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Step 1 Miscellanous > Pathoma Gastrointestinal Pathology > Flashcards

Pathoma Gastrointestinal Pathology Flashcards

1
Q

What is the triad of Behcet syndrome?

A
  1. Recurrent aphthous ulcers
  2. Genital ulcers
  3. Uveitis causing visual disturbances
    - > etiology is unknown
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2
Q

What are the risk factors for squamous cell carcinoma in the oral cavity? Where does it most often arise?

A

Tobacco smoking and alcohol

Chewing betel nut (Asian cultures it’s like chewing tobacco)

Most often arises in the floor of the mouth

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3
Q

What does erythroplakia look like and is it more or less worrying than leukoplakia?

A

Smooth, soft, flat, or slightly eroded red patch which is not otherwise accounted for (looks like a pizza burn)

More worrying -> often highly dysplastic or representing squamous cell carcinoma with subjacent inflammation

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4
Q

What is sialadenitis and what typically causes it?

A

Acute or chronic inflammation of the salivary gland:
Acute:
Bacteria - i.e. Staph aureus, can cause or complicate it by filling it with pus -> generally preceded by sialolithiasis.

Chronic:
Parotid glands - often viral infection like mumps or HIV
Autoimmune disorders like Sjogren’s syndrome may cause it

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5
Q

What are the clinical features of pleomorphic adenoma and what is its most common problem?

A

Painless, slowing-growing mass usually in parotid gland

Treatment is possible with complete surgical excision, but margins are so irregular that the surgeon can miss some and it will come back

Mass is nodular and appears generally well circumscribed, but the surgeon must take a generous margin

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6
Q

What is the uncommon complication of pleomorphic adenoma? How does this present?

A

Carcinoma can arise within it, and it is an aggressive malignancy

Will present with signs of a facial nerve palsy if in parotid gland, as it is neurotropic (similar to how pancreatic adenocarcinoma is)

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7
Q

How does pleomorphic adenoma appear microscopically?

A

Heterogenous (pleomorphic) mixture of epithelial and mesenchymal tissues with no atypia (benign).

Epithelial component - tubules, glands, cords, or nests

Stromal areas - myxoid, fibrous, CARTILAGINOUS, or even osseous

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8
Q

What is a Warthin tumor and who gets it? Is it malignant?

A

Papillary Cystadenoma Lymphomatosum

Always arises in the parotid gland in male smokers

Malignant in 10%, bilateral in 10% (like pheochromocytoma)

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9
Q

How does Warthin tumor appear microscopically?

A

Microscopically - Cystic spaces with tall pink columnar epithelial cells with abundant eosinophilic cytoplasm (arise from striated ducts). They produce chemoattractants so there will be a dense lymphoid stroma underneath which looks like germinal centers

(lymph-node-like stroma)

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10
Q

What is the most common malignant primary salivary gland neoplasm and what cell types are associated? Where does it arise?

A

Mucoepidermoid carcinoma

-> Associated with dysplastic squamous and mucinous cells

Arises in the parotid gland. Because it’s malignant, it can cause facial nerve problems.

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11
Q

What is the characteristic pregnancy finding for a tracheo-esophageal fistula and what is the most common type?

A

Characteristic finding - polyhydramnios (can’t swallow)

Most common type: Esophageal atresia with distal tracheo-esophageal fistula

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12
Q

What are esophageal webs?

A

Protrusion of esophageal mucosa (only the outer layer), most commonly in the upper esophagus

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13
Q

Where does Zenker diverticulum occur? What type of diverticulum is it?

A 
Killian's triangle
Between thyropharyngeus (superiorly) and cricopharyngeus (inferiorly), both of which are components of the inferior constrictor muscle

It is a FALSE diverticulum (just propulsion of the mucosa)

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14
Q

What are the two esophageal syndromes associated with violent wretching and which is more serious? why?

A
  1. Mallory-Weiss syndrome - partial-thickness longitudinal mucosal lacerations at GE junction
  2. Boerhaave syndrome - transmural, distal rupture due to violent wretching -> can cause mediastinitis and subcutaneous emphysema (due to air in mediastinum) and is a surgical emergency
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15
Q

What is the most common cause of death in cirrhosis? How can you differentiate it from Mallory-Weiss syndrome?

A

Ruptured esophageal varices with associated coagulopathy due to liver dysfunction

Presents with painLESS hematemesis (vs Mallory-Weiss which is painful)

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16
Q

Where is the venous drainage from from the upper 1/3, middle 1/3, and lower 1/3 of the esophagus? Where does the portal system connect?

A

Upper 1/3 - Superior vena cava via inferior thyroid veins

Middle 1/3 - azygous system

Lower 1/3 - portal system via left gastric vein (coronary vein (supplies the cardia of the stomach)), which forms an anastamosis with the azygous system

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17
Q

What are the two plexuses found in the wall of the GI tract? Where are they located and what is their function?

A

Meissner’s - subMucosal - receive sensory input from esophageal wall layers

Auerbach’s - Arnold S. = Muscles - Myenteric - sits between IC and OL layers of muscularis propria (externa) -> coordinates peristalsis

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18
Q

What is happening pathophysiologically to cause achalasia?

A

Loss of Auerbach’s (myenteric) plexus, with preferential loss of inhibitor neurotransmitters like NO and VIP -> tonically increased sphincter contraction

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19
Q

What can cause achalasia?

A

Primary - neurological degeneration

Secondary - Chagas’ disease, viral infection, autoimmune diseases

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20
Q

What are the risk factors for GERD / Reflux Esophagitis?

A

Same as sliding hiatal hernias
-> things that decrease sphincter tone or increase intraabdominal pressure

i.e. hiatal hernia, obesity/pregnancy, delayed gastric emptying (increased pressure and acidic stomach pH)

only thing not explained: alcohol and tobacco use -> they are irritants

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21
Q

What are the two types of diaphragmatic hernias and which is most common?

A
  1. Sliding hiatal hernia - GE junction slides into mediastinum, giving hourglass appearance - most common. Sometimes associated with a Schatzki B ring.
  2. Paraesophageal / rolling hiatal hernia - fundus of stomach prolapses into thorax adjacent to GE junction
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22
Q

What are the classic findings and potential complications of paraesophageal hernia?

A
  1. Bowel sounds in the lower lung field (fundus of the stomach is herniated in the mediastinum next to the esophagus)
  2. Lung hypoplasia - last room for lung to expand.
  3. Strangulation and perforation of involved fundic stomach (compromising blood flow)
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23
Q

How is GERD associated with asthma?

A

Reflux of acid down the esophagus can irritate the airway

-> causing coughing and adult-onset asthma.

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24
Q

What are the complications of GERD?

A
  1. Stricture formation
  2. Barrett esophagus -> NONCILIATED columnar epithelium with goblet cells
  3. Erosion / ulceration of esophagus with possible bleed (Rare)
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25
Q

What type of esophageal carcinoma is most common in the US and where does it arise?

A

Adenocarcinoma - progression from Barrett esophagus
-> has all the same risk factors as those for GERD

Arises in the lower 1/3 of the esophagus, especially the GE junction.

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26
Q

What type of esophageal carcinoma is most common in the rest of the world and what are its risk factors? Where does it arise?

A

Squamous cell carcinoma, arises usually mid-esophagus but can be anywhere

RF: alcohol and tobacco smoking**, hot liquids and achalasia (irritation), caustic strictures with repeated injuries, Plummer-Vinson, etc

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27
Q

Where does the upper 1/3, middle 1/3, and lower 1/3 of the esophagus drain its lymph? Note that this is the pattern of spread of esophageal carcinoma.

A

Upper 1/3 - Deep cervical nodes

Middle 1/3 - Mediastinal nodes

Lower 1/3 - Gastric and celiac nodes

Think neck, lung, stomach

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28
Q

Why does squamous cell carcinoma spread easily?

A

Because it lacks an outer serosal layer, it locally extends to other mediastinal organs, even aorta causing a massive bleed

Can also infiltrate lymphatics and spread hematogenously

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29
Q

How does omphalocele occur and is its prognosis better or worse than gastroschisis?

A

Ompalocele - failure of physiologic hernation (which occurs during growth rotation of gut during development) to return from umbilical cord back into abdomen. Will be covered by peritoneum and amnion “cele”‘d.

Prognosis is worse than gastroschisis even though it looks less ugly, because more genetic abnormalities are associated with it

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30
Q

What is gastroschisis? Where does it typically happen?

A

Extrusion of abdominal contents through abdominal wall, not covered by peritoneum or amnion -> typically due to an abdominal wall defect on the right side

Can be repaired surgically

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31
Q

What type of diverticulum is a Meckel diverticulum and what causes it?

A

A true diverticulum - all layers of the abdominal wall

Caused by a failure of the regression of the vitelline duct, which normally communicates with the yolk sac during development -> “vital” for nutrients to the midgut

2 inches long, 2 feet from ileocecal valve, in 2% of the population, occurring in first two years of life

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32
Q

Who tends to get hypertrophic pyloric stenosis, and what are the common presenting signs and symptoms?

A

Firstborn Caucasian males

  1. Olive-shaped mass in epigastric region
  2. Visible peristaltic waves after eating (hypertrophy)
  3. Nonbilious projecting vomiting starting after feedings about 3-4 weeks after birth (takes a while for food to build in stomach)
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33
Q

What things cause decreased mucosal protection to cause acute gastritis?

A
  1. Advanced age - decreased mucus production
  2. NSAIDS, corticosteroids - loss of PGE2, which is needed to maintain blood flow to mucosa / sweep away acid, and maintain production of HCO3- / mucin
  3. Uremia - acidosis will allow less bicarbonate to be excreted
  4. Low O2 -> shock, high altitudes, severe burns -> mucosal ischemia = poor protection
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34
Q

What things tend to induce acute gastric ulcers?

A
  1. NSAIDs
  2. Intracranial trauma - stimulation of vagal nerve increases gastric acid secretion
  3. Major physiologic stress - shock, sepsis, burns leads to decreased perfusion of mucosa (decrease nutrients) and systemic acidosis (Decrease bicarbonate excretion)
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35
Q

What is it called when you have an ulcer due to intracranial injury vs significant burns?

A

Intracranial injury = vagal stimulation -> Cushing ulcer (think of Cushing’s triad)

Burns = hypovolemia -> Curling ulcer (think of Curling irons are hot) -> decreased mucosal perfusion

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36
Q

What is the most common cause of chronic gastritis? Where does it start and how do you get it?

A

H. pylori - 90% of cases

Typically starts in the antrum of the stomach

More a disease of lower SES in the US, thought to spread via oral-oral or fecal-oral

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37
Q

How can the inflammation in autoimmune gastritis be told apart from H. pylori gastritis?

A

Autoimmune - chronic inflammatory infiltrate will be deep within the mucosa near the parietal and chief cells, and will also be in the body / fundus rather than antrum

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38
Q

Other than H. pylori, what are some other causes of Peptic ulcer disease (the more chronic form of acute gastritis)?

A

NSAIDs - especially gastric peptic ulcer disease (i.e. chronic treatment of RA)
Corticosteroid use and psychological stress - inhibit repair by immune system + mucosal protection (blocks PLA2)
Hypercalcemia -> increases gastrin (abdominal pain in hypercalcemia)
Zollinger-Ellison syndrome -> Gastrinoma

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39
Q

What causes Menetrier disease and how does it appear?

A

Increased production of TGF-alpha leads to diffuse hyperplasia of mucous glandular epithelium (foveolar) in body and fundus, with atrophy of parietal and chief cells -> thick rugae

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40
Q

What are the complications of Menetrier disease?

A

Protein-losing enteropathy -> increased mucin secretion leads to diarrhea, weight loss, and hypoproteinemia which can lead to peripheral edema

Increased risk for adenocarcinoma (premalignant)

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41
Q

How does H.pylori-induced gastritis (chronic gastritis) appear grossly and microscopically?

A

Grossly - Erythematous, rough-appearing gastric mucosa with active inflammation. Mucosal atrophy and intestinal G-cell metaplasia and hyperplasia is also present.

Microscopically - acute on chronic inflammation (neutrophils + chronic inflammatory infiltrate) + presence of spiral-shaped bacteria in superficial mucus

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42
Q

How does alcohol cause acute gastritis?

A

Directly damages the mucosa, decreasing mucosal protection.

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43
Q

What is the difference between an erosion and an ulcer? How does this relate to the three stages of acute gastritis?

A
  1. Superficial inflammation - neutrophils and edema.
  2. Erosion - loss of mucosa of the stomach, not into the submucosa (last layer of mucosa is muscularis mucosa)
  3. Ulcer - Penetration into the submucosa or deeper
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44
Q

What causes autoimmune gastritis and what part of the stomach does it affect?

A

Loss of self-tolerance of CD4+ T cells to gastric parietal cells

  • > stimulation of autoantibodies to parietal cells or intrinsic factor. Note that these antibodies are markers but do NOT drive the disease
  • > chronic inflammation and destruction of gastric body and fundus by CD8 activation
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45
Q

What cancer does chronic gastritis predispose to and why?

A

Gastric adenocarcinoma
-> chronic inflammation will induce an intestinal metaplasia (similar to Barrett esophagus) -> increases risk of dysplasia / cancer.

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46
Q

What two tests are considered the most reliable for the diagnosis of H. pylori?

A
  1. Urea breath test, with C13 or C14

2. Stool test - good sensitivity / specificity for picking up H. pylori antigen, and non-invasive

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47
Q

What cells will hypertrophy in duodenal ulcer? And what is the other cause other than H. pylori?

A

Brunner’s glands -> to protect the duodenum from acid

Other cause -> ZE syndrome

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48
Q

What are the signs and symptoms of gastric cancer?

A

Weight loss, pain, NVD, hematemesis, melena

EARLY SATIETY - especially in diffuse type

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49
Q

What is gastric adenoma? When does it typically arise? Where?

A

A premalignant gastric polyp typically arising in the gastric antrum in chronic atrophic gastritis

  • > epithelial dysplasia which will progress to adenocarcinoma
  • > picked up in Japan where they actually screen for it

-> fundic gland polyps are the ones associated with PPI use, and then hyperplastic polyps with chronic inflammation + repair

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50
Q

What are the two types of gastric adenocarcinoma and the risk factors for each?

A

Intestinal-type - Chronic atrophic gastritis of both types (causes intestinal metaplasia), dietary nitrosamines, gastric adenoma, smoking, and blood type “A” (just remember CA).

Diffuse-type - risk factors unknown (thus increasing in prevalence)

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51
Q

Where does intestinal-type gastric adenocarcinoma occur? What does it look like grossly?

A

In the antrum / pyloric region, where intestinal metaplasia is occuring

Appears as heaped up, ulcerating, necrotic or exophytic mass

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52
Q

What is Diffuse-Type Gastric Adenocarcinoma also called? What mutation is associated with it? What does it look like microscopically?

A

Signet-Ring Cell Adenocarcinoma

Associated with loss of E-cadherin (similar to invasive lobular carcinoma of breast with single file cells)

Microscopically - mucin-filled cells with peripheral nuclei, widespread over stomach. Infiltrating singly or in small nests (glandular differentiation with intracellular mucin)

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53
Q

What are some more uncommon presenting symptoms for gastric cancer?

A

Acanthosis nigricans - axillary browning (normally associated with increased insulin resistance)

Leser-Trelat sign - multiple seborrheic keratoses appearing all over body

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54
Q

How does signet-ring cell adenocarcinoma appear grossly?

A

Linitis plastica - “leather bottle” - stomach wall is grossly thickened and leathery due to diffuse invasion by cells with prominent DESMOPLASIA

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55
Q

What lymph node is commonly enlarged in gastric CA?

What are some very specific sites of metastasis for intestinal and diffuse type gastric adenocarcinoma?

A

Virchow - left supraclavicular)

Intestinal type - Sister Mary Joseph nodule -> periumbilical nodular metastasis

Diffuse type - Bilateral Krukenberg tumor -> mucinous adenocarcinoma metastases in the ovaries.

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56
Q

What is the most common place for intestinal atresia to occur and what is thought to cause it? What explains the sign seen on X-ray?

A

Duodenum - failure to recanalize due to loss of blood supply during development

Associated with Down syndrome

Double bubble sign: dilated stomach and proximal duodenum, with intervening constriction via the pyloric sphincter.

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57
Q

What happens in normal rotation of the gut and what will happen if this is done incorrectly?

A

Normally, the gut rotates 270 degrees around the superior mesenteric artery via a counterclockwise rotation from the frontal view. This allows the colon which was pointing downwards to sit on your right side on your posterior abdomen, anterior to the duodenum

If this is done incorrectly -> malrotation occurs

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58
Q

In what condition are Ladd bands observed and what problems may they cause?

A

Malrotation of the midgut, with cecum ending up the RUQ rather than RLQ as it should

  • > Ladd bands are adhesions formed from peritoneal tissue which connect to the cecum
  • > due to mispositioning, these adhesions (which should normally hold the cecum as an intraperoniteal structure) will encircle the duodenum and can cause obstruction of the 2nd part of the duodenum
  • > may also cause volvulus of cecum due to narrowed mesentery

TL;DR: duodenal obstruction (presents like duodenal atresia) or midgut volvulus.

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59
Q

Why do Meckel’s diverticula sometimes cause problems?

A
  1. Often contains heterotopic gastric mucosa which secretes acid, causing bleeding
  2. Volvulus (wrap around the diverticulum which is attached to umbilicus), intussusception, or obstruction can occur
  3. Can present as a fistula which drains into umbilicus
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60
Q

What is it called when a portion of bowel twists on its mesentery and where does it happen in adults? Children

A

Volvulus -> happens in sigmoid colon in adults due to long mesentery
(coffee bean sign on X-ray)

Children -> happens in cecum, due to association with congenital malformations (i.e. Meckel’s, malrotation)

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61
Q

When does intussusception occur in adults and children?

A

Adults - associated with intraluminal mass or tumor acting as a lead point

Children - more common -> due to lymphoid hyperplasia (i.e. rotavirus), with terminal ileum telescoping into cecum

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62
Q

What causes the majority of the injury in ischemic bowel disease, and what area is particularly susceptible? What part of the wall is most susceptible to infarct?

A

Secondary reperfusion injury -> due to oxygen-derived free radicals

Splenic flexure is most vulnerable - watershed area between SMA / IMA

Mucosa is most susceptible to infarct since it is farthest from the blood supply (can be infarcted alone inhypotension)

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63
Q

What is the clinical presentation of ischemic bowel disease / acute mesenteric ischemia?

A

Abdominal pain (especially postprandial) with some bloody diarrhea - “currant jelly stools”, due to hemorrhagic infarct

  • > often seen in post-MI or post-shock patients who have hemorrhagic necrosis
  • > often suspected as a diagnosis of exclusion in older adults, when pain is out of proportion for physical exam findings
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64
Q

What is Chronic mesenteric ischemia also known as? How will it present?

A

“Intestinal angina” - atherosclerosis of celiac artery, SMA, or IMA causes hypoperfusion

  • > postprandial epigastric pain due to increased nutrient requirement for digestion
  • > food aversion and weight loss in an elderly person
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65
Q

What are classical causes of thrombosis / embolism of the SMA?

A

Atrial fibrillation, polyarteritis nodosa

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66
Q

What are the classical causes of venous thrombosis of the mesenteric vein?

A

Hypercoagulable states:
Polycythemia vera
Lupus anticoagulant

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67
Q

What will skin biopsy of Dermatitis Herpetiformis show via H&E and direct immunofluorescence? What forms at the tips of these papillae?

A

H&E - neutrophilic infiltrate of papillary dermal tips

DIF - granular IgA deposition

  • > deposit at papillary tips since this is the farthest they can migrate via blood supply in dermis
  • > herptiform vesicles and blisters form at the dermal tips where IgA is being deposited
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68
Q

What is the pathogenesis of Celiac disease?

A

Gliadin is a soluble portion of grains, is absorbed into GI tract and deaminated by tissue transglutaminase (tTG)

Deaminated gliadin is presented by MHC class II and activates CD4 T cells -> T cell mediated damage of intestinal mucosa

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69
Q

What is seen on anatomic pathology of Celiac disease? Where is the damage worst?

A

Villous atrophy / blunting with crypt hyperplasia for regeneration, and chronic inflammation surrounding

Worst in proximal small intestine (DUODENUM)

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70
Q

What antibodies are detected by serology in Celiac disease? What antibody types should you look for?

A

IgA antibodies:

  1. Anti-tissue transglutaminase (TTG3)
  2. Anti-deaminated gliadin
  3. Anti-endomysial antibodies (anti-TTG2) - connective tissue which ensheaths muscle fibers, thought to be cross-reactive to a tissue transglutaminase

Should also check for IgG antibodies since Celiac’s disease is associated with IgA deficiency

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71
Q

What malignancies is Celiac disease associated with?

A

T-cell lymphoma - due to lymph hyperplasia / inflammation (enteropathy-associated T-cell lymphoma)

Small intestinal adenocarcinoma (rare, associated with the crypt hyperplasia)

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72
Q

What is Tropical sprue and what is the treatment? What is the super high yield difference from Celiac disease?

A

Infectious disease within unknown cause which is responsive to antibiotics, seen in tropics.

Tropical sprue is more associated with jejunum/ileum damage instead of duodenum -> more likely to cause a folate or B12 deficiency

73
Q

What are the intestinal symptoms of Whipple’s disease and why does this happen?

A

Disease of older men, where macrophages get loaded with T. whipplei organisms, which are gram + and stain positive for PAS.

Fat malabsorption and steatorrhea occurs as distended macrophages and compress on the lacteals -> chylomicrons cannot be uptaken.

74
Q

What are the symptoms of Whipple disease?

A

Foamy Whipped cream in a CAN

Foamy - macrophages
Whipped - Whipple disease
CAN:
Cardiac symptoms - invade heart valves
Arthralgias - also live in joint synovial membranes
Neurologic symptoms - invade brain, cause psychiatric complaints

75
Q

What is seen pathologically and on peripheral smear of abetalipoproteinemia?

A

Pathologically - vacuolization of enterocytes due to accumulation of fats which cannot be packaged into chylomicrons

Peripheral smear - acanthocytes (spur cells) - spiky RBCs due to abnormal lipoproteins in their membrane (states of cholesterol dysregulation, also seen in liver disease)

76
Q

What are the differences in presentation between the locations of carcinoid tumors of the GI tract?

A

Small bowel (especially distal ileum) - may cause obstruction due to prominent desmoplasia, and can metastasis

Appendix / rectal - Usually found incidentally, almost never metastasize

77
Q

What are the symptoms of carcinoid syndrome?

A

Bronchospasm, diarrhea (5-HT agonist), wheezing, flushing worsened by emotional stress or alcohol which stimulate serotonin release from the tumor

Damage includes: Left-sided valves not affected due to MAO in the lung.

78
Q

What valve pathogies are seen in carcinoid heard disease and what drives it?

A

Right-sided valvular fibrosis, tricuspid regurgurgation, and pulmonary valve stenosis.

Serotonin drives the myxomatous proliferation of valve endocardium
-> deposition of collagen due to effects of TGF beta

79
Q

How does ulcerative colitis appear grossly in active disease and what is a pseudopolyp? What imaging sign may be seen?

A

Active - superficial, broad-based ulcerations and hemorrhage with intervening inflammatory pseudopolyps

Pseudopolyp - what remains of the mucosa level which as not ulcerated

Loss of haustra on imaging - “Lead pipe sign” -> due to inflammation destroying those folds / pouches

80
Q

How does ulcerative colitis appear microscopically in active disease?

A

Diffuse mucosal acute inflammation with crypt abscesses (neutrophils in crypts) and chronic inflammation, with superficial ulceration

81
Q

Which IBD is p-ANCA positive?

A

Ulcerative colitis

Think UP
Ulcerative colitis, p-ANCA

remember p-ANCA is also positive in microscopic polyangiitis and Churg-Strauss

Also you can remember that Crohn’s is associated with another antibody (anti-Saccharomyces cervisiae), so it can’t be associated with this.

82
Q

How does Crohn disease appear grossly?

A

Firm segments of bowel with serosal “creeping fat” due to transmural inflammation which is very sticky, and the development of deep, narrow, linear ulcers giving a “cobblestone” effect

83
Q

Which IBD is more likely to cause bloody diarrhea and why? How does rectal involvement compare?

A

Definitively ulcerative colitis -> think of all the pus and blood which will be in the crypts.

Crohn’s may or may not present with blood, but remember the rectum is usually SPARED in Crohn’s.

84
Q

What sign is sometimes seen on barium studies of Crohn’s disease and where is it most commonly found?

A

“string sign” due to thickening of smooth muscle and fibrotic strictures (myoepithelial cells constrict granulation tissue, also explains creeping fat)

-> most common in terminal ileum

85
Q

What carcinomas does Crohn’s predispose to? What about Ulcerative colitis?

A

Crohn’s - adenocarcinoma of colon and small intestine
-> may be no increased risk for cancer if colon is not involved at all

Ulcerative - adenocarcinoma of colon only (does not invade small intestine)
-> Ulcerative colitis generally much higher risk, 8-10 years after colonic involvement has started

86
Q

What biliary tract disease is associated with IBD and which one is it associated with?

A

Primary sclerosing cholangitis - Associated with Ulcerative colitis

Think UPP

Ulcerative colitis, p-ANCA, primary sclerosing cholangitis.

87
Q

What type of biopsy is done to assess for Hirschsprung disease? What can be helpful to stain for?

A

Rectal suction biopsy -> normal biopsies would only take mucosa, but this also gets some submucosa in order to evaluate for Meissner’s (submucosal plexus)

Can be helpful to stain for acetylcholinesterase which will be present in greater amounts produced by Preganglionic nerve fibers which have no target to innervate (indicates absence of postganglionic nerves)

88
Q

What are diverticula and where do they appear?

A

Outpouchings of the mucosa / submucosa (false diverticula) of the colon through the muscularis propria (externa) at points where the vasa recta enter (Weak point)

Appear commonly in the sigmoid colon (where the water has been mostly reabsorbed so poop takes more force to propel) -> associated with low fiber diet.

89
Q

What are the symptoms and complications of diverticulosis?

A

Usually asymptomatic or associated with mild left lower quadrant discomfort

Complications:
Diverticulitis -> left-sided appendicitis
Diverticular bleeding (painless hematochezia)
Fistulas -> transmural inflammation could form for instance a colovesical fistula -> presents with air / fecalith in urine

90
Q

What are the symptoms of angiodysplasia? How does it look histologically?

A

Lower GI bleeding (hematochezia) in an older adult -> acquired malformation of mucosal / submucosal capillary

Histologically - huge dilated submucosal veins which can bleed if there is an erosion

91
Q

What does high stress on the left side of the colon / cecum cause? How does it present?

A

Angiodysplasia - an ACQUIRED malformation of the mucosal / submucosal capillary bed -> tortuous dilation
-> due to periodic stress which caused venous distention

Presents as lower GI bleeding / hematochezia in an older adult

92
Q

What vascular disease is also known as Osler-Weber-Rendu syndrome and what are its symptoms? Inheritance pattern?

A

Hereditary hemorrhagic telangiectasia - autosomal dominant

Dilated microvascular swellings (telangiectasias) which start in childhood and increase in adulthood

  • > recurrent epistaxis and bleeding of nasopharynx and GI tract
  • > can lead to iron deficiency anemia and GI hemorrhage
93
Q

What are the skin manifestations of IBD? Describe them?

A

Erythema nodosum - red markings due to fat inflammation on extensor surfaces of skin, common on shins

Pyoderma gangrenosum - ulcerating lesion with heaped up edges

94
Q

What are the joint / eye manifestations of IBD? Which ones are associated with active disease / which ones occur all the time?

A

Peripheral arthritis / migratory polyarthritis - occurs only during exacerbations

Central arthritis - sacroilitis / ankylosing spondylitis, not associated with IBD activity - HLA-B27
-> also associated with uveitis / iritis since HLA-B27 associated.

95
Q

Is irritable bowel syndrome a mechanical or functional disorder? What are the diagnostic criteria?

A

Functional (no structural changes)

Recurrent pain or discomfort at least 3 days / month in last 3 months, associated with 2 or more of:

  1. Improvement with defecation
  2. Onset associated with a change in frequency of stool
  3. Onset associated with a change in appearance of stool
96
Q

What is the cause of IBS?

A

Problem of disordered motility and/or nociception

  • > Psychosocial factors also play a role
  • > may improve with increased fiber
97
Q

What are the types of non-neoplastic polyps and which is most common?

A

Hyperplastic polyps - most common

Juvenile polyps - seen below age 5

Inflammatory polyp and pseudopolyp - i.e. ulcerative colitis

98
Q

How does a hyperplastic polyp appear microscopically? Is it associated with malignancy?

A

Elongated regular glands and crypts with superficial “sawtooth” luminn borders by mature epithelial cells with no cytologic atypia
-> normal cells which are overcrowding

-not associated with malignancy

99
Q

What is a juvenile polyp and what symptoms are they associated with?

A

Hamartomatous mucosal polyp in children <5 years

Associated with bleeding, and autoamputation of polyp into stool is frequent and spooky

Ulceration is common -> causes bleeding

100
Q

What causes Peutz-Jeghers syndrome and how can its polyps be told apart from juvenile polyps? Are they premalignant?

A

Autosomal dominant disorder

GI tract: Large, pedunculated, hamartomatous polyps

Major characteristic difference: Large bundles of smooth muscle within the polyps in PJS

They are NOT premalignant, although PJS is associated with increased risk of cancer

101
Q

What is seen throughout the body in PJS outside the GI tract and what cancers is it associated with?

A

Melanotic pigmented macules along cutaneous and mucosal surfaces, i.e. mouth, lips, genital skin

Cancers: increased risk of breast and GI carcinomas

102
Q

What is the sequence of neoplastic polyps in the APC sequence? What is the usual correspondent growth pattern?

A
  1. Tubular adenoma - most common - low risk of cancer in it - usually grows as pedunculated (flat)
  2. Tubulovillous adenoma - usually grows as midway between sessile / pedunculated
  3. Villous adenoma - worst - 40% chance of having cancer in it - usually sessile

“Villous is the villain”

103
Q

What are the clinical symptoms of tubular adenoma vs villous adenoma? What acid-base disturbance might the latter cause?

A

Tubular - Usually asymptomatic, but some occult bleeding is possible

Villous - rectal bleeding is common.

Villous: Cells are also so dysplastic that they rarely release alot of protein -> hypoproteinemia due to protein-losing enteropathy.

Can also secrete Cl- causing metabolic alkalosis (HCO3- is absorbed in place of Cl). Causes hypokalemia as well (alkalosis state + actual secretion of K+)-> hypokalemia
-> hypokalemic metabolic alkalosis

104
Q

What causes FAP? What is the function of the lost gene? Wat chromosome?

A

Autosomal dominant (first hit) mutation of APC causes familial adenomatous polyposis

APC is a tumor suppressor on chromosome 5 which binds Beta-catenin when it is not associated with E-cadherin. Loss of APC allows beta-catenin to induce transcriptional activation of cell proliferation and decreased apoptosis
-> 100s of polyps are easily formed

105
Q

What are the key associations with Gardner syndrome?

A

FAP +

  1. Fibromatosis - deep, retroperitoneal fibromatoses (fibroblast tumors)
  2. Osteomas - benign tumor of bone
106
Q

What are the key assocations with Turcot syndrome?

A

FAP +

  1. Medulloblastoma
  2. Glial tumors

Turcot = Turban = head

107
Q

Does a syndrome of Juvenile polyposis predispose to cancer?

A

Yes - the hamartomatous polyps arise everywhere (stomach, colon)

  • > actually have increased risk for cancer
  • > Autosomal dominant, still presents before age 5
108
Q

What is Lynch syndrome also called? What causes it?

A

Hereditary Non-Polyposis Colorectal Cancer (HNPCC)

Autosomal dominant mutation of DNA mismatch repair genes with microsatellite instability
-> instability of microsatellites, which are repeating sequences of noncoding DNA.

109
Q

What GI and extra-GI manifestations are a part of Lynch syndrome?

A

GI: increased incidence of sessile serrated adenomas (1st hit in this pathway) which are in right colon, and development of adenocarcinoma

Extra-GI: Endometrial, ovarian, and gastric cancers

110
Q

How does colon cancer in the left colon appear and why? What pathway does it use? What symptoms does it cause?

A

Left colon - most common, via APC pathway - appears as an annular, ulcerated mass with heaped up margins (apple-core or napkin-ring constriction) -> poop is tightly packed so it doesn’t have much room to reach into the lumen

Causes obstruction + pencil thin stools. Bowel habit changes / discomfort. May cause hematochezia.

111
Q

How does colon cancer in the right colon appear and why? What pathway does it use? What symptoms does it cause?

A

Right colon - less common, via the DNA mismatch repair pathway - appears as exophytic, polyploid mass branching into lumen, as the diameter is larger and the poop is fast-moving (tumor less likely to obstruct lumen)

Causes occult, rectal bleeding from sessile carcinoma (occult blood + iron deficiency anemia is common presentation)

112
Q

How do you track for recurrence of colorectal cancer?

A

Recurrence can be tracked by carcinoembryonic antigen (CEA) marker. If it was high before tumor removal and then becomes low, recurrence of cancer can be tracked via this marker

113
Q

When should you start screening for colorectal cancer? How do you do this?

A

Colonoscopy every 10 years starting at age 50

Other options

  1. Yearly fecal occult blood test (FOBT)
  2. Flexible sigmoidoscopy every 5 years - left side only
  3. CT colonography every 5 years
114
Q

When should you have colonoscopies starting before age 50 (assuming no inherited syndromes)?

A

First degree relative who has colon cancer should start screening at age 40 or 10 years before youngest relative at their CRC diagnosis

115
Q

What occurs if the ventral pancreatic bud doesn’t know how to rotate in embryonic development?

A

Annular pancreas - It will rotate in both directions, around the duodenum

Risk is duodenal obstruction

116
Q

What is pancreas divisum and what is the risk?

A

Incomplete fusion of the dorsal and ventral pancreatic buds, including incomplete ductal fusion

  • > since accessory duct of dorsal bud will be smaller than main duct, some secretions will be trapped in body of pancreas
  • > risk of recurrent pancreatitis
117
Q

What are the causes of acute pancreatitis?

A

I GET SMASHED

Idiopathic, Ischemia

**Gallstones
**Ethanol
Trauma - i.e. MVA in children, stabbings

Steroids
Mumps infection
Autoimmune disease
Scorpion stings
Hypercalcemia / hypertriglyceridemia (>1000 mg/dL)
ERCP - to clear stone
Drugs
118
Q

How is alcohol thought to cause pancreatitis?

A

Directly injures acinar cells

**Causes thick, viscous excretions blocking the ducts

Messes up intracellular transport of pancreatic proenzymes -> causes fusion with lysosome and activation within the cell

119
Q

What are the localized complications of acute pancreatitis?

A

Pseudocyst - necrotic cyst lined by fibrotic granulation tissue, not epithelium. Presents with persistently elevated serum amylase, and may rupture into abdominant cavity

Abscess - infected pseudocyst, usually due to E. coli

Biliary obstruction / jaundice

120
Q

What is chronic pancreatitis and what are the causes?

A

Relapsing acute pancreatitis -> progressive injury

Often idiopathic, but:

Adults - alcoholism

Children - Cystic fibrosis

May also be autoimmune, secondary to hyperlipidemia/hypercalcemia, or due to pancreas divisum

121
Q

What are the clinical features of chronic pancreatitis?

A

Fibrotic destruction of gland, with radiologic hallmark of dystrophic calcification

Pancreatic insufficiency - fat malabsorption / steatorrhea

Late complication - secondary diabetes mellitus

Predisposes to adenocarcinoma of the pancreas

122
Q

Are serum amylase / lipase useful for monitoring chronic pancreatitis?

A

No - they may or may not be elevated late in the disease

123
Q

A thin, 70 year old female with no history of pancreatitis presents with diabetes mellitus for the first time. What should be on your differential?

A

Pancreatic adenocarcinoma in the body or tail -> compressing and obstructing the function of the islets

-> generally does not happen in tumors of the head of the pancreas

124
Q

What are the major risk factors for pancreatic adenocarcinoma?

A

Smoking and chronic pancreatitis

Diabetes, being black, male, and old age also play a role

125
Q

What are the gross and microscopic appearance of pancreatic adenocarcinoma?

A

Gross - infiltrative, firm, tan tissue due to desmoplasia

Microscopic - loss of normal lobular architecture with haphazard, irregularly glandular structure, desmoplasia, and perineural invasion -> can be painful
(like pleomorphic adenoma)

126
Q

What is the tumor marker for and treatment of pancreatic adenocarcinoma?

A

CA 19-9. CEA may also be used, but less specific (obvs also for colorectal cancer).

Treatment: Whipple procedure - removal of head / neck of pancreas, proximal duodenum, and gallbladder

127
Q

What are the causes of biliary atresia?

A

Failure to form (rarer) or early destruction (within 2 mo of birth) of extrahepatic biliary tree (probably due to inflammation from infection leading to stricture and destruction of the bile duct)

128
Q

What are the risk factors for cholesterol stone formation?

A
  1. Developed countries
  2. Native American ethnicity
  3. Increased secretion of bile - advanced age, estrogen (obesity, women, OCPs, pregnancy)
  4. Gallbladder hypomotility - rapid weightloss, fasting, or neurogenic
  5. Crohn’s disease - bile salt malabsorption in distal ileum
129
Q

What are the two morphological types of pigment stones? What causes them? Are they radio-opaque or translucent?

A

Black - occur in chronic hemolysis -> majority are radio-opaque from complexing with calcium. Multiple and smooth.

Brown - occur in biliary tract infections (i.e. E. coli, Clonorchis sinensis) -> soft and soapy, radiotranslucent

130
Q

What are the possible complications of cholelithiasis?

A
  1. *Acute cholecystitis
  2. Hydrops of gallbladder due to chronic obstruction
  3. Choledocholithiasis -Obstruction of the common bile duct
  4. Chronic cholecystitis
  5. Gallstone ileus
  6. Carcinoma of the gallbladder
131
Q

What are the predisposing factors to chronic cholecystitis, and what are the characteristic microscopic features?

A

Chronic cholelithiasis

Chronic inflammation - lymphocytes, macrophages, plasma cells, and fibrosis

Rokitansky-Aschoff sinuses - mucosal diverticula seen in gallbladder wall due to contraction against thick gallbladder wall

132
Q

Where does pain radiate in acute cholecystitis?

A

Right scapula - and that is high yield

133
Q

What are the possible complications of choledocholithiasis?

A
  1. Cholestasis with secondary biliary cirrhosis
  2. Ascending cholangitis with possible liver abscess formation - (bile is no longer able to flush the bacteria away, so they can ascend)
  3. Pancreatitis - if stone is wedged right before ampulla of Vater
134
Q

Who tends to get carcinoma of the gallbladder? What IS it?

A

Classically, elderly women with a history of cholelithiasis, especially causing chronic cholecystitis with porcelain gallbladder
-> suspect in an elderly woman who gets cholecystitis since they should not be getting it at this age

It is cancer of the glandular epithelium of the gallbladder wall

135
Q

What is the fate of bilirubin once it enters the intestine?

A

Bacteria in the gut deconjugate it to urobilinogen.

Urobilinogen can be excreted in stool after being made into stercobilin -> gives the stool its brown color.

Some urobilinogen is reabsorbed -> can be excreted again in bile, or transported to kidneys where it is oxidized to urobilin -> gives the urine its yellow color.

136
Q

What will be seen in the blood with excessive extravascular hemolysis / ineffective erythropoesis? What symptoms will the patient have? What is excreted into the urine?

A

Increased unconjugated bilirubin -> waiting for the liver to have time to conjugate

Symptoms:

  1. Increased risk of black pigment gallstones
  2. Increased urine urobilin. Note, this is NOT UCB which is being excreted into urine (which is not water soluble)
137
Q

What is the cause of physiologic jaundice of the newborn? What is the treatment?

A

Newborn has transiently low UGT activity
-> UCB accumulates -> fat soluble so it deposits in basal ganglia, causing kernicterus if untreated

Treatment is phototherapy, which makes the bilirubin water soluble

138
Q

What are the two broad syndromes of congenital unconjugated hyperbilirubinemic states? How are they inherited?

A
  1. Gilbert syndrome
  2. Crigler-Najjar syndrome

Inherited as autosomal recessive

139
Q

What is wrong in Gilbert syndrome? When will the symptoms be seen?

A

Mildly decreased UDP-glucuronosyltransferase (UGT) conjugation activity

  • > symptoms are seen during stress (i.e. illness or fasting)
  • > usually asymptomatic and not clinically significant
140
Q

What is the defect in Crigler-Najjar syndrome and what are its two subtypes?

A

Defect is absent UGT (think about how this sounds like a Disney villain)

Type I - both alleles knocked out -> kernicterus and death

Type II - heterozygotes, will have milder disease as one UGT allele is functional

141
Q

How can Gilbert syndrome and Crigler-Najjar Type II be treated?

A

Treated via phenobarbital - upregulate liver enzyme synthesis via enzyme induction properties
-> improves symptoms if some working allele is still available

142
Q

What is wrong in Dubin-Johnson syndrome and what are the symptoms? How does Rotor syndrome differ?

A

Conjugated hyperbilirubinemia due to defective bilirubin canalicular transport protein

  • > symptoms are benign, with elevated serum CB and slight hepatomegaly
  • > Liver will also grossly appear black due to a melanotic pigment (accumulation of epinephrine metabolites)

Rotor syndrome is the same, except without black liver (no accumulation of metabolites).

143
Q

What is cholangiocarcinoma? What are the risk factors?

A

Carcinoma of the bile duct epithelium (intrahepatic or extrahepatic)

Risk factors: often absent, but include primary sclerosing cholangitis, congenital biliary tract abnormalities leading to stasis, and Clonorchis sinesis (liver fluke)

144
Q

What are the early vs late lab manifestations of cholestasis?

A

Early - Elevated Alk Phos, with corresponding elevated GGT / 5’-nucleotidase

Late - Increased serum bilirubin levels and increased cholesterol levels (all of the bile must be blocked, very late) -> can lead to increased ALT/AST due to bile starting to solubilize cell membranes

145
Q

What are the clinical manifestations of cholestasis?

A
  1. Pruritis - from retention of bile salts / acids
  2. Jaundice / icterus -> conjugated bilirubin increases
  3. Xanthomata / xanthelasma - lipid-laden Macs deposit in dermis due to increased serum cholesterol
146
Q

Describe the general pathogenesis of cirrhosis. What is the key cytokine

A

Chronic liver injury -> cytokine production by Kupffer cells, lymphocytes, and endothelial cells

Ito cells become myofibroblast-like cells due to cytokines, which causes contraction and synthesis of Types I and III collagen into space of Disse (mediated by TGF-beta by stellate cells)

Fibrosis impairs blood flow and diffusion of metabolites -> formation of fibrous septae and portosystemic shunts.

147
Q

Other than feathery degeneration (looks the same as ballooning degeneration, just in the presence of bile), what are some other characteristic pathologic liver changes which occur in cholestasis?

A
  1. Bile ductular proliferation - compensatory response which doesn’t help
  2. Portal tract edema with acute inflammation, even bile lakes forming
  3. Portal tract fibrosis leading to biliary cirrhosis
148
Q

What will happen to urine conjugated bilirubin and urobilinogen in viral hepatitis?

A

Urine conjugated bilirubin - increased

Urobilinogen - normal or decreased, due to damage to liver parenchyma / bile ducts may impair bile secretion into duodenum somowhat, so bacteria cannot convert to urobilinogen for reuptake and making the urine yellow (when urobilinogen -> urobilin)

149
Q

What two non-hepatitis viruses can cause hepatitis?

A

CMV, EBV

-> consider how these viruses cause mononucleosis which can cause hepatosplenomegaly

150
Q

How is hepatitis E virus transmitted and why is it unique? What type of virus is it?

A

Fecal-oral, especially from contaminated water

RNA hepevirus

Unique - only human hepatitis virus with an animal reservoir (swine)

151
Q

Who is at greatest risk for severe disease with HEV? Does it have a carrier state?

A

Expectant (pregnant) women
-> high mortality

No carrier state or chronic liver disease (like hepatitis A) unless immunosuppressed

152
Q

What causes Caput medusae?

A

Anastamoses of paraumbilical (portal) and small epigastric veins of anterior abdominal wall

153
Q

What are the consequences of loss of hepatic function in cirrhosis?

A
  1. Hypoalbuminemia
  2. Coagulopathy
  3. Hepatorenal / hepatopulmonary symptoms
  4. Hyperestrogenism
  5. Jaundice / icterus with cholestasis
  6. Hepatic encephalopathy
154
Q

What factors precipitate worsening of hepatic encephalopathy?

A

Increased NH3 production or absorption:

  1. Excess oral protein intake -> ammonia builds
  2. GI bleed -> increased protein to gut microbes
  3. Constipation / infection

Decreased NH3 removal:

  1. Alkalosis / hypokalemia -> Increased K+ reabsorption stimulates H+ excretion via ammonia, but ammonia can make it back into blood
  2. Renal failure
  3. Diuretics -> hypokalemia
  4. TIPS procedure
  5. Benzos / sedatives
155
Q

How does alcoholic hepatitis appear pathologically? What causes this? What is the major distinguishing feature from viral hepatitis?

A

Swollen and necrotic hepatocytes with a NEUTROPHILIC inflammatory infiltrate (vs viral = lymphocytic).

Mallory-Denk bodies -> alcoholic hyaline - eosinophilic cytoplasmic inclusions which are inclusions of damage keratin filaments (intermediate filaments!)

Consider that Mallory-Weiss and Mallory-Denk are both alcohol related - Mallory Mager

156
Q

Why is the pattern of ALT vs AST different in alcoholics?

A

AST is present in the mitochondria -> preferentially damaged in alcoholism since alcohol is a mitochondrial poison

157
Q

What is the cause of non-alcoholic fatty liver disease? How will AST relate to ALT?

A

Obesity and insulin resistance (metabolic syndrome)

Gives rise to the same spectrum of liver pathology as alcoholic

-> ALT > AST, the typical pattern of all non-alcoholic hepatitis (not mitochondrial toxins)

158
Q

What is the cause of Hereditary hemochromatosis?

A

Autosomal recessive mutation on HFE gene of chromosome 6 (C282Y)
-> increased intestinal absorption of dietary iron, with increased storage of iron in parenchymal cells and cell injury
(abnormal functioning of hepcidin, ferroportin, and transferrin due to HFE product interaction)

159
Q

What causes Wilson’s disease and what three tests are used to definitely test for it with high sensitivity?

A

Autosomal recessive loss of copper-transporting ATPase on chromosome 13 -> decreased secretion of ceruloplasmin from liver, as well as decreased excretion into bile.

  1. Serum ceruloplasmin - decreased in most
  2. 24-hour urine copper - increased in most
  3. Slit-lamp exam - Check for Kayser-Fleischer rings - present in 90% with neurologic presentation
160
Q

What are the consequences of copper excess in all liver and CNS in Wilson disease?

A

Free radical damage:

Liver - fat and glycogen accumulation -> hepatitis -> cirrhosis, HCC

CNS - neuronal injury -> neurologic and psychiatric manifestations.

Basal ganglia involvement: Dystonia, chorea, Parkinsonian symptoms

Elsewhere: depression, anxiety, psychosis

161
Q

What are the consequences of copper excess in cornea, kidneys, and blood?

A

Cornea - Kayser-Fleischer rings (Descemet’s membrane) and sunflower cataracts

Kidneys - Fanconi syndrome

Blood - Hemolytic anemias

162
Q

How is Wilson disease treated?

A

Copper chelation therapy (penicillamine, trientene), and oral zinc (blocks intestinal absorption of copper and binds free copper)

163
Q

What is the cause of primary biliary cholangitis (PBC) and who tends to get it? What is the pathognomonic lesion?

A

Probably an autoimmune disease characterized by progressive T-cell mediated destruction of small to medium sized intrahepatic ducts

As it is autoimmune, it is classically seen in middle-aged women with other autoimmune disorders (i.e. Sjogren’s, RA, Hashimoto)

Pathognomonic = florid duct lesion, giant cells around a duct

164
Q

What is seen in the lab values / tests for PBC?

A

Cholestatic lab profile (increased alk phos, GGT, cholesterol, conjugated bilirubin)

Increased IgM levels -> antimitochondrial antibodies

165
Q

What is the pathogenesis and a few causes of secondary biliary cholangitis?

A

Long-standing extrahepatic biliary obstruction -> increased pressure in intrahepatic ducts -> injury / fibrosis due to bile stasis

Common causes: bile duct strictures, choledocholithiasis, chronic pancreatitis (causes scarring)

166
Q

What ducts are destroyed in primary sclerosing cholangitis (PSC) and who tends to get it?

A

Progressive destruction of large intrahepatic and extrahepatic bile ducts

Found mostly in middle-aged men, strongly associated with ulcerative colitis
-> this disease will be p-ANCA positive (like UC, MP, CS)

167
Q

How does pathology appear in PSC?

A

Think sclerosing + targetting bile ducts

Alternating areas of stricture (concentric, “onion-skin” fibrosis around ducts) and dilatation “beads”.

Basically strictures of fibrosis that look like hyperplastic arteriolosclerosis of ducts, and dilations that are like beads

168
Q

What are the complications of PSC?

A

Progressive biliary obstruction and portal fibrosis -> biliary cirrhosis, like PBC and SBC.

Also, increased risk of cholangiocarcinoma (bile duct cancer due to inflammation) and gallbladder cancer

169
Q

What benign liver neoplasm is most associated with oral contraceptive or estrogen use, and almost never arises outside of this context? How does it present clinically?

A

Hepatocellular adenoma

Present in young women on oral contraceptives. May cause an acute abdomen due to rupture and intraperitoneal hemorrhage -> especially during pregnancy when there is increased estrogen, stimulating growth

170
Q

What is the most common liver malignancy and how does it tend to arise?

A

Metastasis to liver

Colon, pancreas, lung (spreads via systemic circulation thru hepatic artery), and breast (after lung metastasis)

171
Q

What is aflatoxin and why is it carcinogenic?

A

Toxin produced by Aspergillus flavus, which can contaminate stored peanuts and grains -> think of the cow next to the tractor in sketchy

Carcinogenic because it induces p53 mutations

172
Q

How does the liver appear pathologically in A1AT deficiency and what does it stain with?

A

Variable presentation, but often leads to cirrhosis due to damage and chronic hepatitis from the protease in hepatocytes

Stains PAS+ (A1AT is a glycoprotein), as well as eosinophilic cytoplasmic granules

173
Q

What is one marker which is associated with hepatocellular carcinoma?

A

Elevated serum alpha-fetoprotein

-> malignant hepatocytes express the fetal form of albumin

174
Q

What is the prognosis of hepatocellular carcinoma and why?

A

Poor -> usually spreads hematogenously due to lungs by the time there is a presenting complaint (i.e. Budd-Chiari syndrome) -> coagulopathy already spreading outside of liver

May be detected early by frequent ultrasounds in cirrhotic individuals

175
Q

What is the clinical triad of Budd-Chiari syndrome? What is seen in the liver?

A

Sudden liver enlargement (hepatomegaly), pain, and ascites - due to acute hepatic venous occlusion

Centrilobular congestion and necrosis can be seen

176
Q

What should be suspected in a patient with ulcerative colitis, and abdominal pain/distension, bloody diarrhea, fever, and signs of shock? What diagnostic study should be done?

A

Toxic megacolon
(vs cancer, which would not present with fever)

Colonoscopy is relatively contraindicated due to risk of perforation.

Should do an abdominal X-ray.
#410
177
Q

Why do we use the PT as a measure of liver function?

A 
Factor 7 has the shortest halflife, and thereby if liver function is suffering, PT will be the first measure to reflect the decline in the liver's synthesis ability
#1291
178
Q

What does new-onset odynophagia (pain with swallowing) suggest in a patient with GERD?

A 
Likely ulceration of the lower esophagus -> progression to erosive esophagitis
#11591
179
Q

What is seen on pathology of GERD?

A 
Basal zone hyperplasia (regenerating squamous epithelium due to damage), elongation of lamina propria papillae, eosinophilic + neutrophilic infiltrate
#286

Step 1 Miscellanous (40 decks)

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