Chronic Hepatitis
Chronic Hepatitis: Excerpt from The 5-Minute Pediatric Consult
John Y. Tung, MB, BCh, MRCPIVani V. Gopalareddy, MD
Chronic Hepatitis - BASICS
Chronic Hepatitis - description
- Chronic active hepatitis is a continuing inflammation of the liver that may lead to cirrhosis.
- Features include inflammation not caused by acute self-limiting infection or past drug exposure with raised transaminases and histologic evidence of hepatitis.
Chronic Hepatitis - epidemiology
Depends on the cause of the underlying disease:
- Nonalcoholic steatohepatitis (NASH) is a leading cause of elevated AST/ALT.
- Hepatitis B: Common in immigrant children from Asia and Eastern Europe
- Hepatitis C: Common in those who had blood transfusions and blood products before screening became available, users of IV drugs, nasal cocaine users
- Wilson disease presents mainly in older children and adults.
- Autoimmune liver disease is more common in females and older children.
- Autoimmune hepatitis (AIH) may be associated with other autoimmune conditions such as diabetes, ulcerative colitis, autoimmune thyroiditis, and celiac disease.
- Cystic fibrosis and α
Chronic Hepatitis - pathophysiology
Pathology has been traditionally classified as chronic persistent hepatitis, chronic aggressive hepatitis, and chronic lobular hepatitis. The hepatocytes are damaged, with inflammatory cellular infiltration accompanied by liver regeneration.
- Chronic persistent hepatitis:
- Minimal portal tract fibrosis
- Slightly widened portal tracts
- Limiting plate is intact and inflammation does not extend beyond this.
- No bridging fibrosis between portal tracts
- Chronic aggressive hepatitis:
- Perilobular hepatitis, with inflammatory cells extending from portal tracts into parenchyma with fibrosis
- Piecemeal necrosis: Necrotic hepatocytes surrounded by lymphocytes and fibroblasts
- In advanced disease, fibrosis bridges the portal tracts (bridging fibrosis).
- Cirrhosis occurs when there is loss of architecture owing to fibrosis.
- Chronic lobular hepatitis:
- Liver architecture is preserved with scattered changes of acute hepatitis with hepatocyte necrosis in the lobules (perivenular regions).
- These changes are most often associated with hepatitis B and non-A, non-B hepatitis.
Chronic Hepatitis - etiology
- Autoimmune liver disease
- Viral hepatitis
- Obesity (NASH)
- Progressive familial intrahepatic cholestasis syndromes
- Congenital hepatic fibrosis
- Cystic fibrosis
- Metabolic disease:
- Mitochondrial disease
- Lysosomal storage disorders
- Peroxisomal disease
- Lipid storage disease
- Glycogen storage disease
- Wilson disease and others
- Drug hepatotoxicity:
- Methotrexate
- Isoniazid
- Thioguanine
- 6-Mercaptopurine
- Valproate
- Liver disease associated with other chronic diseases:
- Cardiac disease
- Autosomal recessive polycystic kidney disease
- Diabetes mellitus
- Langerhans cell histiocytosis
- Immunodeficiency
- Total parenteral nutrition cholestasis
Chronic Hepatitis - DIAGNOSIS
Chronic Hepatitis - signs & symptoms
Chronic Hepatitis - history
- Preceding clinical signs and symptoms for at least 6 months and complete medical history:
- History of blood transfusions
- Surgery
- Medications
- Foreign travel
- Social circumstances that predispose to liver diseases
- Symptoms of chronic illness can be nonspecific:
- Poor growth
- Intermittent jaundice
- Abdominal pain
- Bleeding
- Malabsorption
- Fever
- Amenorrhea
- Poor school achievement
- Itching
- Variceal bleeding may be a presenting syndrome in patients with portal hypertension.
- A history of jaundice in infancy, family history of liver disease or autoimmune liver disease, blood transfusions, IV drug abuse, or multiple sexual partners can suggest an etiology of hepatitis.
Chronic Hepatitis - physical exam
Stigmata of chronic liver disease are:
- Spider nevi
- Cutaneous shunts
- Palmar erythema
- Cyanosis (hepatopulmonary syndrome)
- Jaundice
- Itching
- Enlarged liver or small, shrunken liver
- Splenomegaly
- Ascites
- Rickets
- Mental changes
- Fetor associated with high ammonia
- Obesity
Chronic Hepatitis - tests
Chronic Hepatitis - lab
- Albumin, creatinine, γ-glutamyl transferase, aspartate aminotransferase, alanine aminotransferase, bilirubin, PT, CBC, blood group, Coombs test
- Other testing as indicated by the specific clinical presentation:
- Viral serologies: Hepatitis B, hepatitis C, hepatitis D
- Autoantibodies: Type 1: Smooth muscle (also called anti-actin), anti-nuclear, anti-soluble liver antigen, Type 2: Liver kidney microsomal, primary sclerosing cholangitis: pericytoplasmic anti-neutrophil (p-ANCA)
- Immunoglobulins: IgG elevated in autoimmune liver disease
- Fasting glucose, insulin levels, CRP, lipid profile (suspected NASH)
- αSerum ceruloplasmin, serum copper, 24-hour urine copper (+/− penicillamine challenge), quantitative liver copper (Wilson disease)
- Cholesterol, triglycerides elevated in cholestatic syndromes, glycogen storage, Alagille syndrome, certain lysosomal disease, steatohepatiti
- Metabolic workup as indicate
- CPK level to rule out muscle source of elevated ALT/AS
- Urinary succinylacetone: Tyrosinemi
- Urinary bile acids: Bile acid synthetic defects and some progressive intrahepatic cholestatic syndrome
- Sweat test and cystic fibrosis genotypin
- α-Fetoprotei
- Fibrosis markers (FibroSURE; FibroTest; ActiTest) are not validated for children but may be useful in older patients.
Chronic Hepatitis - diag proced-surgery
- Ultrasound scan: Focus on liver, spleen with Doppler flow studies. This may also demonstrate steatosis.
- Other testing as indicated by specific clinical presentation:
- MRI can demonstrate percentage steatosis
- Fibroscan can measure liver stiffness/fibrosis.
- Liver biopsy
- Percutaneous transhepatic cholangiography, endoscopic retrograde cholangiopancreatography or magnetic retrograde cholangiopancreatography may be useful if primary sclerosing cholangitis is suspected
- Colonoscopy: Sclerosing cholangitis, inflammatory bowel disease
- Bone marrow aspirate to exclude Niemann-Pick type C or other storage disorders
- Enzyme from white cells or cultured fibroblasts (skin biopsy) to exclude lysosomal storage disease, glycogen storage disease
- Angiography: Congenital or acquired venous or arterial malformations, assessment of portosystemic shunt
- Cardiac catheterization to assess pulmonary hypertension and cardiac status
- Macroaggregated albumin scan to assess hepatopulmonary syndrome and hepatic encephalopathy
- Muscle biopsy to assay respiratory chain enzymes in mitochondrial disorders
- Genotyping: Wilson disease, cystic fibrosis, and others
Chronic Hepatitis - differencial diagnosis
Nonhepatic etiologies of lab or physical exam abnormalities:
- Hepatomegaly: Elevated right-sided cardiac pressures, such as patients with Fontan operations, right-sided heart failure; respiratory diseases with lung hyperexpansion
- Splenomegaly:
- Blood malignancies
- Storage diseases
- Hematologic disease with hemolysis
- Infection
- Vascular
- Jaundice: Often confused with hypercarotenemia
- Elevated transaminases: Consider nonhepatic sources such as skeletal muscles in myopathies. With jaundice, consider hypopituitarism in infancy.
- Alkaline phosphatase: May be elevated in growing children and in rickets; may not indicate biliary obstruction.
- γ-Glutamyl transferase:
- Produced in choroid plexus, renal tubules, pancreatic and biliary ducts
- Often elevated in patients on antiepileptic drugs and in alcoholics
- Abnormal coagulation: Anticoagulant medications, bacterial overgrowth with malabsorption, inherited disorders of coagulation, sepsis
Chronic Hepatitis - TREATMENT
Chronic Hepatitis - general measures
The management of patients is dictated by the underlying diagnosis.
- General management:
- Maintaining growth and development is paramount.
- Fat-soluble vitamins (A, D, E, K) given orally are poorly absorbed in cholestasis, and levels must be monitored.
- Anthropometric parameters must be recorded, including skinfold thickness.
- Body mass index
- Medium-chain triglyceride–rich formulas can reduce fat malabsorption.
- Branched-chain amino acids may be useful in patients with hepatic encephalopathy.
- Ursodeoxycholic acid: Choleretic
- Encourage bolus feedings; minimizing continuous feeding and total parenteral nutrition may reduce gallbladder sludge.
- Proactive involvement of clinical psychologist, play therapist can help alleviate problems such as depression and fear.
- Aggressive weight management in patients with obesity/hypermetabolic syndrome with steatohepatitis. Curbing passive activities such as television, computer games.
- Chronic debilitating pruritus: Indication for liver transplantation after failure of medical therapy. Treatment for pruritus includes:
- Antihistamines
- Cholestyramine
- Naltrexone
- Rifampicin
- Ursodeoxycholic acid
- Monitoring portal hypertension: Assessment of portal flow on ultrasound and splenic size may provide some indication of disease progression.
- Treatment of recurrent cholangitis may decelerate the progression of liver disease.
- Aggressive treatment for spontaneous bacterial peritonitis in patients with ascites
- Early referral to a liver transplant center
- Complete immunization schedule including hepatitis A
- Specific management depends on the underlying liver disease.
Chronic Hepatitis - FOLLOW UP
Chronic Hepatitis - prognosis
Some diseases are treatable while others are progressive and not amenable to treatment. A subset of patients will progress to end-stage liver disease and regular liver transplantation.
Chronic Hepatitis - patient monitoring
- Look for hepatocellular carcinoma developing in patients with chronic liver disease, an ultrasound scan of liver and AFP every 6 months is a reasonable schedule.
- Advise patients with enlarged spleens to wear a spleen guard and avoid activities that can cause splenic rupture.
Chronic Hepatitis - bibliography
- Geller SA. Hepatitis B and hepatitis C. Clin Liver Dis. 2002;6:317–334.
Hardy S, Kleinman R. Cirrhosis and chronic liver failure. In: Suchy F, Sokol R, Balistrer W, eds. Liver Disease in Children, 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:89–127.The liver. In: Walker WA, Durie PR, Hamilton JR, eds. Pediatric Gastrointestinal Disease, 3rd ed. Hamilton, Ontario:BC Decker; 2000: 880–1311.
Chronic Hepatitis - CODES
Chronic Hepatitis - icd9
- 571.49 Chronic active hepatitis
- 572.3 Portal hypertension
- 575.2 Gallbladder obstruction/sludge
- 790.4 Elevated liver function tests
Chronic Hepatitis - FAQ
- Q: What are the risks of providing very young patients with a liver transplant?
- A: Although transplant in the very young is more difficult, with the increased use of split liver techniques, outcomes of orthotopic liver transplantation in infants have improved.
- Q: Why should we be aggressive with vitamin supplementation?
- A: There is significant malabsorption of vitamins A, D, E, and K. Vitamin D and E deficiencies are the most significant, causing rickets and neuropathy.
- Q: Oral supplements of vitamins are sometimes very difficult to administer in the very young. How can I overcome this problem?
- A: It is common practice in some centers to give vitamins D and E as an intramuscular injection on a monthly basis, with levels done in between.
- Q: Why do jaundiced children scratch?
- A: The accumulation of bile salts causes pruritus.
- Q: Are the stigmata of chronic liver disease also seen in children?
- A: Spider nevi, liver palms, splenomegaly, cutaneous shunts, and clubbing are very common.
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 Autoimmune Hepatitis
More Medical Textbooks Online about Autoimmune Hepatitis
Review other book chapters online related to Autoimmune Hepatitis:
Medical Books Excerpts
- JAUNDICE
- "Algorithmic Diagnosis of Symptoms and Signs" (2003)
- [ read ]
- JAUNDICE
- "Differential Diagnosis in Primary Care" (2007)
- [ read ]
- Jaundice
- "Handbook of Signs & Symptoms (Third Edition)" (2006)
- [ read ]
- Jaundice
- "A Pocket Manual of Differential Diagnosis" (1999)
- [ read ]
- Hepatomegaly
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
- [ read ]
- Hepatomegaly
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
- [ read ]
- Jaundice
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
- [ read ]
- Hepatitis
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
- [ read ]
- Jaundice
- "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
- [ read ]
- Hepatomegaly
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
- [ read ]
- Jaundice
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
- [ read ]
- JAUNDICE
- "Differential Diagnosis in Primary Care" (2007)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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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
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