PANCE Blueprint GI and Nutrition (9%)

Cirrhosis (ReelDx)

VIDEO-CASE-PRESENTATION-REEL-DX

Cirrhosis

60-year-old with a distended abdomen

Patient will present as → a 63-year-old white male with a chief complaint of blood in his stool. He is accompanied by his wife, who also reports weight gain, abdominal distension, and swelling of his legs. Physical exam reveals a healthy-appearing male with 3+ bilateral lower extremity edema and distended abdomen with evidence of shifting dullness. You also note several skin changes seen here. The patient is hemoccult positive and has blood on his urine dipstick. He denies tobacco and illicit drug use but admits to drinking 1-2 times per week and has about 6 beers on each occasion.

A patient with cirrhosis develops acute hepatic encephalopathy. Initial pharmacologic treatment of this disorder consists of which of the following?
Lactulose (Enulose)

Cirrhosis is a late stage of hepatic fibrosis that has resulted in a widespread distortion of normal hepatic architecture

  • Characterized by regenerative nodules surrounded by dense fibrotic tissue
  • The liver is unable to regenerate due to large amounts of scar tissue

Cirrhosis is a leading cause of death worldwide and is the ninth leading cause of death among U.S. adults

​The most common cause of cirrhosis is chronic alcohol abuse, but other causes include viral hepatitis B and C, non-alcoholic fatty liver disease, autoimmune hepatitis, and inherited metabolic disorders.

  • Alcohol liver disease (25%): history of alcohol abuse
  • Chronic Hepatitis C (21-25%): ELISA assay for anti-HCV with PCR for HCV RNA if a confirmatory test is necessary
  • Hepatitis C with alcoholic liver disease (15%)
  • Hepatitis B + hepatitis D infection (15%): HBsAg and HBeAg and, in some cases, HBV DNA by hybridization or bDNA assay
  • Nonalcoholic steatohepatitis/obesity (~10%): History of diabetes mellitus or metabolic syndrome. Diagnosis may be suspected by abnormal liver biochemical tests and hepatic imaging showing fatty infiltration and is confirmed by liver biopsy
  • Wilson's disease: ↑ Copper, ↓ Ceruloplasmin + family history

Physical exam may be normal until end-stage disease:

  • Hepatomegaly (small, fibrotic liver in end-stage disease)
  • Terry’s nails (white nail beds)
  • Splenomegaly (if portal hypertension)
  • Central obesity
  • Abdominal fluid wave, shifting dullness (ascites)
  • Gynecomastia
  • Esophageal varices
  • Pulmonary edema/effusion

Classical skin changes: spider angiomata, palmar erythema, jaundice, scleral icterus, ecchymoses, caput medusae, hyperpigmentation

Cirrhosis - skin changes

Hepatic encephalopathy:

  • Asterixis (flapping tremor), dysarthria, delirium, coma

Progressive cirrhosis

  • Elevated ammonia level; BUN, sodium, and potassium
  • α-fetoprotein level at diagnosis to screen for hepatocellular carcinoma (HCC)
  • Abdominal ultrasound every 6 months to screen for hepatocellular carcinoma
  • All patients with cirrhosis should undergo esophagogastroduodenoscopy (EGD) to exclude esophageal varices
  • MRI is the best follow-up test for HCC if α-fetoprotein elevated and/or liver mass found on ultrasound
  • Noninvasive modalities, such as elastography, are being researched as an alternative to liver biopsy
  • Update necessary immunizations and focus on the treatment of the underlying cause of cirrhosis (hepatitis C, alcohol abuse, etc.)
  • Fever and abdominal pain in a patient with cirrhosis think spontaneous bacterial peritonitis
  • Hepatocellular carcinoma: Monitor AFP
  • Budd Chiari (hepatic vein thrombosis) triad of abdominal pain, ascites, and hepatomegaly

Labs: typically, AST > ALT, ↑ the risk for hepatocellular carcinoma: monitor AFP

  • AST/ALT: mildly elevated ⇒ Typically AST > ALT. Enzymes normalize as cirrhosis progresses
  • ↑ ALP and ↑ GGT
  • Anemia from hemolysis, folate deficiency, and splenomegaly
  • Decreased platelet count from portal hypertension with splenomegaly
  • Decreased bilirubin conjugation by the liver ⇒ ↑ unconjugated bilirubin ⇒ jaundice
  • Decreased albumin production by the liver ⇒ Hypoalbuminemia
  • Decreased clotting factor production by the liver ⇒ Prolonged prothrombin (PT), international normalized ratio (INR), partial thromboplastin time (PTT). Vitamin K–dependent clotting factors
  • Ultrasound: helpful to determine the liver size and evaluate for hepatocellular carcinoma
    • Current guidelines recommend performing an abdominal ultrasound every 6 months in patients with cirrhosis to allow for early detection and treatment of HCC. AFP testing can be added to US screening - however, the trade-off is an increase in false positives when using AFP with ultrasound compared to ultrasound alone
  • Liver biopsy is invasive and is subject to sampling error, but it remains the gold standard for the diagnosis of cirrhosis
  • All patients with cirrhosis should undergo esophagogastroduodenoscopy (EGD)Exclude esophageal varices

Child-Pugh Score for Cirrhosis Mortality

  • Class A (5–6 points): one-year survival 100%, two-year survival 85%
  • Class B (7–9 points): one-year survival 81%, two-year survival 57%
  • Class C (10–15 points): one-year survival 45%, two-year survival 35%

Generally, scarring from cirrhosis is irreversible ⇒ Prevent further damage by identifying and treating the underlying cause

  • Stop alcohol
  • Antiviral treatment for Hepatitis C
  • Corticosteroids for autoimmune hepatitis
  • Chelation therapy (e.g., penicillamine) for Wilson's disease
  • Diuretics, antibiotics, laxatives, enemas, thiamine, steroids, acetylcysteine, pentoxifylline for decompensation
  • Nonselective beta-blockers (nadolol and propranolol) for primary prophylaxis against variceal hemorrhage or esophageal variceal ligation (EVL)
  • For advanced cirrhosis ⇒ liver transplant may be necessary
  • Encephalopathy ⇒ lactulose + neomycin
  • Ascites ⇒ sodium restriction, paracentesis
  • Pruritus: ⇒ cholestyramine

Spontaneous bacterial peritonitis is suspected in cases of unexplained fever and abdominal pain

osmosis Osmosis

Picmonic
Cirrhosis Assessment

IM_NUR_CirrohsisAssessment_V1.3_

Cirrhosis is a chronic liver disease that evolves slowly, has a prolonged course, and occurs as a result of excessive alcohol intake, nonalcoholic fatty liver disease (NFLD), or chronic hepatitis C. As a result of these disorders, cirrhosis stems from degeneration and destruction of liver cells.

Play Video + Quiz

Complications of cirrhosis

Patients with cirrhosis are at risk of developing several major complications, such as portal hypertension. This can lead to ascites and esophageal varices. Other complications include decreased liver function, which manifests as coagulation defects. Encephalopathy can occur from ammonia buildup, and hepatorenal syndrome, leading to renal failure

Play Video + Quiz

Cirrhosis Interventions

Care of the patient with cirrhosis involves relieving the discomfort from ascites, excess fluid volume, skin changes, nutritional deficiencies, and preventing complications associated with hematologic problems, esophageal and gastric varices, and hepatic encephalopathy. Patients should avoid alcohol, NSAIDs, and other medications that impair liver function.

Play Video + Quiz

Question 1
A 65-year-old man with liver cirrhosis was brought to the emergency room by his relative due to an altered sleep pattern, irritability, drowsiness, and slowed slurred speech. His relative also said that he has been constipated for the past week. On examination, fetor hepaticus, asterixis, and constructional apraxia were noted. Which of the following is the most appropriate next step in the management of this patient?
A
Administer intravenous fluids
Hint:
While hydration is important in any sick patient, it does not specifically address the primary problem in this case, which is hepatic encephalopathy.
B
Administer lactulose orally or via rectal enema
C
Begin a protein-restricted diet
Hint:
Protein restriction was previously recommended for HE but is no longer advised because malnutrition can exacerbate HE. Adequate protein intake is necessary for liver regeneration and to prevent muscle wasting.
D
Administer haloperidol for his irritability
Hint:
Although haloperidol might calm the patient, it won't treat the underlying cause of his symptoms, which is the accumulation of toxic substances, like ammonia, in the brain due to liver disease. Moreover, haloperidol is metabolized in the liver and may potentially worsen encephalopathy.
E
Prescribe a benzodiazepine for sleep regulation
Hint:
Benzodiazepines are contraindicated in patients with hepatic encephalopathy because they are metabolized by the liver and can exacerbate the condition. They also increase the risk of respiratory depression in these patients.
Question 1 Explanation: 
This patient's presentation is suggestive of hepatic encephalopathy (HE), a complication of cirrhosis characterized by personality changes, intellectual impairment, and a depressed level of consciousness. The signs of HE, such as asterixis (flapping tremor when the wrist is extended), fetor hepaticus (sweet, musty odor of the breath), and constructional apraxia (inability to draw a simple diagram or construct simple figures) are all present in this patient. Constipation can precipitate HE, likely by increasing ammonia production in the colon. The first-line treatment for acute HE is lactulose, which acidifies the colonic contents and converts ammonia (NH3) to ammonium (NH4+), which can't be absorbed, thereby reducing systemic ammonia levels.
Question 2
The ascites associated with cirrhosis generally should be treated by which of the following?
A
sodium restriction
B
water restriction
C
spironolactone
D
furosemide
E
a, c, and d
Question 2 Explanation: 
The treatment of ascites and the edema associated with ascites includes the following: (1) sodium restriction to 800 mg of Na+/day (or 2 g of NaCl); (2) spironolactone (Aldactone), 25 to 100 mg four times per day (effective in 40% to 75% of cases); (3) paracentesis; (4) combination diuretic therapy with furosemide in those patients who do not respond, with either spironolactone plus hydrochlorothiazide or spironolactone plus furosemide; and (5) paracentesis with albumin (or dextran) infusion in refractory cases. There must be complete abstention from alcohol. Fluid restriction is unnecessary unless serum sodium concentration is less than 120 to 125 mEq/L. The 1-year survival rate of patients with cirrhosis with ascites is 50%, compared with 90% in patients with uncomplicated cirrhosis. Formation of ascites results from a combination of portal hypertension, hypoalbuminemia, lymphatic leakage, and sodium retention. The management of ascites in patients with cirrhosis is complicated. Diagnostic paracentesis should be performed in any patient with cirrhosis who undergoes clinical deterioration. Defined indications for the treatment of ascites include significant patient discomfort, respiratory compromise, large umbilical hernia, and recurrent bacterial peritonitis. Sodium restriction is considered the cornerstone of therapy for ascites. Patients with cirrhosis require significant curtailment of sodium intake (800 mg/day) to obtain clinical benefit. Approximately 10% to 20% of patients who maintain a strict low-salt diet achieve complete resolution of ascites without additional therapy. However, hyponatremia may accompany sodium restriction, and severe hyponatremia (serum sodium concentration of less than 125 mEq) is a reason to begin fluid restriction as well. The majority of patients will also require diuretics. Spironolactone (Aldactone), an aldosterone antagonist, is the first-line diuretic of choice in the treatment of cirrhotic ascites. It is effective in controlling up to 50% of patients with cirrhosis. Other potassium-sparing diuretics may be substituted for spironolactone, including triamterene and amiloride. The addition of loop diuretics (e.g., furosemide) is sometimes needed to achieve maximum benefit. Alternatives to furosemide include bumetanide and torsemide. Watch for hypokalemia with diuretics. Up to 90% of patients with cirrhosis and ascites will respond to diuretics and salt restriction. For those who do not, periodic paracentesis with albumin replacement is an alternative, as are portacaval shunts and transjugular intrahepatic portosystemic shunts, the latter for those requiring frequent large-volume paracentesis.
Question 3
One of the following is not a feature of decompensated liver cirrhosis.
A
Jaundice
B
Hepatic encephalopathy
C
Ascites
D
Constipation
Question 3 Explanation: 
Constipation is not a feature of decompensated liver cirrhosis. All other options are.
Question 4
A 60-year-old man presents to you on account of right hypochondrial pain, anorexia, and fatigue. On history taking, patient revealed that he has been taking excessive alcohol for over 20 years. Physical examination reveals icterus, gynecomastia, spider angioma, hepatomegaly, shifting dullness, visibly distended abdominal wall veins. Serum AST, AST, and ALP are elevated. Which of the following is the most likely diagnosis?
A
Liver abscess
Hint:
Patient with liver abscess will have same symptoms and fever. Alcohol ingestion is not a risk factor for abscess.
B
Hepatic encephalopathy
Hint:
Hepatic encephalopathy is a complication of cirrhosis. Patient would have presented with altered sleep pattern, mental confusion, drowsiness.
C
Liver cirrhosis
D
Acute viral hepatitis
Hint:
Patient with acute viral hepatitis may present with the same symptoms and hepatomegaly, but not ascites, gynecomastia, spider angioma, distended abdominal veins.
Question 4 Explanation: 
With a prolonged history of excessive alcohol ingestion and presence of peripheral stigmata of chronic liver disease(icterus, gynecomastia, spider angioma, ascites elicited by shifting dullness, distended abdominal veins , liver cirrhosis is the most likely diagnosis. Patient with acute viral hepatitis may present with the same symptoms and hepatomegaly, but not ascites, gynecomastia, spider angioma, distended abdominal veins. Hepatic encephalopathy is a complication of cirrhosis. Patient would have presented with altered sleep pattern, mental confusion, drowsiness. Patient with liver abscess will have same symptoms and fever. Alcohol ingestion is not a risk factor for abscess.
Question 5
Which of the following is (are) appropriate treatments for cirrhosis of the liver?
A
cessation of alcohol use
B
beta blockers
C
maintenance of proper nutrition
D
liver transplantation
E
all of the above
Question 5 Explanation: 
In uncomplicated cirrhosis, treatment includes cessation of alcohol use, maintenance of proper nutrition, and use of beta blockers to reduce portal hypertension. Patients with uncomplicated cirrhosis may have a relatively benign course of illness for many years. Once the patient has an episode of decompensation, such as fluid retention, variceal bleeding, encephalopathy, spontaneous bacterial peritonitis, or hepatorenal syndrome, mortality is high without transplantation.
Question 6
Which of the following is not a common cause of liver cirrhosis in the United States?
A
Alcoholic liver disease
Hint:
is a common cause of liver cirrhosis in the United States (21%).
B
Chronic hepatitis C infection
Hint:
is a common cause of liver cirrhosis in the United States (26%).
C
Wilson’s disease
D
Nonalcoholic fatty liver disease
Hint:
is a common cause of liver cirrhosis in the United States (18%).
Question 6 Explanation: 
Wilson's disease is a rare inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. It is not a common cause of liver cirrhosis. Patients with Wilson's disease will have abnormal lab values: ↑ Copper, ↓ Ceruloplasmin + family history.
Question 7
Which of the following describes the appearance of distended and engorged superficial epigastric veins, which are seen radiating from the umbilicus across the abdomen seen in patients with cirrhosis?
A
caput medusae
B
spider angiomata
Hint:
Spider angioma is an abnormal collection of blood vessels near the surface of the skin.
C
palmar erythema
Hint:
Palmar erythema is a reddening of the skin on the palmar aspect of the hands, usually over the hypothenar eminence. It may also involve the thenar eminence and fingers. It can also be found on the soles of the feet, when it is termed plantar erythema.
D
scleral icterus
Hint:
The yellowing of the "white of the eye" is thus more properly termed conjunctival icterus. The term "icterus" itself is sometimes incorrectly used to refer to jaundice that is noted in the sclera of the eyes, however its more common and more correct meaning is entirely synonymous with jaundice.
Question 7 Explanation: 
Caput medusae, also known as palm tree sign, is the appearance of distended and engorged superficial epigastric veins, which are seen radiating from the umbilicus across the abdomen.
There are 7 questions to complete.
List
Return
Shaded items are complete.
12345
67
Return

References: Merck Manual · UpToDate

Lesson Intro Video

Acute and chronic hepatitis (ReelDx + Lecture) (Prev Lesson)
(Next Lesson) Nonalcoholic fatty liver disease (NAFLD) – Metabolic dysfunction-associated steatotic liver disease (MASLD)
Back to PANCE Blueprint GI and Nutrition (9%)

NCCPA™ CONTENT BLUEPRINT