Cirrhosis of the liver

Cirrhosis of the liver is a chronic pathology of the liver with the gradual death of hepatocytes, widespread fibrosis and regenerated nodules, gradually replacing the parenchyma; accompanied by insufficiency of hepatocyte functions and a change in the blood flow of the liver, which leads to jaundice, portal hypertension and ascites. The frequency is 2-3% of the population (based on autopsy data), observed 2 times more often in men over 40 years old. The reasons

- Alcoholism
- Viral hepatitis (B, C, D)
- Inherited diseases (a1-antitrypsin deficiency, Wilson-Konovalov disease, hemochromatosis, type IV glycogenosis, galactosemia)
- drugs and toxins
- Diseases of the biliary tract (primary sclerosing cholangitis, cholelithiasis and bacterial cholangitis)
- Parasites (schistosomiasis, clonorchiasis)
- Obstruction of the venous outflow (Budd-Chiari syndrome, veno-occlusive diseases)
- Chronic circulatory failure (right ventricular failure, tricuspid valve stenosis, rheumatic heart disease, cardiomyopathy, constrictive pericarditis)
- Idiopathic (up to 20%). Pathomorphology
- Fibrosis. Classical picture: wide fibrous strands, consisting of dense fibrous connective tissue with blood vessels and bile ducts, surround nests of hepatocytes, dividing the parenchyma of the organ into irregularly shaped fields; there are foci of chronic inflammation, consisting mainly of lymphocytes and macrophages
- Nodules of various sizes consist of hepatocytes that form strands, islets, layers
- disorganization of the parenchyma. Fibrosis, infiltrates and nodules significantly distort the normal architectonics of the organ. Nodules from regenerating liver cells compress the parenchyma. Fibrous bands partially obliterate the vessels and bile ducts. Signs of cholestasis are expressed. The blood supply to the nodules is often insufficient, and their necrosis is possible.
- Obesity and Mallory bodies are typical of alcoholic cirrhosis
- Viral Ag and vitreous nature of hepatocytes are observed in postnecrotic cirrhosis.
- Spherical cytoplasmic globules are noted in a1-antitrypsin deficiency.
- Significant content of hemosiderin in hemochromatosis, copper in Wilson-Konovalov disease
- Fatty degeneration of the liver is characteristic of alcoholics, brown liver – for hemochromatosis. In many forms of cirrhosis, iron accumulates in the liver in the form of brown hemosiderin. A greenish-yellow color is observed in biliary cirrhosis.

Clinical picture

- Loss of appetite, poor tolerance to fatty foods, nausea, vomiting, diarrhea.
- Weakness, high fatigue, decreased work probability.
- Frequent persistent feeling of heaviness and pain in the right hypochondrium.
- Hepatic signs – telangiectasias (asterisks, spiders) in the face and shoulder girdle, erythema of the palmar and digital elevations (liver palms), blanching of the nails (a sign of low serum albumin), deformity of the terminal phalanges of the fingers in the form of drumsticks, red (varnished) language.
- Increase in body temperature.
- Portal hypertension – enlargement of the spleen and / or hypersplenism, ascites, varicose veins of the esophagus, stomach, hemorrhoidal veins, bleeding from them (rectal bleeding is rare).
- Hepatocellular insufficiency – hemorrhagic diathesis (bleeding of the mucous membrane of the nose and gums, subcutaneous petechiae and hemorrhages, localized or generalized purpura), jaundice, hepatic encephalopathy.
- Anemia: microcytic hypochromic anemia (with gastrointestinal bleeding), macrocytic anemia due to impaired metabolism of vitamin B] 2 and folic acid, hemolytic anemia is almost always combined with an enlarged spleen.
- Endocrine disorders – dysmenorrhea, amenorrhea, uterine bleeding, miscarriages or infertility, disorders of secondary hair growth, acne, gynecomastia, testicular atrophy.
- Changes in the liver: compaction with deformation of the plane (often not observed in the small-nodular form) and sharpening of the anterior edge; both lobes initially increase (by no more than 3–10 cm), then the left lobe is predominantly increased with normal or reduced volumes of the right lobe, both lobes decrease in the final stages.

Complications

- Ascites
- Varicose veins (more often noted in the esophagus and hemorrhoidal plexus)
- Hepatic encephalopathy (reversible syndrome with characteristic mood swings, confusion, drowsiness, disorientation, and coma)
- Hepatorenal syndrome (progressive functional renal failure that occurs in patients with severe liver pathology, mortality – 90-100%)
- coagulation disorders. Laboratory research
- An increase in the content of bilirubin and aminotransferases, a decrease in the content of prothrombin and cholesterol, dysproteinemia (an increase in the amount of γ-globulin, hypoalbuminemia, a change in sediment samples) are indicators of functional liver failure
- Detection of HBiAg (hepatitis B) and/or anti-HCV (hepatitis C)
- Peripheral cytopenia (leukopenia, neutropenia, thrombocytopenia, anemia) – in the stage of decompensation, with an advanced picture of hypersplenism.

Special Studies

- Liver biopsy
- Radionuclide liver scan: diffuse decrease in isotope uptake, uneven distribution of the radioactive product, increased accumulation in the spleen
- X-ray examination with a suspension of barium sulfate: varicose veins of the esophagus
- Visceral angiography to determine anatomy, vascular patency, and the presence of collaterals
- FEGDS: degree of severity

varicose veins of the lower 2/3 of the esophagus, fornix and cardia of the stomach

- Ultrasound: dilation of the bile ducts and assessment of the size of the affected area
- Doppler ultrasound: determine the direction of blood flow in the portal and hepatic veins and their patency.

Differential Diagnosis

- Cirrhosis and primary liver cancer
- Diseases accompanied by asymptomatic enlargement of the liver (fatty liver, hepatitis, amyloidosis, glycogenosis)
- Myeloproliferative and oncological diseases
- Constrictive pericarditis with liver enlargement and ascites
- Alveolar echinococcosis
- Metabolic encephalopathies – renal, cardiopulmonary, therapeutic
- Diseases accompanied by bleeding from the upper gastrointestinal tract and / or ascites.

Treatment:

Tactics of conducting

- Gentle mode, limitation of physical activity
- Diet number 5
- With dyspeptic disorders – diet number 5a
- With the appearance of diarrhea, steatorrhea – restriction of fats to 50-60 g / day, exclude milk, honey, jam
- If symptoms of portal hypertension appear, a diet with a normal content of proteins, fats, carbohydrates
- When signs of protein metabolism disorders appear, the number of proteins, salts is sharply reduced.
- Complete exclusion of alcohol
- Prohibition of physiotherapeutic procedures, insolation, vaccination, intake of hepatotoxic products, incl. psychotropic and sleeping pills
- Elimination of dyspeptic disorders with the help of rational nutrition, vitamin therapy (ascorbic acid, vitamin B complex) and enzyme products that do not contain bile acids (pancreatin, mezim-forte, etc.)
- Drugs that improve the metabolism of liver cells (cocarboxylase, lipoic acid, glutamic acid, Essentiale)
- Glucocorticoids in the active stage of viral and biliary cirrhosis, as well as in severe hypersplenism
- In chronic hepatitis B or C – interferon therapy. Therapy of complications
- For varicose veins
- Short-term courses of intravenous vasopressin infusions (traditionally in combination with nitroglycerin) are effective in 60% of patients
- Balloon tamponade (using a Sengstaken-Blackmoor probe) stops bleeding in 80% of patients, but the procedure is accompanied by a high risk of aspiration and rupture of the esophagus. With emergency surgery, mortality is 50%
- In an emergency, somatostatin may be used
- Endoscopic sclerotherapy or endoscopic ligation of vessels is effective in 80-90% of acute cases. To reduce portal pressure and stop bleeding from varicose veins, it is effective to apply (under X-ray control) an intrahepatic portosystemic shunt connecting the hepatic and portal veins
- To reduce the likelihood of recurrent bleeding, long-term treatment is used using multiple endoscopic sclerotherapy or ligation of varicose veins until they are completely obliterated.
- It is possible to use non-selective B-blockers, for example propranolol (anaprilin) to reduce portal pressure
- Surgical portal decompression reduces the incidence of bleeding, but does not increase survival (development of encephalopathy and liver failure). In Japan, semi-

There is a good clinical effect from devascularization of the distal part of the esophagus and the proximal part of the stomach (Sugiura operation).

- With ascites
- In connection with the likely development of hyponatremia – restriction of fluid intake. In the absence of effect – spironolactone 100-400 mg 1 r / day every day. If you need to get a faster effect, additionally – furosemide 40-80 mg / day
- Paracentesis is performed with large amounts of ascitic fluid; for every 1 liter of fluid removed, 10 g of albumin will be needed intravenously
- In resistant cases, surgical shunting (for example, Levin shunt) with prophylaxis of bacteremia and DIC.
- With encephalopathy
- Lactulose 15-30 ml / day orally (as an NH4 acceptor
- in the intestines and a laxative)
- Neomycin (suppresses intestinal flora converting proteins to ammonia)
- Restriction of protein content in the diet (no more than 40 g / day).
- With spontaneous bacterial peritonitis, a combination of ampicillin and an antibiotic aminoglycoside or cefotaxime; subsequent administration of norfloxacin 400 mg/day reduces the likelihood of relapse.
- With hepatorenal syndrome, there are no means of pharmacological correction. All patients are given fluid loading during hypovolemia. In some cases, the clinical effect is achieved by the imposition of a LeVine shunt, portocaval shunts.

Concomitant pathology

- Hepatitis
- Sjögren’s syndrome
- Goiter Hashimoto
- Diseases and injuries of the bile ducts
- Peptic ulcer of the duodenum.

The course of inactive cirrhosis is slowly progressive (many years and decades), there are frequent periods of long-term remission with a satisfactory state of health, liver tests close to normal. With active cirrhosis, the progression of the disease is rapid (several years), clinical and laboratory manifestations of the activity of the process are significant (an increase in the thymol test up to 8 units and y-globulins up to 30% is regarded as moderate activity, and above these limits, as a pronounced activity). Prognosis unfavorable with active cirrhosis, somewhat better with inactive, compensated. In secondary biliary cirrhosis, the prognosis is determined by the causes that caused blockage of the bile duct (tumor, stone, etc.), and the likelihood of their elimination. The prognosis of patients with bleeding from varicose veins of the esophagus and stomach in history worsens; such patients live no more than 1-1.5 years and do not often die from repeated bleeding. See also Acute viral hepatitis, Chronic hepatitis, Cirrhosis

liver biliary, ascites

ICD

- K70.3 Alcoholic liver cirrhosis
- K74 Fibrosis and cirrhosis