QUESTION: 5

A24-year-old male medical student is admitted to the hospital for the evaluation of a 3-month history of bloody stools. The patient has approximately six blood stained or blood streaked stools per day, associated with relatively little, if any, pain. He has not had any weight loss, and he has been able to attend classes without interruption. He denies any fecal incontinence. He has no prior medical history. Review of systems is remarkable only for occasional fevers and the fact that the patient quit smoking approximately 8 months ago. A colonoscopy is performed and reveals a granular, friable colonic mucosal surface with loss of normal vascular pattern from the anal verge to the hepatic flexure of the colon. Biopsies reveal prominent neutrophils in the epithelium and cryptitis with focal crypt abscesses, and no dysplasia. The patient is diagnosed with ulcerative colitis. Which of the following is the best initial treatment for this patient?

colectomy
oral prednisone
oral metronidazole
cortisone enemas
intravenous cyclosporine

Answer(s): B

Explanation:

Oral corticosteroids are a mainstay of firstline treatment for moderate-to-severe ulcerative colitis. Starting doses of 40 mg PO daily of prednisone, with a slow taper, are often effective in reducing colonic inflammation, although some patients are unable to wean steroids or maintain remission once achieved. The patient does not have dysplasia in any biopsy specimens, nor does he have signs of systemic toxicity, so a colectomy would be premature. Oral metronidazole is ineffective in ulcerative colitis. Cortisone enemas would be helpful if the patient had isolated left-sided disease, but it is doubtful that enema therapy would reach his hepatic flexure. Intravenous cyclosporine would be used in severe colitis as a last measure before colectomy but this patient is not yet sick enough to warrant such therapy. PSC occurs in approximately 3% of patients with ulcerative colitis and is its major liver complication. It is a chronic inflammatory condition of the biliary tree. It can typically manifest with elevated alkaline phosphatase and bilirubin levels, and results in diffuse stricturing and pruning of the biliary tree. Wilson disease, hereditary hemochromatosis and alpha-1 antitrypsin deficiency are not associated with ulcerative colitis and are not cholestatic liver diseases. Primary biliary cirrhosis could account for these laboratory findings, but is rare in both males and patients with ulcerative colitis. Patients with PSC are at increased risk of developing cholangiocarcinoma but not the other liver tumors mentioned. Patients with celiac sprue are at increased risk for small bowel cancers (adenocarcinoma, lymphoma). Patients with FAP are at increased risk to develop desmoid tumors.

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QUESTION: 6

A24-year-old male medical student is admitted to the hospital for the evaluation of a 3-month history of bloody stools. The patient has approximately six blood stained or blood streaked stools per day, associated with relatively little, if any, pain. He has not had any weight loss, and he has been able to attend classes without interruption. He denies any fecal incontinence. He has no prior medical history. Review of systems is remarkable only for occasional fevers and the fact that the patient quit smoking approximately 8 months ago. A colonoscopy is performed and reveals a granular, friable colonic mucosal surface with loss of normal vascular pattern from the anal verge to the hepatic flexure of the colon. Biopsies reveal prominent neutrophils in the epithelium and cryptitis with focal crypt abscesses, and no dysplasia. The patient is diagnosed with ulcerative colitis.

While on the inpatient service, the patient is noted to have a serum alkaline phosphatase of 380 U/L and a bilirubin of 2.4 mg/dL. An ERCP is performed, and the following cholangiogram is obtained. In addition to ulcerative colitis, the patient likely has what other illness?

primary biliary cirrhosis
Wilson disease
alpha-1 antitrypsin deficiency
hereditary hemochromatosis
primary sclerosing cholangitis (PSC)

Answer(s): E

Explanation:

Oral corticosteroids are a mainstay of firstline treatment for moderate-to-severe ulcerative colitis. Starting doses of 40 mg PO daily of prednisone, with a slow taper, are often effective in reducing colonic inflammation, although some patients are unable to wean steroids or maintain remission once achieved. The patient does not have dysplasia in any biopsy specimens, nor does he have signs of systemic toxicity, so a colectomy would be premature. Oral metronidazole is ineffective in ulcerative colitis. Cortisone enemas would be helpful if the patient had isolated left-sided disease, but it is doubtful that enema therapy would reach his hepatic flexure. Intravenous cyclosporine would be used in severe colitis as a last measure before colectomy but this patient is not yet sick enough to warrant such therapy. PSC occurs in approximately 3% of patients with ulcerative colitis and is its major liver complication. It is a chronic inflammatory condition of the biliary tree. It can typically manifest with elevated alkaline phosphatase and bilirubin levels, and results in diffuse stricturing and pruning of the biliary tree. Wilson disease, hereditary hemochromatosis and alpha-1 antitrypsin deficiency are not associated with ulcerative colitis and are not cholestatic liver diseases. Primary biliary cirrhosis could account for these laboratory findings, but is rare in both males and patients with ulcerative colitis. Patients with PSC are at increased risk of developing cholangiocarcinoma but not the other liver tumors mentioned. Patients with celiac sprue are at increased risk for small bowel cancers (adenocarcinoma, lymphoma). Patients with FAP are at increased risk to develop desmoid tumors.

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QUESTION: 7

A24-year-old male medical student is admitted to the hospital for the evaluation of a 3-month history of bloody stools. The patient has approximately six blood stained or blood streaked stools per day, associated with relatively little, if any, pain. He has not had any weight loss, and he has been able to attend classes without interruption. He denies any fecal incontinence. He has no prior medical history. Review of systems is remarkable only for occasional fevers and the fact that the patient quit smoking approximately 8 months ago. A colonoscopy is performed and reveals a granular, friable colonic mucosal surface with loss of normal vascular pattern from the anal verge to the hepatic flexure of the colon. Biopsies reveal prominent neutrophils in the epithelium and cryptitis with focal crypt abscesses, and no dysplasia. The patient is diagnosed with ulcerative colitis.

In addition to an increased lifetime risk of colon cancer, the patient is also at increased risk for which of the following tumors?

hepatocellular carcinoma
hepatoblastoma
desmoid tumors
small bowel lymphoma
cholangiocarcinoma

Answer(s): E

Explanation:

Oral corticosteroids are a mainstay of firstline treatment for moderate-to-severe ulcerative colitis. Starting doses of 40 mg PO daily of prednisone, with a slow taper, are often effective in reducing colonic inflammation, although some patients are unable to wean steroids or maintain remission once achieved. The patient does not have dysplasia in any biopsy specimens, nor does he have signs of systemic toxicity, so a colectomy would be premature. Oral metronidazole is ineffective in ulcerative colitis. Cortisone enemas would be helpful if the patient had isolated left-sided disease, but it is doubtful that enema therapy would reach his hepatic flexure. Intravenous cyclosporine would be used in severe colitis as a last measure before colectomy but this patient is not yet sick enough to warrant such therapy. PSC occurs in approximately 3% of patients with ulcerative colitis and is its major liver complication. It is a chronic inflammatory condition of the biliary tree. It can typically manifest with elevated alkaline phosphatase and bilirubin levels, and results in diffuse stricturing and pruning of the biliary tree. Wilson disease, hereditary hemochromatosis and alpha-1 antitrypsin deficiency are not associated with ulcerative colitis and are not cholestatic liver diseases. Primary biliary cirrhosis could account for these laboratory findings, but is rare in both males and patients with ulcerative colitis. Patients with PSC are at increased risk of developing cholangiocarcinoma but not the other liver tumors mentioned. Patients with celiac sprue are at increased risk for small bowel cancers (adenocarcinoma, lymphoma). Patients with FAP are at increased risk to develop desmoid tumors.

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QUESTION: 8

A61-year-old man comes to your office for a checkup. He currently feels well and has no focal complaints. He has a past medical history significant for wellcontrolled hypertension, and his gallbladder was removed 3 years ago in the setting of acute cholecystitis. He does not smoke and drinks one to two alcoholic beverages per day. Family history is remarkable for colon cancer in his mother at age 45 and a brother at age 49. He has a sister who developed endometrial cancer at age 53. He has never undergone colon cancer screening and is interested in pursuing this. The patient's family history is strongly suggestive of which of the following?

familial adenomatous polyposis (FAP) syndrome
hereditary nonpolyposis colorectal cancer (HNPCC) syndrome
Peutz-Jeghers syndrome
Cronkhite-Canada syndrome
Turcot syndrome

Answer(s): B

Explanation:

The patient should undergo screening colonoscopy, especially with his strongly positive family history of first-degree relatives developing colon cancer before age 50. Colonoscopy is the only test that can directly evaluate the entire colon and rectum. Most polyps can be removed completely at colonoscopy, and large lesions or masses can be directly biopsied. Virtual colonoscopy and barium enema combined with flexible sigmoidoscopy are good tests, but any positive findings on either of these tests would warrant further examination with colonoscopy. Barium enema alone is insufficient for screening. Fecal occult blood testing is helpful as a screening tool, but would be inadequate alone in this patient given his family history. The patient satisfies criteria for HNPCC, a syndrome seen in patients with germline mutations in DNA mismatch repair (MMR) genes. He has three first-degree relatives with cancer of the colorectum, endometrium, small bowel, ureter, or renal pelvis (all of whom are first-degree relatives of each other). The colorectal cancers involve at least two generations and at least one case was diagnosed before age
50. FAP involves a mutation of the APC gene and results in dense colonic polyposis, mandibular osteomas, and universal colon cancer at a young age unless colectomy is performed. Peutz Jeghers syndrome results in hamartomatous polyps of the gut as well as mucocutaneous pigmentation changes. Cronkhite-Canada syndrome manifests as GI polyposis, alopecia, cutaneous hyperpigmentation, malnutrition, and dystrophic fingernails. Turcot syndrome is a variant of FAP in which patients can also develop medulloblastoma, glioblastoma multiforme, and hypertrophy of retinal pigmented epithelium.

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