Hepatomegaly

Hepatomegaly: Excerpt from In A Page: Pediatric Signs and Symptoms

Hepatomegaly represents the clinical appearance of liver enlargement and can occur via five mechanisms, including inflammation, excessive storage, infiltration, congestion, and obstruction. The presence of a palpable liver does not always represent hepatomegaly and is determined on the basis of liver span and degree of extension below the right costal margin. Normal liver spans range from 5 to 9 cm depending on age.

Differential Diagnosis

• Inflammation
  –Most common infections: EBV; hepatitis A, B, C; CMV; TORCH
  –Less common infections: HIV, malaria, amebiasis, tuberculosis, toxocariasis, Borrelia burgdorferi
  –Drugs: Acetaminophen (commonly used in overdoses among adolescents), NSAIDs, isoniazid, sodium valproate, propothiouracil, halothane
  –Toxins: Tyrosinemia, galactosemia, vitamin A toxicity
  –Autoimmune hepatitis
  –Systemic lupus erythematosus

• Inappropriate storage
  –Glycogen storage diseases I–V
  –Lipids: Gaucher disease, Wolman disease, Niemann-Pick disease
  –Fat: Fatty acid oxidation defects, mucopolysaccharidoses
  –Metals: Wilson disease (copper), hemochromatosis (iron)
  –Abnormal proteins: α-1 antitrypsin deficiency (store abnormal protein product)
  –Peroxisomal disease: Zellweger
  –Mucopolysaccharidoses, types I–IV

• Infiltration
  –Hepatoblastoma
  –Hepatocellular carcinoma
  –Hemangioma
  –Histiocytosis
  –Extramedullary hematopoiesis
  –Chronic granulomatous disease
• Vascular congestion
  –Congestive heart failure
  –Budd-Chiari syndrome
  –Veno-occlusive disease
  –Suprahepatic web
• Biliary obstruction
  –Biliary atresia represents the most common cause of pediatric liver transplantation
  –Alagille syndrome
  –Cystic fibrosis
  –Primary sclerosing cholangitis
  –Inspissated bile syndrome
• Miscellaneous
  –Reye syndrome, bile acid synthetic disorder

Workup and Diagnosis

• History
  –Abdominal pain, fever, melena, weight loss, medications, age at onset, diarrhea, vomiting, hematemesis, bleeding, bruising, fatigue
  –Exposure to blood products
  –Nutrition history (neonatal formula)
  –Travel history to endemic infectious areas
  –Family history of liver disease, maternal HBV, HCV

• Physical exam
  –Height, weight
  –Liver size, margin, firmness, nodularity, tenderness
  –Ascites, jaundice/scleral icterus
  –Cataracts; Kayser-Fleischer rings (Wilson); posterior embryotoxin (Alagille)
  –Cardiac exam for murmurs; splenomegaly; tone and strength development; hemangiomas/xanthomas

• Labs
  –CBC, ALT, AST, fractionated bilirubin, alkaline phosphatase, total protein, albumin, globulin fraction, PT, U/A
  –Hepatitis serologies, EBV, TORCH titers, plasma amino acids/urine organic acids for metabolic disease
  –Serum AAT with protease inhibitor typing
  –Ceruloplasmin (decreased in Wilson disease)
  –ANA/anti-smooth muscle antibody/anti-liver kidney microsomal antibody, IgG for autoimmune hepatitis

• Ultrasound for echotexture and masses
  –Consider nuclear study/CT for obstruction
• Liver biopsy in chronic disease (>3 months) or to elucidate etiology

Treatment

• Geared towards specific disease
• Cholestasis
  –Ursodeoxycholic acid
  –Supplemental fat soluble vitamins A, D, E, K
• Infections
  –Consider interferon for hepatitis B
  –Consider interferon and ribaviron for hepatitis C
• Toxins
  –Use of NTBC for tyrosinemia
• Metabolic disease
  –Metabolism consultation
  –Often requires specific restricted formulas
• Surgical repair for biliary atresia
  –Kasai portoenterostomy has better outcome if done before 60 days of age
• Mucomyst for acute acetaminophen toxicity
• Immune suppression for autoimmune hepatitis

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

More About Liver failure

• Back to disease: Liver failure: Introduction
• Next Book Extract About Liver failure: Jaundice in Infants – Direct (In A Page: Pediatric Signs and Symptoms)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

 » Next page: Jaundice in Infants – Direct (In A Page: Pediatric Signs and Symptoms)

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• Jaundice in Infants – Direct (In A Page: Pediatric Signs and Symptoms)
• Jaundice in Infants – Indirect (In A Page: Pediatric Signs and Symptoms)
• HEPATOMEGALY (Differential Diagnosis in Primary Care)
• JAUNDICE (Differential Diagnosis in Primary Care)
• Hepatomegaly (Handbook of Signs & Symptoms (Third Edition))

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