Cirrhosis

The term was invented by Laennec to define the disease which gives to the liver reddish granulations. The cirrhosis is a chronic disease of the Foie in which hepatic architecture is upset in a diffuse way by one necroses Hépatocyte S, followed lesions of Fibrose alternating with beaches of cellular regeneration which do not respect any more the initial organization lobulaire.

These zones of regeneration are called “nodules of regeneration”. The cirrhosis is the alcoholic consequence of aggression hépatocytaires prolonged, , viral, or others (Médicament S, enzymatic deficit, Maladie of Wilson, etc).

Cirrhosis has Chronic disease off the Liver in which liver tissue is replaced by Connective tissue, resulting in the loss off liver function. Cirrhosis is caused by ramming from Toxin S (including alcohol), metabolic problems, chronic viral Hepatitis gold other causes. Obsolete Cirrhosis is sometimes referred to by its Eponym Laennec' S cirrhosis after Rene Laënnec. Cirrhosis is irreversible goal treatment off the causative disease will slow fox trot gold even halt the ramming.

Cirrhosis may refer to chronic interstitial ignition off any tissue, goal is rarely used for other diseases than cirrhosis off the liver. -->

Symptoms

Revealing symptoms

For one more or less long period known as noncomplicated only a clinical examination or biological can highlight the marks of cirrhosis. At a more advanced stage, the revealing signs of a cirrhosis can be:
  • asthenia and slimming
  • ascite (presence of liquid inside the cavity péritonéale)
  • digestive hemorrhage, generally by rupture of varixes œsophagiennes
  • encephalopathy with disorders of conscience and flapping, whose physiopathology is multifactorielle (in particular by the means of a hyperammoniemy)
  • ictère (yellow coloring of the skin and mucous membranes)

Clinical signs

  • increase in the volume of the liver (Hepatomegaly) and sharp consistency with palpation
  • signs of insufficiency Hépatocellulaire
    • Ictère
    • stellar Angiomas (
    • erythema palmar (palm of the hand very red)
    • hepatic Foetor, odor characteristic and nauseous of the breath
    • Astérixis or flapping tremor (abrupt and repeated loss of tonicity muscles of the hand)
  • signs of Hypertension portale
    • Splénomégalie (increase in the volume of spleen)
    • abdominal collateral circulation (dilation of the veins under cutaneous of the area abdominal)
  • mixed signs
    • Ascite
    • edema of the pleural lower extremities taking the cup
    • épanchements (presence of liquid in the pleural cavity)
  • biological Signs
    • reduction in the rate of prothrombine (by reduction in certain factors of coagulation, in particular the factor V)
    • Hypoalbuminemie
    • Possibly increase moderate in transaminases and cholestase (increase in alkaline phosphatases and the GGT, sometimes in the combined bilirubine)

Etiology

  • the chronic ethyl intoxication is the first cause in frequency in France, particularly for the men (the situation is different for countries as the United States where the alcoholic cirrhoses are minority for the patients of the two sexes).
  • viral hepatites especially B, (D) and C, in their chronic form.
  • the metabolic Syndrome
  • the Cholestase extra hepatic prolonged can involve a true cirrhosis known as secondary biliary Cirrhose.
  • the right Cardiac failure prolonged involving a cardiac liver can also become complicated cardiac cirrhosis
  • obstruction of the know-hepatic veins (Syndrome of Budd-Chiari)
  • disease genetics (Hémochromatose, Maladie of Wilson, hereditary Déficit in alpha-1 antitrypsin…).
  • autoimmune diseases.
  • Galactosémie .
  • Disease of glycogen overload.
  • combination of certain drugs with alcohol (for example the Méthotrexate).

  • Alcoholic liver disease (ALD). Alcoholic cirrhosis develops after more than has off decade heavy drinking in 15% off all alcoholics. There is great variability in the amount off alcohol needed to causes cirrhosis (3-4 drinks has day in nap men and 2-3 in nap women). Normal Alcohol seems to insult the liver by blocking the metabolism off protein, conceited people, and carbohydrates.
  • Chronic Hepatitis B (with gold without D agent). The Hepatitis B virus is probably the most common off causes cirrhosis worldwide, especially South-East Asia, goal it is less common in the United States and the Western world. Hepatitis B causes liver ignition and injury that over several decades edge lead to cirrhosis. Hepatitis D is dependant one the presence off hepatitis B, goal accelerates cirrhosis in Co-infection.
  • Chronic Hepatitis C . The hepatitis C virus ranks with alcohol ace has major causes off chronic liver disease and cirrhosis. Infection with this virus ignition causes off and low rank ramming to the liver that over several decades edge lead to cirrhosis.
  • Autoimmune hepatitis. This disease is caused by the immune system attacking the liver and causing ignition, ramming, and eventually scarring and cirrhosis.
  • Inherited diseases. Thesis interferes with the way the liver produces, processes, and blinds enzymes, proteins, metals, and other substances the body needs to function properly.
    • Alpha 1-antitrypsin deficiency
    • Hemochromatosis (iron accumulation)
    • Wilson' S disease (copper accumulation)
    • Galactosemia
    • Glycogen storage disease S
    • Cystic fibrosis
  • Non-alcoholic steatohepatitis (NASH). In NASH, conceited builds up in the liver and eventually causes scar tissue. Standard This off hepatitis appears to Be associated with diabetes, protein malnutrition, obesity, coronary artery disease, and treatment with corticosteroid medications.
  • Diseases that lead to chronic obstruction off the Bile duct S. Accumulated bile rammings liver tissue:
    • In babies, blocked bile ducts are most commonly caused by Biliary atresia, has disease in which the bile ducts are absent gold injured.
    • In adults, the most common causes is primary biliary cirrhosis, has disease in which the ducts become inflamed, blocked, and scarred.
    • Secondary biliary cirrhosis edge happen after gallbladder surgery yew the ducts are inadvertently tied off gold injured.
  • Drug S gold Toxin S.
  • Repeated ends off Heart failure with liver congestion.
  • Some parasitic infections (like Schistosomiasis). -->

    • Diagnosis

    The suspicion of a cirrhosis is based on the whole of the clinical and biological arguments found at a patient. The diagnosis of certainty is made by the anatomopathologic analysis of hepatic fabric. The material to be analyzed is generally provided by the hepatic biopsy. The hepatic biopsy is useful to affirm the diagnosis of cirrhosis and contributes to the diagnosis etiologic of this one.

    Positive signs of cirrhosis:

    • clinical: sharp consistency of the liver and clinical signs of hépato-cellular insufficiency (stellar angiomas, etc)
    • biological: fall of the factors of coagulation (rate of prothrombine, factor V) and hypoablbuminemy, block beta-gamma with the electrophoresis of serum proteins
    • an abdominal echography is systematic (heterogeneous and nodular hepatic parenchyma, embossed contours, hypertrophy of segment I or caudé lobe, signs of hypertension portale…)
    • hepathic biopsy ( Seule the hepatic biopsy makes the diagnosis of cirrhosis )

    Diagnosis etiologic:

    • concept of ethyl intoxication chronic, clinical signs of chronic ethyl intoxication and signs biological (thrombopénie, macrocytose, rise - inconstant and aspécifique- of the gammaGT)
    • viral serologies (hepatitis B and C)
    • autoantibody (auto-immune hepatitis, primitive biliary cirrhosis)
    • proportioning of alpha 1 antitrypsin (exceptional deficits in alpha 1 antitrypsin) and proportioning of the céruléoplasmine (in the search of a disease of Wilson)

    For a diagnosis of gravity and a forecast:

    One uses the scores of CHILD and CHILD-PUGH to allow classification in 3 groups according to the hépato-cellular degree of insufficiency. The forecast is strongly related to the continuation of the intoxication Score of Child-Pugh allotted Points :

    • Class a: 5 to 6

    • Class b: 7 to 9,
    • Class C: 10 to 15.

    Pathological anatomy

    The Histologie of the cirrhosis shows:
    • a fibrosis in bridge
    • delimiting nodules of regeneration, fact of sometimes dysplasic spans bicellulaires of hépatocytes

    The liver can be atrophied or conversely hypertrophied according to the prevalence of lesions of destruction or regenerations.

    Other lesions are visible, they are characteristic of the mechanisms or etiologies in the beginning of the cirrhosis.

    • alcoholic Cirrhosis:
      • the hepatic steatosis macrovésiculaire,
      • of the signs of ignition with an infiltration of Lymphocyte S and Macrophage S,
      • the bodies of Mallory,
      • one necroses hépatocytaire,
      • giant Mitochondrie S,
      • hépatosidérose.

    • Disease of Wilson:

      • deposits of copper in the hépatocytes.

    • Hémochromatose :

      • hepatic Iron in high concentration.
      • Deposits of hémosidérine.

    • primitive biliary Cirrhosis:

      • modification of the spans of the bile ducts.

    Methods of hepatic biopsy

    • Way trans-chin-strap.
    • trans-parietal Way (intercostale).
    • laporoscopic Biopsy.

    These techniques present difficulties and advantages suitable for each one.

    Use of colorings éosine-saffron, trichromatic of Masson, Perls and the STEP.

    Proportioning of the iron weight and search for viral antigens.

    Pathology

    Physiopathology

    For a cirrhosis of the liver of origin alcoholic:

    • the day laborer quantity of pure alcohol suitable for induce a cirrhosis is on average of more than 30 grams per day during approximately 10 years at the man (less at the woman)
    • the quantity and the duration of the intoxication by alcohol determine occurred of the cirrhosis
    • it exists variations of individual susceptibility vis-a-vis alcohol consumption, this average quantity being only indicative

    Treatment

    • treatment of the cause
    • complete stop of alcohol
    • prudence in the administration of drugs, a great number being metabolized by the liver.
    • symptomatic treatment: diuretic, punctures of ascite, prevention of the rupture of varixes oesophagiennes (béta-blocking and/or bindings endoscopic according to the cases)
    • transplantation of the liver, in selected indications and in the absence of counter-indication.
    • tracking of hépato-cellular carcinome (cancer of the liver) by echography and proportioning of the alpha-fetoprotein

    Epidemiology

    Consequences and complications

    • hepatic Insufficiency.
    • digestive Hemorrhages especially œsophagiennes.
    • Cancer of the liver (hepatocarcinomist).
    • higher hemorrhagic Risk (it is the liver which synthesizes the factors of coagulation).

    References

    • MedInfos, alcoholic cirrhosis, December 2006

    • Treated medicine, directed by Jean Hamburger, Flammarion, ISBN 2-257-10286-X
    Cirrhosis off the Liver . NIH Publication No 04-1134, December 2003.
    • Iredale JP. Cirrhosis: new research provides has basis for rational and targeted treatments. BMJ 2003;327: 143-7. Fulltext.PMID 12869458
    • CIRCLE, Alain, alcoholism, Paris: Flammarion, 1998,127p.
    • LEDERMANN, Sully, Alcohol, alcoholism, alcoholization, Paris: PUF, 1956,613p.
    • SOURNIA, Jean-Charles, History of Alcoholism, Paris: Flammarion, 1986,324p.
    • Photographs At: Atlas off Pathology-->

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