The Correct Answer is C. Colon carcinoma in a patient with PSC and UC

The correct Answer is E. None of the above

Although all the features are present in PBC, these are not pathognomic of the condition as they are seen in other diseases of the liver as well.

Liver findings in Primary Biliary Cirrhosis

• Granulomas
• Increased expression of class I MHC on bile duct epithelial cells
• Aberrant expression of class II MHC on bile duct epithelial cells
• T cell infiltrate around the bile ducts – CTL may be early event, but more usual to see CD4+ T cells seen around the bile ducts.
• Excess IgM producing B cells around the bile ducts.
• Features have been said to resemble either (i) graft - versus - host disease; or (ii) transplant rejection.
• (Accumulation of excess copper)

The Correct Answer is A. Usually occurs in children, adolescents, and young adults

• The fibrolamellar variant of HCC almost always (in approximately 90% of reported cases) occurs in livers without cirrhosis, although 15-20% of the nonfibrolamellar HCCs have also been observed in noncirrhotic livers.
• Metastatic disease and typical HCC tend to occur predominantly in older patients, and benign lesions, such as adenoma, focal nodular hyperplasia, and fibrolamellar HCC, tend to occur in younger patients.
• No association with HBV or cirrhosis
• Better prognosis

The Correct Answer is F. Retroperitoneal fibrosis


Nodular Regenerative Hyperplasia

• A diffuse lesion with tons of nodules
• Grossly, it looks like cirrhosis, but there is no fibrosis
• Less common than cirrhosis
• It is probably related to focal nodular hyperplasia.
• Portal hypertension may develop with the nodular regenerative hyperplasia because it is diffuse, UNLIKE focal nodular hyperplasia.

The Correct Answer is C. Gut peptides are synthesized as large precursor molecules that undergo a series of post translational processing steps which result in the formation of biologically active hormone

The Correct Answer is B. The extracellular components of the receptor are critical in determining ligand recognition

The Correct Answer is D. Diarrhea occurs commonly in Zollinger-Ellison patients and is due to multiple factors including gastric acid hypersecretion and mild steatorrhea

Zollinger Ellison (hyper-acidity which overwhelms neutralization of the duodenal contents; therefore pancreatic lipase is inactive leading to steatorrhea and diarrhea)

The Correct Answer is A. Adrenal insufficiency

Hypergastrinemia can result from a variety of causes. Hypergastrinemia that results from administration of antisecretory agents is an appropriate response caused by loss of feedback inhibition of gastrin release by luminal acid. Lack of acid causes a reduction in somatostatin release, which in turn causes increased release of gastrin from antral G cells. Hypergastrinemia can also occur in the setting of pernicious anemia or uremia or following surgical procedures such as vagotomy or retained gastric antrum after gastrectomy. In contrast, gastrin levels increase inappropriately in patients with gastrinoma (Zollinger-Ellison syndrome). These gastrin-secreting tumors are not located in the antrum and secrete gastrin autonomously. The clinical triad of Zollinger-Ellison syndrome is the finding of gastric acid hypersecretion, severe peptic ulcer disease, and a non-beta islet cell tumor of the pancreas .

The correct Answer is B. False

The Correct Answer is A. Prednisone

The Correct Answer is C. Serum Amylase > 3x the upper limit of normal


Predictors of Severity of Acute Pancreatitis

• Clinical signs: peritonitis, shock, respiratory distress
• Ranson’s signs: 11 signs that include prognostic significance during the 1st 48 hours. A higher score means more severe disease.
• APACHE-II: sensitive and specific, can be calculated on admission

Ranson’s Criteria

On Admission

• Age > 55 years
• WBC > 16,000/mm³
• Blood Glucose > 200 mg/dl
• LDH > 350 IU/ L
• AST > 250 U/L

During the first 48 Hours

• Fall in Heamatocrit > 10%
• Serum Calcium <>
• Base deficit > 4m Eq/L
• Fluid Sequestration > 6 L
• BUN rises > 5mg/dl
• PaO₂ <>

Risk of morbidity and mortality depends on patient’s score at 28 Hours

• Number of signs 0-2 3-4 5-6 7-8
• Percent mortality 0.9 16 40 100
• Percent morbidity 3.7 40 93 100
• > 7Days in the ICU

The correct answer is E. Tobramycin

• Acute pancreatitis may be clinically mild or severe.
• Severe acute pancreatitis is usually a result of pancreatic glandular necrosis.
• The morbidity and mortality associated with acute pancreatitis are substantially higher when necrosis is present
• has a rapid onset manifested by upper abdominal pain, vomiting, fever,tachycardia, leukocytosis, and elevated serum levelsof pancreatic enzymes
• Gallstones and alcohol abuse are the most common causes in the United States.
• Ranson's score is based on 11 clinical signs with prognostic importance; 5 are measured at the time of admission and the other 6 in the first 48 hours after admission
• Necrosis is present in approximately 20 to 30 percent of acute pancreatitis per year in the United States.
• In a recent prospective trial: the incidence of gram-negative pancreatic infection and late mortality were significantly reduced in patients with necrotizing pancreatitis who were treated with selective gut decontamination.
• At the present time, intravenous administration of imipenem-cilastatin is recommended. ,continue for at least two to four weeks.
• Selection of appropriate agent should be made based on ability to penetrate the pancreas. Third generation cephs, pipericillin, metronidazole, imipenem/cilistatin, and certain quinolones.

The Correct Answer is D


Diagnosis of chronic pancreatitis

• no true gold standard exists, unlike with the liver, where biopsies are less risky and hence are the gold standard
• it is true that pancreatic biopsy is not seldom performed, but it is not often indicated in chronic pancreatitis—it is more commonly performed on pancreatic masses, etc.
• imaging studies: pancreatic calcification is a good marker of chronic rather than acute pancreatitis
• secretin stimulation: decreased duodenal bicarbonate secretion. doesn’t work perfectly
• fecal fat > 7 gm / 24 hr

Calcifications in chronic pancreatitis

• calcifications are white
• dilated pancreatic duct is also seen

• dilated pancreatic duct with side branches are visualized
• normally, the duct is thin and wispy

The correct answer is D

• an inflammatory process centered around the large pancreatic ducts
• presents as a pancreatic mass or diffuse swelling that mimics pancreatic cancer
• can also cause biliary strictures, mimicking PSC (primary sclerosing cholangitis)
• may affect other organs such as the lung or kidney
• imaging: on CT, pancreas is diffusely, abnormally enlarged
• microscopically, there are numerous plasma cells and lymphocytes around pancreatic ducts
• hallmark feature: IgG subtype 4 is elevated
• imaging: on ERCP, very narrowed pancreatic duct
• this is clinically important because treatment is simply medical (steroids) and the disease can be confused with cancer

Answer B.

Genetic testing for mutation of the APC gene is the screening test of choice in these patient. If genetic testing cannot be done, answer C would be the screening regimen.

Answer A.


A slit-lamp examination since the combination of liver and neuropsychiatric abnormalities suggest Wilson's disease. Although the ceruloplasmin may be within the normal range in as many as 5-25% of patients with Wilson's disease (more frequently so in younger patients), Kayser-Fleischer rings are usually present in those with established neuropsychiatric disease. Measurement of urinary copper would also be appropriate. Liver biopsy is also an acceptable answer if it was combined with quantitative hepatic copper concentration measurement.