A 42-year-old female presented with anorexia and fatigue. Workup revealed elevated aminotransferases approximately 10 times the upper limit of normal. Immunoglobulin G levels are elevated, and she is found to have positive titers for antinuclear antibodies. Liver biopsy reveals inflammation at the boundaries of the hepatocytes and portal triad. The patient is started on therapy with prednisone.
The addition of which medication would be MOST helpful?
Correct Answer: C
This patient has autoimmune hepatitis with classic features such as elevated IgG and positive ANA (this is most common autoantibody present in autoimmune hepatitis, but there are other multiple possible autoantibodies), and inflammatory changes on biopsy. Initial treatment is either prednisone monotherapy or combined therapy with azathioprine. Often azathioprine is added to prednisone monotherapy as the dose of steroid is tapered. More steroids would not necessarily be helpful. Tenofovir is a treatment for hepatitis B virus. Cefotaxime is used for SBP prophylaxis.
A 62-year-old man with history of ethanol use and hepatic cirrhosis presents with upper gastrointestinal bleeding. Endoscopy reveals multiple varices with signs of recent bleeding. Bands are placed on the varices and the patient is started on an octreotide infusion. His hemoglobin responds well to the single unit of packed red blood cells he was given before endoscopy. Thirty hours later large amount of blood is suctioned out of his nasogastric tube, he becomes hypotensive, and his hemoglobin does not increase despite transfusion of packed red blood cells.
What is the MOST effective additional treatment option?
Correct Answer: D
Elevated portal pressure is the primary reason for variceal bleeds. Transjugular intrahepatic portosystemic shunt (TIPS) allows for decompression of the elevated portal venous system pressures. This can stop bleeding in up to 90% of cases of variceal hemorrhage and is a commonly recommended second line treatment for variceal hemorrhage.
A 52-year-old female presents with jaundice and right upper quadrant pain. Obstruction of her hepatic vein was found on ultrasonography. After anticoagulation and interventional angioplasty her aminotransferases and bilirubin remain elevated and she is now developing ascites.
Which treatment option should be considered next?
Correct Answer: A
Budd–Chiari syndrome is caused by hepatic venous outflow obstruction. The majority of patients have an underlying disorder putting them at risk for thrombosis. The venous outflow obstruction causes ischemia to the hepatocytes and increases liver sinusoidal pressure causing liver congestion and portal hypertension. TIPS may increase survival in patients refractory to anticoagulation or angioplasty/stenting from around 45% to around 70%. Liver transplantation may still be required, but TIPS would generally be attempted prior. Increasing the anticoagulation will likely only increase the risk of bleeding. Placement of an inferior vena cava filter can help prevent clots from transmitting to the pulmonary vasculature but will not affect the hepatic vein. Paracentesis may relieve complications directly related to ascites but will not treat the underlying cause.
A patient with no history of chronic liver disease is in the ICU with acute fulminant hepatic failure. Their family is asking you if they will survive. You tell them they are very ill and that based on their liver failure alone the chances they will still be alive in three weeks is around:
Although there are many variables that go into predicting survival/mortality for any individual patient, patients with acute liver failure overall have around a 70% survival rate at 21 days.
Which of the following is NOT a risk factor for the development of significant cerebral edema in the setting of liver failure?
Correct Answer: B
Elevated intracranial pressures related to cerebral edema in 20% or more of patients with acute liver failure. Risk factors associated with developing significant cerebral edema in the setting of liver failure are hyperacute presentation, younger individuals (<35 years), high-grade HE, serum ammonia >150 µmol/L, presence of systemic inflammatory response syndrome or concurrent infection, high Sequential Organ Failure Assessment score, and requirement for vasopressors or renal replacement therapy. In addition, older individuals are protected against development of intracranial hypertension with cerebral edema due to reduction in cerebral volume with age.
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