Rosen & Barkin's 5-Minute Emergency Medicine Consult (160 page)

• Congenital
  
• Arteriohepatic dysplasia, biliary atresia, cystic fibrosis, α
  ₁
  -antitrypsin deficiency
  
• Metabolic
  
• Fructosemia, tyrosinemia, galactosemia, glycogen storage diseases
  
• Infectious
  
• Congenital hepatitis B
  

DIAGNOSIS

• May be silent
  
• Insidious onset with nonspecific findings:
  
  • Malaise
    
  • Fatigue
    
  • Anorexia
    
  • Nausea and vomiting
    
  • Weight loss
    
  • Pruritus
    
  • Hyperpigmentation
    
• Jaundice
  
• Abdominal collateral circulation including caput medusae
  
• Hepatomegaly
  
• Splenomegaly
  
• Abdominal discomfort or tenderness
  
• Fever
  
• Fetor hepaticus
  
• Asterixis
  
• Hypotension
  
• Cruveilhier–Baumgarten murmur
  
• Renal insufficiency
  
• Spider telangiectasias
  
• Palmar erythema
  
• Dupuytren contractures
  
• Parotid and lacrimal gland enlargement
  
• Terry nails
  
• Muehrcke lines
  
• Clubbing
  
• Feminization:
  
  • Testicular atrophy
    
  • Impotence
    
  • Loss of libido
    
  • Gynecomastia
    
• Amenorrhea
  
• Complications:
  
  • When complications develop, patient is considered to have decompensated disease.
    
  • Ascites
    
  • Spontaneous bacterial peritonitis (SBP)
    
  • Hepatic encephalopathy (HE)—may be precipitated by:
    
    • GI bleed
      
    • Infections
      
    • Increased dietary protein
      
    • Hypokalemia
      
    • Sedatives
      
    • Constipation
      
    • Azotemia
      
    • Alkalosis
      
  • Variceal hemorrhage:
    
    • 1/3 of patients with variceal bleed.
      
    • Each bleeding episode carries a 33% mortality rate.
      
    • Hepatic venous pressure gradient >12 mm Hg increases risk of bleed.
      
  • Portal hypertensive gastropathy or peptic ulcer disease
    
  • Hepatorenal failure:
    
    • Caused by decreased renal perfusion during severe decompensated cirrhosis
      
    • May be iatrogenic: Secondary to diuretics, NSAIDs, IV contrast, aminoglycosides, large-volume paracentesis
      
    • High mortality rate
      
  • Hepatopulmonary syndrome:
    
    • Intrapulmonary vascular dilation and hypoxia
      
    • Results in increased alveolar–arterial gradient
      

Detailed history and physical exam to search for clues to liver disease

• CBC:
  
  • Anemia
    
  • Macrocytosis
    
  • Leukopenia and neutropenia
    
  • Thrombocytopenia
    
• Impaired liver function:
  
  • High bilirubin
    
  • Low albumin
    
  • High globulins
    
  • Prolonged PT
    
  • Varying degrees of DIC
    
  • Hypoglycemia
    
• Increased liver enzymes:
  
  • Aspartate alanine aminotransferase (AST, SGOT), alanine aminotransferase (ALT, SGPT)—reflect injury
    
  • Ratio of AST:ALT ≥2 in alcoholic liver disease
    
  • Alkaline phosphatase and 5′-nucleotidase reflect cholestasis.
    
  • γ-Glutamyltranspeptidase (GGT)
    
  • May be normal in inactive cirrhosis
    
• Electrolytes, BUN, and creatinine
  
• Hyponatremia:
  
  • Renal dysfunction and hepatorenal syndrome
    
• Arterial blood gases or pulse oximeter for:
  
  • Suspected pneumonia
    
  • CHF
    
  • Hepatopulmonary syndrome
    
• Search for cause:
  
  • Hepatitis B surface antigen
    
  • Hepatitis C antibody
    
  • Antinuclear antibody (ANA) and antismooth muscle antibody (autoimmune hepatitis)
    
  • Antimitochondrial antibody (PBC)
    
  • Serum iron, transferrin saturation, and ferritin (hemochromatosis)
    
  • Ceruloplasmin (Wilson disease)
    
  • α
    ₁
    -Antitrypsin deficiency
    
  • Serum immune electrophoresis (high IgM in PBC)
    
  • Cholesterol (chronic cholestasis)
    
  • α-Fetoprotein (hepatocellular cancer)
    

• US for liver architecture, biliary obstruction, ascites, portal vein thrombosis, splenomegaly
  
• CT scan to explore abnormal finding on ultrasound
  
• CXR for pleural effusion, cardiomegaly, and CHF
  

• Esophagogastroduodenoscopy (EGD) indicated for upper GI bleeding or variceal surveillance
  
• Variceal ligation or endoscopic sclerotherapy
  
• Paracentesis for significant ascites or SBP
  

• Ascites:
  
  • Increased right heart pressure
    
  • Hepatic vein thrombosis
    
  • Peritoneal malignancy/infection
    
  • Pancreatic disease
    
  • Thyroid disease
    
  • Lymphatic obstruction
    
• Upper GI bleeding:
  
  • Peptic ulcer disease
    
  • Gastritis
    
• Encephalopathy:
  
  • Metabolic
    
  • Toxic
    
  • Intracranial process
    

TREATMENT

• Naloxone, dextrose (or Accu-Chekk), and thiamine for altered mental status
  
• Reverse hypotension with IV fluids to prevent acute ischemic hepatic injury.
  

Treat complications such as GI bleeding or HE.

• For suspected variceal bleed:
  
  • IV proton pump inhibitors
    
  • IV octreotide-splanchnic vasoconstrictor
    
  • Reverse coagulopathy:
    
    • Fresh-frozen plasma 1 IU/hr until bleeding is controlled
      
    • Desmopressin (DDAVP)—improves bleeding time and prolonged PTT
      
  • Balloon tamponade with Sengstaken–Blakemore tube or a variant for variceal compression (rarely used anymore, prophylactic intubation recommended)
    
  • Emergent endoscopic sclerotherapy
    
• Initiate broad-spectrum antibiotics in suspected sepsis or SBP:
  
  • Cefotaxime
    
  • Ticarcillin–clavulanate
    
  • Piperacillin–tazobactam
    
  • Ampicillin–sulbactam
    
• Treat complicating conditions such as ascites, HE, SBP.
  
• Treat pruritus with:
  
  • Diphenhydramine 25–50 mg IM/IV q4h
    
  • Cholestyramine, ursodeoxycholic acid, or rifampin
    
  • Naloxone infusion 0.2 μg/kg/min for temporary relief for extreme cases
    
• β-Blocker (propranolol) for esophageal varices:
  
  • Titrated to pulse rate of 60 or 25% reduction of resting pulse
    
  • With or without isosorbide dinitrate
    
  • Decreases rebleeding rate
    
  • May delay or prevent occurrence of 1st bleed
    
• Relieve biliary obstruction (e.g., stricture) by endoscopic, radiologic, or surgical means.
  
• Provide nutritious diet, high in calories and adequate in protein (1 g/kg), unless there is complicating HE
  
• Consult transplantation coordinator whenever postliver transplantation patient presents to the ED with liver dysfunction, suspected sepsis, or possible treatment-related complication.
  

• Hemochromatosis: Phlebotomy or deferoxamine (iron-chelating agent)
  
• Autoimmune hepatitis: Prednisone with or without azathioprine
  
• Chronic hepatitis B or C: α-Interferon (avoid in decompensated cirrhosis)
  
• PBC: Ursodeoxycholic acid
  
• Wilson disease: Penicillamine
  
• The only cure for most advanced cirrhosis is liver transplantation.
  

• Azathioprine: 1–2 mg/kg PO daily
  
• Cefotaxime: 1–2 g q6–8h (peds: 50–180 mg/kg/d q6h) IV
  
• Cholestyramine: 4 g PO 1–6 times per day
  
• Desmopressin (DDAVP): 0.3 μg/kg in 50 mL saline infused over 15–30 min
  
• Dextrose: D
  ₅₀
  W 1 amp (50 mL or 25 g; peds: D
  ₂₅
  W 2–4 mL/kg) IV
  
• Naloxone: 0.2–2 mg (peds: 0.1 mg/kg) IV or IM initial dose
  
• Lactulose: 15–30 mL TID—goal is 2–3 stools per day
  
• Octreotide: 25–50 μg IV bolus followed by 50 μg/hr IV infusion
  
• Piperacillin–tazobactam: 3.375 g IV q6h (peds: 100–400 mg/kg/d div. q6–8h; renal dosing required)
  
• Prednisone: 40 mg (peds: 1–2 mg/kg) PO daily
  
• Propranolol: 40 (initial) to 240 mg (peds: 1–5 mg/kg/d) PO TID
  
• Rifampin: 600 mg (peds: 10–20 mg/kg) PO daily
  
• Thiamine: 100 mg (peds: 50 mg) IV or IM
  
• Ursodeoxycholic acid: 8–10 mg/kg/d TID
  

FOLLOW-UP

• Acute decompensation or complicating conditions
  
• 1st presentation with clinically evident cirrhosis, unless close outpatient workup is possible
  
• Advanced grades HE, sepsis, active GI bleed, and hepatorenal and hepatopulmonary syndromes require ICU.
  
• Advanced stages of hepatocellular carcinoma
  

Most patients with compensated cirrhosis can be treated as outpatients.