Cirrhosis

The liver cirrhosis (Greek κίρρωσις kírrosis, yellow - orange ', by René Laënnec term coined ) is the end stage of chronic liver diseases. This stage is considered to be irreversible, even if individual reports of healings exist. Typically, cirrhosis developed over years to decades, rarely are faster gradients of less than one year. Almost all chronic liver diseases lead to end-stage liver cirrhosis. In Europe, alcohol abuse and chronic viral hepatitis are the most common causes.

Due to the chronic period of decline and regeneration of liver tissue results in a disordered tissue architecture with nodular changes. In addition, excessive connective tissue ( fibrosis ) forms. This scarred areas can occupy more than 50 % of the entire fabric a cirrhotic liver. Thus, the blood flow to the liver is disturbed, in the portal vein, the blood accumulates in front of the liver ( portal hypertension ).

The incidence, ie the number of new cases is in industrialized countries 250 per 100,000 inhabitants and year. The ratio of affected men to women is 2:1.

Liver cirrhosis is not to be confused with the much more common fatty liver.

Causes

Classification according to etiology:

• Alcoholic liver cirrhosis ( in developed countries with 50 % common cause)
• Cirrhosis in chronic viral hepatitis ( in industrialized countries with 20-25 % second leading cause in Africa with 90 % natural abundance ) - mostly hepatitis C, which remains chronic in 85% and leads to cirrhosis in 35%
• Cryptogenic cirrhosis ( without demonstrable etiology )
• Stauungszirrhose ( cirrhosis cardiaque ), typical of right heart failure
• Autoimmune hepatitis
• Fatty liver hepatitis ( ASH, NASH )
• Rare forms of cirrhosis with a defined etiology hemochromatosis
• Wilson's disease
• Galactosemia
• Hereditary fructose intolerance
• Cystic fibrosis
• Glycogen Storage Disease
• Cholangiodysplastische liver cirrhosis
• Primary sclerosing cholangitis
• Budd -Chiari syndrome
• Tropical Diseases
• Cirrhosis in alpha - 1 antitrypsin deficiency
• Primary biliary cirrhosis
• Secondary biliary cirrhosis
• Hepatopathy in celiac disease (gluten - sensitive enteropathy )

Pathogenesis

The cause of cirrhosis is the necrosis (death ) of liver cells such as those caused by viruses or toxins. The cells release freely cytokines that activate one hand, liver macrophages ( Kupffer cells) and fat storage cells of the liver ( Ito cells ) and on the other hand, monocytes and granulocytes from the blood. These cells generate the organ structure with parenchymal necrosis, formation of regenerative nodules ( Pseudolobuli ) and fibrous septa becomes destructive rebuilt. Through this Bindegewebsknoten the channels of the liver to be interrupted to bring the bile through the bile canaliculi ( canaliculi and duct ) to the gallbladder, lead nutrients from the portal blood into the body, the hepatocytes wash ashore pollutants to detoxify and supply the liver with oxygenated blood. Bile ducts form may be new, but end blindly. The consequences are congestion between the liver and digestive tract ( portal hypertension ), through which ascites forms and increases the spleen. In the worst case, there is Ösophagusvarizenblutungen. The failure of hepatocytes leads to other secondary diseases such as hepatic encephalopathy and hepatic coma.

The pathologist distinguishes the micronodular, macronodular and the gemischtknotige cirrhosis after the appearance of the organ. The liver shrinks, its surface is wrinkled and gnarled. Microscopically can be active or florid (ie progressive ) and inactive cirrhosis differ. The preliminary stage of cirrhosis is the fatty liver.

Symptoms

Cirrhosis of the liver affects the subjective perception of the affected patient often only at a fairly late stage. The liver function may be affected very differently with respect to the synthetic function ( coagulation factors, albumin ) and the detoxification function ( hepatic coma ). In addition, the secondary diseases ( following from esophageal varices = varicose veins in the esophagus ) ( lack of platelets) and hepatic encephalopathy often play as collateral circulation, portal hypertension, ascites or splenomegaly an important role. Other typical symptoms include redness of the palms ( palmar erythema ), caput medusae, spider nevi ( nevus araneus ), fissures, paint and tongue edema. In the so-called Child -Pugh score classification more of these factors are included (bilirubin, prothrombin time, albumin, encephalopathy and ascites) and from a score calculated; the resulting division into stages A to C allows a statement about the prognosis of the disease, patients with stage C according to Child -Pugh have a very poor prognosis in terms of survival time. The encephalopathy and ascites only be judged in three severity levels, which leads to more or less subjective, inaccurate values ​​. That's why since 2002, they also uses the MELD score, which is calculated according to a formula based on laboratory parameters ( creatinine, bilirubin and INR).

Liver cirrhosis are considered facultative precancerous condition, that is, that a malignant tumor may develop on the floor of liver cirrhosis, hepatocellular carcinoma ( HCC).

As the central symptoms of cirrhosis of the liver, also known as cirrhosis of the liver reduced performance, poor concentration and fatigue are. Added to this are the so-called liver skin characters that make, among other things through red colored little finger, by a yellowish skin ( jaundice ) and Nevus noticeable.

Diagnosis

In the investigation often fall jaundice, an increased waist circumference ( ascites due ), edema, gynecomastia, skin bleeding, as well as hepatic encephalopathy, a flapping tremor and impaired consciousness on.

Prurigo Spider nevi (vascular spider), " milk glass nails " ( Opaque white discolouration of the nails with distal longitudinal red - brown staining, also called " Terry - nails " ), ( eczema ), paint tongue: Typical, but not until late occurring, are the so-called liver skin characters, skin atrophy ( " Thinning of the skin ") and palmar or Plantarerytheme (redness of the palms and the soles ). Occasionally, however, early fingernails changes such as the 1954, the first time mentioned by the British physician Dr. Richard Terry Terry's nails are an important indicator of an underlying systemic disease, eg liver cirrhosis. These signs are, however, not only to the hepatic cirrhosis specific, but can also be in discrete form with other liver disorders, such as fatty liver occurs. This can also be temporary, for example during pregnancy, the case. Other liver signs are caput medusae, Dupuytren's contractures, as well as a lack of Bauchbehaarung in men ( Abdominalglatze, "belly bald ").

In the laboratory, reduced values ​​for cholinesterase, albumin, and some coagulation factors fall by the decreased synthetic capacity of the liver ( decreased prothrombin time ). The liver enzymes GOT (AST ), GPT (ALT ) and γ -GT, bilirubin and ammonia can be increased.

In ultrasound, the liver is inhomogeneous dar. The liver edge is wavy, inland vessels are rarefied. The caudate lobe may be enlarged. Very good ascites and splenomegaly (splenomegaly ) can be detected with ultrasound. An improved form of sonography, the so-called FibroScan, also known as transient elastography, dar. This can be used to determine the fibrosis, ie the Bindegewebsumbau, the liver, which is a very reliable result for the diagnosis and provides the future liver biopsy (see below) could replace. With the color-coded duplex sonography can be divided into the hepatic vein decreased elasticity of the liver, measuring an increased peripheral resistance in the portal vein as well as a reduced flow in the hepatic artery.

The definitive diagnosis is made by a liver biopsy.

For various examination findings of the Child-Pugh score is created, which serves both for staging (Child AC) as well as for estimation of prognosis.

Therapy

The base form of therapy General measures such as avoidance of all potentially hepatotoxic substances (alcohol, drugs), balancing a vitamin deficiency (eg, vitamin B1 in alcoholism ) and a sufficient supply of energy, in hepatic encephalopathy restriction of protein intake.

The underlying disease must be treated at an alcoholism detoxification treatment is attempted, patients with autoimmune hepatitis are treated with immunosuppression in a chronic hepatitis virus elimination can be attempted with interferons.

On complications will react with specific actions: hemostasis of esophageal varices, surgical shunt in hepatic encephalopathy, aspiration for persistent ascites.

Important are regular examinations for early detection of liver cancer. A last resort is the liver transplantation in many cases.

Probably caffeine protects the liver from the formation of cirrhosis and delayed their development.

In a clinical study approved by ethical principles in the center of Abdominal Surgery in Zurich, a new form of treatment in severe chronic cirrhosis of the liver has been studied for several years successfully by Hans Ulrich Baer, ​​be used in the body's liver cells to improve liver function. In a minimal liver operation, pieces of tissue of the diseased liver and pancreas, the function here as stimulating cells, collected and processed in a special laboratory. These are mixed and applied to a carrier substance. Two days later, the cell mixtures are reintroduced into the patient's body in the suspension straps of the small intestine. This cell mixture according to take about two weeks important functions of diseased liver and partially or completely normalize hepatic function. The cells used are not stem cells, so that many ethical concerns of stem cell research for this procedure do not exist.

Forecast

The prognosis depends on the cause, the successful causal treatment, the complications and the stage. Thus, the one-year survival rates for patients with stage Child A nearly 100 %, with about 85% Child B and Child C at 35%. The MELD score is statements for survival in the next three months can make. Thus, a patient in the hospital with a score of 20-30, a 25% risk of dying in the next three months. A cirrhotic with a MELD of 40 died safely in three months.

History

The first macroscopic description of liver cirrhosis in the history of medicine can be found in the notes to the drawing del Vecchio by Leonardo da Vinci (1452-1519 ). The drawings on the vascular anatomy of the liver based on an autopsy, the Leonardo da Vinci in 1508 undertook in Florence at a more than 100 - year-old.