Question 1

A 65-year-old man on chronic peritoneal dialysis (PD) for two years complains of 1 day of diffuse abdominal pain.  He has not noticed any changes in his catheter exit site, changes in bowel movements, fever, or chills.
His temperature is 37.2 C (99.0 F), blood pressure is 140/90 mm Hg, and pulse is 90/min.  Bowel sounds are normal.  There is mild, diffuse abdominal tenderness without definite rebound tenderness.  The catheter exit site looks normal.
Analysis of peritoneal fluid reveals a white blood cell count of 110 cells/µL.  Gram stain of the peritoneal fluid is negative.
Laboratory results are as follows:

Which of the following would be the most appropriate next step in management?

Accepted Answer

11ec193a_97f7_3276_853d_8b8faf27ce8e_MD0002_00 This patient's presentation is concerning for PD-related peritonitis, most commonly due to touch-contamination from skin bacteria or catheter-related infection. Patients usually present with abdominal pain, low grade fever (in up to 50% of patients), and nausea. Examination typically reveals abdominal tenderness, sometimes with cloudy abdominal fluid and rebound tenderness. Diagnosis is highly suggested by peritoneal fluid showing > 100 WBCs. However, fluid with > 50% neutrophils strongly favors PD-related peritonitis regardless of the absolute fluid WBC count. Gram stain is usually negative and diagnosis is confirmed by positive peritoneal fluid cultures (80-95% of cases). Although Gram-positive skin bacteria is the most common cause of PD peritonitis, patients should be treated empirically for both Gram-positive and Gram-negative organisms. Current guidelines recommend intraperitoneal vancomycin and a 3^rd or 4^th generation cephalosporin (e.g., cefepime) or an aminoglycoside. Intraperitoneal antibiotics appear to be more efficacious than intravenous antibiotics (Choice E). ^rd (Choice A) Abdominal CT scan is indicated in patients with suspected secondary peritonitis due to enteric pathology (e.g., appendicitis, incarcerated hernia, ruptured diverticula). However, secondary peritonitis tends to present with more systemic symptoms (e.g., fever, chills, hypotension, significant leukocytosis), which are usually not present in PD-related peritonitis. If a CT scan is performed, it is important to recall that PD patients can have asymptomatic free peritoneal air from the PD catheter. (Choice B) Colonoscopy is not usually indicated in PD peritonitis. (Choice C) Catheter removal is considered only for tunnel infections or refractory intraperitoneal infections. However, this patient's normal appearing catheter site makes this less likely. Educational objective: Peritoneal dialysis-associated peritonitis usually presents with abdominal pain and nausea. Fever and leukocytosis may not be present. Diagnosis is suggested by peritoneal fluid showing > 100 WBCs or > 50% neutrophils (regardless of fluid WBC count), and is confirmed with positive peritoneal fluid cultures. __________ References: Treatment for peritoneal dialysis-associated peritonitis. (http://www.ncbi.nlm.nih.gov/pubmed/18254075) Microbiological profile of peritoneal dialysis peritonitis and predictors of hospitalization. (http://www.ncbi.nlm.nih.gov/pubmed/22073826)

Question 2

A 57-year-old man is evaluated for a 6-month history of persistent diarrhea.  The patient describes frequent soft to watery bowel movements that sometimes awaken him from sleep.  He has had fecal incontinence on several occasions along with abdominal bloating and excessive flatulence but has had no fever, abdominal or rectal pain, tenesmus, melena, hematochezia, or weight loss.  Medical history includes hypertension, peripheral vascular disease, and a 40-year history of diabetes mellitus treated with insulin.  The patient has not traveled recently.
Temperature is 36.9 C (98.4 F), blood pressure is 136/84 mm Hg supine and 122/77 mm Hg standing, and pulse is 72/min.  The lungs are clear on auscultation and heart sounds are normal.  The abdomen is nondistended, soft, and nontender with no palpable mass or hepatosplenomegaly.  Bowel sounds are normal.  Stool is negative for occult blood.  Light touch and vibratory sensation are decreased on both feet, and bilateral ankle reflexes are absent.
Laboratory results are as follows:
 Stool is negative for ova and parasites, and stool culture grows no pathogenic bacteria.  Colonoscopy reveals grossly normal mucosa; random biopsies are unrevealing.  Small bowel follow-through barium studies and upper gastrointestinal endoscopy with random biopsies are normal.
Which of the following is most likely contributing to this patient's condition?

Accepted Answer

11ec193a_97f8_6afa_853d_a58e93767fff_MD0002_00 This patient has symptoms consistent with diabetic diarrhea; a painless, watery diarrhea that can occur at night and is often associated with fecal incontinence. Patients may have periods of normal bowel movements and/or constipation. The etiology is likely multifactorial but is thought to be largely due to autonomic neuropathy leading to small bowel and colonic hypomotility, increased intestinal fluid secretion, and anorectal dysfunction. Diabetic diarrhea is a diagnosis of exclusion; workup includes stool studies, serum chemistries and endocrine studies, and endoscopic biopsy. Small intestinal bacterial overgrowth (SIBO) commonly contributes to diabetic diarrhea as gastrointestinal hypomotility allows for excessive colonization. This leads to abdominal discomfort, bloating, and flatulence; severe disease can cause malabsorption with steatorrhea and vitamin deficiency. Patients with bacterial overgrowth often have secondary lactose intolerance as well. The diagnosis of SIBO can be confirmed with a carbohydrate breath test and treated with antibiotics (eg, rifaximin); endoscopic and histopathologic studies are often nonrevealing. Patients with diabetic diarrhea in the absence of SIBO are often treated with antimotility agents (eg, loperamide). (Choices B and D) Celiac disease is common in diabetic patients due to the similar genetic predisposition. Microscopic colitis may also occur with increased frequency in patients with diabetes. Both diseases cause diarrhea; however, this patient's normal small bowel biopsy essentially rules out these disorders. (Choice C) Irritable bowel syndrome (IBS) can present with alternating diarrhea and constipation. However, the presence of nocturnal diarrhea suggests an organic cause rather than IBS. (Choice E) Pancreatic insufficiency is a rare cause of diarrhea/malabsorption in the diabetic population. It leads to steatorrhea but not watery stools. Educational objective: Autonomic dysfunction in longstanding diabetes mellitus can lead to gastrointestinal dysmotility. Diabetic diarrhea is characterized by painless, watery diarrhea, fecal incontinence, and/or alternating normal bowel movements and constipation. Small intestinal bacterial overgrowth is a common complication and may contribute to symptoms. __________ References: Diabetic diarrhea. (http://www.ncbi.nlm.nih.gov/pubmed/19765362)

Question 3

A 32-year-old man comes to the office due to malaise and fatigue over the last 3 months.  The patient also reports mild generalized abdominal discomfort and nausea.  He has lost approximately 2.3 kg (5 lb) in the last month.  The patient has had no fever, vomiting, diarrhea, back pain, or urinary symptoms.  He has no medical issues and takes no medication.  The patient is a lifetime nonsmoker.  Family history is notable for liver cancer in his father.
Physical examination shows right upper quadrant tenderness.  The liver edge is soft and palpable 2 cm below the right costal margin.  Rectal examination shows no abnormalities, and a stool occult blood test is negative.
Laboratory results are as follows:
 A hepatitis panel shows immunity against the hepatitis B virus.  Ultrasonography shows no gallstones and no common bile duct dilation.  Liver biopsy reveals intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis.
Which of the following is the best next step in management of this patient?

Accepted Answer

11ec193a_97f4_4c3e_853d_134198ef2921_MD0002_00 This patient's presentation is consistent with cholestasis due to primary sclerosing cholangitis (PSC), which is associated with underlying inflammatory bowel disease (IBD) (usually ulcerative colitis) in nearly 90% of patients. PSC is a chronic progressive disorder of unknown etiology with inflammation, fibrosis, and stricturing, most commonly of medium and large biliary ducts. Patients are usually asymptomatic but can present with fatigue, pruritus, and a cholestatic pattern of elevations in liver function tests. Continued bile duct destruction leads to end-stage liver disease and portal hypertension. Ultrasound is usually nondiagnostic; a cholangiogram (either endoscopic or magnetic resonance) may show multifocal narrowing and dilation of both intrahepatic and extrahepatic bile ducts. Liver biopsy is helpful for disease staging and prognosis, and can reveal intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis. PSC patients with IBD are at significantly increased risk of colorectal cancer. Current guidelines recommend colonoscopy with full biopsies in newly diagnosed patients with PSC regardless of the presence of other symptoms. Patients found to have IBD require yearly follow-up with colonoscopy; those with PSC but without IBD should have colonoscopy every 5 years. Liver transplantation is the preferred treatment for PSC as medical therapy (eg, cyclosporine) does not appear to slow progression of the disease (Choice B). (Choice C) Although glucocorticoids are an important treatment for IgG4-related cholangitis, they have not been shown to improve survival in PSC and can worsen bone loss. (Choice D) Patients with PSC are also at increased risk for cholangiocarcinoma; some authorities have advocated for annual monitoring of cancer antigen 19-9 levels for screening. However, the evidence in support of this practice is not conclusive. (Choice E) Ursodeoxycholic acid is a mainstay of treatment for primary biliary cholangitis (PBC), which can present similarly to PSC. However, PBC usually affects women (90% of cases), and biopsy shows mononuclear cell-mediated destruction and/or periductal granulomatous inflammation of intrahepatic bile ducts. Ursodeoxycholic acid does not have a clear role in management of PSC. Education objective: Primary sclerosing cholangitis presents with cholestasis and can reveal intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis. Most patients have comorbid inflammatory bowel disease (IBD) (usually ulcerative colitis) and a significantly increased risk of colorectal cancer. Colonoscopy is recommended in all patients to diagnose underlying IBD and for colorectal cancer surveillance. __________ References: Primary sclerosing cholangitis. (http://www.ncbi.nlm.nih.gov/pubmed/27653566)

Question 4

A 38-year-old woman comes to the office due to constipation for the last 3-4 months.  She has not had previous problems with bowel movements; however, she now spends 20-25 minutes having a bowel movement once every 3-4 days.  The patient reports excessive straining; hard, pebble-like stools; incomplete evacuation; and intermittent digital disimpaction.  She also has abdominal discomfort, bloating, and increased flatulence; all are relieved with bowel movements.  Her appetite is good and her weight is stable.
The patient's other medical problems include anxiety disorder, vitiligo, migraine headaches, and fibromyalgia.  She uses ibuprofen occasionally and takes no other medications.  She underwent cognitive-behavioral therapy for anxiety and panic attacks approximately 3 years ago.
The patient does not use tobacco, alcohol, or illicit drugs.  Her diet consists mainly of hamburgers, pizza, and fried foods.  Family history is negative for colon cancer.
Vital signs are normal.  Physical examination shows no abnormalities.
Laboratory results are as follows:
 Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

11ec193a_97fa_db02_853d_a73230a6fffc_MD0002_00 Chronic constipation is defined as >3 months of symptoms, including a combination of <3 bowel movements per week and >25% of bowel movements requiring straining (ie, lumpy and hard stools, sensation of incomplete evacuation, requirement of digital evacuation). Poor dietary habits or adverse medication effects are often responsible, but patients with alarm features (eg, prominent, unexplained change in bowel habits) should be evaluated for other secondary causes (eg, malignancy, hypothyroidism). This patient's mild hyponatremia and history of autoimmune disease (vitiligo) raise suspicion for hypothyroidism; macrocytic anemia is present in approximately 10% of hypothyroidism cases due to concomitant pernicious anemia. All patients with chronic constipation should undergo initial evaluation with detailed history, physical examination, and basic laboratory testing (eg, complete blood count, serum glucose, serum creatinine, electrolytes). Those with alarm features should undergo further evaluation, including additional laboratory testing (eg, TSH, serum calcium), abdominal imaging, and/or colonoscopy, as suggested by the history. In patients with no alarm features and an unremarkable initial laboratory evaluation, a therapeutic trial of medication adjustment and/or dietary fiber intake and lifestyle modification is appropriate before additional testing is performed (Choice D). (Choice B) Anorectal manometry assesses anal sphincter and pelvic floor function to assist in identifying neurologic causes of chronic constipation (eg, pelvic floor dyssynergia, Hirschsprung disease). This and other motility testing is typically reserved for patients who fail a trial of diet and lifestyle modification. (Choice C) Colonoscopy is indicated in patients with unexplained chronic constipation who have alarm features and have not recently undergone colonoscopy screening. However, TSH testing is less invasive and more clinically appropriate in this patient and should be performed first. (Choice E) Cognitive-behavioral therapy may have a role in management of patients with severe irritable bowel syndrome symptoms and psychiatric comorbidity. However, common secondary causes should be excluded before employing psychological treatments. Educational objective: The initial evaluation of patients with chronic constipation should include detailed history, physical examination, and basic laboratory testing. Those without alarm features can undergo a therapeutic trial of medication adjustment and/or dietary fiber intake and lifestyle modification. Those with alarm features should undergo further evaluation based on history, including additional laboratory testing (eg, TSH level), abdominal imaging, and colonoscopy. __________ References: Diagnostic approach to chronic constipation in adults. (http://www.ncbi.nlm.nih.gov/pubmed/21842777)

Question 5

A 47-year-old woman is evaluated for a 6-month history of increasing fatigue, malaise, pruritus, and arthralgias. She has had no shortness of breath, chest pain, or fever. The patient was found to have abnormal liver chemistry tests on 2 occasions over the past 3 months. She takes no medications, drinks a glass of wine with dinner every day, and does not use tobacco or illicit drugs. The patient received a blood transfusion in childhood after surgery for a leg fracture. Temperature is 36.5 C (97.7 F), blood pressure is 110/60 mm Hg, pulse is 86/min, and respirations are 12/min. BMI is 25 kg/m². There is no scleral icterus, lymphadenopathy, or skin rash. Cardiac examination reveals a 2/6 holosystolic murmur at the apex radiating to the axilla. The liver is palpable 4 cm below the right costal margin with no splenomegaly. There is no peripheral edema. Several of the small joints of the hands are mildly tender without erythema or swelling. Laboratory results are as follows: Which of the following is the most appropriate pharmacotherapy for this patient?

Accepted Answer

11ec193a_97f4_e880_853d_3d9f49f2de04_MD0002_00 This patient's clinical presentation is consistent with autoimmune hepatitis (AH), which is characterized by gradual hepatocyte damage and nonspecific symptoms. Patients have persistently abnormal liver function studies (predominantly transaminases); multiple circulating autoantibodies, including antinuclear antibody (ANA), antismooth muscle antibody (ASMA), anti-liver/kidney microsomal-1 antibody (LKM-1), and anti-liver cytosol-1 antibody (LC-1), may also be present. Current guidelines recommend treatment for patients with suspected AH and any of the following: Transaminases >10 times normalTransaminases >5 times normal with hyperglobulinemiaEvidence of necrosis on liver biopsyIncapacitating symptoms Transaminases >10 times normal Transaminases >5 times normal with hyperglobulinemia Evidence of necrosis on liver biopsy Incapacitating symptoms Prednisone is the preferred therapy, usually combined with azathioprine to allow for lower glucocorticoid doses and fewer adverse effects. Such treatment leads to clinical, laboratory, and histological improvement in up to 90% of patients with 10%-40% achieving sustained remission; however, patients who develop cirrhosis usually require liver transplant. (Choice A) Patients intolerant of or refractory to prednisone and azathioprine can be treated with a calcineurin inhibitor (eg, tacrolimus, cyclosporine) or with other immunosuppressive agents (eg, sirolimus, methotrexate, mycophenolate mofetil). (Choice B) This patient's positive hepatitis C virus (HCV) antibody with negative HCV RNA may indicate spontaneously cleared prior infection or may be a false-positive HCV antibody result, which can occur in the setting of hypergammaglobulinemia from AH. Nonetheless, the negative HCV RNA assay rules out active infection and the need for treatment. (Choice C) Ursodeoxycholic acid is a bile acid supplement used to treat primary biliary cholangitis (PBC), which is characterized by autoimmune destruction of intrahepatic biliary ducts with a predominant elevation in alkaline phosphatase. Antimitochondrial antibodies are nearly 100% sensitive for PBC but are not specific. These antibodies are also positive in up to 20% of patients with AH; this patient's transaminitis >500 U/L and positive ASMA, both of which would be unusual in PBC, strongly favor a diagnosis of AH. (Choice E) Nucleotide analogues targeting the hepatitis B virus (eg, tenofovir, entecavir) are commonly used to treat active hepatitis B; however, this patient's hepatitis B serology indicates prior infection with subsequent immunity, and treatment is not indicated. Educational objective: Autoimmune hepatitis is characterized by persistently abnormal liver function studies (eg, transaminitis) and circulating autoantibodies, including antinuclear antibody (ANA), antismooth muscle antibody (ASMA), anti-liver/kidney microsomal-1 antibody (LKM-1), and anti-liver cytosol-1 antibody (LC-1). Prednisone is the preferred therapy and is usually combined with azathioprine to allow for lower glucocorticoid doses and fewer side effects. __________ References: Auto immune hepatitis. (http://www.ncbi.nlm.nih.gov/pubmed/27217697) Update on autoimmune hepatitis. (http://www.ncbi.nlm.nih.gov/pubmed/26357634)

Question 6

A 44-year-old woman comes to the office due to fatigue.  Medical history is notable for hypertension, obesity, and hypothyroidism; current medications include hydrochlorothiazide, amlodipine, and levothyroxine.  The patient does not use tobacco but drinks 1 or 2 glasses of wine on weekends.  She stopped using recreational drugs approximately 10 years ago.
Examination shows multiple skin xanthelasmas, a few scattered hyperpigmented spots, and tenderness in the right upper quadrant of the abdomen.  The liver is palpable 3 cm below the costal margin and mild splenomegaly is present.
Laboratory results are as follows:
 Which of the following is this patient at the highest risk of developing?

Accepted Answer

11ec193a_97f6_6f24_853d_af2c1438be4d_MD0002_00 This patient's presentation is consistent with primary biliary cholangitis (PBC, formerly primary biliary cirrhosis). PBC is due to autoimmune destruction of intralobular bile ducts and predominantly affects women age 30-65. Patients with PBC typically have a cholestatic injury pattern on liver biomarkers (disproportionate elevation in alkaline phosphatase and bilirubin compared to transaminases), and anti-mitochondrial antibodies are positive in ~95% of cases. Antinuclear antibodies are present in approximately 70% of patients and correlate with a poor prognosis. The diagnosis can be confirmed with liver biopsy. Up to 50% of patients develop metabolic bone disease, which may manifest as osteoporosis and/or osteomalacia. The cause of osteoporosis in PBC is unknown but is thought to be due to cholestasis leading to an accumulation of substances that inhibit bony turnover. Additionally, the disease predominately affects middle aged female patients who are already at risk for osteoporosis. Patients may have malabsorption of fat-soluble vitamins, but those with PBC-related osteoporosis usually have normal vitamin D levels. Nevertheless, patients with PBC should receive routine supplementation with calcium and vitamin D in addition to regular bone density screening. (Choices A and D) This patient has evidence of prior hepatitis B infection and recovery, with positive hepatitis B surface and core antibodies. Aplastic anemia may rarely occur in hepatitis B, but it is not usually a complication of PBC (this patient's mild thrombocytopenia is most likely due to portal hypertension). Polyarteritis nodosa is a possible complication of chronic hepatitis B but not PBC. (Choice B) Cryoglobulinemia is typically associated with hepatitis C, but it is not usually seen with PBC. This patient has a negative hepatitis C antibody screen. (Choice E) Patients with PBC can develop significant hypercholesterolemia (sometimes >1,000 mg/dL), which leads to xanthelasmata and xanthomas. However, these patients are not at increased risk for coronary artery disease, possibly due to marked elevation of HDL but only mildly elevated LDL and VLDL. Educational objective: Primary biliary cholangitis predominantly affects women age 30-65. Patients typically have a cholestatic injury pattern on liver biomarkers and positive anti-mitochondrial antibodies. Up to 50% of patients also develop metabolic bone disease, which may manifest as osteoporosis and/or osteomalacia. __________ References: Osteoporosis in patients with primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/20926953) Primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/21601785)

Question 7

A 76-year-old woman comes to the physician for routine follow-up. She complains of frequent right knee pain and swelling that is relieved by daily naproxen. She was offered knee replacement surgery for her severe osteoarthritis, but she refused. She was diagnosed with stable angina five years ago and has not undergone any coronary intervention. She denies chest pain with mild exertion, but admits to occasional chest discomfort with moderate exertion. Her other medical problems include atrial fibrillation, hypertension, hyperlipidemia, and diet-controlled type 2 diabetes mellitus. Her current medications include warfarin with target INR 2-3, low-dose aspirin, metoprolol, enalapril, and rosuvastatin. She is an ex-smoker with a 35 pack-year smoking history. Her blood pressure is 130/69 mm Hg and pulse is 80/min and irregular. Her BMI is 42 kg/m². A 2/6 mid-systolic murmur is heard at the left sternal border. Her lungs are clear. The right knee is mildly swollen and tender. Laboratory results are as follows: Which of the following additional medications would most likely benefit the patient?

Accepted Answer

11ec193a_97f5_d2e2_853d_a76ee84fac00_MD0002_00 Non-steroidal anti-inflammatory drugs (NSAIDs) cause significant morbidity and mortality due to gastrointestinal (GI) toxicity, particularly in elderly patients. This patient has multiple risk factors for NSAID-associated GI toxicity. Current guidelines recommend switching high or moderate risk patients to a selective COX-2 inhibitor or combining NSAIDs with a PPI or misoprostol. Lansoprazole is approved in the U.S. for prevention of NSAID-induced ulcers. Additionally, patients should take the lowest possible dose of NSAIDs. This high risk patient should be placed on prophylaxis, since she will likely require long-term NSAIDs for osteoarthritis. (Choice A) The patient is on therapeutic doses of warfarin (goal INR 2-3) plus aspirin without embolic phenomena from atrial fibrillation. Adding clopidogrel would not reduce her embolization risk and it could significantly increase her bleeding risk. (Choice B) The patient's LDL cholesterol is below the recommended goal of < 70 mg/dL with the use of a statin alone. Adding a fibrate would not benefit the patient and would increase the risk of statin myopathy. (Choice C) Elderly diabetics with multiple comorbidities have a recommended target HbA1c of 7.0-7.9%, rather than the commonly used goal of < 7.0% in younger patients. A HbA1c < 7.0% can increase the risk of hypoglycemic events in elderly patients, which further increases the risk of adverse cardiac events and cognitive impairment. Giving this patient glyburide would decrease her HbA1c of 7.2% and increase hypoglycemia risk. (Choice E) Niacin raises HDL but it has a higher side effect profile and lower cardiac benefit in a 76-year-old patient with optimal LDL and stable medical conditions. Educational objective: Patients taking chronic NSAIDs at high or moderate risk for GI toxicity should be switched to either a selective COX-2 inhibitor or be given a PPI or misoprostol in combination with the NSAID. __________ References: Clinical efficacy of esomeprazole in the prevention and healing of gastrointestinal toxicity associated with NSAIDs in elderly patients. (http://www.ncbi.nlm.nih.gov/pubmed/18331072) NSAID-associated adverse effects and acid control aids to prevent them: a review of current treatment options. (http://www.ncbi.nlm.nih.gov/pubmed/16454539)

Question 8

A 65-year-old man is evaluated for a 3-month history of malaise and abdominal distension with increasing abdominal girth.  He has also had progressive shortness of breath and generalized weakness.  Past medical problems include hypertension, hypercholesterolemia, type 2 diabetes mellitus, heart failure, and hepatitis C.  The patient also has a history of prostate cancer, which was treated with radical prostatectomy and radiation therapy a year ago.  He drinks 1 or 2 cans of beer a day and has smoked a pack of cigarettes daily for 20 years.  He admits to using injection drugs in the past.
Vital signs are within normal limits.  Chest examination reveals basilar crackles in the lungs and a grade 2 systolic murmur along the left sternal border.  The abdomen is nontender and distended with ascites.  There is no rebound tenderness or rigidity.  The liver edge is palpable 2 cm below the right costal margin and mild splenomegaly is present.  There is 2+ bilateral lower extremity pitting edema.
Laboratory results are as follows:
 Which of the following is the most likely cause of this patient's ascites?

Accepted Answer

11ec193a_97f2_9e8a_853d_653bf3e94630_MD0002_00 Evaluation of the etiology of ascitic fluid should proceed in a stepwise fashion. In this patient: etiology Ascitic fluid leukocytes <500/mm³ and neutrophils <250/mm³ argue against infection (eg, bacterial peritonitis) or a significant inflammatory process (eg, malignancy, pancreatitis).Serum-to-ascites albumin gradient (SAAG) ≥1.1 g/dL (1.7 g/dL in this patient) is indicative of portal hypertension, occurring in conditions including cirrhosis, heart failure, and Budd-Chiari syndrome (hepatic vein thrombosis).Ascitic fluid total protein ≥2.5 g/dL makes an underlying low-protein state such as cirrhosis (decreased protein production) or nephrotic syndrome (increased urinary protein excretion) less likely. Ascitic fluid leukocytes <500/mm³ and neutrophils <250/mm³ argue against infection (eg, bacterial peritonitis) or a significant inflammatory process (eg, malignancy, pancreatitis). Ascitic fluid leukocytes <500/mm³ and neutrophils <250/mm³ argue against infection (eg, bacterial peritonitis) or a significant inflammatory process (eg, malignancy, pancreatitis). ³ ³ Serum-to-ascites albumin gradient (SAAG) ≥1.1 g/dL (1.7 g/dL in this patient) is indicative of portal hypertension, occurring in conditions including cirrhosis, heart failure, and Budd-Chiari syndrome (hepatic vein thrombosis). Serum-to-ascites albumin gradient (SAAG) ≥1.1 g/dL (1.7 g/dL in this patient) is indicative of portal hypertension, occurring in conditions including cirrhosis, heart failure, and Budd-Chiari syndrome (hepatic vein thrombosis). Ascitic fluid total protein ≥2.5 g/dL makes an underlying low-protein state such as cirrhosis (decreased protein production) or nephrotic syndrome (increased urinary protein excretion) less likely. Ascitic fluid total protein ≥2.5 g/dL makes an underlying low-protein state such as cirrhosis (decreased protein production) or nephrotic syndrome (increased urinary protein excretion) less likely. This patient with SAAG ≥1.1 g/dL and ascitic fluid total protein ≥2.5 g/dL has known heart failure and no evidence to suggest acute Budd-Chiari syndrome (eg, right upper quadrant pain, history of thrombophilia); therefore, decompensated heart failure is the most likely cause of ascites. (Choice A) Although cirrhosis (eg, due to hepatitis C virus) is the most common cause of ascites, it is a low-protein state; the SAAG is usually elevated due to portal hypertension, but the ascites total protein is usually low (<2.5 g/dL). (Choice C) Nephrotic syndrome is a low-protein state that does not cause portal hypertension. Therefore, ascites due to nephrotic syndrome typically has low total protein and low SAAG (<1.1 g/dL). This patient's proteinuria is most likely due to hypertensive or diabetic nephropathy in the absence of nephrotic syndrome. (Choices D and E) Peritoneal carcinomatosis and tuberculous peritonitis typically cause a high-protein ascites similar to heart failure. The SAAG is typically low, unlike in this patient. Portal hypertension can sometimes develop, resulting in a high SAAG. However, ascites due to heart failure is more likely in this patient with known history and evidence of current decompensation (eg, lower extremity edema, congestive hepatopathy). Educational objective: The etiology of ascites can often be determined by evaluation of the ascitic fluid cell count, serum-to-ascites albumin gradient (SAAG), and fluid total protein. Low levels of inflammatory cells and elevations in the SAAG (1.1 g/dL) and fluid total protein (2.5 g/dL) are consistent with ascites due to heart failure. __________ References: Evaluation of new-onset ascites. (http://www.ncbi.nlm.nih.gov/pubmed/27434446)

Question 9

A 33-year-old man comes to the office for a routine follow-up visit. He was recently treated for esophageal achalasia with a pneumatic dilation. He reports a significant improvement in symptoms of dysphagia and nocturnal regurgitation. His past medical history is also significant for borderline hypertension and an anxiety disorder. He is an ex-smoker with a 5 pack-year smoking history. His mother suffered from Sjogren's disease and lymphoma. His medications include omeprazole and clonazepam. His blood pressure is 135/85 mmHg and his pulse is 67/min. His BMI is 30 kg/m². His physical examination is otherwise unremarkable. Over the next 10 years, the patient is at the highest risk for which of the following?

Accepted Answer

Achalasia is of unknown etiology and involves decreased peristalsis of the distal esophagus with failed relaxation of the lower esophageal sphincter (LES). Patients typically present with dysphagia with solids (91%) and liquids (85%), difficulty belching (85%), and other nonspecific symptoms (40-60%, including weight loss, chest pain, heartburn). Achalasia confers an increased risk for esophageal cancer (usually squamous cell carcinoma). Stasis and fermentation cause mucosal inflammation, epithelial hyperplasia, multifocal dysplasia, and eventual squamous cell carcinoma in some patients. As a result, these patients may need periodic surveillance, although a firm surveillance interval has not yet been established for these patients. (Choice A) Fibrosing mediastinitis is a rare but potentially serious disease caused by long-standing mediastinal inflammation. It involves diffuse fibrosis of the soft tissues of the mediastinum, and it is usually a consequence of prior granulomatous disease (e.g., histoplasmosis, tuberculosis, etc.) or radiation treatment, but not with achalasia. (Choice B) Achalasia does not usually increase the risk of lymphoma. However, non-Hodgkin's lymphoma may lead to pseudoachalasia by causing gastroesophageal obstruction. (Choice C) Patients with achalasia may have or develop other GI motility disorders such as gastroparesis, intestinal pseudoobstruction, etc. Isolated pyloric stenosis is usually either congenital (seen mainly in the first 2 years of life) or secondary to a mucosal disease such as a mass lesion or peptic ulcer. (Choice E) Zenker's diverticulum is a pulsion diverticulum which occurs in the upper esophagus. It is related to cricopharyngeal dysfunction and not achalasia, which affects the LES and not the UES. Educational objective: Patients with achalasia are at an increased risk of subsequent development of squamous cell cancer of the esophagus. __________ References: Long-term risk of esophagitis, Barrett's oesophagus and oesophageal cancer in achalasia patients (http://www.ncbi.nlm.nih.gov/pubmed/16782616)

Question 10

A 41-year-old woman comes to the office for a routine follow-up visit. The patient has had malaise and mild abdominal discomfort over the last 3-4 months. Her other medical problems include hypertension, type 2 diabetes mellitus, hyperlipidemia, asthma, and osteoarthritis. The patient also has a history of breast cancer treated with lumpectomy 5 years ago. She smokes a pack of cigarettes daily and drinks 1 or 2 glasses of wine on the weekends. Medications include glyburide, metformin, lisinopril, amlodipine, albuterol, and acetaminophen. The patient stopped taking a statin 2 months ago due to muscle aches. Blood pressure is 141/78 mm Hg and pulse is 78/min. BMI is 32 kg/m². There is no jugular venous distension. Bilateral scattered wheezes are heard on expiration. The liver edge is palpated 3 cm below the costal margin. No splenomegaly is present. There is 1+ pitting edema of both ankles. Laboratory results are as follows: Which of the following is the most likely mechanism of hepatomegaly in this patient?

Accepted Answer

11ec193a_97f1_3ef7_853d_539a44155e71_MD0002_00 This patient with metabolic syndrome (diabetes, hypertension, hyperlipidemia, and obesity) who has abdominal discomfort and a mild aminotransferase elevation likely has nonalcoholic fatty liver disease (NAFLD). NAFLD is a diagnosis of exclusion and is marked by the presence of hepatic steatosis not due to a secondary cause (eg, heavy alcohol use). NAFLD resembles alcohol-induced liver injury on histology; cirrhosis is more likely to develop in those with inflammation present (nonalcoholic steatohepatitis) than those without it (fatty liver disease). The underlying pathogenesis of NAFLD is likely multifactorial but may be partially due to insulin resistance, leading to increased lipid synthesis and decreased lipolysis, which cause hepatic steatosis and hepatocellular injury. NAFLD is the most common cause of liver disease in the Western world, likely due to the widespread prevalence of metabolic syndrome, a risk factor for the disease. Patients with NAFLD may have mild to moderate aminotransferase elevations (2-5 times the upper limit of normal). As opposed to alcoholic liver disease, the ratio of aspartate aminotransferase to alanine aminotransferase in NAFLD is typically <1. Iron studies are often abnormal, likely due to chronic inflammation; however, they are not elevated to levels noted in hereditary hemochromatosis (eg, ferritin >1,000 ng/mL, transferrin saturation >45%-50%) (Choice B). (Choice A) Autoimmune hepatitis usually causes a much higher elevation of aminotransferases (ranging from 300-1,000 U/L) than seen in this patient. Also, the negative antinuclear antibody titer in this patient makes this diagnosis even less likely. (Choice C) Increased central venous pressure suggests congestive hepatopathy/cardiac cirrhosis from right heart failure. This patient's lack of elevated jugular venous pressure makes cardiac cirrhosis unlikely. The ankle edema may be caused by amlodipine. (Choice E) Toxin-mediated liver injury (eg, from acetaminophen) often causes marked elevations in aminotransferases ranging from 5-25 times the upper limit of normal, hyperbilirubinemia, and increased prothrombin time. This patient's relatively mild elevations in liver function tests make this condition unlikely. Educational objective: Nonalcoholic fatty liver disease is a common cause of elevated liver enzymes in patients with metabolic syndrome and minimal or no alcohol history. Laboratory abnormalities include mild to moderate aminotransferase elevation (2-5 times the upper limit of normal), with an aspartate aminotransferase to alanine aminotransferase ratio typically <1. Iron studies are often abnormal, likely due to chronic inflammation. __________ References: Adiponectin, a key adipokine in obesity related liver diseases. (http://www.ncbi.nlm.nih.gov/pubmed/21734787) Nonalcoholic fatty liver disease: a review of the spectrum of disease, diagnosis, and therapy. (http://www.ncbi.nlm.nih.gov/pubmed/21947639)

Question 11

A 78-year-old woman is brought to the office from a nursing home for evaluation of urinary and fecal incontinence.  She has longstanding, episodic urinary incontinence but started to also have fecal incontinence over the last week.  The stool is described as loose and moderate in volume.  The patient has chronic constipation treated with a stable dose of laxatives, but these were discontinued at the onset of fecal incontinence.  Her other medical problems include moderate dementia, hypertension, type 2 diabetes mellitus, osteoporosis, and well-controlled congestive heart failure.  The patient is in a wheelchair for about 4 hours daily, but otherwise remains in bed.  No new medications have been added recently.
Vital signs are stable and unchanged from baseline.  The abdomen is soft and nontender with distant but normoactive bowel sounds.
Which of the following is the most likely mechanism of fecal incontinence in this patient?

Accepted Answer

11ec193a_97f2_0248_853d_7b81d99a1b60_MD0002_00 This elderly patient with a history of dementia, relative immobility, and chronic constipation is likely experiencing overflow fecal incontinence due to fecal impaction. Fecal impaction is common in the elderly and is associated with impaired mental function, immobility, inadequate fluid/dietary fiber intake, decreased sensation of stool in the vault, and chronic constipation. Impacted stool can apply pressure to the bladder and contribute to urinary incontinence. Fecal overflow incontinence occurs when loose or liquid stool flows around the impaction in the rectum. Evaluation of fecal incontinence begins with a perineal inspection and digital rectal examination. Patients with fecal impaction should undergo manual disimpaction and be placed on a chronic bowel regimen to prevent recurrence. (Choices A and E) Diabetic autonomic neuropathy can cause weakness of the anal sphincter and lead to fecal incontinence. Other causes of anal sphincter weakness include trauma (eg, operative vaginal delivery, anal surgery), spinal cord injury, and atrophy (eg, age-related, infiltration with systemic sclerosis). Although this patient has diabetes, diabetic autonomic neuropathy more commonly affects the upper gastrointestinal tract (eg, gastroparesis). When fecal incontinence does occur in autonomic neuropathy, it is typically associated with chronic, painless, watery diarrhea and the inability to retain stool in the rectum, rather than chronic constipation. (Choice B) Decreased rectal compliance results in the reduced ability to store fecal matter, leading to an increase in stool frequency, urgency, and fecal incontinence. However, it is most common in patients with a history of ulcerative or radiation proctitis. This patient's lack of prior diarrhea or history of radiation makes this unlikely. (Choice D) Rectal prolapse is associated with chronic constipation but usually presents with an anal mass, abdominal pain, blood and mucus in the stool, and a sensation of incomplete rectal emptying, which are not seen in this patient. Educational objective: Fecal impaction is common in elderly patients and can lead to stool overflow incontinence. Evaluation of fecal incontinence should begin with a perineal inspection and digital rectal examination. Management includes manual disimpaction and a chronic bowel regimen to prevent recurrence. __________ References: Fecal incontinence in the elderly. (http://www.ncbi.nlm.nih.gov/pubmed/19699410) Approach to fecal incontinence and constipation in older hospitalized patients. (http://www.ncbi.nlm.nih.gov/pubmed/21441765)

Question 12

A 40-year-old woman comes to the clinic due to fatigue and generalized weakness for the last 3 months. She has vague, intermittent right upper quadrant abdominal discomfort without pain, nausea, vomiting, or diaphoresis. The patient also reports low back pain and bilateral knee pain. She has had no weight loss or stool changes. Her other medical problems include polycystic ovarian disease, type 2 diabetes, and hyperlipidemia. The patient's menstruations are regular, and she does not use any hormonal contraception. Her medications include glyburide and metformin. She stopped taking a statin due to muscle cramps. The patient drinks 1 or 2 beers (350 mL each) every weekend. Her father was diagnosed with gallbladder carcinoma at age 65. Her mother has hypothyroidism and vitiligo. Blood pressure is 142/78 mm Hg and pulse is 92/min. BMI is 36 kg/m². Examination reveals an obese and nontender abdomen. The right knee has mild crepitus. Pinprick sensitivity is decreased below the knees, and the ankle reflex is absent bilaterally. Laboratory results are as follows: Ultrasonogram shows gallstones and a hyperechoic, enlarged liver with a 16-cm span. There is no gallbladder wall thickening or pericholecystic fluid. Which of the following would most likely improve this patient's liver condition?

Accepted Answer

11ec193a_97fa_18b0_853d_d5d901718eb4_MD0002_00 This patient with morbid obesity, diabetes, and hyperlipidemia who presents with a mild aminotransferase elevation and hepatomegaly likely has nonalcoholic fatty liver disease (NAFLD). NAFLD is a diagnosis of exclusion; it is marked by the presence of hepatic steatosis not due to a secondary cause (eg, heavy alcohol use). It is the most common cause of liver disease in Western industrialized countries, likely due to the widespread prevalence of metabolic syndrome (eg, central obesity, diabetes mellitus/insulin resistance, hyperlipidemia, and hypertension) that is a risk factor for the disease. Patients are often asymptomatic but can have nonspecific symptoms (eg, fatigue) with mildly elevated aminotransferases and aspartate aminotransferase/alanine aminotransferase (AST/ALT) ratio <1. After other causes of steatosis are ruled out, diagnosis is usually made clinically with noninvasive techniques (eg, ultrasound, CT scan, MRI) showing a fatty liver. Patients in whom diagnosis is unclear may require liver biopsy. NAFLD can progress to fibrosis and cirrhosis. Treatment involves addressing comorbidities (eg, diabetes, hypertension, hyperlipidemia) as well as promoting aggressive lifestyle changes, including gradual weight reduction (rapid loss can worsen NAFLD), exercise regimen, and increased aerobic activity. (Choice A) Alcoholic liver disease typically causes an AST/ALT ratio ~2:1 and presents in patients with significant alcohol consumption (eg, >15 drinks/week for men and >10 drinks/week for women). (Choice B) Iron chelation therapy is indicated for hemochromatosis, which can be associated with hepatitis, arthritis, and diabetes mellitus. However, premenopausal women are unlikely to develop symptomatic iron overload from hemochromatosis due to the regular iron excretion during menses. In addition, arthritis found in hemachromatosis is classically noted in the second and third metacarpophalangeal joints; this patient's knee and back pain are likely obesity-related. (Choice C) Hypothyroidism can cause fatigue and weakness; however, this patient has subclinical hypothyroidism (elevated TSH with normal free T4), which does not typically cause symptoms. True hypothyroidism is also associated with weight gain, irregular menses (in contrast to this patient's regular menses), constipation, and cold intolerance. (Choice D) Primary biliary cholangitis can cause fatigue, hyperlipidemia, and hypothyroidism and is treated with ursodeoxycholic acid. However, it typically causes a markedly elevated alkaline phosphatase and pruritus. Educational objective: Nonalcoholic fatty liver disease is commonly associated with metabolic syndrome (diabetes, hyperlipidemia, hypertension, and obesity) in patients with minimal alcohol history. Patients should be counseled for gradual weight loss and optimal control of diabetes and hyperlipidemia to help prevent or slow the progression of cirrhosis. __________ References: Diagnosis and evaluation of nonalcoholic fatty liver disease. (http://www.ncbi.nlm.nih.gov/pubmed/22110476) Nonalcoholic fatty liver disease: a review of the spectrum of disease, diagnosis, and therapy. (http://www.ncbi.nlm.nih.gov/pubmed/21947639)

Question 13

A 43-year-old woman comes to the office due to nausea, vomiting, and intermittent epigastric pain for the last 6 months.  Her symptoms have gradually worsened and now she cannot finish a regular meal without feeling full quickly.  Although the patient has a decent appetite, she is afraid to eat due to postprandial bloating and heartburn.  Fatty foods and salads are particularly bothersome.  Consequently, the patient has lost 6.8 kg (15 lb).
Other medical problems include hypertension, type 1 diabetes mellitus complicated by diabetic nephropathy and neuropathy, hypercholesterolemia, and nonalcoholic fatty liver disease.  Medications include insulin glargine, insulin lispro, lisinopril, gabapentin, simvastatin, low-dose aspirin, and ranitidine.  The patient has no known drug allergies.
Examination reveals a distended and nontender abdomen with an obvious succussion splash.  Rectal examination shows brown stool negative for occult blood.
Laboratory results are as follows:
 Which of the following is the most appropriate next step in management of this patient?

Accepted Answer

11ec193a_97f3_88ec_853d_8f95c0d8405e_MD0002_00 This patient's presentation (postprandial reflux, vomiting, succussion splash) is most consistent with impaired gastric emptying, which can be due to the following: Gastric dysmotility (eg, gastroparesis)Gastric outlet obstruction (GOO) due to mechanical (eg, pancreatic cancer, gastric polyp) or mucosal (eg, scarring from peptic ulcer disease) causes Gastric dysmotility (eg, gastroparesis) Gastric outlet obstruction (GOO) due to mechanical (eg, pancreatic cancer, gastric polyp) or mucosal (eg, scarring from peptic ulcer disease) causes Although this patient likely has gastric dysmotility due to diabetic gastroparesis, the definition of gastroparesis requires proving delayed gastric emptying in the absence of obstruction. Therefore, the first step in the workup of any patient with impaired gastric motility is to exclude mechanical or mucosal etiologies using upper endoscopy to evaluate for causes of GOO. Patients should also undergo imaging studies, such as CT/MR enterography (eg, to exclude small bowel mass). Once mechanical and mucosal causes have been excluded, the next step is to establish the diagnosis of gastroparesis by proving the presence of delayed gastric emptying (eg, gastric-emptying scintigraphy showing >10% gastric retention 4 hours following a solid meal) (Choice D). Once gastroparesis has been established, evaluation for the underlying etiology, such as diabetes mellitus, thyroid disease, and autoimmune disease, should occur. Gastroduodenal manometry and autonomic testing can also be helpful for distinguishing between myopathic and neuropathic causes. (Choice A) This patient has multiple signs of poorly controlled diabetes (eg, glycosuria), and modifying the insulin regimen is an important step in managing diabetic gastroparesis; however, prior to treating diabetic gastroparesis the patient should undergo an evaluation to exclude mechanical or mucosal etiologies. (Choice B) Ultrasound is useful for diagnosing gallstones, but it is not as sensitive or specific for gastric obstruction. This patient's symptoms of postprandial epigastric pain, early satiety, and weight loss are more concerning for impaired gastric emptying, which requires endoscopic evaluation, than for gallstone disease. (Choice E) Metoclopramide helps treat confirmed gastroparesis, but it should not be started empirically because of its significant adverse effects (eg, extrapyramidal symptoms). Gastroparesis should first be confirmed with a nuclear scintigraphic study. Educational objective: Impaired gastric emptying usually presents with early satiety, postprandial reflux, nausea, vomiting, and a succussion splash on examination. All patients should first be evaluated for mechanical or mucosal causes with an upper endoscopy. __________ References: Epidemiology, mechanisms, and management of diabetic gastroparesis. (http://www.ncbi.nlm.nih.gov/pubmed/20951838)

Question 14

A 24-year-old man comes to an urgent care center because of a 2 week history of watery diarrhea.  The patient's stools are pale, voluminous, foul smelling, and non-bloody.  He has lost 5 pounds (2.3 kg) over the past week despite eating normally.  He denies fever or abdominal pain, but does admit to bloating and flatulence.  He is an avid hiker, but denies recent travel.
His vital signs are within normal limits.  His abdomen is soft and non-tender with normal bowel sounds.  The remainder of his examination shows no abnormalities.
Laboratory results are as follows:

Which of the following is the most appropriate next step in management?

Accepted Answer

This patient's presentation of diarrhea with steatorrhea (indicating malabsorption) is most likely due to Giardiasis. Giardia cysts survive well in cold water (e.g., rivers and streams), and water dwelling mammals (e.g., beavers) can become infected and continue infecting the water. Hikers in wilderness areas are at high risk for giardiasis if they drink water without adequate filtration, treatment, or boiling. Patients may be asymptomatic or present with the symptoms in the above table. Stool microscopy testing for G. lamblia trophozoites and cysts is initially preferred and can detect 50-70% of cases after one specimen and > 90% of cases after 3 specimens. Immunoassays for G. lamblia antibodies are more sensitive and specific than stool microscopy and can be performed if stool microscopy is negative. If noninvasive tests fail to reveal the organism, both endoscopy with duodenal biopsy and colonoscopy are very sensitive and specific for diagnosing G. lamblia. Stool microscopy (Choice A) Increased absolute eosinophil count (usually > 2000 cells/mm³) may suggest eosinophilic gastroenteritis, which is an uncommon cause of malabsorption. The diagnosis is confirmed with a biopsy demonstrating > 20-25 eosinophils/hpf. However, infectious etiologies should first be ruled out in this patient. ³ (Choice B) Colonoscopy with biopsies can confirm diagnoses such as celiac sprue, inflammatory bowel disease, and microscopic colitis. However, it is usually indicated after negative or equivocal noninvasive testing. (Choice C) Fecal fat testing will confirm malabsorption but it will not diagnose the underlying cause of the patient's diarrhea. (Choice E) Tissue transglutaminase IgA antibodies are frequently positive in celiac disease, which can present with malabsorption. However, this test should only be performed if the initial infectious studies are negative. Educational objective: Patients presenting with acute diarrhea associated with steatorrhea should first be evaluated for infectious etiologies. Giardia lamblia (also known as intestinalis or duodenalis) is the most common infectious cause of malabsorption and is diagnosed with stool microscopy or stool immunoassay. __________ References: Comparison of microscopy, real-time PCR and a rapid immunoassay for the detection of Giardia lamblia in human stool specimens. (http://www.ncbi.nlm.nih.gov/pubmed/17949440) An updated review on Cryptosporidium and Giardia (http://www.ncbi.nlm.nih.gov/pubmed/16880067) Giardia duodenalis: the double-edged sword of immune responses in giardiasis. (http://www.ncbi.nlm.nih.gov/pubmed/20599999)

Question 15

A 56-year-old man comes to the office due to 2 months of upper abdominal discomfort, bloating, and anorexia. Over this period, the patient has had no nausea, vomiting, or black/tarry stools but has lost 8 kg (17.6 lb). He has a history of hypertension and gout. The patient is an active smoker with a 45 pack-year history and drinks alcohol occasionally. He traveled to Mexico 3 months ago for vacation. Temperature is 37.2 C (99 F), blood pressure is 120/70 mm Hg, pulse is 86/min, and respirations are 12/min. BMI is 29 kg/m². Scleral icterus is present. The abdomen is soft and nondistended. There is mild right upper quadrant discomfort on deep palpation. The liver edge is palpable 3 cm below the right costal margin. Stool testing for occult blood is negative. The remainder of the physical examination shows no abnormalities. Laboratory results are as follows: Abdominal ultrasound shows dilated intrahepatic and extrahepatic bile ducts. The gallbladder is enlarged but no gallstones are visualized. Which of the following is the best next step in management of this patient?

Accepted Answer

11ec193a_97f7_ceb8_853d_891416e371be_MD0002_00 This patient's presentation is most consistent with extrahepatic biliary obstruction. The differential diagnosis includes choledocholithiasis, stricture, pancreatic pathology, and malignancy of the biliary tree. Diagnostic evaluation usually begins with transabdominal ultrasound. However, this test may miss pancreatic lesions due to overlying bowel gas, small lesion size (<3 cm), or lack of skill of the operator. Given the high pretest probability of cancer in this patient (weight loss, anorexia, jaundice, smoking history), the best next step is a helical abdominal CT scan to evaluate for pancreatic malignancy. If CT scan reveals a pancreatic mass, histological diagnosis is then generally made with endoscopic ultrasound-guided fine-needle aspiration. (Choice A) Primary biliary cholangitis is also associated with elevated alkaline phosphatase and cholestatic jaundice but is not typically associated with extrahepatic biliary duct dilation or gallbladder enlargement. Liver biopsy shows inflammation confined to the portal and periportal areas, bridging fibrosis, and/or cirrhosis. (Choices C and D) Both endoscopic retrograde cholangiopancreatography (ERCP) and MR cholangiopancreatography can be used to evaluate the pancreatobiliary tree; ERCP has the added benefit of being able to provide a therapeutic intervention (eg, stent placement). These tests are superior to abdominal CT scan when choledocholithiasis is suspected. However, patients who require imaging of the pancreas for suspected cancer (as in this patient with heavy tobacco use, weight loss, abdominal pain, jaundice, and no gallstones on ultrasound) should first undergo abdominal CT scan as it is lower cost, less invasive, associated with fewer complications, and can provide staging information. If CT shows pancreatic cancer causing biliary obstruction, ERCP with stenting is sometimes used for palliation or as a bridge to surgery. (Choice E) Viral hepatitis typically causes elevation of both AST and ALT without dilated intrahepatic or extrahepatic bile ducts. Although this patient went to Mexico recently, the relatively normal AST and ALT makes this diagnosis less likely. Educational objective: Patients with jaundice and abdominal pain should be evaluated for gallstones with ultrasound. Those who have no evidence of gallstones should undergo CT scan of the abdomen to evaluate for pancreatic pathology, particularly if there are signs (eg, unintentional weight loss) concerning for malignancy. Depending on CT scan findings, further evaluation with endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, or MR cholangiopancreatography is often required. __________ References: Diagnosis of pancreatic cancer. Page(s): PMC2020745 (http://www.ncbi.nlm.nih.gov/pubmed/18333085)

Question 16

A 24-year-old man is brought to the physician for a follow-up appointment. He initially presented 4 weeks ago with an acute episode of psychosis and personality changes, and he was prescribed low dose risperidone. He subsequently developed tremor and gait abnormalities. The patient's father died from liver failure at age 40. His mother is alive at age 60 and is a smoker with mild emphysema. He has two siblings who are alive and healthy. The patient drinks 2-3 beers daily and has smoked two packs of cigarettes per day for the last five years. His vital signs are within normal limits. The patient's BMI is 23 kg/m². Lungs are clear to auscultation. Examination reveals a nontender abdomen. The liver is palpable 3 cm below the right costal margin. A bilateral resting tremor is noted in his hands. Laboratory studies obtained 4 weeks ago during his first visit are shown below. Complete blood count, renal function panel, urinalysis, and thyroid function testing are within normal limits. Urine toxicology screen is negative. Which of the following is the most appropriate next step in management?

Accepted Answer

11ec193a_97f8_e02c_853d_87612e66def4_MD0002_00 This patient presents with psychosis and liver disease (non-hepatitis B or C). His tremor and gait disturbances could be due to drug-induced Parkinsonism. However, the combined presence of liver abnormalities, family history of liver disease, psychiatric symptoms, and young age suggest likely Wilson disease (hepatolenticular degeneration). Dopamine antagonism due to risperidone use may have precipitated his more acute Parkinsonian symptoms. Wilson disease is an autosomal recessive disorder that causes defective copper transport leading to copper accumulation, most commonly in the liver and brain. Nearly 35% of patients present with neurologic symptoms (e.g., Parkinsonian-like tremor, rigidity, clumsy gait, slurred speech), usually before age 30. Kayser-Fleischer rings (copper deposition in the eye) are present in 50-60% of Wilson disease patients with isolated hepatitis and > 90% of patients with neurological symptoms. All patients should undergo slit lamp examination, but the absence of Kayser-Fleischer rings does not rule out the diagnosis. Diagnosis is supported by increased serum and urine copper levels, and decreased serum ceruloplasmin levels. Early diagnosis and medical therapy with penicillamine or trientine may prevent many of the permanent sequelae. (Choice A) Anti-smooth muscle antibodies are found in autoimmune hepatitis, which often presents with nonspecific complaints (e.g., arthralgias, fatigue, and weight loss) and is associated with extrahepatic conditions (e.g., immune thrombocytopenia, type 1 diabetes mellitus, thyroiditis, celiac disease, and ulcerative colitis). This patient's neurologic symptoms and lack of autoimmune symptoms makes this diagnosis unlikely. (Choice C) Knee pain, chondrocalcinosis, and liver function abnormalities are also seen with hemochromatosis. However, neurologic disease is rare in hemochromatosis. (Choice D) Lumbar puncture is indicated for suspected meningitis or encephalitis. However, this patient's lack of fever, altered mentation, and headache makes this diagnosis less likely. (Choice E) Alpha-1 antitrypsin (A1AT) deficiency can lead to liver and lung disease; development of panacinar emphysema in smokers can be accelerated by as early as 19 years. Isolated liver disease is less common in A1AT deficiency, and this patient's neurologic findings and lack of pulmonary findings make this consideration unlikely. Educational objective: Chronic liver disease associated with neuropsychiatric manifestations suggests Wilson disease, which is caused by a defect in copper transport. Kayser-Fleischer rings are characteristic, but their absence on slit lamp examination does not rule out the diagnosis. __________ References: Clinical molecular diagnosis of Wilson disease (http://www.ncbi.nlm.nih.gov/pubmed/21901653) Wilson disease: pathogenesis and clinical considerations in diagnosis and treatment (http://www.ncbi.nlm.nih.gov/pubmed/21901655) Wilson's disease. Psychiatric manifestations may be the clinical presentation. (http://www.ncbi.nlm.nih.gov/pubmed/8202418)

Question 17

A 65-year-old woman with a known history of Crohn's disease comes to the physician to receive the influenza and pneumonia vaccines.  She has never received these vaccines in the past.  She has no current complaints.  She denies abdominal pain, diarrhea, vomiting, and weight loss.  Her other medical problems include hypertension and osteoarthritis.
Her surgical history includes hysterectomy for fibroids, partial small bowel resection, and cholecystectomy.  Her last colonoscopy was 9 months ago, which showed mild inflammation.  She quit smoking 2 years ago and has 30 pack-year smoking history.  Her medications include hydrochlorothiazide, omeprazole, naproxen, sulfasalazine, azathioprine, and infliximab.
Which of the following is the most appropriate course of action?

Accepted Answer

Patients with inflammatory bowel disease (IBD) taking immunosuppressive medications such as anti-TNF agents (infliximab) and antimetabolites (azathioprine) should receive the standard adult vaccinations. However, live vaccines (e.g., intranasal influenza) should not be given with anti-TNF therapy (Choice D). In addition, vaccines ideally should be given before starting anti-TNF therapy. Patients with hepatitis B risk factors should also receive the hepatitis B vaccine. (Choices B and C) This patient qualifies for both vaccines and should receive both the intramuscular influenza and pneumococcal vaccines. (Choice E) Patients requiring live vaccines (e.g., varicella, zoster, etc.) should hold their immunosuppressants for 2-3 months before vaccination. However, this patient can be vaccinated with the inactivated influenza vaccine instead of the intranasal form. Educational objective: Inflammatory bowel disease (IBD) patients taking anti-TNF therapy should not receive live vaccines, but they should receive the standard adult immunizations. Intramuscular influenza and pneumococcal vaccines are recommended for IBD patients on anti-TNF therapy. __________ References: Vaccines and inflammatory bowel disease (http://www.ncbi.nlm.nih.gov/pubmed/20926882)

Question 18

A 46-year-old man comes to the physician because of recurrent abdominal discomfort. He describes episodes of moderate, dull, epigastric pain lasting 2-3 days that have occurred 1-2 times/month over the last 2 years. He also describes occasional nausea but denies any vomiting, diarrhea, black stools, blood in the stool, abdominal distention, or weight loss. He takes ibuprofen for chronic low back pain. He drinks 8-10 beers over the weekends and frequently throughout the week. His vital signs are within normal limits and his BMI is 28 kg/m². Physical examination is unremarkable. Fasting blood glucose is 127 mg/dL. Abdominal x-ray shows focal calcifications anterior to the spine over the epigastric area. Upper GI endoscopy reveals a normal esophagus, gastric varices in the fundus of the stomach, and a normal duodenum. Which of the following is the most likely diagnosis?

Accepted Answer

11ec193a_97fb_7743_853d_b1a3ba7a1869_MD0002_00 This patient's presentation is consistent with chronic pancreatitis due to excessive alcohol intake. Abdominal x-rays typically show calcifications in the area of the pancreas. The splenic vein runs along the posterior surface of the pancreas, where chronic pancreatic inflammation can lead to thrombosis. Splenic vein thrombosis leads to gastric varices near the gastric fundus only; esophageal varices are not seen. Variceal hemorrhage is the most common symptom of splenic vein thrombosis and splenectomy is usually curative. (Choice A) Cirrhosis causes scarring in the liver that leads to increased sinusoidal vascular pressures and portal hypertension. These patients typically present with esophageal varices, diffuse gastric varices (not just gastric fundus), and rectal varices (hemorrhoids). (Choice B) Gastroparesis is usually a complication of diabetes (rather than chronic pancreatitis) and causes delayed gastric emptying. Patients typically present with nausea/vomiting and bloating, and endoscopy will not show varices. (Choice C) Helicobacter pylori is a common cause of ulcer disease and may be associated with dyspepsia. This patient's use of alcohol and NSAIDs increases his risk of peptic ulcer disease, but his endoscopy showing varices make this less likely. (Choice D) Non-ulcer dyspepsia is usually diagnosed after excluding other etiologies (e.g., peptic ulcer disease, GERD, and malignancy). However, this patient's abnormal endoscopy showing varices makes this unlikely. Educational objective: Chronic pancreatitis can lead to splenic vein thrombosis. Gastric varices localized to the gastric fundus confirm the diagnosis. Bleeding from these varices can be cured by splenectomy. __________ References: Splenic vein thrombosis and gastrointestinal bleeding in chronic pancreatitis (http://www.ncbi.nlm.nih.gov/pubmed/14502405) Significance of splenic vein thrombosis in chronic pancreatitis (http://www.ncbi.nlm.nih.gov/pubmed/18585674)

Question 19

A 25-year-old man comes to the physician for evaluation of abnormal liver tests.  Blood work performed for graduate school enrollment revealed elevated bilirubin levels.  He has no complaints and feels well.  He reports that he had a history of jaundice and hepatitis at the age of 5.  He took azithromycin for an upper respiratory infection approximately 2 months ago.  He has no other medical problems.  He does not smoke or drink alcohol, and he denies any recreational drug use.
Physical examination is unremarkable.
Laboratory results are as follows:

Which of the following is the best next step in management for this patient?

Accepted Answer

11ec193a_97f9_a37e_853d_a5aa8a621251_MD0002_00 This patient's isolated unconjugated hyperbilirubinemia is most likely due to Gilbert's syndrome. Gilbert's syndrome results in decreased production (< 30% of normal) of UDP-glucuronyl transferases, deficiency of which leads to decreased glucuronidation of bilirubin and unconjugated hyperbilirubinemia. Patients typically are asymptomatic or may present with nonspecific symptoms (e.g., fatigue) and icterus on examination. The bilirubin levels are usually < 3-4 g/dL, but they can significantly increase with fasting for 24 to 48 hours, hemolysis, febrile illness, or medication use (e.g., nicotinic acid). Diagnosis is suggested by unconjugated hyperbilirubinemia with otherwise normal laboratory results (e.g., CBC, liver function tests, and hemolysis tests) and confirmed with repeat testing in 6-12 months that shows continued isolated unconjugated hyperbilirubinemia. Genetic testing for the UGT1A1 gene mutation is usually reserved for patients with an unclear diagnosis (Choice D). Gilbert's syndrome requires no further testing or specific therapy. This patient's normal liver enzymes and hepatic function rule out pre- and post-hepatic causes. The normal reticulocyte count and LDH do not suggest hemolysis. Given his immunity to hepatitis A (positive IgG HAV antibodies), Gilbert's syndrome is the most likely diagnosis by exclusion. (Choice A) Antinuclear antibodies can evaluate for SLE or autoimmune hepatitis, but this patient's normal liver function tests (except for bilirubin) and lack of symptoms make these unlikely. (Choice B) CT scan of the abdomen can help evaluate cholestatic disease or possible hepatic parenchymal lesions. However, this patient's otherwise normal liver function tests (except for bilirubin) make these scenarios unlikely. (Choice C) Phenobarbital is a hepatic enzyme inducer that can increase glucuronidase activity and decrease bilirubin levels. However, patients do not routinely need hepatic inducers (e.g., phenobarbital) since Gilbert's syndrome is a benign disease. Educational objective: Gilbert's syndrome usually presents with asymptomatic isolated unconjugated hyperbilirubinemia with normal liver enzymes and negative work-up for hemolysis. Further invasive tests or treatments are not necessary due to the benign nature of the disease. __________ References: The genetic basis of the reduced expression of bilirubin UDP-glucuronosyltransferase 1 in Gilbert's syndrome (http://www.ncbi.nlm.nih.gov/pubmed/7565971) Eponym : Gilbert syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/22160004)

Question 20

A 77-year-old man is brought to the office by his daughter because of weight loss and poor appetite. The patient denies having any problems and states that his daughter worries unnecessarily. The daughter agrees that she has become overly concerned about her father's health since his stroke three months ago. According to her, he has lost 10-15 pounds since then and sometimes takes a long time to finish his meals. He also suffers from coughing spells during his meals which usually lasts a couple of minutes. She has not noticed any other symptoms except for some slurred speech. His medical history is significant for an ischemic stroke, hypertension, diabetes mellitus, arthritis, and depression. He had a recent hospitalization for pneumonia 1 month ago. He is currently taking atenolol, baby aspirin, insulin, and trazodone. He intermittently takes over-the-counter naproxen. His dentist started him on an unknown antibiotic 5 days ago. He has a 20-pack-year smoking history, but quit smoking 30 years ago. He stopped drinking alcohol after the stroke, but otherwise says he has enjoyed a glass of wine everyday of his life. Physical examination shows a thin-built elderly man. His blood pressure is 152/72mmHg, pulse is 55/min, and BMI is 19 kg/m². He has some halitosis, decaying molars bilaterally, and dry oral mucosa. Minimal crackles are heard at the right lung base. There is trace peripheral edema on both lower extremities. Which of the following is the best next step in evaluating this patient?

Accepted Answer

In this elderly patient with a history of stroke, recent pneumonia, and symptoms of coughing spells after liquid intake, concern for oropharyngeal dysphagia is high. Oropharyngeal dysphagia is caused by disrupted neuromuscular coordination required for deglutition and is commonly seen in stroke patients. The main symptoms of oropharyngeal dysphagia are: coughing/choking spells, nasal regurgitation, prolonged swallowing effort, or difficulty in initiation of swallows with a feeling of obstruction at the level of the neck. 11ec193a_97f0_5495_853d_93bd541f3555_MD0002_00 Oropharyngeal dysphagia is best diagnosed/assessed by a modified barium swallow (MBS) or videofluoroscopy performed in the presence of an expert speech pathologist and radiologist. It is important to diagnose it early as failure to do so can lead to aspiration pneumonia, which carries a high morbidity and mortality. This patient has already had an episode of pneumonia, thus making it even more important to diagnose and treat oropharyngeal dysfunction. (Choice A) Various mechanical factors (e.g., osteophytes, Zenker's diverticulum, etc.) could cause oropharyngeal dysphagia. In the presence of a classic history, the next step would be to confirm oropharyngeal dysfunction before working up the etiology. Additionally, these mechanical factors may be discovered on the MBS itself and would not require a CT of the neck/chest. (Choice C) Dietary adjustments may be required, but confirmation of the diagnosis and assessment of the aspiration risk is essential to determine the needed consistency of a modified diet (e.g., thick, mechanically soft, puree diet, etc.) as well as the route of administration (e.g., oral, PEG tube, etc.). (Choice D) Endoscopy is good tool to assess esophageal abnormalities, but it would not help with oropharyngeal dysphagia. (Choice E) Pill-esophagitis is possible given the patient's new-onset chest pain, but stopping the antibiotic without confirming its role in causation would not be recommended. Furthermore, this does not explain the high aspiration risk. Educational objective: Oropharyngeal dysphagia is commonly seen after a neurologic injury such as a stroke, and is best diagnosed with a modified barium swallow (MBS). Prompt diagnosis is essential to prevent aspiration pneumonia, which carries high morbidity and mortality rates. __________ References: Dysphagia in stroke patients (http://www.ncbi.nlm.nih.gov/pubmed/16754707)

Exam 3: Office/Health Center