Updated: 8/21/2019

Cirrhosis and Portal Hypertension

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Snapshot
  • A 50-year-old man presents to his primary care physician with yellowing skin and increased abdominal girth. He believes that he has gained weight and is worried about obesity. He also reports being concerned about increased breast size. He denies having a history of alcohol abuse, but when he is questioned further, he admits to drinking a bottle of vodka daily to cope with stressors in his life. On exam, he has spider angiomas on his abdomen, jaundice, and gynecomastia.
Introduction
  • Overview
    • cirrhosis is a liver disease characterized by hepatic fibrosis, regenerative nodules, and dysfunction
    • portal hypertension is a complication of cirrhosis, resulting in increased pressure in the portal venous system
  • Epidemiology
    • incidence
      • very common cause of death
    • risk factors
      • alcoholic liver disease (most common)
      • nonalcoholic steatohepatitis
      • chronic viral hepatitis
      • autoimmune hepatitis
      • hepatocellular carcinoma
      • primarily biliary cirrhosis
      • α1-antitrypsin deficiency
      • Wilson disease
      • hemochromatosis
  • Pathogenesis
    • mechanism
      • chronic liver damage
        • results in regenerative nodules surrounded by bridging fibrosis
          • abnormal wound healing with continued connective tissue deposition, resulting in fibrosis
          • micronodular
            • nodules < 3 mm following metabolic insult
          • macronodular
            • nodules > 3 mm following hepatic necrosis
            • increased risk of hepatocellular carcinoma
        • results in damage to hepatic vasculature
          • fibrosis causes shunting of portal and arterial blood
  • Prognosis
    • Model for End-Stage Liver Disease (MELD) score
      • predicts 3-month mortality in patients with cirrhosis
      • creatinine
      • bilirubin
      • INR
Presentation
  • Symptoms
    • common symptoms
      • fatigue
      • weakness
      • weight loss
      • loss of appetite
      • pruritus
      • upper gastrointestinal bleeding
  • Physical exam
    • inspection
      • mental status changes
      • signs of liver disease (BAD JPEGS)
        • Bleeding
          • prothrombin time
        • Asterix
          • “flapping” tremor
        • Dupuytren contracture
        • Jaundice
          • ↓ excretion of bilirubin
        • Palmar erythema
        • Encephalopathy
          • ↓ excretion of ammonia
        • Gynecomastia
          • ↓ degradation of estrogen
        • Spider angiomata
          • ↓ degradation of estrogen
      • portal hypertension
        • hepatosplenomegaly
        • caput medusa
        • ascites
Imaging
  • Computed tomography (CT) of abdomen
    • indications 
      • other imaging studies are inconclusive
      • patients often undergo CT of the abdomen during workup to exclude other pathologies
    • findings
      • nodularity
      • hypertrophy
      • ascites
  • Liver ultrasound
    • indications
      • all patients
    • findings
      • fibrosis
      • nodularity
      • increased echogenicity
      • atrophy or hypertrophy of liver lobes
  • Transient elastography
    • indications
      • measures liver stiffness
      • all patients
    • findings
      • hepatic fibrosis and increased stiffness
Studies
  • Serum labs
    • multiple severity scores exist for cirrhosis but often include
      • ↑ liver enzymes
      • ↑ prothrombin time
        • treat with fresh frozen plasma or factor replacement
      • ↑ direct bilirubin
      • ↓ platelets
      • creatinine
    • determining etiology
      • hepatitis B and C serology
      • alpha-1 antitrypsin levels
      • antinuclear antibody
      • anti-smooth muscle titers
      • anti-mitochondrial antibody
      • ferritin
      • transferrin saturation levels
      • 24-hour copper level in the urine
      • ceruloplasmin
      • hemochromatosis genetic testing
  • Invasive studies
    • liver biopsy   
      • indications
        • patients who cannot undergo transient elastography
        • clinical presentation is not consistent with a diagnosis
Differential
  • Acute viral hepatitis
    • key distinguishing factor
      • acute onset of symptoms with viral prodrome, nausea, vomiting, and abdominal pain
      • lacks findings of chronic liver disease
Treatment
  • Management approach
    • treat underlying cause if possible
  • Lifestyle
    • diet with restricted sodium
      • indications
        • all patients with ascites
    • alcohol and smoking cessation
  • Medical
    • antibiotic prophylaxis for spontaneous bacterial peritonitis
      • indications
        • cirrhosis and gastrointestinal bleeding or ascites
    • vaccinations
      • indications
        • all patients
      • modalities
        • hepatitis A
        • hepatitis B
        • 23-valent pneumococcal vaccine
  • Surgical
    • liver transplantation
      • indications
        • refractory liver cirrhosis
        • major complications
          • ascites
          • variceal bleeding
          • hepatic encephalopathy
    • portal shunting
      • indications
        • portal hypertension
Complications
  • Hepatocellular carcinoma
    • screening
      • liver ultrasound and alpha-fetoprotein every 6 months
  • Esophageal or gastric varices
    • screening
      • esophagogastroduodenoscopy (EGD)
  • Spontaneous bacterial peritonitis
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.GI.4750) A 63-year-old male with a history of alcohol abuse is admitted to the hospital after his wife found him to be confused. On exam, he is disoriented and fatigued. The whites of his eyes and his skin have a yellow hue. Furthermore, his hands flap when he holds his arms out straight in front of him and extends his wrists. His abdomen is distended with muffled bowel sounds and a fluid wave. His abdominal exam is remarkable for the finding of abnormal masses as shown in Figure A. Which of the following processes is most likely responsible for the finding seen in this clinical photograph? Review Topic

QID: 108689
FIGURES:
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1

Capillary dilation due to excess estrogen

5%

(5/91)

2

Hyperammonemia

8%

(7/91)

3

Weakness in the fascia overlying the superficial inguinal ring

2%

(2/91)

4

Anastomoses between the superficial epigastric veins and the paraumbilical veins

80%

(73/91)

5

Anastomoses between the superficial epigastric veins and the gastric veins

1%

(1/91)

M1

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