Genetics
Which of the following is NOT a possible genetic mutation for a patient with Lynch Syndrome?
A) MLH1
B) MSH2
C) MSH6
D) PMS2
E) STK11
What is E) STK11
(That is the mutation for Peutz Jegher Syndrome)
What is the classic triad for Heyde Syndrome?
What is:
1) GI bleeding from Angiodysplastic lesions
2) Acquired vWD in a patient with
3) Aortic stenosis
Name a medication that can lead to DILI with liver biopsy finding of microvesicular steatosis.
What is:
- Aspirin (Reye syndrome)
- Valproic acid
- Amiodarone
- High dose IV tetracycline
What is the mechanism of action of Resmetirom?
What is thyroid hormone receptor B (THR-B) agonist
Name 3 FDA approved medications for the treatment of PBC
What are: Ursodiol, Obeticholic acid, Elafibranor, Seladelpar
*Elafibranor - PPAR-a/d agonist (FDA approved 6/10/2024
*Seladelpar - PPAR-d agonist (FDA approved 8/14/24)
Which of the following patients is most likely to have a gastrointestinal AVM? (Hint: the question refers to a true congenital AVM rather than an angioectasia)
A) An elderly patient with a systolic heart murmur
B) A younger patient with mucosal telangiectasias
C) A patient with heart failure s/p LVAD
What is B) A younger patient with mucosal telangiectasias
(HHT is associated with congenital AVMs)
What is the diagnostic cutoff for a positive breath test for SIBO based on the North American Consensus as noted in the ACG 2020 clinical guideline (rise in *** ppm by *** minutes)
What is a rise in 20+ ppm by 90 minutes.
Note: The diagnosis of IMO would be a rise in 10+ ppm at any point during testing.
Which of the following patient's is most likely to have clinically significant portal hypertension?
A) A patient with platelets 130, VCTE LSM 19 kPA
B) A patient with platelets 140, MRE LSM 6 kPA
What is B) A patient with platelets 140, MRE LSM 6 kPA
Note difference in scale for a fibroscan (VCTE) liver stiffness measurement and an MRE. 6 kPA for an MRE is very high.
How do calcium oxalate stones form in patients with Crohn's disease?
What is small bowel inflammation (ileum) leads to malabsorption of fat soluble vitamins and bile acids. Less fat absorption leads to more fat in the GIT to bind calcium therefore releasing oxalate. This leads to increased oxalate absorption which then end up in the kidneys and precipitate calcium oxalate stones.
What were the locations of the last 3 ACG conferences?
What is:
1. Vancouver (2023)
2. Charlotte (2022)
3. Las Vegas (2021)
A patient has family history of Peutz Jegher Syndrome and on exam you find perioral pigmentation. At what age should he have an MRCP?
What is 35 years old.
Peutz-Jeghers syndrome: pancreatic screening at age 35 or 10 years earlier than the youngest relative with pancreatic cancer (ASGE 2022 guidelines for pancreatic cancer screening)
Per the Lyon consensus 2.0, what is the most likely diagnosis for a patient with the following results on pH-impedance testing?
- Baseline impedance <1500 ohms, Acid exposure time (AET) >6.0% for 2 days, total reflux episodes >80/day
What is GERD
You are following a liver patient on the inpatient GI service. They have a ferritin of 2700. What is the transferrin saturation (TSAT) cutoff to help determine if this is hemochromatosis or (more likely) due to secondary iron overload?
What is TSAT <45%
Noted in EASL 2022 guidelines for hemochromatosis.
How do PPIs help prevent rebleeding?
Name a microorganism/infectious agent that can present with "Faget's sign" ("temperature-pulse dissociation" or "fever with relative bradycardia").
Preferably a GI related bug!!
What is Salmonella typhi/paratyphi OR Legionella pneumophilia.
A patient had a colonoscopy and 5 juvenile polyps were found. Which of the following mutations are they most likely to have?
A) SMAD4
B) STK11
C) APC
D) MLH1
E) PTEN
What is A) SMAD4
(Juvenile polyposis syndrome)
A patient has elevated liver enzymes. You send a ceruloplasmin which is low. You send a follow up test (24 hr urine copper). What is the conventional diagnostic cutoff for this test to suggest Wilson's disease (in micrograms/24hr)
What is >100 micrograms/24 hr.
Also acceptable is >40micrograms/24 hr which is the upper limit of normal (as mentioned in the 2022 ACG Practice Guidance). As the 100 microgram/24 hr cutoff may miss 16-23% of patients with WD.
You receive a consult for a patient with known liver disease. He has orthodeoxia and platypnea. What would be 2 tests to order to assess for hepatopulmonary syndrome?
What is:
1) Upright ABG
2) TTE w/ bubble study
Why is LR theoretically a better isotonic fluid choice than NS in patient's with pancreatitis, as noted in the ACG 2024 guidelines for the management of acute pancreatitis? (Give two explanations)
What is:
- LR is more pH balanced
- LR provides calcium which binds nonesterified fatty acids (which are associated with more severe disease)
- Wu et al. found patients given LR were less likely to develop SIRS
- Lee et al. found patients given LR were less likely to be admitted to ICU and had a shorter hospitalization
The GI tract makes about 7L of fluid + 2L of oral intake = 9L. A large percentage of that fluid is reabsorbed under normal physiologic conditions. What percentage of fluid ends up in the stool?
What is 1%
Which of the following genotype is most likely to be found in an Irish male with a ferritin level 400 and a transferrin saturation of 75%?
A) H63D heterozygotę
B) H63D homozygote
C) C282Y/H63D compound heterozygote
D) Other
What is other: C282Y homozygote
Which of the following IBD extraintestinal manifestations is independent of disease activity?
A) Erythema nodosum
B) Episcleritis
C) Uveitis
What is C) Uveitis
Per the 2024 AASLD Practice Guidance on risk stratification and management of portal hypertension and varices in cirrhosis, which statin has the most data for its use in decompensated cirrhosis with a possible benefit in decreasing portal pressures?
What is simvastatin (20 mg daily max in decompensated cirrhotics)
According to the Baveno 7 consensus, why is carvedilol the preferred NSBB in compensated cirrhotics?
What is: carvedilol has intrinsic anti-alpha adrenergic vasodilatory effects that contribute to its greater portal pressure reducing effect.
- More effective at reducing HVPG
- Tendency towards greater benefit in preventing decompensation & better tolerance than traditional NSBBs
- Has been demonstrated to improve survival
What is the ferritin goal during maintenance for phlebotomy for a patient with hereditary hemochromatosis?
What is 50-100 μg/L.
Noted in EASL 2022 guidelines for hemochromatosis.