USMLE USMLE-STEP-3 - Questions & Answers

Although this is the first time that your patient has been noted to have an elevated blood pressure reading, given his family history and obesity, it is important to consider the coexistence of other cardiovascular risk factors. His evaluation should include, among other things, screening for DM and dyslipidemia along with an ECG. It is reasonable to ask the patient to submit himself to a strict diet (low in fat and salt) and to increase his exercise and activity, since these lifestyle modifications will likely result in weight loss, decreased blood pressure, and improve his risk profile for cardiovascular disease. Nonetheless, it is rarely enough to normalize blood pressure in all but the earliest stages of hypertension.
Provided that no other comorbidities exist, the patient should return to clinic in no more than 2 months for a repeat blood pressure check. There is no need to consider secondary causes of hypertension, given his age and presentation.

You should not start antihypertensive medications until further evaluation is completed, and a second elevated reading confirms your diagnosis of hypertension. In the initial evaluation of hypertension (as per the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure [JNC-7], 2003), it is important to evaluate the patient for end-organ damage. This should include the heart, kidneys, eyes, and nervous system.
It is recommended to obtain a urinalysis to assess for proteinuria, glucosuria, or hematuria; to obtain an ECG to evaluate the heart for potential hypertrophy or early signs of cardiovascular disease; to obtain a fasting lipid profile, particularly after the age of 35, to assess the cardiovascular risk profile; and to check the patient's renal function to assess for damage or dysfunction. Thyroid function tests are only indicated in the workup of secondary causes of hypertension. According
to the JNC-7, this patient's blood pressure falls into the stage 2 hypertension classification in which either systolic blood pressure (SBP) is at least 160 mmHg or diastolic blood pressure (DBP) is at least 100 mmHg.

Stage 1 hypertension is characterized by a SBP of 140159 mmHg and a DBP of 9099 mmHg. Prehypertension is characterized by a SBP of 120139 mmHg and a DBP of 8089 mmHg. Normal blood pressure is characterized by a SBP of less than 120 mmHg and a DBP of less than 80 mmHg. In classifying a patient's blood pressure and determining appropriate therapy, the higher of the two categories corresponding to the SBP and DBP is the one that is used. Per JNC-7 guidelines, treatment of stage 2 hypertension should involve the consideration of a two-drug regimen initially. The goal blood pressure in patients with diabetes is a SBP less than 130 mmHg and a DBP less than 80 mmHg. An ACE inhibitor should be used as the drug class has been shown to slow the progression of diabetic nephropathy and reduce albuminuria. Thiazide diuretics, betablockers, and calcium channel blockers are appropriate choices to consider in this patient in addition to an ACE inhibitor.

Although this is the first time that your patient has been noted to have an elevated blood pressure reading, given his family history and obesity, it is important to consider the coexistence of other cardiovascular risk factors. His evaluation should include, among other things, screening for DM and dyslipidemia along with an ECG. It is reasonable to ask the patient to submit himself to a strict diet (low in fat and salt) and to increase his exercise and activity, since these lifestyle modifications will likely result in weight loss, decreased blood pressure, and improve his risk profile for cardiovascular disease. Nonetheless, it is rarely enough to normalize blood pressure in all but the earliest stages of hypertension.
Provided that no other comorbidities exist, the patient should return to clinic in no more than 2 months for a repeat blood pressure check. There is no need to consider secondary causes of hypertension, given his age and presentation.

You should not start antihypertensive medications until further evaluation is completed, and a second elevated reading confirms your diagnosis of hypertension. In the initial evaluation of hypertension (as per the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure [JNC-7], 2003), it is important to evaluate the patient for end-organ damage. This should include the heart, kidneys, eyes, and nervous system.
It is recommended to obtain a urinalysis to assess for proteinuria, glucosuria, or hematuria; to obtain an ECG to evaluate the heart for potential hypertrophy or early signs of cardiovascular disease; to obtain a fasting lipid profile, particularly after the age of 35, to assess the cardiovascular risk profile; and to check the patient's renal function to assess for damage or dysfunction. Thyroid function tests are only indicated in the workup of secondary causes of hypertension. According
to the JNC-7, this patient's blood pressure falls into the stage 2 hypertension classification in which either systolic blood pressure (SBP) is at least 160 mmHg or diastolic blood pressure (DBP) is at least 100 mmHg.

Stage 1 hypertension is characterized by a SBP of 140159 mmHg and a DBP of 9099 mmHg. Prehypertension is characterized by a SBP of 120139 mmHg and a DBP of 8089 mmHg. Normal blood pressure is characterized by a SBP of less than 120 mmHg and a DBP of less than 80 mmHg. In classifying a patient's blood pressure and determining appropriate therapy, the higher of the two categories corresponding to the SBP and DBP is the one that is used. Per JNC-7 guidelines, treatment of stage 2 hypertension should involve the consideration of a two-drug regimen initially. The goal blood pressure in patients with diabetes is a SBP less than 130 mmHg and a DBP less than 80 mmHg. An ACE inhibitor should be used as the drug class has been shown to slow the progression of diabetic nephropathy and reduce albuminuria. Thiazide diuretics, betablockers, and calcium channel blockers are appropriate choices to consider in this patient in addition to an ACE inhibitor.

The patient has chronic diarrhea superimposed on a long history of loose stools, steatorrhea, and significant weight loss. While these features could be seen in several diseases, the presence of the pruritic vesiculopapular lesions on his extensor surfaces makes the diagnosis highly likely to be celiac sprue, with its frequently accompanying skin manifestation dermatitis herpetiformis. Crohn's disease is not usually associated with steatorrhea, and ulcerative colitis is often associated with bloody stools. Chronic pancreatitis and Whipple disease could cause a similar clinical picture but would not have the associated skin findings. A small bowel biopsy would confirm histopathologic features consistent with celiac sprue, such as villous atrophy and crypt hyperplasia. A small bowel biopsy could also diagnose or rule out Whipple disease by looking for the pathognomonic PAS (periodic acid-Schiff) positive organism Tropheryma whippelii. Colonic biopsies would be unhelpful in celiac sprue. A fecal fat quantification would likely confirm and assess the degree of steatorrhea, but would offer little other diagnostic information. A small bowel x-ray is too nonspecific to confirm the diagnosis and an abdominal CT scan would likely be normal unless the patient had developed a complication of advanced sprue, such as intestinal lymphoma. Patients with celiac sprue are at increased risk for malignancies of the small bowel with adenocarcinoma and lymphoma being the two most commonly encountered. Patients with celiac sprue are not at greatly increased risk of the other malignancies listed. Limited data suggest that strict adherence to a glutenfree diet may decrease the incidence of malignancy in these patients.

The patient has chronic diarrhea superimposed on a long history of loose stools, steatorrhea, and significant weight loss. While these features could be seen in several diseases, the presence of the pruritic vesiculopapular lesions on his extensor surfaces makes the diagnosis highly likely to be celiac sprue, with its frequently accompanying skin manifestation dermatitis herpetiformis. Crohn's disease is not usually associated with steatorrhea, and ulcerative colitis is often associated with bloody stools. Chronic pancreatitis and Whipple disease could cause a similar clinical picture but would not have the associated skin findings. A small bowel biopsy would confirm histopathologic features consistent with celiac sprue, such as villous atrophy and crypt hyperplasia. A small bowel biopsy could also diagnose or rule out Whipple disease by looking for the pathognomonic PAS (periodic acid-Schiff) positive organism Tropheryma whippelii. Colonic biopsies would be unhelpful in celiac sprue. A fecal fat quantification would likely confirm and assess the degree of steatorrhea, but would offer little other diagnostic information. A small bowel x-ray is too nonspecific to confirm the diagnosis and an abdominal CT scan would likely be normal unless the patient had developed a complication of advanced sprue, such as intestinal lymphoma. Patients with celiac sprue are at increased risk for malignancies of the small bowel with adenocarcinoma and lymphoma being the two most commonly encountered. Patients with celiac sprue are not at greatly increased risk of the other malignancies listed. Limited data suggest that strict adherence to a glutenfree diet may decrease the incidence of malignancy in these patients.

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.

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.

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.

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.

The patient has a peptic stricture, seen in the setting of long-standing untreated gastroesophageal reflux with esophagitis. The history of progressive solid food dysphagia without weight loss is typical. Tobacco, alcohol, thiazide diuretics, and spicy foods do not predispose to benign esophageal strictures. The patient has developed a peptic stricture, a serious complication of GERD. The patient needs esophageal dilation (either with mechanical or pneumatic dilators) and maximal acid suppression. PPI therapy is superior to histamine receptor antagonist therapy in terms of healing erosive esophagitis. Patients with long-standing GERD are at increased risk of developing Barrett esophagus, a risk factor for esophageal adenocarcinoma. GERD is not a risk factor for esophageal squamous cell cancer, gastric cancer, or duodenal cancer. Patients with chronic H. pylori infection (which this patient did not have) are at increased risk for a form of gastric lymphoma known as a MALT-oma.

The patient has a peptic stricture, seen in the setting of long-standing untreated gastroesophageal reflux with esophagitis. The history of progressive solid food dysphagia without weight loss is typical. Tobacco, alcohol, thiazide diuretics, and spicy foods do not predispose to benign esophageal strictures. The patient has developed a peptic stricture, a serious complication of GERD. The patient needs esophageal dilation (either with mechanical or pneumatic dilators) and maximal acid suppression. PPI therapy is superior to histamine receptor antagonist therapy in terms of healing erosive esophagitis. Patients with long-standing GERD are at increased risk of developing Barrett esophagus, a risk factor for esophageal adenocarcinoma. GERD is not a risk factor for esophageal squamous cell cancer, gastric cancer, or duodenal cancer. Patients with chronic H. pylori infection (which this patient did not have) are at increased risk for a form of gastric lymphoma known as a MALT-oma.