Cirrhosis

Overview

Definition 
Cirrhosis of the liver is a chronic degenerative liver disease associated with a diffuse pathological process in which normal liver cells are damaged and then replaced by scar tissue, forming excessive fibrosis and structural-anatomical regenerative nodes.
Causes of cirrhosis of the liver
You cannot get cirrhosis, but if a person is ill with viral hepatitis b, c or d, then cirrhosis can develop as a complication of a viral infection.
Cirrhosis occurs if the liver has been exposed to destructive factors for a long time. This may be, for example, systematic alcohol abuse.



Symptoms

With a highly active cirrhosis, in addition to general fatigue, lightening of the stool and darkening of the urine, there may be a dull pain in the right hypochondrium and bloating.
During the examination often reveal:
•    Subictericity (jaundice) of the sclera;
•    Expansion of the veins of the abdominal wall, resembling the head of a jellyfish;
•    Venous murmur during auscultation in the epigastric region of the abdomen (cruvelier-baumgarten murmur);
•    Gray-brown color of the neck;
•    Gynecomastia (breast enlargement);
•    Hypogonadism (in men);
•    Dupuytren's contracture (shortening of the tendons of the palms).



Causes

Metabolic disorders (for example, non-alcoholic steatohepatitis, hereditary hemochromatosis, wilson’s disease)

•    Diseases of the biliary tract (for example, extrahepatic obstruction of the biliary tract);

•    Disruption of the liver's venous outflow; +

•    Harmful substances for the liver; 

•    Immunological conditions (such autoimmune hepatitis);

•    Other causes (syphilis, schistosomiasis, sarcoidosis, hypervitaminosis a).
 



Prevention

Prevention of the development of the disease involves:
•    Timely access to medical care;
•    The need for a salt-free diet;
•    Fluid balance control (measurement of body weight, abdominal volume and daily diuresis, taking into account fluid intake);
•    Control of self-consciousness (handwriting test and physiotherapy exercises - the fight against hypodynamia).
Prevention of infectious complications is carried out by vaccination and timely administration of antimicrobial drugs. 
 



Treatment

Diet
Therapeutic measures begin with a rational diet. It should be high-calorie and high-protein (with the exception of severe forms of hepatic encephalopathy), with ascites - low-salt, with protein intake in a certain amount (based on 1.5 g / kg of body weight and up to 40 kcal / kg per day).
As a nutritional support, additional meals in the form of enteral nutrition with mixtures enriched in dietary fiber, with a low content of aromatic amino acids, are recommended.
Etiotropic treatment
Etiotropic therapy involves the treatment of the underlying disease, against which cirrhosis developed:
•    In viral hepatitis - antiviral therapy with nucleoside analogues;
•    With alcoholic steatohepatitis - the exclusion of alcohol intake;
•    With drug steatohepatitis as a result of the simultaneous administration of multiple drugs - limiting hepatotoxic and nephrotoxic drugs while maintaining the drug only for health reasons;
•    With hemochromatosis - the abolition of iron preparations.
Symptomatic treatment
Often, against the background of the course and decompensation of the cirrhosis, persistent widespread itching develops. For its relief, cholestyramine, enterosorbens and hemosorbents (activated carbon, enterosgel, polysorb) are used. Plasmapheresis has a positive effect.
To improve metabolic processes, vitamin deficiency is replenished by adding udca preparations. Some patients with primary biliary cirrhosis take these drugs for life.
Surgery
With the ineffectiveness of conservative treatment, the saved reserves of the body and taking into account age indicators, a decision is made on liver transplantation. This is especially necessary for severe hypoalbuminemia (less than 25 g/l) and persistent hyperbilirubinemia (more than 100 µmol/l).



Tests Required for Diagnosis

Patient History 
When questioning the patient, special attention is paid to the presence of a history of jaundice, pruritus, acute, chronic or hereditary liver diseases, drug use, alcohol, transfusions of blood products, etc.
A clinical blood test is necessary to determine the condition of the liver cells. People with cirrhosis have thrombocytopenia and a tendency to decrease the number of leukocytes and neutrophils in the blood with hypersplenism. ESR is often increased. Anemia of any severity may indicate recent bleeding.
A biochemical blood test depends on the cause and severity of cirrhosis. If a disease is suspected in the blood serum, the level of activity of aminotransferases (alt, ast), total and direct bilirubin is determined. To assess the protein-synthetic function of the liver, the prothrombin index is used, the amount of ammonia and the level of afp are set, and more.
Specific laboratory tests to determine the cause of cirrhosis involve determining the presence of autoantibodies and conducting serological diagnosis of hepatotropic viruses (hbsag, hbeag, anti-hbc, anti-hcv).
A functional examination in 80-90% of patients reveals an increased activity of aminotransferases (usually 2-6 times higher than normal), with autoimmune liver damage, the indicators may be higher.
Serum gamma globulin (increased in 90% of patients with cirrhosis), sublimate test (positive in 85% of patients), bilirubin level (increased by 2-5 times in 80-90% of patients) and ammonia content (increased in 80% of patients) are important. 
Fibromax test: Non-invasive (without biopsy) structural assessment of the state of the liver tissue. It consists of several laboratory studies, the results of which are used to calculate the five main indicators of the state of the liver tissue:
•    Fibrosis stage according to the metavir scale (fibro test);
•    The degree of necroinflammatory reaction (acti test);
•    The degree of steatosis and fatty degeneration of the liver tissue (steato test);
•    Diagnosis of non-alcoholic steatohepatitis (nash test);
•    Diagnosis of alcoholic liver damage with determination of its activity (ash test).
 Fibromax is an alternative to liver biopsy. It allows you to comprehensively assess the changes caused by liver disease. 
Ultrasound examination (USG) is a highly informative method for diagnosing cirrhosis, which allows you to make a preliminary diagnosis. With its help, it is possible to detect an increase in the density of the liver, nodular deformation of the contours, expansion of the intrahepatic branches of the portal and splenic veins, as well as the size of the spleen. With highly active cirrhosis, with the help of ultrasound and ultrasound, clear changes are detected in 80% of the examined, with low-active forms of cirrhosis - in 40-60%.
Computed tomography (CT) is superior in diagnostic information to ultrasound, so it is necessarily performed in doubtful cases and in case of suspicion of oncological diseases.
Magnetic resonance imaging (MRI) has no advantage over CT, but a type of MRI such as magnetic resonance cholecystopancreatography should be performed in people with primary biliary cirrhosis.
Ultrasound (doppler) is carried out mainly for the diagnosis and assessment of the severity of portal hypertension.
Ultrasound elastography (elastometry) is a non-invasive determination of liver elasticity associated with the stage of fibrosis according to the metavir scale.
Esophagogastroduodenoscopy in 60-70% of cases reveals nodular dilatation of the veins of the esophagus (sometimes the veins of the cardial part of the stomach), and in 15-20% - signs of various stages of peptic ulcer.
Liver biopsy is a morphological invasive study with high information content and specificity. It is the gold standard in the diagnosis of cirrhosis and other liver diseases. 
Differential diagnosis
Differential diagnosis of cirrhosis is carried out with non-cirrhotic causes of liver enlargement, ascites, portal hypertension and encephalopathy.
In highly active forms of cirrhosis, it is important to differentiate the disease from acute (viral, alcoholic or drug-induced) hepatitis. For this, markers of hepatitis viruses, pcr and immunology are widely used.
Also, differential diagnosis of liver cirrhosis is carried out with:
•    Obstruction of the bile ducts;
•    Toxic effects of various substances, heavy metals and drugs;
•    Autoimmune hepatitis;
•    Primary and secondary biliary cirrhosis;
•    Primary sclerosing cholangitis;
•    Liver metastasis;
•    Non-alcoholic fatty liver disease ;
•    Congenital pathologies (inherited hemochromatosis, wilson-konovalov disease, alpha 1 antitrypsin deficiency).



Useful info

Classification and stages of development of liver cirrhosis
Histological classification:
•    Micronodular (small-nodular) form - nodes of the same size with a diameter of 1-3 mm, the node consists of parts of one lobule;

•    Macronodular (large-nodular) form - nodes of various sizes with a diameter of 3 mm to several cm, irregular septa, vary in width, the node consists of parts of several lobules;

•    Mixed form or large- and small-nodular cirrhosis - the number of small and large nodes is approximately the same;

•    Incomplete septal cirrhosis - full-fledged nodes are not formed, but septa cross the parenchyma. Part of the septa can be classified as incomplete, as they are incomplete.
Classification according to illness progression:
•    Latent cirrhosis - clinical, biochemical and morphological signs of cirrhosis activity are not observed. Portal hypertension and liver failure are very rare. This form of cirrhosis in most patients does not affect life expectancy unless other causes of cirrhosis are activated.

•    Sluggish cirrhosis - clinical signs are often absent, biochemical markers of the disease appear only when cirrhosis is activated, morphological signs are moderately expressed. Portal hypertension is formed extremely slowly, and functional liver failure often does not develop. 

•    Slowly progressive (active) cirrhosis - biochemical and morphological signs are expressed, clinical manifestations are fuzzy. Portal hypertension develops slowly. The life expectancy of most patients is 10 years or more.

•    Rapidly progressing (active) cirrhosis - all signs of high cirrhosis are present. Progressive portal hypertension and liver failure. The life expectancy of most patients is about five years.
•    Subacute cirrhosis is an almost immediate transition of acute hepatitis to cirrhosis with a fatal outcome. It is characterized by symptoms of the initial stage of cirrhosis, which develops against the background of acute hepatitis. The duration of the disease is 4-12 months.

Classification by etiology:
•    Biliary cirrhosis of the liver ;
•    Alcoholic cirrhosis of the liver;
•    Toxic cirrhosis of the liver (due to drugs, dietary supplements, poisons, etc.);
•    Viral cirrhosis of the liver;
•    Autoimmune;
•    Genetic (metabolic);
•    Cardiac;
•    With an unknown cause (cryptogenic).

Complications of cirrhosis of the liver
•    Gastrointestinal bleeding;
•    Ascites;
•    Acute kidney injury (hepatorenal, or hepatorenal, syndrome);
•    Pulmonary hypertension;
•    Hepatopulmonary syndrome - insufficient blood oxygen saturation;
•    Heart failure.
Also complications of liver cirrhosis can be hepatic encephalopathy (a reversible syndrome accompanied by mood swings, confusion, drowsiness, disorientation, and in severe cases coma) and bleeding disorders.
If a patient with cirrhosis develops ascites, infection of the ascitic fluid with bacteria and the development of spontaneous bacterial peritonitis (inflammation of the peritoneum) can complicate it.
Also, with cirrhosis of the liver, hepatocellular carcinoma, a malignant tumor, can become a complication.



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