I85PREP
⌘ K
ABP Transplant Hepatology study materials
ABP Transplant Hepatology study resources

ABP Transplant Hepatology practice questions

One question at a time. Build your ABP Transplant Hepatology understanding.

First 50 of 200 questions. Each one lists the choices, the correct answer, and an explanation. The checker at the top of this page saves progress in this browser when it is available.

Continue in the practice tool above to load more questions, 50 at a time.

Question 1Evaluation and management of acute and chronic liver disease in children

An 8-week-old infant has persistent jaundice and dark urine. Which laboratory result confirms cholestasis rather than unconjugated hyperbilirubinemia?

  1. Direct bilirubin >1 mg/dL with total >5 mg/dL Correct answer.
  2. Alkaline phosphatase of 300 U/L with normal bilirubin
  3. Total bilirubin of 12 mg/dL with a normal direct fraction
  4. Hemoglobin of 9 g/dL with increased reticulocytes

Explanation

Cholestasis in infants is defined by a conjugated (direct) bilirubin level greater than 1 mg/dL when the total bilirubin is above 5 mg/dL. Jaundice persisting beyond 2 weeks of age should prompt fractionation of bilirubin. Unconjugated hyperbilirubinemia alone does not indicate cholestasis.

Question 2Evaluation and management of acute and chronic liver disease in children

A term infant with acholic stools and direct hyperbilirubinemia is suspected to have biliary atresia. Which management step most improves the chance of native liver survival?

  1. Elective liver transplantation at 1 year of age
  2. Kasai portoenterostomy before 60 days of age Correct answer.
  3. Observation with repeat liver tests in 6 weeks
  4. Ursodeoxycholic acid alone until 6 months of age

Explanation

Kasai portoenterostomy restores bile flow in biliary atresia, and outcomes are best when it is performed before 60 days of age. Delaying surgery increases the risk of progressive cirrhosis and need for transplantation. Ursodeoxycholic acid is supportive but not definitive.

Question 3Evaluation and management of acute and chronic liver disease in children

A newborn with galactosemia is started on an appropriate diet. Which feeding choice is correct?

  1. Breast milk with lactase supplementation
  2. Soy-based, lactose-free formula Correct answer.
  3. Elemental formula containing medium-chain triglycerides
  4. Standard cow's milk-based formula

Explanation

Classic galactosemia requires strict avoidance of galactose and lactose. Soy-based formula is lactose-free and is the standard initial feeding choice. Breast milk and cow's milk contain lactose and are contraindicated.

Question 4Evaluation and management of acute and chronic liver disease in children

A 10-year-old child develops jaundice, vomiting, and elevated transaminases after a family trip. Which statement about hepatitis A is correct?

  1. It typically causes chronic infection in children
  2. It requires lifelong antiviral therapy
  3. It is transmitted by blood and body fluids
  4. Fecal-oral spread with a self-limited course Correct answer.

Explanation

Hepatitis A is transmitted by the fecal-oral route and causes acute, self-limited hepatitis in children. It does not establish chronic infection, and treatment is supportive. Vaccination and hand hygiene are key prevention measures.

Question 5Evaluation and management of acute and chronic liver disease in children

A 3-year-old with persistent transaminase elevation has suspected alpha-1 antitrypsin deficiency. Which test is the most appropriate initial screen?

  1. Sweat chloride test
  2. Liver biopsy with PAS-positive globules
  3. Genetic testing for HFE mutations
  4. Serum alpha-1 antitrypsin concentration Correct answer.

Explanation

Serum alpha-1 antitrypsin concentration is the initial screening test; low levels are followed by phenotype or genotype testing. Liver biopsy may show characteristic globules but is not the first-line screen. HFE testing and sweat chloride evaluate different diseases.

Question 6Evaluation and management of acute and chronic liver disease in children

A 12-year-old girl has fatigue, jaundice, elevated IgG, and interface hepatitis on biopsy. Which autoantibody pattern most supports type 1 autoimmune hepatitis?

  1. Antinuclear and smooth muscle antibodies Correct answer.
  2. Anti-liver-kidney microsomal antibody
  3. Anti-mitochondrial antibody
  4. Anti-tissue transglutaminase antibody

Explanation

Type 1 autoimmune hepatitis is associated with antinuclear antibodies and smooth muscle antibodies. Anti-liver-kidney microsomal antibody characterizes type 2 disease. Anti-mitochondrial antibodies are typical of primary biliary cholangitis.

Question 7Evaluation and management of acute and chronic liver disease in children

A 14-year-old presents with tremor, dysarthria, and jaundice. Which finding most specifically supports Wilson disease?

  1. Low serum ferritin
  2. Kayser-Fleischer rings on slit-lamp Correct answer.
  3. Elevated serum ceruloplasmin
  4. Positive antinuclear antibody

Explanation

Kayser-Fleischer rings reflect copper deposition in Descemet membrane and are highly specific for Wilson disease when present. Serum ceruloplasmin is usually low, not elevated. Antinuclear antibodies and ferritin are not specific for Wilson disease.

Question 8Evaluation and management of acute and chronic liver disease in children

An obese 13-year-old has ALT 90 U/L and hepatic steatosis on ultrasound. Which is the first-line management for presumed metabolic dysfunction-associated steatotic liver disease?

  1. Vitamin E 800 IU daily
  2. Diet, exercise, and gradual weight loss Correct answer.
  3. Low-dose aspirin
  4. Metformin 1000 mg daily

Explanation

First-line management of pediatric steatotic liver disease is structured lifestyle change with gradual weight loss. There is no routinely approved pharmacologic therapy for pediatric metabolic dysfunction-associated steatotic liver disease. Metformin, vitamin E, and aspirin are not first-line treatments.

Question 9Evaluation and management of acute and chronic liver disease in children

A child with progressive cholestasis and severe pruritus has markedly elevated serum bile acids but normal gamma-glutamyl transferase. Which disorder fits this pattern?

  1. PFIC type 3
  2. Biliary atresia
  3. Alagille syndrome
  4. PFIC type 1/2 Correct answer.

Explanation

Progressive familial intrahepatic cholestasis types 1 and 2 typically produce low or normal gamma-glutamyl transferase despite marked cholestasis. PFIC type 3, biliary atresia, and Alagille syndrome usually raise gamma-glutamyl transferase. The normal value points away from biliary obstruction.

Question 10Evaluation and management of acute and chronic liver disease in children

A child with Alagille syndrome has a heart murmur. Which cardiac lesion is most characteristic?

  1. Tetralogy of Fallot with pulmonary atresia
  2. Patent ductus arteriosus with left-to-right shunt
  3. Ventricular septal defect with left-to-right shunt
  4. Peripheral pulmonary stenosis Correct answer.

Explanation

Alagille syndrome is caused by JAG1 or NOTCH2 abnormalities and commonly includes peripheral pulmonary stenosis. Other cardiac defects can occur, but peripheral pulmonary stenosis is the classic lesion. The syndrome also features bile duct paucity and butterfly vertebrae.

Question 11Evaluation and management of acute and chronic liver disease in children

An infant with failure to thrive, hepatomegaly, and coagulopathy has elevated tyrosine and methionine. Which test confirms tyrosinemia type 1?

  1. Serum phenylalanine
  2. Urine organic acids showing methylmalonic acid
  3. Sweat chloride
  4. Blood succinylacetone Correct answer.

Explanation

Succinylacetone is pathognomonic for tyrosinemia type 1 and reflects deficiency of fumarylacetoacetate hydrolase. Confirmation is important because nitisinone plus dietary restriction can prevent liver failure. Phenylalanine, methylmalonic acid, and sweat chloride test other disorders.

Question 12Evaluation and management of acute and chronic liver disease in children

A child with cystic fibrosis develops elevated liver enzymes and hepatomegaly. Which liver manifestation is most typical?

  1. Autoimmune hepatitis
  2. Focal biliary cirrhosis Correct answer.
  3. Wilson disease
  4. Primary biliary cholangitis

Explanation

Cystic fibrosis liver disease commonly manifests as focal biliary cirrhosis due to inspissated bile and ductal obstruction. Ursodeoxycholic acid is often used, although evidence for hard outcomes is limited. Primary biliary cholangitis, autoimmune hepatitis, and Wilson disease are unrelated.

Question 13Evaluation and management of acute and chronic liver disease in children

A 7-year-old boy has acute hepatitis, high aminotransferases, and positive anti-liver-kidney microsomal antibody. Which diagnosis is most likely?

  1. Type 2 autoimmune hepatitis Correct answer.
  2. Type 1 autoimmune hepatitis
  3. Celiac hepatitis
  4. Primary sclerosing cholangitis

Explanation

Anti-liver-kidney microsomal antibody is the marker of type 2 autoimmune hepatitis, which tends to affect younger children and can be severe. Type 1 disease is marked by antinuclear and smooth muscle antibodies. Primary sclerosing cholangitis and celiac hepatitis have different serologic and histologic features.

Question 14Evaluation and management of acute and chronic liver disease in children

A child with acute liver failure after an acetaminophen overdose is being managed. Which therapy is indicated?

  1. Pegylated interferon
  2. N-acetylcysteine Correct answer.
  3. Ribavirin
  4. Corticosteroids

Explanation

N-acetylcysteine replenishes glutathione and is the antidote for acetaminophen-induced liver injury. It is given early and continued in severe cases. Corticosteroids, ribavirin, and interferon do not treat acetaminophen toxicity.

Question 15Evaluation and management of acute and chronic liver disease in children

A pregnant woman is positive for hepatitis B surface antigen. Which protocol best prevents perinatal transmission to her newborn?

  1. Cesarean delivery and no immunoprophylaxis
  2. Hepatitis B vaccine at 2 months only
  3. Vaccine and HBIG within 12 hours of birth Correct answer.
  4. Hepatitis B immune globulin at 6 months

Explanation

Newborns of hepatitis B surface antigen-positive mothers should receive hepatitis B vaccine and hepatitis B immune globulin within 12 hours of birth. The vaccine series is then completed according to schedule. Cesarean delivery does not replace immunoprophylaxis.

Question 16Evaluation and management of acute and chronic liver disease in children

A 4-year-old has recurrent abdominal pain, jaundice, and a cystic dilation of the common bile duct. Which statement is correct?

  1. It usually resolves spontaneously by age 10
  2. It carries no increased cancer risk after drainage
  3. It is best managed with ursodeoxycholic acid alone
  4. Excision is advised because of cancer risk Correct answer.

Explanation

Choledochal cysts are excised with biliary reconstruction because they carry an increased risk of cholangiocarcinoma. Spontaneous resolution does not occur, and ursodeoxycholic acid alone is inadequate. Drainage without excision leaves malignant risk.

Question 17Evaluation and management of acute and chronic liver disease in children

A child with cirrhosis has hematemesis and signs of portal hypertension. After stabilization, which initial therapy is appropriate for suspected variceal bleeding?

  1. Immediate transjugular intrahepatic portosystemic shunt
  2. Intravenous octreotide and urgent endoscopy Correct answer.
  3. Oral propranolol started during active bleeding
  4. Large-volume paracentesis

Explanation

Acute variceal bleeding is managed with vasoactive therapy such as octreotide plus urgent endoscopic evaluation and band ligation. Beta blockers are used for prophylaxis, not during active hemorrhage. Transjugular intrahepatic portosystemic shunt is generally rescue therapy.

Question 18Evaluation and management of acute and chronic liver disease in children

A newborn with severe liver failure, coagulopathy, and hypoglycemia is diagnosed with gestational alloimmune liver disease. Which treatment is appropriate?

  1. Penicillamine and zinc
  2. Nitisinone and a tyrosine-restricted diet
  3. Ursodeoxycholic acid and fat-soluble vitamins
  4. IVIG and exchange transfusion Correct answer.

Explanation

Gestational alloimmune liver disease is treated with intravenous immunoglobulin and exchange transfusion to blunt the alloimmune injury. Liver transplantation is considered if supportive therapy fails. Chelation, nitisinone, and ursodeoxycholic acid treat other liver diseases.

Question 19Evaluation and management of acute and chronic liver disease in children

A 2-year-old with hepatomegaly, fasting hypoglycemia, lactic acidosis, and hypertriglyceridemia is diagnosed with glycogen storage disease type I. Which dietary strategy is correct?

  1. High-protein diet with no carbohydrate
  2. Prolonged fasting to reduce glycogen stores
  3. Ketogenic diet with medium-chain triglycerides
  4. Frequent carbs and uncooked cornstarch Correct answer.

Explanation

Glycogen storage disease type I causes fasting hypoglycemia and lactic acidosis, so treatment aims to prevent fasting and maintain glucose. Frequent carbohydrate feedings and uncooked cornstarch are standard. Prolonged fasting is dangerous.

Question 20Evaluation and management of acute and chronic liver disease in children

A teenager develops jaundice and elevated transaminases after starting isoniazid. Which management step is most important?

  1. Add rifampin
  2. Increase the isoniazid dose
  3. Continue isoniazid and monitor liver tests closely
  4. Stop isoniazid and give supportive care Correct answer.

Explanation

Isoniazid can cause drug-induced liver injury, and the key step is to stop the offending drug promptly. Supportive care and monitoring follow, and rechallenge is avoided. Continuing or increasing the drug can lead to severe injury.

Question 21Evaluation and management of acute and chronic liver disease in children

A child with PFIC type 2 has intractable pruritus despite ursodeoxycholic acid. Which non-surgical therapy directly reduces intestinal bile acid reabsorption?

  1. Phenobarbital
  2. IBAT inhibitor Correct answer.
  3. Cholestyramine resin
  4. Corticosteroid

Explanation

Ileal bile acid transporter inhibitors block intestinal reabsorption of bile acids, lowering serum bile acids and pruritus in PFIC. Cholestyramine binds bile acids in the gut but does not directly inhibit the transporter. Corticosteroids and phenobarbital do not address bile acid reabsorption.

Question 22Evaluation and management of acute and chronic liver disease in children

A child with Wilson disease has elevated urinary copper and liver involvement. Which chelating agent is commonly used as initial therapy?

  1. Zinc acetate therapy
  2. Rifampicin
  3. D-penicillamine Correct answer.
  4. Colchicine

Explanation

D-penicillamine chelates copper and promotes urinary excretion, making it a standard initial treatment for symptomatic Wilson disease. Trientine is an alternative chelator. Zinc blocks intestinal copper absorption but is not a chelator for initial treatment of active disease.

Question 23Evaluation and management of acute and chronic liver disease in children

A child with citrin deficiency has hyperammonemia and prefers high-protein foods. Which amino acid supplementation is often used?

  1. Valine
  2. Arginine Correct answer.
  3. Methionine
  4. Tryptophan

Explanation

Arginine supplementation is used in citrin deficiency to support the urea cycle and help manage hyperammonemia. Management also includes avoiding fasting and providing appropriate calories, often with medium-chain triglycerides. Valine, tryptophan, and methionine are not the standard supplement.

Question 24Evaluation and management of acute and chronic liver disease in children

A child with cirrhosis and hepatic encephalopathy is being treated. Which agent is first-line to reduce ammonia production in the gut?

  1. Rifaximin
  2. Sodium benzoate
  3. Lactulose Correct answer.
  4. Pegylated interferon

Explanation

Lactulose acidifies the colon and traps ammonia, reducing absorption, and is first-line for hepatic encephalopathy. Rifaximin is often added for recurrent or refractory episodes. Sodium benzoate and interferon do not act primarily on gut ammonia.

Question 25Evaluation and management of acute and chronic liver disease in children

A 10-year-old with Wilson disease presents with fulminant hepatic failure and coagulopathy. Which management is most appropriate?

  1. Observation with serial ceruloplasmin
  2. Zinc acetate monotherapy
  3. Long-term penicillamine alone
  4. Urgent liver transplantation evaluation Correct answer.

Explanation

Fulminant Wilson disease with coagulopathy is life-threatening and requires urgent liver transplantation evaluation. Chelation and zinc are too slow to reverse fulminant hepatic failure. Observation or monotherapy would delay definitive treatment.

Question 26Evaluation and management of acute and chronic liver disease in children

A 3-week-old breastfed infant remains jaundiced. Which laboratory test best identifies whether the hyperbilirubinemia is cholestatic rather than due to unconjugated bilirubin?

  1. Serum alanine aminotransferase activity
  2. Total serum bilirubin concentration
  3. Serum gamma-glutamyl transferase activity
  4. Direct (conjugated) bilirubin fraction Correct answer.

Explanation

Cholestasis in an infant is defined by an elevated direct or conjugated bilirubin fraction, which indicates that bilirubin is being conjugated but not excreted normally. Total bilirubin cannot distinguish conjugated from unconjugated hyperbilirubinemia, so it does not establish cholestasis. Liver enzymes may be abnormal in many patterns of injury but do not directly measure bilirubin conjugation.

Question 27Evaluation and management of acute and chronic liver disease in children

A 6-week-old infant has persistent jaundice, pale stools, and conjugated hyperbilirubinemia; biliary atresia is confirmed. Which intervention offers the best chance of native liver survival?

  1. Long-term ursodeoxycholic acid monotherapy
  2. Kasai portoenterostomy performed promptly Correct answer.
  3. Prophylactic cholecystectomy
  4. Immediate liver transplantation for all affected infants

Explanation

Kasai portoenterostomy restores bile drainage and is most effective when performed before approximately 60 days of age, improving the chance of native liver survival. Ursodeoxycholic acid is supportive rather than definitive, and transplantation is reserved for children in whom drainage fails or cirrhosis progresses. Cholecystectomy does not relieve the extrahepatic biliary obstruction.

Question 28Evaluation and management of acute and chronic liver disease in children

An infant with prolonged cholestasis is found to have alpha-1 antitrypsin deficiency with a PI*ZZ genotype. Which process most directly explains the liver injury?

  1. Autoimmune destruction of interlobular bile ducts
  2. Retention of misfolded alpha-1 antitrypsin in hepatocytes Correct answer.
  3. Excess iron deposition within periportal hepatocytes
  4. Deficient antiprotease activity within the pulmonary alveoli

Explanation

The PI*ZZ genotype produces a misfolded alpha-1 antitrypsin protein that accumulates within hepatocyte endoplasmic reticulum, causing hepatocellular injury and cholestasis. Deficient antiprotease activity in the lung explains emphysema rather than liver disease. Iron overload and autoimmune bile duct destruction are separate disorders.

Question 29Evaluation and management of acute and chronic liver disease in children

A 12-year-old with ulcerative colitis has elevated alkaline phosphatase and gamma-glutamyl transferase. MRCP shows multifocal intrahepatic and extrahepatic bile duct strictures. Which antibody is most commonly associated with this condition?

  1. Anti-smooth muscle antibody directed against hepatocyte antigens
  2. Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) Correct answer.
  3. Anti-mitochondrial antibody directed against bile duct antigens
  4. Anti-liver-kidney microsomal antibody directed against cytochrome P450

Explanation

The child has primary sclerosing cholangitis, which is strongly associated with inflammatory bowel disease and with perinuclear anti-neutrophil cytoplasmic antibodies. Anti-mitochondrial antibodies are associated with primary biliary cholangitis, while anti-smooth muscle and anti-liver-kidney microsomal antibodies are associated with autoimmune hepatitis. MRCP demonstrating multifocal strictures is characteristic of primary sclerosing cholangitis.

Question 30Evaluation and management of acute and chronic liver disease in children

A 9-year-old with epilepsy treated with valproate develops vomiting, lethargy, and anorexia. Labs show elevated transaminases, ammonia, and prolonged INR. Which step is most appropriate?

  1. Reduce the valproate dose and monitor weekly
  2. Start isoniazid prophylaxis
  3. Stop valproate and give L-carnitine Correct answer.
  4. Continue valproate and add ondansetron

Explanation

Valproate can cause mitochondrial hepatotoxicity with hyperammonemia and coagulopathy, so the drug should be stopped promptly and L-carnitine should be provided. Continuing or merely reducing valproate risks progression of liver failure. Isoniazid is irrelevant to this presentation and is itself potentially hepatotoxic.

Question 31Evaluation and management of acute and chronic liver disease in children

A 7-month-old develops vomiting, pallor, jaundice, and hepatomegaly shortly after fruit juice and sweetened cereal are introduced. Symptoms resolve with fasting and avoidance of those foods. Which diagnosis is most likely?

  1. Cystic fibrosis-associated liver disease
  2. Classic galactosemia
  3. Hereditary fructose intolerance Correct answer.
  4. Glycogen storage disease type I

Explanation

Hereditary fructose intolerance results from aldolase B deficiency, so ingestion of fructose or sucrose causes vomiting, hypoglycemia, jaundice, and hepatomegaly that improve with dietary exclusion. Classic galactosemia typically presents after milk feeding rather than after fruit or sucrose. Glycogen storage disease type I causes fasting intolerance and lactic acidosis, while cystic fibrosis liver disease is chronic and not reproducibly food-triggered in this way.

Question 32Evaluation and management of acute and chronic liver disease in children

A 10-day-old presents with fever, lethargy, vesicular skin lesions, coagulopathy, and markedly elevated aminotransferases. Which empiric therapy is most urgently indicated?

  1. Oral acyclovir
  2. Intravenous acyclovir Correct answer.
  3. Oral valacyclovir
  4. Intravenous ceftriaxone alone

Explanation

The combination of vesicular lesions, fever, coagulopathy, and marked aminotransferase elevation in a neonate suggests disseminated herpes simplex virus infection, for which intravenous acyclovir is the urgent treatment. Oral antiviral agents do not achieve reliable adequate exposure in severe neonatal disease. Ceftriaxone alone would not treat herpes simplex virus.

Question 33Evaluation and management of acute and chronic liver disease in children

A 14-year-old with abdominal pain and ascites is found to have absent hepatic vein flow on Doppler ultrasound. Which initial management is most appropriate for this vascular disorder?

  1. Immediate surgical portosystemic shunt for all patients
  2. High-dose corticosteroids
  3. Observation until liver transplant evaluation
  4. Anticoagulation with monitoring for liver failure Correct answer.

Explanation

Absent hepatic vein flow with ascites indicates Budd-Chiari syndrome, and initial management generally includes anticoagulation to limit thrombus extension while monitoring hepatic function. Portosystemic shunting is reserved for selected patients with refractory symptoms or progressive disease, not as universal first-line therapy. Corticosteroids and simple observation do not address the underlying venous obstruction.

Question 34Evaluation and management of acute and chronic liver disease in children

A child with ornithine transcarbamylase deficiency has recurrent hyperammonemic crises despite dietary protein restriction and nitrogen-scavenging drugs. Which definitive treatment should be considered?

  1. Liver transplantation Correct answer.
  2. Long-term lactulose monotherapy
  3. Arginine supplementation alone
  4. Partial portacaval shunting

Explanation

Ornithine transcarbamylase deficiency is a urea cycle enzyme defect expressed in the liver, and liver transplantation replaces the defective enzyme function, preventing recurrent hyperammonemia. Lactulose and arginine are adjunctive therapies rather than definitive correction. Partial portacaval shunting is not standard definitive management for this disorder.

Question 35Complications of cirrhosis and portal hypertension

A patient with new-onset ascites has a serum-ascites albumin gradient of 1.8 g/dL. Which mechanism best explains this finding?

  1. Tuberculous peritonitis
  2. Peritoneal carcinomatosis
  3. Nephrotic syndrome
  4. Portal hypertension Correct answer.

Explanation

A gradient of 1.1 g/dL or more reflects a transudative process driven by hydrostatic pressure and is roughly 97% accurate for portal hypertension. Values below 1.1 g/dL point to peritoneal or other non-portal causes. The gradient is calculated by subtracting ascitic albumin from serum albumin.

Question 36Complications of cirrhosis and portal hypertension

Which ascitic fluid polymorphonuclear leukocyte count establishes the diagnosis of spontaneous bacterial peritonitis?

  1. At least 1000 cells per mm3
  2. At least 250 cells per mm3 Correct answer.
  3. At least 100 cells per mm3
  4. At least 500 cells per mm3

Explanation

A polymorphonuclear count of 250 cells per mm3 or more is the diagnostic threshold and should prompt antibiotics, even before cultures return. Lower thresholds such as 100 cells per mm3 are not diagnostic in ascites. Waiting for culture results delays treatment and increases mortality.

Question 37Complications of cirrhosis and portal hypertension

A patient with compensated cirrhosis has medium-sized esophageal varices with red wale marks on screening endoscopy. Which intervention is most appropriate?

  1. Non-selective beta-blocker Correct answer.
  2. Immediate TIPS
  3. Repeat endoscopy in two years
  4. Long-term proton pump inhibitor therapy

Explanation

Medium or large varices, or varices with red wale marks, warrant primary prophylaxis with a non-selective beta-blocker or endoscopic variceal ligation. Either approach lowers the risk of first bleeding and is recommended over observation. TIPS is reserved for secondary prophylaxis failures, not initial prevention.

Question 38Complications of cirrhosis and portal hypertension

Which agent is first-line treatment for an acute episode of overt hepatic encephalopathy?

  1. Oral neomycin
  2. Rifaximin monotherapy
  3. Branched-chain amino acid infusion
  4. Lactulose Correct answer.

Explanation

Lactulose is the first-line agent for acute overt encephalopathy and is titrated to two to three soft stools daily. Rifaximin is typically added for recurrence prevention rather than used alone for the acute episode. Neomycin and branched-chain amino acids are adjuncts at best.

Question 39Complications of cirrhosis and portal hypertension

Which daily sodium intake is generally recommended for adults with cirrhotic ascites?

  1. 500 mg of sodium
  2. 2 g of sodium Correct answer.
  3. 6 g of sodium
  4. No sodium restriction

Explanation

A daily sodium intake of about 2 g (88 mmol) is standard and is paired with diuretics when tolerated. More severe restriction below 1 g is not recommended because it worsens nutrition and does not improve outcomes. Unrestricted intake makes ascites control very difficult.

Question 40Complications of cirrhosis and portal hypertension

A patient with cirrhosis and ascites has a serum sodium of 124 mEq/L and is currently taking furosemide and spironolactone. Which initial measure is most appropriate?

  1. Rapid infusion of hypertonic saline
  2. Higher doses of furosemide
  3. Demeclocycline
  4. Fluid restriction to about 1 L daily Correct answer.

Explanation

Hypervolemic hyponatremia below roughly 125 mEq/L is managed by stopping diuretics and restricting fluid to about 1 L per day. Rapid correction with hypertonic saline risks osmotic demyelination and volume overload. Demeclocycline is hepatotoxic and not used in cirrhosis.

Question 41Complications of cirrhosis and portal hypertension

A patient with cirrhosis recovers from an episode of spontaneous bacterial peritonitis. Which management is indicated afterward?

  1. No further prophylaxis
  2. Prophylactic TIPS
  3. Daily oral norfloxacin Correct answer.
  4. Weekly therapeutic paracentesis

Explanation

After an episode of spontaneous bacterial peritonitis, secondary prophylaxis with a daily fluoroquinolone such as norfloxacin or ciprofloxacin reduces recurrence and improves survival. It should be continued until transplant or indefinitely. Albumin is also given during the acute episode to prevent renal failure.

Question 42Complications of cirrhosis and portal hypertension

A patient with cirrhosis and ascites develops acute kidney injury. Which finding best favors hepatorenal syndrome over prerenal azotemia from volume depletion?

  1. Fractional excretion of sodium above 2%
  2. Resolution of injury after 1 L of saline
  3. No response to 48 hours of albumin Correct answer.
  4. Proteinuria of 2 g per day

Explanation

Hepatorenal syndrome is a diagnosis of exclusion that requires no response to at least 48 hours of albumin with diuretic withdrawal, after excluding shock, nephrotoxins, and structural disease. The urinary sediment is usually bland and proteinuria is minimal or absent. Volume-responsive injury suggests a prerenal cause instead.

Question 43Complications of cirrhosis and portal hypertension

A patient with cirrhosis presents with hematemesis and is resuscitated. Which pharmacologic approach is standard once bleeding is suspected?

  1. Octreotide alone with no antibiotic
  2. Ceftriaxone alone without vasoactive therapy
  3. Propranolol plus fresh frozen plasma
  4. Octreotide plus ceftriaxone Correct answer.

Explanation

Suspected variceal hemorrhage is treated with a vasoactive drug such as octreotide or terlipressin plus prophylactic antibiotics, started before endoscopy. Antibiotics reduce bacterial infection, rebleeding, and mortality. Propranolol is not used during acute hemorrhage because it blunts the compensatory tachycardia.

Question 44Complications of cirrhosis and portal hypertension

A patient with cirrhosis has a large right-sided pleural effusion, and thoracentesis shows a transudate with a serum-pleural albumin gradient above 1.1 g/dL and no pleural infection. Which initial therapy is most appropriate?

  1. Thoracotomy with pleurectomy
  2. Immediate chest tube placement
  3. Sodium restriction with diuretics Correct answer.
  4. Chemical pleurodesis

Explanation

Hepatic hydrothorax is managed like ascites, with sodium restriction, diuretics, and treatment of the underlying portal hypertension. Chest tubes are avoided because of large protein losses, infection risk, and failure of the effusion to resolve without correcting the portal pressure. TIPS is reserved for refractory cases.

Question 45Complications of cirrhosis and portal hypertension

A patient with cirrhosis and known gastric varices presents with active gastric variceal bleeding at endoscopy. Which endoscopic therapy is preferred?

  1. Cyanoacrylate glue injection Correct answer.
  2. Endoscopic sclerotherapy
  3. Balloon tamponade
  4. Endoscopic band ligation

Explanation

Cyanoacrylate glue injection is the preferred endoscopic treatment for acute gastric variceal hemorrhage because it obliterates the large submucosal vessels. Band ligation is used for esophageal varices and is technically difficult in the stomach. Balloon tamponade is a temporizing rescue measure.

Question 46Complications of cirrhosis and portal hypertension

Which finding is an absolute contraindication to creation of a transjugular intrahepatic portosystemic shunt?

  1. Child-Pugh class B cirrhosis
  2. Refractory ascites
  3. Recurrent variceal bleeding
  4. Severe portopulmonary hypertension Correct answer.

Explanation

Severe portopulmonary hypertension, uncontrolled heart failure, and systemic infection are absolute contraindications because the shunt abruptly increases right-sided venous return and can precipitate right heart failure. Refractory ascites and recurrent variceal bleeding are common indications, not contraindications.

Question 47Complications of cirrhosis and portal hypertension

A patient with cirrhosis reports dyspnea that worsens on standing and improves when supine. Arterial blood gas on room air shows hypoxemia, and contrast echocardiography demonstrates intrapulmonary vascular dilatation appearing after several cardiac cycles. What is the diagnosis?

  1. Cirrhotic cardiomyopathy
  2. Portopulmonary hypertension
  3. Hepatic hydrothorax
  4. Hepatopulmonary syndrome Correct answer.

Explanation

Hepatopulmonary syndrome consists of liver disease, intrapulmonary vascular dilatation, and arterial deoxygenation, with platypnea and orthodeoxia as classic features. Delayed appearance of bubbles on contrast echocardiography indicates intrapulmonary shunting. Portopulmonary hypertension instead shows elevated pulmonary arterial pressure without shunting.

Question 48Complications of cirrhosis and portal hypertension

Which statement best describes the coagulation system in cirrhosis?

  1. A uniformly hypocoagulable state
  2. Rebalanced hemostasis Correct answer.
  3. A uniformly hypercoagulable state
  4. Normal coagulation tests

Explanation

Cirrhosis involves reduced synthesis of both procoagulant and anticoagulant factors, so the net effect is rebalanced hemostasis rather than a simple bleeding tendency. This is why patients can both bleed and thrombose, and why INR does not reliably predict bleeding risk. Routine coagulation tests do not capture this balance.

Question 49Complications of cirrhosis and portal hypertension

A patient with cirrhosis is found to have a portal vein thrombus. Which feature most strongly supports starting anticoagulation?

  1. Presence of red wale marks
  2. Thrombus limited to a small intrahepatic branch
  3. Platelet count of 60000 per mm3
  4. Superior mesenteric vein involvement Correct answer.

Explanation

Anticoagulation is generally favored when the thrombus extends into the superior mesenteric vein, when it is progressive, or when the patient is a transplant candidate, because extension can cause intestinal ischemia and complicates transplantation. Low-molecular-weight heparin is typically used first. Bleeding risk, especially from varices, must be weighed.

Question 50Complications of cirrhosis and portal hypertension

A patient with tense ascites undergoes large-volume paracentesis with removal of 6 L of fluid. Which adjunct is recommended to prevent post-paracentesis circulatory dysfunction?

  1. Two liters of normal saline
  2. Albumin 8 g per liter removed Correct answer.
  3. Fresh frozen plasma
  4. Intravenous furosemide 80 mg

Explanation

Albumin at roughly 6 to 8 g per liter of ascites removed prevents post-paracentesis circulatory dysfunction, which otherwise causes rapid reaccumulation, hyponatremia, and renal impairment. It is generally given when more than 5 L is removed. Crystalloid and plasma are inferior substitutes.