Miscellaneous

Question 1

A 58-year-old man is found to have anemia. He has a history of HIV and HCV. He reports shortness of breath on exertion and constipation. He has not noticed any rectal bleeding. He has not had endoscopic workup for anemia. On physical exam, there is diffuse violaceous coin-shaped papulosquamous rash on trunk and extremities [figure A]. Laboratory testing reveals hemoglobin 8.8 g/dL (normal: 14-17 g/dL), MCV 82 fL (normal: 80-96 fL), CD4 count 16 cells/µL (normal: 677-1401 cells/µL). His liver chemistry was normal except low albumin levels of 2.1 g/dL (normal: 3.5-5.0 g/dL). EGD- reddish, nodular lesion in second part of duodenum [figure B]. IHC- human herpesvirus 8 on biopsy specimen. What is the most appropriate next step in the management of this patient?

A. Oral iron supplementation

B. Thermal energy to ablate the lesions

C. Initiation of antiretroviral therapy

D. Treatment of hepatitis C infection

Answer 1

• Kaposi Sarcoma (KS) with cutaneous and gastrointestinal manifestations - can have GI w/o skin
• Gastrointestinal lesions can be asymptomatic or can present with weight loss, abdominal pain, nausea and vomiting, upper or lower gastrointestinal bleeding, malabsorption, intestinal obstruction, and/or diarrhea.
• Gastrointestinal KS lesions are typically hemorrhagic nodules that can be either isolated or confluent and may occur in any portion of the gastrointestinal tract.
• Dx: histopathology with biopsies positive for human herpesvirus 8
• Tx: start antiretroviral therapy (ART). Some patients will also require chemotherapy

Question 2

A 20-year-old man presented to the emergency department (ED) with fever, headache, and diarrhea after returning 3 weeks ago from a 1-week trip to Indonesia. He presented to the ED 7 days ago, and malaria and dengue fever were ruled out. GI multiplex PCR was performed with no pathogen identified. He was given empiric azithromycin for suspected febrile bacterial diarrhea. His symptoms worsened, which caused him to return to the ED.

The patient reports that his travel included fresh water swimming, hiking in rain forests, spending time on the beaches, and adventurous eating of unique foods in remote villages and settings. He does not recall having any tick bites but did have a couple of scabs on his shins and lower ankle [figure], which he attributes to skin puncture injuries while hiking off trail in the jungles. What test would be most helpful to determine the potential etiology of this patient’s clinical condition?

A. Tuberculin skin test

B. Leptospirosis IgM serology

C. Endoscopy and duodenal biopsy

D. Stool culture for Campylobacter spp.

Answer 2

• multiple potential exposures given the adventurous travel itinerary, including exposure to fresh water (leptospirosis).
• A multiplex PCR panel that was negative, as well as lack of response to azithromycin, makes Campylobacter unlikely.
• Endoscopy and duodenal biopsy could support tropical sprue, but his travel duration was short (under 2 weeks), and other infectious agents should first be excluded.

Question 3

A 56-year-old man with a history of hypertension and benign prostatic hyperplasia presents with a complaint of bloating for the last 2 months. His bowel movements are normal, and he reports no weight loss or abdominal pain. He does not believe that his bloating is associated with meals. He takes losartan and tamsulosin. He has no history of surgery. His family history is notable for his mother having cancer of the pancreas that was diagnosed at age 56, his maternal grandmother was diagnosed with ovarian cancer at age 62, and his maternal aunt had breast cancer at age 47. His maternal aunt did have genetic testing performed and was positive for a germline mutation in BRCA2. Comprehensive metabolic panel and CBC are normal. H. pylori stool antigen is normal. Celiac serologies are also normal. CT scan demonstrates fatty infiltration of the pancreas [figure]. What would you do next?

A. Order Ca19-9 test.

B. Start pancreatic lipase.

C. Repeat CT scan in 1 year.

D. Refer to genetic counseling.

Answer 3

• This patient has a family history concerning for hereditary breast and ovarian cancer (HBOC) syndrome. He should be referred to a genetic counselor for genetic testing to determine if he also harbors the BRCA2 mutation or other associated mutations.
• This mutation is not only associated with breast and ovarian cancer syndromes but also associated with pancreatic cancer.
• Patients with BRCA2 and a family member with pancreatic cancer have a 2-9-fold greater risk than the general population and should be enrolled in a surveillance program.
• This patient may have exocrine pancreatic insufficiency, but the fatty infiltration noted on imaging may be early PanIN (Pancreatic intraepithelial neoplasia)lesions.
• A CA 19-9 is helpful if a diagnosis of pancreatic cancer is being formally entertained but not for initial workup.

Question 4

A 17-year-old Armenian boy who had an appendectomy a year ago presents with recurring fever and abdominal pain. For the past 3 years, he has had paroxysms of fever up to 104°F (40°C) that rise quickly, plateau and then gradually resolve over 48 hours. Abdominal pain usually occurs with these paroxysms and often is associated with constipation. Diarrhea typically follows each episode. He also has been noted to have rashes over his lower legs and has complained of testicular pain on several occasions. When you see him in the office, he is between attacks and his physical examination is normal. All basic laboratory tests are normal including a CBC, liver biochemical tests, BMP, ESR, and CRP. Urinalysis, however, reveals microscopic hematuria and a dip-stick test for albumin is strongly positive. Subsequently, you learn that a 24-hr urine collection contains 1.5 grams of albumin. What is the most appropriate management at this time?

A. Observation

B. Prednisone

C. Colchicine

D. Etanercept

Answer 4

• Familial Mediterranean Fever (FMF) or recurrent polyserositis
  
  • recurrent episodes of peritonitis, pleuritis, synovitis, and arthritis, usually accompanied by fever.
  • rates are much higher for certain Mediterranean populations including individuals of Armenian, Turkish, Arabic, and North African Jewish descent than in other ethnicities.
  • FMF is caused by mutations in the MEFV gene, which normally produces pyrin or marenostrin, a protein expressed mostly in neutrophils.
  • Symptoms usually develop by age 20.
  • Abdominal pain occurs in almost all FMF patients and peritonitis may mimic appendicitis, cholecystitis, or renal colic. Other symptoms include rashes on the lower extremities, scrotal pain from inflammation of the tunica vaginalis, myalgia and symptoms of associated vasculitis, (e.g., Henoch-Schönlein, polyarteritis nodosa, and Behçet’s disease).
  • Routine blood tests (CRP, ESR, WBC) performed during an acute attack are nonspecifically abnormal.
  • Genetic testing is now used to confirm the diagnosis.
  • Treatment, both prophylactic and for acute attacks, is with colchicine.
  • Because colchicine may prevent, arrest, and even reverse renal amyloidosis, such treatment should be life-long.
  • Hemodialysis is used for renal failure and peritoneal dialysis tends to increase the number of abdominal attacks.

Question 5

A 59-year-old man who underwent laparoscopic sleeve gastrectomy 7 months ago is referred to you because he has developed worsening reflux, dysphagia, nausea, and emesis. He has developed a fear of going out to eat, as he is inconsistently tolerating solid food and cannot predict when he will need to vomit. Physical exam is notable for a well-appearing male in no distress. Laboratory analysis is notable for a normal basic metabolic panel and complete blood count. What is the best next step in the management of this patient?

A. Perform a CT of the abdomen - no role, UGI yes

B. Refer to surgery.

C. Obtain laboratory studies.

D. Perform an upper endoscopy.

Answer 5

• Reflux and symptoms of obstruction following sleeve gastrectomy suggest sleeve stenosis, a condition that classically occurs at the incisura. Symptoms of obstruction can occur depending on the severity of the narrowing.
• This diagnosis is typically made by endoscopy or upper gastrointestinal series, and endoscopic dilation is the primary mode of management.
• Referral to surgery for consideration of RYGB should only be considered if serial pneumatic dilations with increasing balloon size and pressure is not effective

Question 6

1. Cecal intubation rate for healthy individuals undergoing screening colonoscopy?
2. ADR for average risk patients undergoing screening colon?
3. Reversal agent for Dabigatran?
4. Reversal for RiavaroXaban and ApiXiban?

Answer 6

1. greater than or equal to 95%
2. 30% men and 20% women
3. IDarucizumab
4. AndeXanet

Question 7

A 68-year-old woman with a history of Roux-en-Y gastric bypass 5 years ago for obesity reported excellent initial weight loss after her bariatric surgery. However, she has now been gaining weight in the last year. Her barium upper GI series is shown in figure A. An upper endoscopy showed a normal esophagus, normal gastrojejunal anastomosis, and normal examined jejunum. An endoscopic photo of her gastric pouch is shown in figure B. Which of the following is the likely cause of patient’s weight gain?

A. Dilated stoma

B. Gastro-gastric fistula

C. Gastric ulcer

D. Gastrojejunal anastomotic stricture

Answer 7

Weight gain after gastric bypass surgery is often multi-factorial and related to diet, exercise, psychosocial behavior and anatomical changes after surgery. The barium upper GI series shows flow of contrast from the gastric pouch to the excluded stomach through a gastro-gastric fistula. This is clearly seen in the endoscopic photo as well which shows a fistulous opening in the pouch leading to the excluded stomach. This is the most likely cause of weight gain in this patient. Endoscopy showed a normal nondilated gastrojejunal anastomosis or stoma. Of note, stricture at the gastrojejunal anastomosis would cause gastric outlet obstruction and would not result in weight gain. The endoscopic photo does not show a gastric ulcer which typically causes abdominal pain, nausea, and vomiting without weight gain.

Question 8

A 41-year-old woman presented with progressive abdominal pain, nausea, and postprandial emesis. Over the past 2 weeks, she has lost 5 lb. Her history is significant for breast augmentation and bariatric weight loss balloon placement 1 month prior. CT scan demonstrates gastric wall pneumatosis with an overdistended balloon in the gastric body with a proximal fluid level. Her only medication is ibuprofen 800 mg every 12 hours for osteoarthritis pain. On exam, her vitals are stable. She has tenderness over the left quadrant but no rebound or guarding. She has active bowel sounds and a soft abdomen (although you can feel a mass over her epigastrium). Which of the following do you tell her?

A. She is having an allergic reaction to the balloon and should take diphenhydramine to manage it.

B. She likely has complications from balloon placement and will need endoscopic removal of the balloon to feel better.

C. Her balloon is in proper position, and she should be worked up for viral gastroenteritis.

D. She should stop taking Ibuprofen, and you will reassess her symptoms in 1 week.

E. She needs the balloon surgically removed as soon as possible.

Answer 8

• pneumatosis, not free air
• complication from balloon placement with imaging demonstrating gastric wall pneumatosis.
• 30 to 40 mg/kg² and who have failed to lose weight with diet and exercise.
• Early complications after balloon placement include prosthetic slippage or perforation.
• Delayed complications include migration of the balloon, infection, partial, or complete obstruction with gastro-esophageal reflux as in this patient, and pressure ulceration. Imaging suggests that the balloon is causing pressure along the gastric wall leading to pneumatosis (an uncommon finding for gastroenteritis).
• Endoscopic removal is a reasonable start with a benign exam and no free air on imaging.
• There are no reported allergic reactions to bariatric balloons to date. NSAIDs can cause ulceration, but the timeline is consistent with a delayed complication from the balloon. It is important to remove the balloon once complications are seen. She is able to undergo endoscopic removal due to benign exam and normal vitals.

Question 9

A 38-year-old man presented for evaluation of elevated liver enzymes. He developed fatigue and was found to have elevated AST and ALT on routine laboratory testing. He has no known past medical history, and there is no family history of known liver disease. He denies alcohol or substance abuse. Physical examination was remarkable for macular erythema and blisters on his hands and neck, with hyperpigmentation on his cheeks and eyebrows.

Laboratory testing revealed:
Hemoglobin 14.1 g/dL (normal: 14-17 g/dL)
Leukocyte count 9,000/µL (normal: 4,000-10,000/µL)
Platelet count 250,000/µL (normal: 150,000-350,000/µL)
ALT 127 U/L (normal: 0-35 U/L)
AST 110 U/L (normal: 0-35 U/L)
Alkaline phosphatase 80 U/L (normal: 36-92 U/L)
Total bilirubin 1.1 mg/dL (normal: 0.3-1.2 mg/dL)
Lipase normal
Hepatitis A IgM: negative
HepBsAg: negative
Anti-HCV: positive

What is the next step in the management of this patient?

A. Measurement of plasma total porphyrin levels

B. Measurement of erythrocyte uroporphyrinogen decarboxylase activity

C. Measurement of urinary delta-aminolevulinic acid

D. Measurement of urinary porphobilinogen

Answer 9

• This is an example of porphyria cutanea tarda (PCT) associated with hepatitis C. Measurement of total porphyrins in plasma (or serum) is the preferred first step in the laboratory evaluation for suspected PCT.
• Measurement of total urine porphyrins on a spot urine sample can also be used, with the caveat that urine porphyrins are more often nonspecifically elevated in other medical conditions. A 24-hour urine collection is not required.
• Some patients have reduced erythrocyte uroporphyrinogen decarboxylase activity, indicating the presence of a heterozygous UROD gene mutation. Urinary ALA is usually normal in PCT and hepatoerythropoietic porphyria (HEP) but may be mildly elevated; urinary PBG is normal.

Question 9

A 38-year-old woman presents to your clinic because she is interested in an endoscopic bariatric procedure for weight loss. She has failed to sustain her weight loss through lifestyle modification. Her past medical history includes controlled acid reflux, large hiatal hernia, and pre-diabetes. Her BMI is 32.5 kg/m². Her medications include PPI 20 mg daily and metformin 500 mg daily. Which endoscopic bariatric procedure would you recommend?

A. Intragastric balloon

B. Endoscopic sleeve gastroplasty

C. Aspiration therapy

D. Thin plastic sleeve lining the first 60 cm of the small bowel

Answer 9

• Both severe acid reflux and a large hiatal hernia are contraindications for intragastric balloon placement. However, for this patient, her acid reflux is well controlled on low-dose PPI.
• Additionally, in contrast to sleeve gastrectomy, acid reflux is not a contraindication for endoscopic sleeve gastroplasty (ESG). In fact, most patients report improvement in their reflux symptoms following ESG.
• Aspiration therapy is approved for patients with a BMI of 35 to 55 kg/m². Therefore, this patient’s BMI is too low for aspiration therapy.
• Last but not least, thin plastic sleeve lining the first 60 cm of the small bowel (e.g., EndoBarrier) is not currently FDA approved and is being performed under investigation only. One of the inclusion criteria for the trial is a history of poorly controlled diabetes. Therefore, a history of pre-diabetes is not sufficient for consideration of EndoBarrier placement under clinical investigation.