GI Path Flashcards
(264 cards)
1
Q
A 55-year-old man presents with a solitary enlarged lymph node over his left clavicle. The lymph node measures 8 mm in greatest dimension. A biopsy from the lymph node shows infiltrating round, mucin-filled cells with flat, peripheral nuclei (signet ring cells).
What organ is the most likely origin of this malignancy?
A
Stomach
“Signet cell carcinoma”
Diffuse type of gastric cancer infiltrates the stomach wall (linitis plastica) and histologically shows signet ring cells (round, mucin-filled cells with flat, peripheral nuclei). Diffuse type of gastric cancer (signet cell carcinoma) is associated with mutations involving E-cadherin (cells lose the ability to adhere together).
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Where are parietal cells located in stomach (anatomical location)?
A
The superficial upper glandular layer of the gastric body and fundus
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Autoimmune metaplastic atrophic gastritis is associated with an increase risk for what malignancies?
A
Gastric adenocarcinoma and carcinoid tumors
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Risk factors for esophageal adenocarcinoma
A
Long-standing GERD
Obesity
Smoking
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Histologic marker for Barrett esophagus
A
Presence of goblet cells
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What disease is characterized by reduced numbers of inhibitory ganglion cells in the myenteric (Auerbach) plexus?
A
Achalasia
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Where is the Auerback (myenteric plexus) located in the esophagus?
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Between the inner circular and outer longitudinal layers of the muscularis propria in the esophageal wall
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CMV esophagitis
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Painful (vs. varices which is painless)
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Sliding hiatal hernia
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Know cause - circumferential laxity of phrenoesophageal membrane
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## Footnote
Mets to ovary - When linitis plastica (a type of gastric cancer) metastasizes to the ovary, it's known as a Krukenberg tumor.
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Signet cell carcinoma
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Menetrier disease
## Footnote
Associated w/ overproduction of TGF-alpha and cause parietal cell hypoplasia
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An activating (gain-of-function) mutation involving which one of the following genes is most likely responsible for colon adenoma formation and also the transformation of an early adenoma into a late adenoma (ie, increasing size of the adenoma)?
KRAS
## Footnote
The adenoma to carcinoma sequence is a series of gene mutations that leads to the development of colon adenocarcinoma. Increase in the size of the adenoma results from nutation of the KRAS protooncogene, while malignant transformation of adenoma into carcinoma requires mutation of TP53, an antioncogene (tumor suppressor gene) that codes for protein p53, which triggers apoptosis of cells with damaged DNA.
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What type of colorectal cancer is typically more aggressive with a higher histopathologic grade, often evolves from flat (nonpolypoid) lesions, and is frequently multifocal origin?
Colitis-associated colorectal cancer
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What gene is responsible for familial adenomatous polyposis (FAP) - and what's the mode of inheritance?
-Germline mutation to the tumor suppressor gene adenomatous polyposis coli (APC)
-Inherited in an autosomal dominant fashion
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Inheritance pattern of Lynch syndrome:
Autosomal dominant
## Footnote
Most cases of Lynch syndrome involve mutations in MSH2 and MLH1, which are part of the mismatch repair system.
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Most common type of colon polyps
Hyperplastic
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A pathologist examining microscopic routine sections of the appendix finds a tumor in the tip of the appendix that is composed of uniform tumor cells with round nuclei, salt and pepper chromatin, and eosinophilic cytoplasm. What is the best diagnosis for this tumor?
Carcinoid tumor
## Footnote
A biopsy of the appendix that shows a small mass in the tip composed of uniform tumor cells with round nuclei, salt and pepper chromatin, and eosinophilic cytoplasm, is consistent with a well-differentiated neuroendocrine tumor (WDNET) (ie, carcinoid tumor).
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## Footnote
Pre-malignant
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Adenoma to carcinoma sequence
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Adult w/ Hx of renal transplant - with intermittent rectal bleeding (worse w/ defecation), and ulcerated mass found extending from anal verge into rectum.
Anal carcinoma
## Footnote
Typically squamous cell carcinoma - associated with HPV 16/18
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A 39-year-old woman presents with prolonged abdominal cramps and periodic facial flushing. Workup reveals several masses within her liver and in her ileum. A biopsy from one of these masses reveals nests of bland-appearing monotonous cells that have a salt-and-pepper appearance of chromatin. These tumor cells stained positively with chromogranin A. Which one of the listed substances is most likely to be elevated in the urine of this individual as a result of her disease?
5-hydroxyindoleacetic acid (5-HIAA)
## Footnote
An adult with recurrent facial flushing, wheezing, and watery diarrhea, along with increased 5-hydroxyindoleacetic acid (5-HIAA) in the urine, likely has carcinoid syndrome. Intestinal carcinoids that metastasize to the liver (metastatic carcinoid) release vasoactive substances that avoid first-pass metabolism, resulting in carcinoid syndrome (eg, flushing, diarrhea, bronchospasm).
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A 60-year-old healthy man undergoes a screening colonoscopy. He has been staying active, does not smoke, denies any bloody or dark stools, changes in stool, abdominal pain, or fatigue. A large pedunculated growth is found in the sigmoid colon. A biopsy of that mass reveals a polyp with fingerlike projections with fibrovascular cores lined by dysplastic epithelium. What is the best diagnosis for this lesion?
Villous adenoma
## Footnote
A colon biopsy that demonstrates a polyp composed of long glands with finger-like (villous) projections extending from the surface as well as dysplastic epithelium, is characteristic of a villous adenoma, a type of colon polyp that tends to be larger, sessile, and more dysplastic (greater association with malignant transformation)
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Lynch syndrome, aka hereditary non-polyposis colorectal cancer syndrome (HNPCC), results from a germline mutation in one of four mismatch repair (MMR) genes called MLH1, MSH2, MSH6, and PMS2. An individual with Lynch syndrome is most at risk for developing which one of the following types of cancer?
Mucinous adenocarcinoma of the colon
## Footnote
In addition to having an increased risk of colorectal cancer (especially mucinous adenocarcinomas on the right side of the colon), individuals with the Lynch syndrome are more likely to develop other cancers such as endometrial and ovarian carcinomas.
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An adult undergo a screening endoscopy because of a family history of colon cancer and a physical examination that finds pigmented lesions around the oral cavity. Endoscopy finds numerous hamartomatous polyps of the small intestines. What is the most likely cause of these clinical findings?
Peutz-Jeghers syndrome
## Footnote
The combination of multiple gastrointestinal polyps, mucocutaneous discoloration (pigmented lesions on the lips), and a family history of gastrointestinal malignancy is consistent with Peutz-Jeghers syndrome (PJS). Hamartomatous polyps are the most likely type of GI polyp to be found in a patient with PJS.
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Abx's associated with *Clostridium difficile* infection:
-Cephalasporins
-Fluoroquinolones
-Clindamycin
-Ampicillin
## Footnote
Penicillins +/-lactamase-inhibitor combinations such as ticarcillin/clavulanate and piperacillin/tazobactam pose significantly less risk
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Toxins associated with *Clostridium difficile*
Toxin A - exotoxin
Toxin B - cytotoxin
Binary toxin CDT (increases bacterial adhesion to host cells)
| Both toxins AB glucosylate the GTP-binding proteins of the Rho subfamily
## Footnote
These toxins initiate processes resulting in the disruption of epithelial-cell barrier function, diarrhea, and pseudomembrane formation
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Why does *Clostridium difficile* infection result in fluid leakage from endothelial cells
Toxins TcdA and TcdB, causes fluid leakage from endothelial cells by disrupting the integrity of the intestinal lining and disrupting tight junctions.
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The pseudomembranes of PMC are confined to what layer of the intestines?
The colonic mucosa - appear as 1-2 mm whitish-yellow plaques
## Footnote
The whole colon is usually involved, but 10% of patients have rectal sparing
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Histological characterization of PMC
Pseudomembranes have a mucosal attachment point and contain necrotic leukocytes, fibrin, mucus, and cellular debris.
The epithelium is eroded and necrotic in focal areas, with neutrophil infiltration of the mucosa.
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Most sensitive test for *C. difficile*
Stool culture
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What test detects toxigenic C. difficile in stool; is newly approved for clinical testing, but appears to be more sensitive than enzyme immunoassay toxin testing and at least as specific
PCR for *C. difficile* toxin B gene in stool
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What test detects glutamate DH found in toxigenic and nontoxigenic strains of C. difficile (and others)
Enzyme immunoassay for *C. difficile* common antigen in stool
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*Vibrio cholarae* characteristics:
* Gram (-) curved rods
* Polar flagella
* Oxidase (+)
* Facultative anaerobes
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*Vibrio cholarae* tropism:
Lumen of the small intestines
## Footnote
Enteric Pathogen (Extracellular/Non-invasive/Toxemia)
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How to distinguish *Vibrio cholarae* from other Vibrio species
Yellow colonies on Thiosulfate-Citrate-Bile Salts-Sucrose Agar (TCBS), **other Vibrio species do not ferment sucrose** and are greenish color.
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What serotypes of Vibrio produce cholera toxin?
**O1 & O139** (associated w/ epidemics)
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Toxin subunit that ADP-ribosylates the Galpha-s protein, constituitevly activating adenylate cyclase?
Subunit A of cholera toxin
## Footnote
increases Na+ dependent Cl- secretion into the lumen of the intestine
o Prevention of Na+ and Cl- absorption by NaClcotransport mechanism
o Rapid secretion of water and electrolytes into lumen of
small intestine
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Clinical effects of cholera toxin
* Secreted fluid is isotonic with 2X the bicarbonate and 4 to 8X the K+ concentration of plasma
## Footnote
V. cholerae bacteria that lack cholera toxin can still produce significant diarrhea due to the action of other toxins it has; however, the diarrhea is much less severe.
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Dx of *Vibrio cholerae*
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Tx of *Vibrio cholerae*
-Measure fluid loss and replete
-Doxy is sensitive (azithromycin if not)
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Rotavirus structure
Naked, double-shelled nucleocapsids
Linear dsRNA (segmented - can undergo genome reassortment)
## Footnote
Replication occurs in the cytoplasm - cannot use host ribosomes
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Norovirus structure
Naked, icosahedral capsid
(+) ssRNA genome
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Norovirus tropism
INfect jejunal villus epithelial cells - causes villus blunting
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A 45-year-old female presents to her primary care physician complaining of sudden onset vomiting and diarrhea. She mentions attending a family picnic gathering a few days ago where several relatives experienced similar symptoms about two days after the picnic like she did. On examination, she appears dehydrated but otherwise stable. What is the most likely cause of the gastrointestinal illness in this scenario?
Norwalk virus
| Norwalk is most common in US
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What strain of *C. difficile* is most likely to have the worst outcome?
An infection with the NAP1 *C. difficile* strain
## Footnote
The epidemic organism is characterized by (1) an ability to produce 16–23 times as much toxin A and toxin B as control strains in vitro; (2) the presence of a third toxin (binary toxin CDT); and (3) high-level resistance to all fluoroquinolones.
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In 1933 at the Chicago World Fair, there was an outbreak of diarrhea that was linked to contaminated drinking water. There were more than 1000 cases and about 100 deaths associated with the infection. If one were to have examined the stool contents of the ill individuals, both cysts with 4-nuclei and trophozoites with a single-nuclei would have been evident. Which of the following would best match these characteristics?
a) Vibrio cholera
b) Entamoeba histolytica
c) Cryptosporidium parvum
d) Clostridium difficile
e) Giardia lamblia
b) Entamoeba histolytica
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In Mexico City, there is a severe outbreak of infantile gastroenteritis in a day-care center. The gastroenteritis is characterized by watery, non-inflammatory diarrhea, fever, abdominal pain, and vomiting. The symptoms last 1-2 weeks in the afflicted children only. Which of the following is the MOST LIKELY etiologic agent?
Rotavirus
## Footnote
Rotavirus affects primarily children and duration is longer (1-2 days of sxs for Norwalk)
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A young adult from South East Asia visiting the US presents with a curious skin lesion on her abdomen. Her history indicates that in previous weeks, she had an extensive bout of diarrhea that was at first very watery, but turned bloody over time with considerable pain; however, she stated that the symptoms resolved and she thought nothing more about it. Examination of her stool sample showed cysts with four nuclei. What is most likely the cause of the skin lesion?
*Entamoeba histolytica*
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In 1933 at the Chicago World Fair, there was an outbreak of diarrhea that was linked to contaminated drinking water. There were more than 1000 cases and about 100 deaths associated with the infection. If one were to have examined the stool contents of the ill individuals, both cysts with 4-nuclei and trophozoites with a single-nuclei would have been evident. Which of the following would best match these characteristics?
A. *Vibrio cholera*
B. *Entamoeba histolytica*
C. *Cryptosporidium parvum*
D. *Clostrium difficile*
E. *Giardia lamblia*
B. *Entamoeba histolytica*
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A. Stool bacteria culture
D. Stool for C-Diff PCR
| PPi's increase risk of getting C-Diff
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A. Colonization
## Footnote
Treatment for symptomatic
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What is the recurrence rate of *C. difficile*?
15-30%
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A. Probiotics
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C. Flask-shaped lesions
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B. Liver - leads to diffuse coagulative necrosis of the liver
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Which of the following is an infectious agent that can cause a protracted diarrhea and is associated with failure to thrive in children?
A. Giardia
B. Rotavirus
C. Norovirus
D. Vibrio cholera
A. Giardia
B. Rotavirus
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Rule of F's for Giardia
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D. Cryptosporidium
## Footnote
Cryptosporidium oocysts contain mycolic acids - stain acid fast
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B. Malabsorption
C. Villus blunting
## Footnote
Cryptosporidium - herald disease for AIDs
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Tx for *Cryptosporidium*
Nitazoxanide - interferes with anaerobic metabolism
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C. Oxidase negative
## Footnote
*Campylobacter* and *Vibrio* are both oxidase positive
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D. G-alpha-s
## Footnote
Increase in cAMP leads to opening of CFTR chloride channel (leads to **efflux of chloride**) and blockade of NaCl channels (NaCl cannot be reabsorbed)
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*Vibrio cholerae* can ferment what substrate?
| What agar demonstrates this?
Sucrose
## Footnote
Other Vibrio species do not ferment sucrose
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A. Increases bacterial adhesion
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What is a volvulus?
wisting of intestines around its mesentery
## Footnote
"coffee bean" sign on KUB
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What is intussusception and who is most at risk?
Telescoping of bowel into another segment (usually at the ileocecal junction)****
Risk: weaning infants and adults with a polypoid mass.
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What is the classic triad of intussusception?
-Sudden colicky abdominal pain
-abdominal distention
-currant jelly stool
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What imaging finding is typical in intussusception?
"Target sign" on ultrasound or CT.
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What stain is used to screen for steatorrhea?
Sudan III stain (detects fat).
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What is celiac disease and which HLA type is associated?
Autoimmune sensitivity to gliadin (gluten); associated with **HLA-DQ2**
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What are two early histological findings in celiac disease?
Increased intraepithelial lymphocytes and later villous atrophy.
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Name three comorbid conditions associated with celiac disease.
-Dermatits herpetformis
-IgA nephropathy
-Enteropathy-associated T-cell lymphoma
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How does tropical sprue differ from celiac disease?
Tropical sprue is likely infectious and responds to antibiotics.
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What is the main diagnostic test for lactose intolerance?
Lactose hydrogen breath test.
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What is dumping syndrome and what causes it?
Rapid gastric emptying after gastric surgery; rapid carbohydrate absorption leads to insulin surge and hypoglycemia.
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What is abetalipoproteinemia and what histologic finding is classic?
Deficiency in ApoB synthesis; lipid-laden enterocytes and acanthocytes on smear.
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What is the typical location and pattern of ulcerative colitis?
Starts in the rectum and extends proximally without skip lesions.
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What lab finding is associated with ulcerative colitis?
Positive p-ANCA
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What are two major complications of ulcerative colitis?
Colorectal cancer and toxic megacolon.
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What is the hallmark distribution of Crohn disease?
Skip lesions and transmural inflammation, often involving terminal ileum.
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What is a characteristic lab marker in Crohn disease?
Anti-Saccharomyces cerevisiae antibodies (ASCA).
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What are common signs of Crohn disease?
Non-bloody diarrhea, RLQ abdominal pain, weight loss, malabsorption (especially B12 and fat-soluble vitamins).
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What classic imaging finding is associated with Crohn disease?
"String sign" due to bowel wall thickening and narrowing.
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What distinguishes IBS from IBD?
IBS lacks inflammation and presents with pain that improves with defecation; no nighttime diarrhea.
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What is ischemic colitis and who is most at risk?
Decreased blood supply to the large intestines, especially at watershed areas like the splenic flexure; more common in elderly.
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What is the hallmark imaging finding of ischemic colitis?
"Thumbprint sign" on imaging due to mucosal edema and hemorrhage.
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What artery is commonly occluded in acute mesenteric ischemia?
Superior mesenteric artery.
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What are the signs of acute mesenteric ischemia?
Acute severe abdominal pain, bloody diarrhea, decreased bowel sounds.
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What is the key histologic feature of melanosis coli?
Lipofuscin pigment in the lamina propria.
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What causes melanosis coli?
Chronic laxative use (especially anthraquinones).
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What are the two types of microscopic colitis?
**Collagenous colitis** (thickened collagen layer) and **lymphocytic colitis** (increased intraepithelial lymphocytes).
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What is a mimic of appendicitis caused by Yersinia enterocolitica?
Mesenteric adenitis.
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What is a trichobezoar and what is it associated with?
Accumulation of ingested hair; associated with trichotillomania.
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What is mucosal prolapse syndrome and a major risk factor?
Rectal mucosal prolapse due to mucosal ischemia; risk factor: multiple childbirths.
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What serologic test confirms celiac disease?
Elevated tissue transglutaminase IgA antibodies.
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What dermatologic condition is strongly associated with celiac disease?
Dermatitis herpetiformis
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What HLA types are associated with celiac disease?
HLA-DQ2 and HLA-DQ8
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What is the key diagnostic feature of IBS?
Recurrent abdominal pain associated with ≥2: pain improving with defecation, change in stool frequency, or change in stool form.
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What cytokine plays a central role in Crohn disease pathogenesis?
TNF-alpha
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What causes diverticular bleeding?
Disruption of vasa recta in the colonic wall.
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What area of the colon is most susceptible to ischemic colitis?
Watershed areas: splenic flexure and rectosigmoid junction.
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What is angiodysplasia?
Dilated, tortuous submucosal vessels, often seen in older adults, especially with aortic stenosis.
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What smear finding is classic for abetalipoproteinemia?
Acanthocytes (spur cells).
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What is the histological hallmark of Whipple disease?
PAS-positive foamy macrophages in the lamina propria.
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What bacteria causes Whipple disease?
*Tropheryma whipplei*
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What cells are initially invaded by Shigella species?
M cells over Peyer's patches.
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What two bacteria are most associated with intra-abdominal abscesses?
Bacteroides fragilis and Escherichia coli.
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What staining method identifies Cryptosporidium in stool?
Acid-fast stain
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What is gallstone ileus and what radiologic finding is common
Gallstone obstructing bowel; pneumobilia (air in biliary tree).
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What is the most sensitive test for detecting malabsorption?
Sudan III stain for fecal fat.
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What causes dumping syndrome after gastric surgery?
Rapid delivery of hyperosmolar chyme into small intestine, causing fluid shifts.
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What is diabetic diarrhea and what causes it?
**Secretory diarrhea** due to diabetic autonomic neuropathy.
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Gross of intussesception
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Young male presents with iron-deficiency anemia should be worked up for?
Celiac disease
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## Footnote
p-ANCA = myeloperoxidase Ab
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A 23-year-old young man presents with bloating, abdominal pain, flatulence, and chronic diarrhea. He states that his stools are foul smelling and float in the water of the toilet. Laboratory examination reveals the presence of IgA anti-tissue transglutaminase antibodies. A small intestinal biopsy reveals intraepithelial lymphocytosis and villous atrophy. What is the best diagnosis?
Celiac disease
## Footnote
HLA-associated autoimmune disorder triggered by dietary gluten that develops almost exclusively in patients with HLA-DQ2 or -DQ8 serotype
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A 24-year-old man with a several year history of intermittent abdominal pain and diarrhea presents with bowel obstruction. Emergency surgery finds bowel obstruction with peritoneal adhesions and ileoileal fistulas. Histologic sections of the resected bowel shows transmural inflammation along rare non-caseating granulomas. What is the best diagnosis?
Crohn disease
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A patient presents with signs of malabsorption. Examination of the peripheral smear reveals that many of the red blood cells have numerous spikes on their surface (these cells being called acanthocytes). A mucosal biopsy of the small intestines shows enterocytes with clear and foamy cytoplasm that is more prominent at the tips of the villi. What is the best diagnosis?
Abetalipoproteinemia
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A 37-year-old woman presents with fever, migratory polyarthritis, generalized lymphadenopathy, and steatorrhea. A small intestinal biopsy reveals numerous macrophages having PAS-positive cytoplasm within the lamina propria of the small intestines. Antibiotic therapy with tetracycline produces a prompt response in her signs. What is the best diagnosis for this individual?
Whipple disease
## Footnote
A patient with abdominal pain and malabsorption along with an intestinal biopsy showing foamy macrophages in the lamina propria filled with bacilli that are PAS positive likely has Whipple disease, which is caused by a chronic bacterial infection with Tropheryma whipplei, a gram-positive bacterium. Treatment is with antibiotics.
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Histologic characteristics of Crohn disease
- Non-caseating granulomas along with
- Transmural inflammation
- Distortion of normal mucosal architecture
- Paneth cell metaplasia
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How does Crohn disease result in GI strictures?
Bowel wall edema, fibrosis, and hypertrophy (thickening) of the muscularis mucosae, which narrows the intestinal lumen.
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During fetal development, when does the midgut herniate through the umbilicus?
| What artery supplies the midgut?
Week 6
| SMA supplies the midgut
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What symptoms are most likely to result from a pancreatic islet cell tumor that secretes vasoactive intestinal polypeptide (VIPoma)?
* Watery diarrhea
* Hypokalemia
* Achlorhydria
## Footnote
Signs of VIPoma include watery diarrhea, hypokalemia, and achlorhydria, which is called the WDHA syndrome (pancreatic cholera).
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A 45-year-old man with hyperglycemia due to mild, non-ketotic diabetes mellitus along with a necrolytic migratory skin rash is found to have a 3 cm mass in the tail of the pancreas. These clinical signs and symptoms are most characteristic of what tumor?
Glucagonoma
## Footnote
An adult with hyperglycemia (often as newly diagnosed diabetes mellitus) and necrolytic migratory erythema (blistering erythematous plaques with central clearing) affecting the groin, face, and extremities likely has a glucagonoma , a rare tumor arising from the alpha cells of the pancreatic islets of Langerhans. The diagnosis is made by detecting elevated glucagon levels.
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Pancreatic adenocarcinoma is associated w/ elevated levels of what tumor marker?
CA 19-9
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A 31-year-old man who has had a history of repeated attacks of acute pancreatitis related to binge drinking is found to have a non-motile, fluid-filled mass in the posterior portion of the pancreas. The mass is resected and is found to be a cystic structure that is lined by fibrous and granulation tissue. What is the best diagnosis for this lesion?
Pancreatic pseudocyst
## Footnote
A cystic lesion found in the area of the pancreas is suggestive of a pancreatic pseudocyst. Pancreatic pseudocyst, which is a common complication of pancreatitis, is lined by fibrous and granulation tissue (not by epithelium as seen in true cysts).
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An abdominal CT scan from 67-year-old man who presented with intermittent RUQ abdominal pain reveals a rim of calcification in the gallbladder wall. Lab findings were within normal limits. This gallbladder abnormality is most closely associated with an increased risk for what condition?
Gallbladder adenocarcinoma
## Footnote
A firm, palpable gallbladder and extensive calcification throughout the gallbladder wall is characteristic of porcelain gallbladder, a potential manifestation of chronic cholecystitis and is often found in association with multiple gallstones. Porcelain gallbladder is associated with an increased risk of gallbladder adenocarcinoma, therefore, treatment for a porcelain gallbladder is surgical removal of the gallbladder.
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Pathenogenesis of cholesterol gall stones:
| Diagram
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## Footnote
Tx = surgical resection
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What is a pancreas divisum?
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What GI complication is associated w/ CF in newborns?
Meconium ileus
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What GU abnormaility is associated with males dx'd with CF?
Absence of the vas deferens
## Footnote
Azospermia
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What clinical signs are associated with hemorrhagic acute pancreatitis?
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True cycst are differentiated from pseudocyts by?
True cysts are internally lined by epithelial cells
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What labs are elevated with obstructive jaundice?
* ALP
* Bilirubin
## Footnote
Caused by either gallstones or pancreatic cancer
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Is the gallbladder palpable due to chronic cholecystitis
No
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Is the gallbladder palpable due to obstruction caused by pancreatic cancer?
Yes
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Insulinoma
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Insulinoma
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What is a benign condition that presents with marked jaundice due to congenital cholestasis accompanied by conjugated hyperbilirubinemia?
Dubin-Johnson syndrome
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What is the rate-limiting step in bile acid synthesis?
7α-hydroxylase or CYP7A1, an enzyme found only in the liver
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Primary bile salts are synthesized by conjugating the primary bile acids with?
Glycine or taurine (a sulfated amino acid).
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Most common bile acids
**Cholic acid** and **chenodeoxycholic acid**,
are called primary bile acids
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Niemann-Pick C1-like1 protein (NPC1L1) inhibitor:
Ezetimibe
| Prevents absorption of cholesterol
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________ can be absorbed directly into the portal blood and are transported as free fatty acids
bound to serum albumin.
Short and medium-chain-length fatty acids can be absorbed directly into the portal blood and are transported as free fatty acids bound to serum albumin.
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Secondary bile acids/salts
**Deoxycholate** (from cholate) and **lithocholate** (from chenodeoxycholate)
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Canalicular ABC
transporters are used for
Excretion of bile salts from hepatocytes
## Footnote
These transporters have broad specificity and transport bile acids, phospholipids, and other organic molecules, such as steroids, anticancer drugs, glutathione, and conjugated or free bilirubin
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Sinusoidal ABC transporters are used for
Reuptake of bile acids from portal circulation
## Footnote
These transporters have broad specificity and transport bile acids, phospholipids, and other organic molecules, such as steroids, anticancer drugs, glutathione, and conjugated or free bilirubin
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Acetaminophen tox - hepatocellular necrosis (mostly affects acinar zone III)
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Main cause of ascites with portal HTN?
Splanchnic arterial vasodilation
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Bilirubin metabolish
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CMV hepatitis
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What is the next course of action when serology returns + for HBc IgM, but negative for all other Ag/Ab?
Acute infection within the window period - supportive Tx
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The gross findings of a liver reveal an classic nutmeg appearance due to passive hepatic congestion in centrilobular regions (zone 3). What is the most likely cause of this liver abnormality?
Right-sided heart failure
## Footnote
A liver biopsy that shows passive hepatic congestion in centrilobular regions (zone 3) is evidence of congestive hepatopathy, which is a complication of right-sided heart failure. The centrilobular congestion, combined with relatively normal-appearing periportal regions (zone 1), creates an overall heterogenous gross appearance referred to as nutmeg liver.
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A moderately elevated alkaline phosphatase of unclear etiology should be followed up with which one of the following lab tests?
Gamma-glutamyl transferase
## Footnote
Bone and liver are the primary sources of alkaline phosphatase. A moderately elevated alkaline phosphatase of unclear etiology should be followed up with gamma-glutamyl transpeptidase, which is useful in determining whether an elevated alkaline phosphatase is of hepatic or bone origin.
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A 41-year-old man with a history of intravenous drug use presents for a routine health check. He feels well and has no current symptoms. Laboratory testing reveals elevated alanine aminotransferase (ALT) and aspartate aminotransferase (AST). Hepatitis C virus (HCV) antibody is positive. A follow-up HCV RNA assay confirms detectable viral RNA. He has never received antiviral therapy. What is the most likely long-term outcome of this patient's condition without treatment?
Lifelong persistent infection with possible progression to cirrhosis
## Footnote
The most common outcome of hepatitis C infection, which can be confirmed with HCV RNA assay, without antiviral treatment is lifelong persistent infection (ie, chronic hepatitis C). Of those with chronic hepatitis C, approximately 20% develop cirrhosis, and only a minority experiences complications (eg, ascites, variceal bleeding, hepatocellular carcinoma).
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A 28-year-old woman in her third trimester of pregnancy presents to the clinic with a 5-day history of fever, fatigue, nausea, and right upper quadrant abdominal pain. She recently returned from visiting relatives in a rural region of South Asia. On exam, she appears mildly jaundiced. Laboratory studies reveal elevated AST and ALT levels, and her bilirubin is also increased. She is admitted for monitoring. Over the next several days, her condition deteriorates, and she develops signs of acute liver failure. What virus is the most likely cause of her condition?
Hepatitis E virus
## Footnote
A high mortality rate observed in infected pregnant women is suggestive of infection with hepatitis E virus, which is an unenveloped, single-stranded RNA virus spread through the fecal-oral route. In contrast to the disease in pregnant women, hepatitis E infection is typically self-limited and not associated with either chronic liver disease or a carrier state (like hepatitis A infection).
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What causes Budd-Chiari syndrome?
Thrombosis of hepatic vein
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n adult with a history of erythrocytosis who presents with the acute onset of tender hepatomegaly with elevated serum transaminases without jugular venous distention likely has?
Budd-Chiari syndrome
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Which one of the following abnormalities would most likely result from the obstruction of blood flow through the hepatic sinusoids by deposits of collagen from the cells of Ito (cirrhosis)?
A. Atrophy of the tongue
B. Bronze discoloration of the skin
C. Red pulp expansion of the spleen
C. Red pulp expansion of the spleen
## Footnote
Portal hypertension, as seen in alcoholic liver disease (cirrhosis), produces splenomegaly by causing congestion of blood within the spleen, which produces red pulp expansion. Hematemesis, orthostatic symptoms, and normocytic anemia are seen with acute bleeding from esophageal varices.
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A liver biopsy from an adult with a history of alcohol use who presented with fever, right upper quadrant pain, and jaundice, shows marked intrahepatic neutrophil infiltration, hepatocellular ballooning, Mallory bodies, and steatosis. Further work-up finds elevated aminotransferases with an AST/ALT ratio greater than 2:1. What is the most likely cause of these findings?
Alcoholic hepatitis
## Footnote
An adult with a history of heavy alcohol use who presents with fever, right upper quadrant pain, and jaundice along with elevated aminotransferases with an AST/ALT ratio >2:1 likely has alcoholic hepatitis. The disproportionate elevation of AST relative to ALT is caused by ethanol-induced mitochondrial damage (in hepatocytes, large amounts of AST are found in the mitochondria whereas ALT is present only in the cytosol). Histopathology is characterized by marked intrahepatic neutrophil infiltration, Mallory bodies (alcoholic hyaline), and steatosis.
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A 5-year-old girl presents because of severe vomiting that suddenly developed 5 days after she had a viral infection. Upon questioning, her parents indicate that she was given aspirin for several days to treat a fever that occurred with this viral illness. She is hospitalized and quickly develops signs of cerebral edema. Liver tissue from this young girl reveals microvesicular steatosis. What is the best diagnosis?
Reye syndrome
## Footnote
A child with a febrile illness (eg, influenza, varicella) treated with salicylates (eg, aspirin) is at risk for developing Reye syndrome, which is characterized by liver failure and encephalopathy. Aspirin-induced mitochondrial dysfunction causes impaired fatty acid metabolism and microvesicular steatosis of the liver.
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An adult non-smoker with signs of panlobular emphysema and liver dysfunction likely has?
Alpha-1-antitrypsin deficiency
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Histologic findings of primary biliary cholangitis:
Dense lymphocytic inflammation of portal tracts with granulomatous destruction of interlobular bile ducts
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What sxs characterize primary biliary cholangitis?
- Fatigue
- Pruritus
- Jaundice
- Cholestasis (i.e. increase ALP, direct hyperbilirubinemia)
- Antimitochorndrial antibodies
## Footnote
**Anti-smooth muscle antibodies are seen in lupoid hepatitis **
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What type of HSR is Schistosoma infection?
Type II
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What disease process is histologically characterized by "fibrosis and nodular parenchymal regeneration?"
Cirrhosis
| Parenchymal regeneration = increased risk for cancer
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Kayser-Fleischer rings
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Hepatocellular carcinoma (intracytoplasmic bile is characteristic)
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A 42-year-old man comes to the clinic due to increasing abdominal distension and discomfort. Exam shows splenomegaly and prominent abdominal wall veins. Esophageal varices are seen on endoscopy. Liver biopsy is normal. What is the most likely cause of his findings?
Portal vein thrombosis
## Footnote
A patient with portal hypertension, splenomegaly, and varicosities at portocaval anastomoses along with a normal liver biopsy likely has portal vein thrombosis, which does not cause histologic changes to the hepatic parenchyma. Ascites is uncommon as the obstruction is presinusoidal; ascites typically only develops in conditions that cause sinusoidal hypertension.
257
A 62-year-old man with liver failure secondary to cirrhosis develops a pungent odor in his breath (fetor hepaticus). He is also noted to have marked ascites, gynecomastia, spider angiomas, and palmar erythema. His serum ammonia levels are found to be elevated. The gynecomastia, palmar erythema, and spider angiomas in this patient most likely developed due to which one of the following?
Impaired estrogen metabolism
## Footnote
In patients with cirrhosis, gynecomastia and palmar erythema often occurs due to hyperestrinism, which can also promote the formation of spider angiomata, which are subcutaneous vascular lesions consisting of a central arteriole surrounded by smaller vessels that blanch on compression. Cirrhosis increases adrenal production of estrogen precursors, which are subsequently aromatized to estrogens, which induce sex hormone-binding globulin production, which results in increased testosterone binding and a further decrease in the free testosterone/estrogen ratio
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A 49-year-old man presents with slowly progressing RUQ pain in his abdomen. Physical examination reveals his liver to be enlarged and tender. A CT scan reveals a large 6.5 mass within the right lobe of his liver. A biopsy of this mass reveals abnormal thickened trabecular plates composed of atypical hepatocytes (some with intracytoplasmic bile). Elevation in the serum of what is most characteristic of this hepatic lesion?
Alpha fetoprotein (AFP)
## Footnote
Biopsy of a liver mass in a patient with cirrhosis and worsening signs that shows abnormal thickened trabecular plates composed of atypical hepatocytes (some with intracytoplasmic bile) is consistent with hepatocellular carcinoma. Alpha-fetoprotein (AFP) is a serum tumor marker that is often moderately elevated in patients with chronic viral hepatitis (especially hepatitis C virus), but markedly elevated levels or a sudden rise in level can be a sign of hepatocellular carcinoma.
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Exposure to polyvinyl chloride is most closely associated with the development of what hepatic malignancy?
Angiosarcoma
## Footnote
An older man with a history of working in a factor that produced polyvinyl chloride who presents with abdominal pain, weight loss, and a hepatic mass, likely has a hepatic angiosarcoma, which is an aggressive vascular malignancy associated with exposure to vinyl chloride. The tumor cells express CD31, an endothelial cell marker important for leukocyte transmigration.
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What increases peripheral lipolysis in metabolic syndrome?
Insulin resistance
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Why is ferritin elevated in alcoholic hepatitis?
Ferritin is an acute phase protein
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What inheritable liver disease is associated with pyschiatric abnormalities (especially in adolescence)?
Wilson's
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What does palmar erythema and spider angiomas share in common as a result of liver disease?
Increase estrogen is causative of both
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What is a poor prognostic indicator in liver disease?
Increased PT-INR
