Chronic liver disease and cirrhosis have a number of etiologies that are treatable

Chronic liver disease and cirrhosis have a number of etiologies that are treatable: Excerpt from Avoiding Common Pediatric Errors

Author: Madan Dharmar, MD

What to Do - Gather Appropriate Data, Interpret the Data, Make a Decision

They present with a similar complex of symptoms including hepatosplenomegaly, nevi, varices, or hemorrhoids. The treatment principles need to be known and followed.

Injury to the liver results in alterations in hepatic structure and function due to cell (hepatocytes) injury. This results in inflammation or cell death, which leads to scar formation (fibrosis), and potentially nodule formation (regeneration). Chronic hepatitis is defined as a persistent inflammatory condition of the liver in which the biochemical and histopathological abnormalities are present for >6 months. Cirrhosis is the end result of virtually any progressive liver disease.

Chronic liver disease can be caused by a wide spectrum of etiologies including infectious, metabolic, genetic, drug-induced, idiopathic, structural, and autoimmune diseases. Many of these diseases have similar presentations and initial laboratory findings that definitive diagnosis can be made only by specialized laboratory tests and the histological examination of the liver tissue. Most acute hepatitis, if lasting >3 months, needs to be aggressively investigated to determine the etiology of the disease, because most of these diseases lead to chronic hepatitis, which may respond to specific medical therapy. Patients with chronic liver disease present with clubbing, spider telangiectasia, and hepatosplenomegaly (stigmata of chronic liver disease); and/or with evidence of severe liver disease (hepatic failure).

Based on the location of the inflammatory lesion on histopathology, chronic hepatitis can be chronic persistent hepatitis (CPH) or chronic active hepatitis (CAH). In CPH the inflammatory cells are limited to the portal triad preserving the lobular architecture, whereas in CAH, they are not restricted to the portal triad and enter the lobule thereby disturbing the lobular architecture. CPH is typically due to a slow resolving viral hepatitis, α1-antitrypsin deficiency or Wilson disease. CPH is insidious in its presentation, with the patients being asymptomatic or with mild symptoms, whereas CAH presents with prominent clinical manifestations of liver disease.

Cirrhosis is considered to be a clinical-pathologic state that represents the end stage of any chronic liver disease. Histologically, the liver shows scarring and regeneration nodules devoid of central veins, surrounded by bands of fibrous tissues which distorts the lobular architecture. The progressive scarring of the tissue in the liver leads to altered hepatic flow and increased resistance to portal blood flow, causing portal hypertension and loss of hepatic function. Patients with cirrhosis may be either in a compensated or decompensated state. In the compensated state, the patient is asymptomatic with physical findings of enlarged liver, spleen, or both; whereas in a decompensated state, patients present with symptoms of hepatic dysfunction, portal hypertension, or both.

Presentation and Diagnostic Workup. Chronic liver disease causes profound cholestasis, which can lead to nonspecific symptoms of liver disease such as fatigue, anorexia, abdominal pain, malnutrition and growth failure, and bleeding. These symptoms precede signs of jaundice, dark urine, and light- colored stools. The presence of physical signs of liver disease such as palmar erythema, spider angiomas, xanthomas, ascites, muscle wasting, and an enlarged firm liver and spleen indicates the chronic nature of the disease. Patientswithcirrhosispresentwithsplenomegaly,ascites,andgastroesophageal varices, which are indicative of portal hypertension. Hepatorenal syndrome is a functional renal failure in patients with end-stage liver disease. Hepatopulmonary syndrome is characterized by the triad of hypoxemia, intrapulmonary vascular dilations, and liver disease. Variceal bleeding and ascites are lethal complications of chronic liver disease and cirrhosis.

Evaluation for chronic liver disease involves a careful history and physical examination, and skillful interpretation of signs and symptoms. Previous history of acute hepatitis, jaundice during infancy, maternal and patient risk factors for hepatitis (transfusion, intravenous drug abuse, and ethnic background),familyhistoryofchronicliverdiseaseandexposuretohepatotoxins. Initial laboratory evaluation includes a complete blood count; erythrocyte sedimentation rate; and liver function test, such as serum aminotransferases, totalandfractionatedbilirubinlevels;alkalinephosphatase,and5. nucleotidase or gamma-glutamyltransferase. The hepatocellular function (synthetics ability) is specifically determined by measuring the serum albumin, and prothrombin time. These tests reveal the extent of liver disease and also help in determining the prognosis and response to therapy in a patient. These must be followed up with specific diagnostic test to determine the etiology of the liver disease.

Specialized diagnostic test to evaluate chronic liver disease includes measuring levels of serological markers for chronic hepatitis B virus (HBV) namely HBV DNA, hepatitis B surface antigen (HBsAg) and hepatitis E antigen (HBeAg). When these tests are negative, appropriate serological tests to diagnose other viral infections such as hepatitis C virus infection, cytomegalovirus infection, and Epstein-Barr virus infection need to be performed. Specific testing includes α1-antitrypsin level in the blood to diagnose α1-antitrypsin deficiency; serum ceruloplasmin level, a 24-hour urinary copper excretion to, and an examination of the eyes for Kayser-Fleischer rings or sunflower cataracts to diagnose Wilson disease. In addition, a normal sweat sodiumlevelwouldruleoutcysticfibrosisandautoantibodiesforantinuclear, smooth muscle, mitochondrial and liver kidney microsome to help to make the diagnosis of autoimmune hepatitis.

Plain x-rays and abdominal ultrasonography help in determining the size and shape of the liver. Ultrasound may also help to define the architecture of the liver, the hepatobiliary tract and the blood flow in the liver. Ultrasound identifies anatomical abnormalities such as biliary atresia, and choledochalcystsandstones.Althoughcomputedtomographyandmagnetic resonance imaging may not play an important role in the initial diagnosis of liver disease, they do detect subtle differences in the parenchyma (storage disorders) and characterize liver masses clearly. Radionuclide scanning helps to detect abnormalities in liver uptake and parenchymal concentration and excretory abilities. Endoscopy is the most reliable method for detecting esophageal varices and bleeding. Liver biopsy (typically obtained percutaneously)isconsideredthedefinitivemethodbywhichtodiagnoseunderlying liver disease. Histopathological examination of the liver tissue can identify the structural, genetic and metabolic disorders causing chronic liver disease.

Management of Chronic Liver Disease. The management of chronic liver disease involves mainly supportive or palliative care to relieve symptoms of the diseases. In patients with chronic viral hepatitis, interferon-a can induce long-term remission in 25% of treated children. In patients with Wilson disease, chelating agents such as penicillamine can increase the urinary copper excretion and slowly improve the clinical condition. The administration of ursodeoxycholic acid decreases xanthomas and relieves pruritus. In addition, it helps to decrease symptoms due to cholestasis by increasing bile flow. Patients must be treated for malabsorption that arises directly or indirectly from cholestasis and due to loss of hepatic function. Malnutrition due to malabsorption, and vitamin and micronutrient deficiencies are treated with dietaryformulaorsupplementswithmedium-chaintriglycerides,fat-soluble vitamins, calories, water-soluble vitamins, and micronutrients.

In patients with portal hypertension, a variety of surgical procedures allow diversion of portal blood flow and a reduction in portal pressure. Portocaval shunts and transjugular intrahepatic portosystemic shunt (TIPS) divert the portal blow flow and reduce pressure in the portal system. These procedures are prone to thrombosis and the diversion of the blood flow from the liver could also result in encephalopathy. Variceal bleeding is a life- threatening complication of portal hypertension which needs emergency treatment directed at prevention of initial and subsequent bleeding. Fluid resuscitation with crystalloid infusions, followed by the replacement of red blood cells must be performed with care because overresuscitation could increase bleeding. The correction of coagulopathy by the administration of vitamin K or the infusion of platelets or fresh frozen plasma may be required. In patients with prolonged bleeding, pharmacologic therapy to decrease portal pressure and endoscopic sclerosis of the esophageal varices need to be considered to stop bleeding. Ascites, another complication due to progressive liver disease is treated with salt restriction and spironolactone, the diuretic of choice in these patients. A paracentesis may be performed if the abdomen is tense, the child uncomfortable, or if abdominal distension causes respiratory distress. Central venous pressure should be monitored, and albumin provided in cases of low pressure.

Finally, liver transplantation with immunosuppression is the standard therapy for children with end-stage liver disease, life-threatening hepatic metabolic disorders, and cancers of the liver. The use of reduced-size transplants and living donors increases the ability to treat small children successfully. The prognosis for survivals is very encouraging. Most children have improvement in growth and development and the stigmata of chronic liver disease resolve.

Suggested Readings

Behrman RE, Kliegman R, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia: Saunders; 2004.
D’Agata ID, Balistreri WF. Evaluation of liver disease in the pediatric patient. Pediatr Rev. 1999;20(11):376–390.
Mews C, Sinatra F. Chronic liver disease in children. Pediatr Rev. 1993;14(11):436–444. Rosenthal P, Lightdale JR. Laboratory evaluation of hepatitis. Pediatr Rev. 2000;21(5):178.

Book Source Details

• Book Title: Avoiding Common Pediatric Errors
• Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
• Year of Publication: 2008
• Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Avoiding Common Pediatric Errors Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7489-6

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