Cirrhosis

Cirrhosis: Excerpt from The 5-Minute Pediatric Consult

Rose C. Graham-Maar, MD, MSCE

Cirrhosis - BASICS

Cirrhosis - description

Cirrhosis is the end stage of progressive hepatic fibrosis and regenerative nodule formation that may occur as a result of many different liver diseases. In its advanced form, it is irreversible and often requires liver transplantation for survival of the patient.

Cirrhosis - epidemiology

• Based on the varying causes, no specific epidemiologic pattern can be identified.
• Cirrhosis due to chronic HCV infection is the most common indication for liver transplantation in adults.
• Biliary cirrhosis due to biliary atresia is the most common indication for liver transplantation in children.

Cirrhosis - risk factors

Cirrhosis - genetics

• Many distinct genetic disorders can cause cirrhosis, such as Wilson disease and hereditary hemochromatosis.
• Human leukocyte antigen (HLA) associations have been identified in several autoimmune disorders, including sclerosing cholangitis.

Cirrhosis - DIAGNOSIS

Cirrhosis - signs & symptoms

• Compensated (latent) cirrhosis: Asymptomatic, with no signs or symptoms of liver disease. Discovered incidentally either during routine physical examinations with an enlarged liver and/or palpable spleen, or as a result of an investigation for an unrelated condition.
• Decompensated (active) cirrhosis: As cirrhosis progresses, overt signs and symptoms may occur including failure to thrive, muscle weakness, fatigue, fever, jaundice, pruritus, edema, abdominal pain, ascites, steatorrhea, and spontaneous bleeding (i.e., epistaxis) or bruising. This stage may also present with acute, precipitous liver failure or a life-threatening complication such as an esophageal variceal hemorrhage.

Cirrhosis - history

Based on the varying etiologic agents, one should elicit pertinent historical features characteristic of each specific problem as detailed:

• Exposure to hepatitis, antecedent viral illnesses
• Exposure to hepatotoxins
• Family or personal history of genetic, metabolic, or autoimmune diseases
• Neurologic problems, deteriorating school performance, depression (Wilson disease)

Cirrhosis - physical exam

• General: Poor growth, malnutrition, fever, cachexia
• Skin: Jaundice, flushing, pallor, cyanosis, palmar erythema, spider angiomata, fine telangiectasia (face and upper back), easy bruising
• Abdomen: Ascites (distention, fluid wave, shifting dullness), caput medusa (prominent periumbilical veins), splenomegaly, rectal varices, hepatomegaly, or a shrunken liver
• Extremities: Digital clubbing, hypertrophic osteoarthropathy, muscle wasting, peripheral edema
• Endocrine: Gynecomastia, testicular atrophy, delayed puberty
• Central nervous system: Asterixis, positive Babinski sign, mental status changes, hyperreflexia, muscle wasting
• Eyes: Kayser-Fleischer rings (Wilson disease)

Cirrhosis - tests

Cirrhosis - lab

These tests focus on determining the etiology and the severity of liver disease prior to a liver biopsy.

• Tests of liver cell injury: Alanine aminotransferase (ALT), aspartate aminotransferase (AST), lactic dehydrogenase (LDH)
• Tests of synthetic function: Albumin and other serum proteins, prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), ammonia, plasma and urine amino acids, serum lipids and lipoproteins, cholesterol and triglycerides
• Tests of cholestasis: Fractionated bilirubin, alkaline phosphatase, γ-glutamyltransferase, cholesterol, serum and urine bile acids
• Tests of fibrosis: Serum markers may be useful to evaluate hepatic fibrosis noninvasively, however, these are still being investigated for clinical utility. Possible markers include procollagen III peptide, type IV collagen, laminin, hyaluronic acid, and prolyl-hydroxylase.
• Miscellaneous disease-specific serum tests:
  • Viral serologies: Toxoplasma, rubella, cytomegalovirus, herpesvirus, hepatitis B, hepatitis C, Epstein-Barr virus, cytomegalovirus, other viruses
  • Wilson disease: Ceruloplasmin, 24-hour urine copper, and slit-lamp exam for Kaiser-Fleischer rings
  • αAutoimmune hepatitis: Sedimentation rate, autoantibodies (antinuclear, anti-smooth muscle, anti-liver kidney microsomal, anti-F-actin), serum immunoglobulins
  • Hemochromatosis: Serum iron, total iron binding capacity, ferriti
  • Metabolic/Genetic: Fasting blood sugar, lactate, pyruvate, uric acid, sweat test, carnitine, creatine phosphokinase (CPK), porphyrins, serum amino acids, urine organic acids, urine reducing substances, urine succinylacetone, fatty acid degeneration products, alpha-fetoprotein

Cirrhosis - imaging

• Ultrasound with Doppler images: Evaluates for anatomic variation or obstruction of the biliary tree, presence of ascites, portal hypertension and vascular obstruction
• Hepatobiliary radioisotope scanning: Assess for biliary excretion in neonatal cholestasis.
• Cholangiography (magnetic resonance cholangiopancreatography [MRCP]): Assess for intra- and extrahepatic biliary disease (stones, choledochal cyst, sclerosing cholangitis).

Cirrhosis - diag proced-surgery

• Liver biopsy:
  • Percutaneous needle biopsy, intraoperative wedge biopsy, transjugular liver biopsy
  • Confirm the presence, type, and degree of activity of cirrhosis.
  • Various hepatic diseases that progress to cirrhosis have characteristic histologic findings. However, the process of cirrhosis may obscure the nature of the original insult, rendering morphologic and histologic classifications unhelpful.
• Cholangiography:
  • Intraoperative cholangiography: Assess for extrahepatic biliary atresia in neonates.
  • Endoscopic retrograde cholangiopancreatography (ERCP): Assess for extrahepatic biliary disease in older patients where MRCP is not helpful or therapeutic interventions possible (i.e., stent placement).

Cirrhosis - differencial diagnosis

• Biliary:
  • Extrahepatic biliary atresia
  • Choledochal cyst
  • Tumors
  • Common bile duct and biliary lithiasis
  • Alagille syndrome
  • Biliary hypoplasia
  • Sclerosing cholangitis
  • Graft-versus-host disease
  • Vanishing bile duct syndrome due to drugs (e.g., trimethoprim-sulfamethoxazole)
  • Langerhans cell histiocytosis
• Hepatic:
  • Infectious hepatitis, including toxoplasma, rubella, cytomegalovirus, herpesvirus (TORCH) infections, viral hepatitis B, C, D; Epstein-Barr virus, other viruses
  • Autoimmune hepatitis
  • Nonalcoholic steatohepatitis (NASH), associated with obesity
  • Drugs/toxins and alcohol
• Genetic/Metabolic (examples for each category, not a complete list):
  • Cystic fibrosis
  • αCongenital hepatic fibrosis
  • Progressive familial intrahepatic cholestasis (PFIC)
  • Wilson disease
  • Hereditary hemochromatosis
  • Carbohydrate defects: Galactosemia, hereditary fructose intolerance, glycogen storage III and IV
  • Amino acid defects: Tyrosinemia, urea cycle disorders
  • Lipid storage diseases: Gaucher disease, Niemann-Pick type C
  • Mitochondrial disorders: Fatty acid β-oxidation defects, respiratory chain defects
  • Peroxisomal disorders: Zellweger syndrome
  • Porphyrias: Erythropoietic protoporphyria
• Vascular:
  • Budd-Chiari syndrome
  • Veno-occlusive disease
  • Congestive heart failure

Cirrhosis - TREATMENT

Cirrhosis - general measures

Cirrhosis - diet

• Malnutrition is common in chronic liver diseases because of several metabolic derangements, fat malabsorption, anorexia, and increased energy requirements.
• Adequate caloric intake is critical and often will require supplemental nocturnal nasogastric tube feedings.
• Some of the dietary fat should be provided as medium-chain triglycerides, which do not require bile for absorption.
• Fat-soluble vitamins should be supplemented.
• Careful attention must also be paid to fluid and electrolyte balance; sodium restriction (<2 mEq/kg/d) may be necessary in the presence of ascites.

Cirrhosis - activity

Spleen guard and avoidance of abdominal trauma if significant splenomegaly

Cirrhosis - medication

Cirrhosis - first line

• Fat-soluble vitamin supplementation: Vitamins A, D, E, and K
• Diuretic therapy (furosemide, spironolactone, chlorothiazide) for patients with ascites
• Albumin infusions for patients with refractory ascites
• Beta-blockers have been shown to decrease portal pressure and reduce the risk of variceal bleeding in adults.
• Antibiotics if suspicious for spontaneous bacterial peritonitis (avoid nephrotoxic agents)
• Lactulose and neomycin are used for patients with hepatic encephalopathy.

Cirrhosis - surgery

• Endoscopic variceal band ligation or sclerotherapy for GI bleeding
• Paracentesis for refractory ascites or diagnosis of spontaneous bacterial peritonitis
• Portosystemic shunt placement (surgical or radiologic transjugular intrahepatic portosystemic shunting [TIPS] procedure) for uncontrolled portal hypertension
• Liver transplantation

Cirrhosis - FOLLOW UP

Cirrhosis - prognosis

• The prognosis for cirrhosis leading to decompensation depends on the underlying cause.
• Most available liver tests have poor predictive value until liver decompensation has occurred.
• Poor prognostic features in children include prolonged PT unresponsive to vitamin K, ascites, malnutrition, low plasma cholesterol, elevated bilirubin level, PTT prolonged by >20 seconds, and presence of hepatorenal syndrome.

Cirrhosis - complications

• Malnutrition and growth failure
• Malabsorption (diarrhea, steatorrhea, fat-soluble vitamin deficiencies)
• Portal hypertension and variceal bleeding
• Chronic gastritis, peptic ulcer disease, gastroesophageal reflux
• Ascites
• Encephalopathy
• Hypersplenism (associated with anemia, thrombocytopenia, and neutropenia)
• Anemia
• Coagulopathy
• Hepatopulmonary syndrome (hypoxemia, cyanosis, dyspnea, digital clubbing)
• Hepatorenal syndrome (rapidly progressive renal failure in patients with cirrhosis)
• Bacterial infections, spontaneous bacterial peritonitis
• Hepatocellular carcinoma

Cirrhosis - bibliography

1. Kelly DA. Current results and evolving indications for liver transplantation in children. J Pediatr Gastroenterol Nutr. 1998;27:1214.
2. Gupta SK, Peters-Golden M, Fitzgerald JF, et al. Obese children with steatohepatitis can develop cirrhosis in childhood. Am J Gastroenterol. 2002;97(9):2460–2462.
3. Poupon R, Chazouilleres O, Poupon RE. Chronic cholestatic diseases. J Hepatol. 2000;32(1 suppl):129.

Cirrhosis - CODES

Cirrhosis - icd9

571.5 Cirrhosis of liver without mention of alcohol

Cirrhosis - FAQ

• Q: Will my child with cystic fibrosis develop cirrhosis?
• A: The medical literature cites a 5–20% incidence of cirrhosis in children with cystic fibrosis. Many factors seem to relate to the development of cirrhosis in these children, but there is no proven association with the specific mutation in the cystic fibrosis disease gene.
• Q: Will every child with cirrhosis need a liver transplant?
• A: Most children who develop cirrhosis from causes such as biliary atresia or metabolic disease will ultimately require a liver transplant.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Primary biliary cirrhosis

• Back to disease: Primary biliary cirrhosis: Introduction
• Next Book Extract About Primary biliary cirrhosis: Surveys relating to Primary biliary cirrhosis
• More Book Extracts: All Online Books for Primary biliary cirrhosis

More Medical Textbooks Online about Primary biliary cirrhosis

Review other book chapters online related to Primary biliary cirrhosis:

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

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

 » Next page: Surveys relating to Primary biliary cirrhosis

Rate This Website

What do you think about the features of this website? Take our user survey and have your say:

Website User Survey

Medical Tools & Articles:

Next articles:

• Surveys relating to Primary biliary cirrhosis
• Ask Question about Primary biliary cirrhosis

Tools & Services:

• Bookmark this page
• Symptom Search
• Symptom Checker
• Medical Dictionary
• Give your feedback

Medical Articles:

• Disease & Treatments Search
• Misdiagnosis Center
• Full list of interesting articles

Forums & Message Boards

• Ask or answer a question at the Boards:
• I cannot get a diagnosis. Please help.
• Tell us your medical story.
• Share your misdiagnosis story.
• What is the best treatment for my condition?
• See all the Boards.

Enter your search terms Submit search form
Search The Web Search wrongdiagnosis.com

Common Health Mistakes

Symptom
Checker

Search Specialists by State and City