Cirrhosis Blog

The liver supports almost every organ in the body and is vital for survival. Because of its strategic location and multidimensional functions, the liver is also prone to many diseases.

The most common include: Infections such as hepatitis A, B, C, E, alcohol damage, fatty liver, cirrhosis, cancer, drug damage (especially acetaminophen (also known as paracetamol) and cancer drugs)

Many diseases of the liver are accompanied by jaundice caused by increased levels of bilirubin in the system. The bilirubin results from the breakup of the Hemoglobin of dead red blood cells; normally, the liver removes bilirubin from the blood and excretes it through bile.

There are also many pediatric liver diseases including biliary atresia, alpha-1 antitrypsin deficiency, alagille syndrome, progressive familial intrahepatic cholestasis, and Langerhans cell histiocytosis, to name but a few.

Diseases that interfere with liver function will lead to derangement of these processes. However, the liver has a great capacity to regenerate and has a large reserve capacity. In most cases, the liver only produces symptoms after extensive damage.

Liver diseases may be diagnosed by liver function tests, for example, by production of acute phase proteins.

The term biliary tree is derived from the arboreal branches of the bile ducts. The bile produced in the liver is collected in bile canaliculi, which merge to form bile ducts. Within the liver, these ducts are called intrahepatic (within the liver) bile ducts, and once they exit the liver they are considered extrahepatic (outside the liver). The intrahepatic ducts eventually drain into the right and left hepatic ducts, which merge to form the common hepatic duct. The cystic duct from the gallbladder joins with the common hepatic duct to form the common bile duct.

Bile can either drain directly into the duodenum via the common bile duct, or be temporarily stored in the gallbladder via the cystic duct. The common bile duct and the pancreatic duct enter the second part of the duodenum together at the ampulla of Vater.

The liver receives a dual blood supply from the hepatic portal vein and hepatic arteries. Supplying approximately 75% of the liver’s blood supply, the hepatic portal vein carries venous blood drained from the spleen, gastrointestinal tract, and its associated organs. The hepatic arteries supply arterial blood to the liver, accounting for the remainder of its blood flow. Oxygen is provided from both sources; approximately half of the liver’s oxygen demand is met by the hepatic portal vein, and half is met by the hepatic arteries.
Blood flows through the sinusoids and empties into the central vein of each lobule. The central veins coalesce into hepatic veins, which leave the liver and empty into the inferior vena cava.

The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion. The liver is necessary for survival; there is currently no way to compensate for the absence of liver function long term, although liver dialysis can be used short term.

This organ plays a major role in metabolism and has a number of functions in the body, including glycogen storage, decomposition of red blood cells, plasma protein synthesis, hormone production, and detoxification. It lies below the diaphragm in the abdominal-pelvic region of the abdomen. It produces bile, an alkaline compound which aids in digestion via the emulsification of lipids. The liver’s highly specialized tissues regulate a wide variety of high-volume biochemical reactions, including the synthesis and breakdown of small and complex molecules, many of which are necessary for normal vital functions

The Model for End-Stage Liver Disease, or MELD, is a scoring system for assessing the severity of chronic liver disease. It was initially developed to predict death within three months of surgery in patients that had undergone a transjugular intrahepatic portosystemic shunt (TIPS) procedure.

It uses the patient’s values for serum bilirubin, serum creatinine, and the international normalized ratio for prothrombin time (INR) to predict survival. This score is also used by the United Network for Organ Sharing (UNOS) and Eurotransplant for prioritizing allocation of liver transplants. It is calculated according to the following formula:

* The maximum score given for MELD is 40. All values higher than 40 are given a score of 40

* If the patient has been dialyzed twice within the last 7 days, then the value for serum creatinine used should be 4.0

* Any value less than one is given a value of 1 (i.e. if bilirubin is 0.8, a value of 1.0 is used).

Patients with a diagnosis of liver cancer will be assigned a MELD score based on how advanced the cancer is. This staging system is known as the TNM system. T stands for the local extent of the tumor, N stands for the presence or absence of lymph node metastases, and M stands for the presence or absence of distant metastasis (tumor spread to another organ such as the lung in the case of liver cancer).

Some of the following signs and symptoms may occur in the presence of cirrhosis or as a result of the complications of cirrhosis. Many are nonspecific and may occur in other diseases and do not necessarily point to cirrhosis. Likewise, the absence of any does not rule out the possibility of cirrhosis.

* Spider angiomata or spider nevi. Vascular lesions consisting of a central arteriole surrounded by many smaller vessels due to an increase in estradiol. These occur in about 1/3 of cases.
* Palmar erythema. Exaggerations of normal speckled mottling of the palm, due to altered sex hormone metabolism.
* Nail changes.
o Muehrcke’s nails – paired horizontal bands separated by normal color due to hypoalbuminemia (inadequate production of albumin).
o Terry’s nails – proximal two thirds of the nail plate appears white with distal one-third red, also due to hypoalbuminemia
o Clubbing – angle between the nail plate and proximal nail fold > 180 degrees
* Hypertrophic osteoarthropathy. Chronic proliferative periostitis of the long bones that can cause considerable pain.
* Dupuytren’s contracture. Thickening and shortening of palmar fascia that leads to flexion deformities of the fingers. Thought to be due to fibroblastic proliferation and disorderly collagen deposition. It is relatively common (33% of patients).
* Gynecomastia. Benign proliferation of glandular tissue of male breasts presenting with a rubbery or firm mass extending concentrically from the nipples. This is due to increased estradiol and can occur in up to 66% of patients.
* Hypogonadism. Manifested as impotence, infertility, loss of sexual drive, and testicular atrophy due to primary gonadal injury or suppression of hypothalamic or pituitary function.
* Liver size. Can be enlarged, normal, or shrunken.
* Splenomegaly (increase in size of the spleen). Due to congestion of the red pulp as a result of portal hypertension.
* Ascites. Accumulation of fluid in the peritoneal cavity giving rise to flank dullness (needs about 1500 mL to detect flank dullness). It may be associated with hydrocele and penile flomation (swelling of the penile shaft)[citation needed] in men.
* Caput medusa. In portal hypertension, the umbilical vein may open. Blood from the portal venous system may be shunted through the periumbilical veins into the umbilical vein and ultimately to the abdominal wall veins, manifesting as caput medusa.
* Cruveilhier-Baumgarten murmur. Venous hum heard in epigastric region (on examination by stethoscope) due to collateral connections between portal system and the remnant of the umbilical vein in portal hypertension.
* Fetor hepaticus. Musty odor in breath due to increased dimethyl sulfide.
* Jaundice. Yellow discoloring of the skin, eye, and mucus membranes due to increased bilirubin (at least 2–3 mg/dL or 30 mmol/L). Urine may also appear dark.
* Asterixis. Bilateral asynchronous flapping of outstretched, dorsiflexed hands seen in patients with hepatic encephalopathy.
* Other. Weakness, fatigue, anorexia, weight loss.

Complications

As the disease progresses, complications may develop. In some people, these may be the first signs of the disease.

* Bruising and bleeding due to decreased production of coagulation factors.
* Jaundice due to decreased processing of bilirubin.
* Itching (pruritus) due to bile salts products deposited in the skin.
* Hepatic encephalopathy – the liver does not clear ammonia and related nitrogenous substances from the blood, which are carried to the brain, affecting cerebral functioning: neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, or changes in sleep habits.
* Sensitivity to medication due to decreased metabolism of the active compounds.
* Hepatocellular carcinoma is primary liver cancer, a frequent complication of cirrhosis. It has a high mortality rate.
* Portal hypertension – blood normally carried from the intestines and spleen through the hepatic portal vein flows more slowly and the pressure increases; this leads to the following complications:
o Ascites – fluid leaks through the vasculature into the abdominal cavity.
o Esophageal varices – collateral portal blood flow through vessels in the stomach and esophagus. These blood vessels may become enlarged and are more likely to burst.
* Problems in other organs.
o Cirrhosis can cause immune system dysfunction, leading to infection. Signs and symptoms of infection may be aspecific are more difficult to recognize (e.g. worsening encephalopathy but no fever).
o Fluid in the abdomen (ascites) may become infected with bacteria normally present in the intestines (spontaneous bacterial peritonitis).
o Hepatorenal syndrome – insufficient blood supply to the kidneys, causing acute renal failure. This complication has a very high mortality (over 50%).
o Hepatopulmonary syndrome – blood bypassing the normal lung circulation (shunting), leading to cyanosis and dyspnea (shortness of breath), characteristically worse on sitting up.
o Portopulmonary hypertension – increased blood pressure over the lungs as a consequence of portal hypertension.

Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged, bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis and cirrhosis. It was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3-4,000 people; the sex ratio is at least 9:1 (female to male)

Cirrhosis (pronounced /sɪˈroʊsɪs/, si-ROH-sis) is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrous scar tissue as well as regenerative nodules (lumps that occur as a result of a process in which damaged tissue is regenerated), leading to progressive loss of liver function. Cirrhosis is most commonly caused by alcoholism, hepatitis B and C, and fatty liver disease but has many other possible causes. Some cases are idiopathic, i.e., of unknown cause.

Ascites (fluid retention in the abdominal cavity) is the most common complication of cirrhosis and is associated with a poor quality of life, increased risk of infection, and a poor long-term outcome. Other potentially life-threatening complications are hepatic encephalopathy (confusion and coma) and bleeding from esophageal varices. Cirrhosis is generally irreversible once it occurs, and treatment generally focuses on preventing progression and complications. In advanced stages of cirrhosis the only option is a liver transplant.

The word “cirrhosis” derives from Greek κίρῥος, meaning tawny (the orange-yellow colour of the diseased liver). While the clinical entity was known before, it was René Laennec who gave it the name “cirrhosis” in his 1819 work in which he also describes the stethoscope.

Amitrano L, Guardascione MA, Ames PR.
Gastroenterology Unit, A. Cardarelli Hospital, Naples, Italy. luamitra@tin.it

The liver has a central role in the clotting process and an altered haemostasis is common in advanced liver disease. Nevertheless, recent studies have questioned the historical belief that impaired haemostasis in liver disease means an increased risk of bleeding.
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Coagulation and anticoagulation mechanisms are still balanced but are set at a lower level. Platelet function and number also play a role. The prevalence of thrombotic events is similar in both cirrhotic patients and in the general population but the cirrhotic patients have an increased risk for thrombosis in the splanchnic area. Portal blood flow stasis is the main underlying change favouring thrombosis even if other local, systemic, congenital and acquired factors are present. The onset of portal vein thrombosis strongly affects the prognosis of liver cirrhosis, worsening both portal hypertension and liver function. Some of the known risk factors for venous thrombosis–G20210A mutation of prothrombin, factor V Leiden, endoscopic treatment of esophageal varices and abdominal surgery–have a specific role in the development of splanchnic thrombosis in cirrhotic patients. The knowledge of the pathophysiological aspects of portal vein thrombosis and clotting alterations in liver disease will allow determination of the indication, duration and timing of anticoagulation therapy.

Kravetz D.
San Diego Veterans Affairs Medical Center, University of California at San Diego, San Diego, CA 92161, USA. dkravetz@ucsd.edu

Variceal rebleeding is a very frequent and severe complication in cirrhotic patients; therefore, its prevention should be mandatory. Lately several studies demonstrated that the rate of rebleeding was decreased by 40% and overall survival is improved by 20% with beta-blockers. However, this treatment presents some problems, such as the number of nonresponders and contraindications for its use. Recent trials found that the combination of beta-blockers with mononitrate of isosorbide to be superior to beta-blockade alone. Furthermore, endoscopic band ligation also shown to decrease the frequency of rebleeding, complications, and death compared with sclerotherapy and should be the preferred endoscopic treatment. In addition, the comparison between combined pharmacologic treatment with endoscopic treatment present similar rebleeding and mortality rates. More recently, the addition of nadolol to endoscopic band ligation increased the efficacy of endoscopy alone in the prevention of variceal rebleeding. These studies suggest that banding plus drugs could be the treatment of choice for the prophylaxis of rebleeding. When these treatments fail, the recommendation is to use transjugular intrahepatic portosystemic shunt (TIPS) or surgical shunts. Both treatments are effective in preventing rebleeding; however, they are associated with a greater risk of encephalopathy. The comparison of portacaval shunts with TIPS demonstrated that TIPS patients presented higher rebleeding, treatment failure, and transplantation. Another randomized controlled trial comparing distal splenorenal shunt with TIPS shows that variceal rebleeding was similar in both groups without differences in encephalopathy and mortality. The only difference observed was the higher rate of reintervention observed in the TIPS group to maintain his patency.