase:

10 days after the start of oral administration of phenobarbital 0 times a day or zixorin 0.2 - 3 times a day after meals in people with Gilbert's syndrome, the level of unconjugated bilirubin significantly decreases or normalizes.

It is carried out primarily with hemolytic jaundice, mainly with hereditary microspherocytosis. Criteria such as the appearance of the first clinical symptoms (jaundice) of Gilbert's syndrome in adolescence are taken into account, while hemolytic jaundice appears much earlier, in childhood. Microspherocytosis is characterized by splenomegaly and moderate anemia, which is not the case with SF. Serum bilirubin levels are usually lower in FS than in hemolytic jaundice.

Unlike chronic hepatitis, which can also be predominantly unconjugated hyperbilirubinemia, Gilbert's syndrome does not show signs of carriage of hepatotropic viruses. Unlike hepatitis, there is no hepatomegaly laboratory data indicating the presence of an active inflammatory process in the liver. The analysis of liver biopsies does not reveal signs of inflammation, necrosis of liver cells, active fibrosis. In hepatocytes, the presence of a pigment, lipofuscin, is determined.

General blood analysis.

Biochemical blood test: bilirubin, cholesterol, AST, ALT, gamma-glutamyl transpeptidase.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

Provocative tests with restriction of the energy value of food or the intake of nicotinic acid.

Exercise tests with glucuronyl transferase inducers - phenobarbital or zixorin.

SD is not a reason for prescribing any specific treatment. Prophylactic complex vitamin therapy may be indicated. It should be remembered that such people need a complete, high-calorie diet with a sufficient amount of fat in the diet. They must stop drinking alcohol. In vocational guidance, the undesirability of emotional and physical overload is taken into account. Drugs that can induce jaundice (niacin) should be avoided. In the presence of concomitant gallstone disease, cholecystectomy using minimally invasive, laparoscopic surgery is an effective way of treating it.

In the classical course of the process, the prognosis is favorable.

Dabin-Johnson syndrome (SDS) is a genetically determined enzymopathy that causes disruption of bilirubin transport in the liver, which is manifested by an increase in the content of conjugated bilirubin in the blood, jaundice, and the accumulation of melanin-like pigment in hepatocytes.

ICD10: E80.6 - Other disorders of bilirubin metabolism.

Diabetes mellitus is an inherited disorder. Individuals with DMD have an autosomal recessive genetic defect that causes impairment of the transfer of organic anions, including the transport of conjugated bilirubin from hepatocytes to the bile ducts. In men, SDD occurs more often than in women.

As a result of a violation of the mechanism of directed transport of bilirubin from hepatocytes into the lumen of the bile ducts, part of the conjugated bilirubin returns to the blood. Postmicrosomal hepatocellular jaundice occurs with a moderate increase in direct bilirubin in the blood. Pathogenetically, SDS is identical to the Rotor syndrome, from which it differs in one feature - the accumulation in hepatocytes of a large amount of melanin-like pigment, which gives the liver a dark bluish-green, almost black color. In patients with diabetes mellitus, calculi from bilirubin salts may form in the gallbladder.

Complaints of recurrent jaundice of the sclera, skin, sometimes together with slight itching are characteristic. During the period of jaundice, many patients feel general weakness, physical and mental fatigue, loss of appetite, mild nausea, bitterness in the mouth, sometimes dull aching pain in the right hypochondrium. When jaundice occurs, the urine becomes dark in color.

Jaundice can be provoked by physical and psycho-emotional stress, fever caused by a respiratory viral infection, alcoholic excess, and the use of anabolic steroids.

Biliary cholelithiasis is usually asymptomatic, but sometimes it manifests itself as biliary colic, symptoms of calculous cholecystitis, and in some cases it can cause obstructive jaundice.

Among the objective manifestations, there is a moderate icterus of the sclera and skin, a slight increase in the volume of the liver. Palpation of the liver is not compacted, painless.

Complete blood count: no deviations.

General urine analysis: dark color, high content of bilirubin.

Biochemical blood test: an increase in the content of bilirubin due to the conjugated fraction.

Samples with a load of bromsulfalein, radioisotope hepatography reveal a pronounced violation of the excretory function of the liver.

Ultrasound: the liver is of normal structure. Intra- and extrahepatic bile ducts are not dilated. Portal hemodynamics is not impaired. In the gallbladder, dense, echo-positive calculi can be detected.

Laparoscopy: the liver surface is dark bluish green or black.

Puncture biopsy: the morphological structure of the liver is not changed. Melanin-like pigment is detected in hepatocytes.

It is performed with obstructive jaundice, from which SDD is distinguished by the absence of an increase in blood cholesterol levels, the activity of enzymes specific to cholestasis - alkaline phosphatase, gamma-glutamyl transpeptidase. An ultrasound scan with SDD does not show the expansion of the intra- and extrahepatic bile ducts - a specific sign of obstructive jaundice.

General blood analysis.

General urine analysis with determination of bilirubin, urobilin, hemosiderin.

Coprogram with the definition of stercobilin.

Biochemical blood test: bilirubin, cholesterol, alkaline phosphatase, AST, ALT, gamma-glutamyl transpeptidase.

Test with bromsulfalein to assess the excretory function of the liver.

Radioisotope hepatography to assess the excretory function of the liver.

Immunological analysis: markers of infection with hepatitis B, C, G viruses.

Ultrasound of the abdominal organs.

Puncture biopsy of the liver.

No special treatment is required. Individuals with SDD should completely stop drinking alcohol. They should avoid any intoxication, limit the intake of medications as much as possible. They can be advised to take complex multivitamin preparations. In the presence of gallstone disease, especially if it proceeds with attacks of colic, cholecystectomy using minimally invasive surgery methods is indicated.

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Fatty hepatosis

Description of the disease

Fatty hepatosis is the accumulation of fat in the liver cells, which is often a reaction of the liver to various intoxications (toxic effects).

Causes

The main causes of fatty hepatosis are:

• alcohol abuse
• diabetes mellitus combined with obesity,
• obesity,
• Cushing's syndrome
• myxedema,
• unbalanced nutrition (protein deficiency),
• chronic diseases of the digestive system with malabsorption syndrome,
• exposure to toxic substances.

Symptoms

Patients with fatty hepatosis usually do not present complaints. The course of the disease is erased, slowly progressing. Over time, constant dull pains appear in the right hypochondrium, there may be nausea, vomiting, and stool disorders.

Diagnostics

The physician-therapist can suspect fatty degeneration already during a clinical examination by an increase in the liver in size during palpation of the abdomen. Liver enlargement is confirmed by abdominal ultrasound. In a biochemical blood test, an increase in liver enzymes (AST, ALT, alkaline phosphatase) is detected. In some cases, CT, MRI, and liver biopsy are performed to confirm the diagnosis.

Treatment

Traditional medicine around the world, in the treatment of fatty hepatosis, hepatomegaly and cirrhosis of the liver, offers drug, substitution and post-syndrome therapy, which can slightly improve the patient's well-being, but inevitably leads to the progression of diseases, since any presence of chemicals in a person's blood has a detrimental effect on altered liver.

However, proper nutrition, refusal of alcohol, correction of metabolic disorders, as a rule, lead to an improvement in the condition.

Fatty hepatosis in the ICD classification:

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Drug treatment of fatty liver hepatosis

The main reason for the appearance of fatty liver hepatosis is a disturbance in the work of metabolic processes. When the disease is activated, healthy liver cells are replaced with adipose tissue. The disease can be inflammatory or non-inflammatory in nature, but in any case, when the underlying causes manifest themselves, the disease must be treated appropriately.

Treatment of fatty liver hepatosis with drugs

When diagnosing fatty hepatosis, the patient needs to start timely treatment with medications, which are prescribed by the doctor in each case only individually.

There is a general basis for therapy, which is aimed at eliminating the root causes of the disease that has appeared, as well as eliminating the factors that provoked the manifestation of fatty liver hepatosis. Necessarily prescribed therapy aimed at normalizing metabolic internal processes, as well as restoring the functions of an internal organ. The patient is required to carry out intoxication therapy aimed at cleansing the liver from harmful pesticides and hazardous substances.

What drugs are indicated for patients with fatty liver hepatosis?

• A group of medications, which is aimed at protecting and restoring the basic functions of the liver, - Phosphogliv, Essentiale;
• Sulfoamino acids that stabilize internal processes - Methionine, Dibikor;
• Phytopreparations - Karsil, Liv 52.

The most effective remedy for the treatment of fatty hepatosis

Any, even the most effective medicine for unpleasant fatty hepatosis, is prescribed to patients only on an individual basis. But it is important to remember that a high-quality cure for such an ailment is impossible without fulfilling important conditions that apply to all patients with this disease:

• complete elimination from everyday life of all factors that provoked the disease to be active;
• careful correction of the habitual diet, as well as adherence to only a healthy lifestyle of daily life;
• taking prescribed medications, which are actively aimed at normalizing metabolism, as well as protecting and cleansing the liver from harmful factors.

Metformin for fatty liver hepatosis

With fatty liver hepatosis, which is not provoked by the factor of abuse of alcohol-containing liquids, Metformin is often prescribed to patients. This medication acts as a normalizer of metabolic processes and protects the internal organ from negative harmful factors.

Along with Metformin, patients may be prescribed medications such as Pioglitazone or Rosiglitazone.

Is it possible to cure fatty liver hepatosis completely?

Most patients are sure that fatty hepatosis is not fully cured. But this opinion is deeply mistaken. The specified process in the liver is reversible. And with the appointment of the correct course of treatment for fatty hepatosis, you can get rid of it forever.

The further vital activity of a person who has recovered from the underlying disease also plays an important role here. The latter must be regularly observed by the attending doctor, as well as adhere to the regular implementation of the rules of a healthy and wholesome diet.

Fatty hepatosis - microbial code 10

According to the International Classification of Diseases, fatty liver hepatosis (fatty liver degeneration) is classified under code 76.0.

All photos are taken from a free source Yandex Pictures

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