Pathology Flashcards
Components of VACTERL syndrome
Vertebral Anal atresia Cardiac TE fistula Renal Limb
Difference between omphalocoele and gastroschisis
Omphalocoele = herniation through the ventral membranes
Gastroschisis = herniation all the way through the skin
What percentage of omphalocoeles present with other defects?
40%
What is the name for the most common site of ectopic gastric mucosa, and where is it typically located?
Inlet patches in Upper esophagus
Gastric heterotopia in the colon or SI
What are the consequences of inlet patches in the esophagus?
Occult blood loss due to ulceration, Barrett’s metaplasia, adenocarcinoma
What percentage of Meckel diverticula produce gastric acid and therefore confer high risk of perforation and intestinal bleeding?
50%
Which pathology is caused by failed involution of the vitelline duct?
Meckel diverticulum
What is the morphologic difference between a Meckel versus typical diverticulum?
Only Meckel has muscle
What is the most common location for a typical diverticulum?
Sigmoid colon
Which pathology is associated with Trisomy 18 and Turner Syndrome?
(because it’s genetic, it’s also more common in twins)
Pyloric stenosis
Which gender is at higher risk for pyloric stenosis?
Males
What is the treatment for pyloric stenosis?
Myotomy (splitting of the pyloric sphincter)
Which congenital disorder is (surprisingly) associated with incomplete penetrance?
Hirschsprung (RET gene)
What stain can be used to confirm the biopsy diagnsosis for Hirschsprung?
Acetylcholinesterase
Which part of the colon is typically most enlarged in Hirschsprung?
Cecum (because the sigmoid colon and rectum don’t work)
Which type of creature causes Chagas disease?
A protozoan
Which 3 arteries supply the esophagus?
upper - inferior thyroid
middle - aorta
lower - left gastric
Which condition is associated with loss of coordination between longitudinal and circular esophageal smooth muscle?
Nutcracker esophagus
Which muscular layers are associated with diffuse esophageal spasm?
Both
How can you distinguish Hypertensive LES from achalasia?
With hypertensive LES you still have normal peristalsis
Which muscular layers are associated with Chagas disease?
Myenteric only – difference from Hirschsprung!
Which pathology is related to diabetic neuropathy, Sarcoidosis, Down syndrome, and Sjogren’s?
Achalasia
In which part of the esophagus do you normally find diverticula? What is the exception?
Lower. Exception: Zenker
Which esophageal pathology may be associated with graft vs. host and GERD?
Esophageal webs
What is the difference between esophageal Schatzki A and B rings?
A = squamous B = columnar
BOTH DISTAL
GI bleeds are most common where?
Esophagus
Desquamative skin diseases like bullous pemphigoid and epidermolysis bullosa may predispose you to these 2 conditions
Esophagitis
Crohns
Which type of esophagitis causes necrosis?
Toxic – including pill esophagitis
Which virus causes esophagitis with cytoplasmic inclusions?
CMV
Which pathology is this?
Hyperemic mucosa with eosinophilia and some neutrophils; possibly with basal zone hyperplasia
Esophageal reflux
What’s special about the presentation of eosinophilic esophagitis?
It presents with dysphagia but NO REFLUX
How many cirrhosis cases lead to portal HTN?
50%
How are esophageal varices treated?
Beta blockers
In which gender is Barrett’s more common?
Males
Which esophageal pathology presents with velvety salmon-colored mucosa with squamous islands in a sea of columnar cells?
Barretts
What is the length that differentiates long segment Barretts from short segment? What is the significance there?
3cm
LONGER = MORE LIKELY TO BECOME DYSPLASTIC
Which pathology has pale blue wine goblet shaped cells?
Barrett
Most common benign mesenchymal esophageal tumor
leiomyoma
Which are more common in the esophagus: benign tumors or malignant?
Benign
2 cancers with signet ring cells
Esophageal adenocarcinoma
Diffuse gastric cancer
Which pathology are people with CagA negative H. pylori generally spared from?
Esophageal adenocarcinoma
What is tylosis and what does it signify?
Hyperkeratosis of palms and soles
White patch in the mouth
HOWEL EVANS SD (SCC ESOPHAGUS)
SOX2
Esophageal SCC
Location of adenocarcinoma vs SCC in the esophagus
Adeno = distal SCC = middle
Which type of esophageal cancer is more likely to metastasize?
SCC
2 diseases that cause hypertrophic gastropathy
Menetrier
Zollinger-Ellison
GI consequence of altitude sickness
Acute gastritis
Why are the elderly more susceptible to gastritis?
Reduced mucin and bicarb secretion
Surface epithelium with corkscrew profiles
Gastritis (mucus is gone so you can see the curvature of the cells)
How many critically ill patients develop ulcers in the first 3 days of their illness?
75%
What is a Dieulafoy lesion and what causes it?
A dilated artery in the gastric submucosa (in the lesser curvature) that may rupture
Cause: NSAIDS
What is GAVE (gastric antral vascular ectasia) and what causes it?
Longitudinal stripes of red mucosa alternating with pale bits (“watermelon stomach”); may rupture and bleed
Cause: Cirrhosis or SCLERODERMA
Association with CagA h. pylori and cancer
CagA POSITIVE = high risk
CagA NEGATIVE = protective
Nodular gastric mucosa with thickened rugal folds
H. pylori chronic gastritis
Which cause of chronic gastritis presents with elevated SERUM gastrin (hypergastrinemia)?
Autoimmune gastritis
What are the antibodies against in AIG?
Parietal cells & IF
specifically HK ATPase
Atrophic glossitis = which anemia?
B12
Chromogranin A
Autoimmune gastritis or carcinoid tumor
Type of tumor that may result from autoimmune gastritis
Carcinoid (may present as “NE hyperplasia”)
Onset time for autoimmune gastritis
20-30 years
Predisposing condition for MALToma
H. pylori chronic gastritis
Stomach region affected in eosinophilic gastritis
pylorus
Typical cause of eosinophilic gastritis
milk allergy
Patient has celiac disease. Their whole stomach is covered in aphthous ulcers. They have increased T cells.
Varioloform gastritis (Also known as lymphocytic gastritis )
Primary cause of granulomatous gastritis
Crohns
This malformation may predispose to PUD
Meckels
Most common location for PUD
proximal duodenum
Recurrence rate for H. pylori
20%
How can achlorhydria lead to cancer?
Permits the growth of bacteria that secrete carcinogenic nitrosamines
Gastritis cystica may present as polyposa (in the submucosa) or profunda (in the deep gastric wall). What causes it?
Trauma! (chronic gastritis and stomach surgery)
Multiendocrine neoplasia
Zollinger Ellison
Prognosis for gastrinoma: what might they become?
Up to 90% are malignant! May become carcinoid tumors
Prognosis for Menetrier disease
Associated with adenocarcinoma
Cerebriform rugal folds with enormous overstuffed mucus cells and Anti-TNF-Alpha Ab’s
Menetrier’s disease
Typical cause of a fundic gland polyp (2)
FAP or long term use of PPI’s (hypertrophy of oxyntic glands)
Gastric antral polyp with intestinal metaplasia
Gastric adenoma
CDH1 tumor suppressor
gastric adenocarcinoma
Discohesive non-glandular structures with flattening of rugal folds with mucin lakes
Diffuse gastric adenocarcinoma (linitis plastica)
Which type of gastric cancer has no precursor lesions: diffuse or intestinal?
Diffuse
Chimeric fusion gene: API2-MLT fusion product of Ch 11, 18
MALToma
Which type of cancer does MALToma progress to>
DIffuse large B cell lymphoma
Lymphoepithelial lesions in the pylorus should raise suspicion of ___
Maltoma
Body’s most common organ for a carcinoid tumor
Ileum
Synaptophysin
Carcinoid tumor
Typical progression of an ileal carcinoid tumor – where does it metastasize?
Liver
Forgegut vs midgut vs hindgut carcinoid tumors: prognosis
Only midgut is malignant (jejunum and ileum)!
Most common mesenchymal tumor of the abdomen
GIST
KIT
GIST
Prognosis for a GIST
benign, especially in the stomach (may be worse in intestine)
Carney Triad
1- GIST
2- paraganglioma
2- pulmonary chondroma
Best guess for a tumor that’s larger than 30 cm
GIST
Typical presentation of a GIST
Mass effect + anemia
Most common cause of intestinal obstruction worldwide
Hernia
Which type of hernia is most likely to lead to obstruction?
Inguinal
Biggest negative consequence of a hernia
Bowel infarction
Typical location for a volvulus
Sigmoid colon (LLQ)
Which childhood GI complication is associated with rotavirus and its vaccine?
Intussusception
This vessel has the worst prognosis in bowel ischema
SMA
Mortality rate in bowel ischemia
10%
Pathogen that can cause bowel ischemia
CMV
Presentation for bowel ischemia
bloody diarrhea with LLQ pain
What are the diagnostic criteria for angiodysplasia?
Exclusionary diagnosis for bloody diarrhea with no polyps or mucosal changes, and with ectatic nests
Commonly in cecum/RLQ
May be associated with Meckel
2 vitamin deficiencies that can cause peripheral neuropathy
B12; A
Gliadin
The wheat protein that triggers Celiac disease
Part of the GI tract most sensitive to gluten
duodenum, jejunum
Prognosis for Celiac
Mucosa returns to normal within 6-24 months of new diet
Dermatitis herpetiformis
Celiac
Small intestine is atrophic and flat, with no visibile villi
Celiac
Which cancer is associated with Celiac?
Enteropathy Associated T Cell Lymphoma (EATL)
also
Small intestinal adenocarcinoma
IPEX syndrome/FOXP3 mutation
Autoimmune enteropathy
Diffuse antibodies to every type of intestinal cell, some stomach cells, and some pancreatic cells, with mixed amounts of lymphocytic infiltrate
IPEX syndrome
Which organism can cause transient lactose intolerance?
Giardia
MTP protein (microsomal triglyceride transfer protein)
Abetalipoproteinemia
Intracellular lipid accumulations –> acanthocytic red cells
Abetalipoproteinemia
Hemagglutinin
Cholera
CFTR channel (infectious)
Cholera
What is the most common enteric pathogen in the US?
Campylobacter
This pathogen causes arthritis in patients who are HLA-B27+
Campylobacter
Guillain-Barre syndrome
Campylobacter
Cryptitis with abscess (but crypts are still preserved)
Campylobacter
How many cases of campylobacter progress to dysentery?
15% (but 50% of children)
What is the most common worldwide cause of dysentery?
Shigella
This organism has an intracellular tropism for APC’s (specifically M cells)
Shigella
This pathogen is non-motile
Shigella
This pathogen has a tropism for the left colon and ileum
Shigella
Pseudomembranes with aphthous ulcers
Shigella
May cause triad of reactive arthritis, urethritis, conjuctivitis
Shigella
May cause HUS
Shigella, EHEC
This intestinal bug has a surprising vaccine
Salmonella
May cause severe dissemninated disease in asplenic patients
Salmonella
This pathogen can cause gallstones
Salmonella typhi or paratyphi
Which type of salmonella can cause lymphatic hyerplasia?
Typhi (not dysenteriae)
Infection that causes grossly enlarged Peyers patches with mesenteric lymph node involvement
Salmonella
Infection that may cause osteomyelitis in sickle cell patients
Salmonella
Infection associated with rose spots on the chest and abdomen
Salmonella
This pathogen causes a worse infection in people with increased free iron, ie anemia
Yersinia
This pathogen may lead to protein loss and hypoalbuminemia
C. diff
Presents with the classic triad of diarrhea, weight loss, and joint pain
Whipple’s disease
The only pathogen that causes prolonged malabsorption syndromes
Whipple’s disease
The most common cause of acute gastroenteritis in the SU
Norovirus
The most common cause of severe childhood diarrhea
Rotavirus
This type of worm infection may persist for life because the worms can have their larval stage inside the body
Strongyloides
This type of worm also invades the lungs as part of its life cycle
Ascaris lumbricoides
Eterobius vermicularis
Pinworms
This worm causes anemia and significant mortality
Necator duodenale (hookworm)
This worm is famous for causing B 12 anemia
D. latum
This protozoan is an obligate glucose fermenter
Entamoeba
What are the path findings in IBS?
THERE ARE NONE!
Which disease is classified by the Rome Criteria?
IBS
This disease may be related to over-secretion of 5HT3
IBS
Malignant potential: Crohns & UC
Both are high!
Which can lead to toxic megacolon: Crohns or UC?
UC
Which has a higher recurrence rate: Crohns or UC?
Crohns
Helminths may be protective for this disease
IBD
Which is more likely to cause strictures: Crohns or UC?
Crohns
Which presents with finger clubbing: UC or Crohns?
Crohns
Which can be treated with Anti-TNF antibodies: Crohns or UC?
Crohns
Which biliary pathology is associated with UC?
PSC
Middle aged woman with colitis (diarrhea but no blood or weight loss); biopsy reveals dense subepithelial fibrous tissue with lymphocytes
Collagenous collitis
Patient with a history of UC had a colostomy and now has non-bloody diarrhea. Biopsy reveals mucosal lymphoid follicles, increased macrophages, and increased plasma cells.
Diversion colitis
Epithelial apoptosis of crypt cells
graft vs host
How may patients with diverticulae are symptomatic?
20%
Most common location for a colon polyp
Left colon
Etiology of a colon polyp
decreased epithelial turnover & shedding
Colon lesion with mature goblet and absorptive cells
hyperplastic polyp
SMAD4+
Juvenile polyps
How many patients with juvenile polyposis get colon cancer
50%
Retention polyps
Single juvenile polyps
Typical age of a patient with juvenile polyps who presents with rectal bleeding
<5
STK11
Peutz Jeghers
These juvenile polyp syndromes confer an increased risk of pancreatic cancer
Peutz Jeghers; Juvenile Polyps
Typical location for a Peutz Jeghers polyp
Small intestine
Rectal polyp in a child with digital clubbing and increased risk of ANY Gi cancer
Juvenile polyposis
Ileal polyp in a child with mouth freckles and increased risk of colon cancer
Peutz Jeghers
Which polyp presents with Congenital RPE hypertrophy?
Classic FAP
Colon polyp with a serrated surface
Hyperplastic polyp
Cowden syndrome, Cronkhite-Canada syndrome, and TSC all cause this type of polyp
Hamartomatous
Outcome of most colon adenomas
Nothing – rare to become cancerous (30% of adults in the US have them)
FAP has an association with this condition, taught by Dr. Galbraith
Nasopharyngeal angiofibroma
Primary syndromic cause of colon cancer (though still only 3%)
HNPCC (Lynch Syndrome)
Patients with colon polyps whose first degree relatives died of breast or pancreatic cancer may tip you off to this syndrome
HNPCC (Lynch Syndrome)
MSH2 or MLH1 (microsatellite instability/mismatch repair error)
HNPCC (Lynch Syndrome)
Patients with this syndrome present with colon cancer at a relatively young age, often located in the cecum
HNPCC (Lynch Syndrome)
Effect of aspirin and NSAIDS on colon cancer
Protective!
Most common mutation in colon cancer
APC (WNT/B catenin)
Location differences!
Polypoid exophytic colon cancer vs. napkin ring/apple core lesions
Exophytic = right colon (as in Lynch syndrome/HNPCC)
Napkin ring = left colon
Anemia in the elderly is this type of colon cancer until proven otherwise
Right sided (Exophytic)
This gross pathogen can cause appendicitis
Pinworms (vermicularis)
Prognosis for a carcinoid tumor of the appendix
Benign!
Bacteria with this type of metabolism predominate in periodontitis
Anaerobes
People with a variety of systemic and auto-immune diseases present with this dental complication
Periodontitis
This vascular disease is characterized by oral ulcers, genital ulcers, and uveitis
Behcet disease
“Pregnancy tumors” that often regress
Pyogenic granuloma
This type of herpes causes cold sores
HSV-1
This part of the mouth is most affected in a primary acute oral herpes infection
gingiva (acute herpetic gingivostomatitis)
Acantholysis with intracellular and extracellular edema
Oral HSV-1
How many people have candida as part of their normal oral flora?
50%
Raspberry/strawberry tongue
Strep A pharyngitis
Warthin Finkeldey giant cells
Measles
This infectious disease causes palatal petechiae
Mono
Most common cause of erythema multiforme
Stevens Johnson syndrome
Ludwig’s angina cellulitis is a consequence of this group of disorders
Pancytopenias
Leukoplakia or erythroplakia – which is raised? Which is depressed?
Raised – leukoplakia
Depressed – erythroplakia
Location of erythroplakia
Anywhere in the mouth
What percentage of HEENT cancers are SCC?
95%
70% of oropharyngeal cancers are caused by this
HPV-16
Which malignancy has the highest rate of secondary tumor growth, at 7%?
SCC of the oropharynx
What effect does HPV positivity have on prognosis of SCC?
Improves it!
Primary gene involved: classic (tobacco) oral SCC
p53 (53 cigarettes)
Primary gene involved: HPV oral SCC
p16 (for HPV16)
Which type of oral SCC has no precancerous lesions associated with it?
HPV+
This type of oral SCC presents with tumor cells hiding in tonsillar cryps or the base of the tongue, and may present as a sore throat
HPV+
This cancer is characterized by plaques with indurated (rolled) borders
Oral SCC
Extension and metastasis of oral SCC:
Extension: submandibular and cervical nodes
Mets: mediastinal nodes, lung, liver, bone
This very aggressive cyst is found in the posterior mandible of young males. It is lined by keratinized stratified SCC and has a very high recurrence rate.
Odontogenic Keratocyst
If a patient has more than 1 Odontogenic Keratocyst, they should be evaluated for this syndrome and gene.
Gorlin Syndrome (PTCH+)
The most common salivary gland lesion
Mucocele
Primary cause of a mucocele
Trauma
Fluctuant oral pseudocyst filled with inflammatory granulation tissue
Mucocele
Regression rate, mucocele
High
Large sublingual epithelial-lined lesion penetrating the myelohyoid muscle
Ranula
2 most common bacterial causes of sialadenitis
Staph and strep viridans
Typical patient with a salivary gland tumor (except Warthin tumors)
60 year old female
PLAG1
pleomorphic adenoma
How many pleomorphic adenomas will become malignant?
2%
Which 2 salivary gland tumors can be bilateral?
Warthin (benign) Acinic cell (malignant)
Oncocytic tumors
Warthin
Benign or malignant: Warthin tumor
Benign & unlikely to recur
MECT/MAML fusion gene and prognosis
Mucoepidermoid carcinoma – malignant!
Which malignant salivary gland tumor tends to be perineural?
adenoid cystic carcinoma
CD14+ cell
Kupffer cell
What is the function of Endothelin?
It is a vasoconstrictor that also causes scar contraction
Range of time for acute hepatitis
6 months
Two diseases that present with diffuse microvesicular steatosis
Fatty liver of pregnancy
Tetracycline toxicity
Which clotting factors are produced in the liver?
5, 7, 9, 10
Friable, hard, small liver with obliteration of the central vein
Cirrhosis
Why would you measure pulmonary venous wedge pressure in a patient with liver disease?
To determine the progression of cirrhosis
“Compaction of scars” in the liver indicataes this
Imminent regression
What percentage of people with chronic liver disease are asymptomatic?
40%
The risk of these 2 conditions continues to be elevated even if the patient’s cirrhosis is reversed.
HCC
Portal HTN
Spider angioma indicates this, which causes this
Chronic liver failure; hyperestrogenemia
Portal hypertension and ascites are almost always (>90%) indicative of this condition
Cirrhosis
What percentage of cirrhosis patients develop esophageal varices? What percentage will die as a result?
40%; 20%
Thrombocytopenia and pancytopenia indicate this complication of cirrhosis.
Congestive splenomegaly (“hypersplenism”) 2ry to portal HTN
If a patient with chronic liver failure has hepatopulmonary syndrome, in what position are they most comfortable and able to breathe?
Lying supine – standing = bad
How many cirrhosis patients get hepatopulmonary syndrome?
30%
Extreme over-dilation of pulmonary capillaries due to over-production of NO indicates this complication of cirrhosis and portal HTN
Hepatopulmonary syndrome
Patient has chronic liver failure, dyspnea, and digital clubbing. Dyspnea is NOT improved in the supine position.
Portopulmonary HTN (unknown physiology)
What is the mortality rate for patients with acute on chronic liver failure?
> 50%
During which period of viral infection is someone with hepatitis most contagious?
Incubation period (asymptomatic)
Which 2 types of hepatitis may lead to immune complex glomerulonephritis?
B, C
What is the most common symptom of chronic hepatitis?
FATIGUE not jaundice
Which antigen is elevated in HBV carriers?
HBsAg
Patient has elevated HBsAg and moderately elevated AST/ALT. They are asymptomatic. What type of HBV do they have?
ACTIVE! If it’s anything other than just HBsAg – even with no symptoms – it’s not a carrier state.
Mottled infiltrate with spotty necrosis (lobular hepatitis), hepatic dropout, pyknosis and no portal inflammation
Acute viral hepatitis
Presents with an extremely high plasma cell count in hepatic fluid
Acute Hep A
Extensive fibrosis and ductular reactions with inflammatory cells and interface hepatitis (inflammation of the junctions between hepatocytes and vessels)
Chronic viral hepatitis
This disease presents with hepatocytes filled with lipids and increased lymphoid follicles
Chronic Hep C
What causes the ground glass appearance of chronic Hep B?
Accumulation of HBsAg
50% of Americans are seropositive for this type of hepatitis
A
What is the most common disease course for HBV?
Acute hepatitis followed by full recovery
How many HBV patients develop cirrhosis? How many developing chronic disease without cirrhosis?
10% each
What is the most common mode of transmission for HBV worldwide?
Childbirth
Which hepatitis has an incubation period of 2-6 months?
B
Which Hep B protein confers increased risk to HCC? What is its function?
HBx; controls viral replication
Which cell marker is the only one you’ll see in the “window phase” of HBV infection
IgM
Continually elevated HBeAg indicates this
Progression to chronic liver disease
Which HBV protein confers resistance to drugs?
HBsAg
Which hepatitis presents 30% of the time in someone with no risk factors?
HCV
Which hepatitis can cause metabolic syndrome?
C (genotype 3)
E2 envelope protein
Confers HCV resistance to our antibodies
How does co-infection of HBV/HDV affect prognosis?
Prognosis is actually better if you get them both at once
In patient with HBV-HDV coinfection who has very elevated AST/ALT, which infection is predominating?
HDV
Which presents with an elevated T cell count – acute or chronic hepatitis?
BOTH
Which forms of hepatitis confer increased risk for HCC?
B, C
Which unpleasant GI bacteria can cause direct infection of the liver?
Salmonella typhi
PBC and PSC are both associated with this liver disease
Auto-immune hepatitis
Identify the patient and prognosis for each type of autoimmune hepatitis:
I (ANA/ASMA+)
II (LKM/CYP2D6+)
I = patients > 40 with better prognosis II = patients <20 with worse prognosis
Patient has high plasma cell count in hepatic fluid but is HAV negative
Autoimmune hepatitis
Hepatocyte rosettes
Autoimmune hepatitis
How many patients with autoimmune hepatitis will die on their first attack?
40%! It’s much worse than viral hepatitis
Which liver disease is associated with elevated NADH levels?
Alcoholic fatty liver
Which liver cell type is Keratin 8+ and Keratin 18+?
Mallory Denk bodies
In what conditions would you expect to see Mallory Denk bodies?
Alcoholic fatty liver, NAFLD, Wilson, Biliary tract disease
Micronodular perisinusoidal cirrhosis
Alcoholic steatofibrosis (Laennec cirrhosis)
What is the primary cause of chronic liver disease in the US?
NAFLD
> 5% of hepatocytes are triglyceride +
NAFLD
Which type of metabolic liver disease is more likely to lead to cirrhosis: AFLD or NAFLD?
NAFLD – but it’s still super uncommon
C282Y vs H63D mutation in Hemochromatosis
C282Y = more severe; more common in whites H63D = less severe
Causes pseudogout
Hemochromatosis
Which metabolic liver disease presents with absolutely 0 evidence of inflammation?
Hemochromatosis
Which metabolic liver disease causes cardiomyopathy?
Hemochromatosis
Which condition increases risk of HCC by 200x regardless of treatment?
Hemochromatosis
This liver condition is diagnosed via buccal biopsy
Secondary (fetal) Hemochromatosis
ATP7B
Wilson disease
Which metabolic disease cannot be diagnosed based on a blood test?
Wilson – serum copper is unreliable
Rhodamine-Orceine stain
Wilson disease
Cutaneous necrotizing panniculitis (SQ fat)
A1AT
The most commonly diagnosed inherited liver disease in children
A1AT
Cytoplasmic globular inclusions in hepatocytes that are PAS+
A1AT
Feathery degeneration
Kuppfer cells filled with bile
Charcot triad for ascending cholangitis
RUQ pain, fever, jaundice
Jigsaw-shaped liver nodules
Biliary cirrhosis
2 conditions that cause periportal accumulation of Mallory Denk bodies
Wilson; biliary cirrhosis
Which is caused by sepsis: canalicular cholestasis, or ductular cholestasis?
Ductular
Which people are at highest risk for intrahepatic gallstone formation?
Asians
Chemical composition of an intrahepatic gallstone?
Calcium
Which liver disease is the primary cause of liver-related death in children under age 2?
Biliary atresia
This LIVER disease is related to a rotavirus, reovirus, or CMV infection in infancy
Perinatal biliary atresia
This autoimmune cholangiopathy is AMA+
Primary biliary cirrosis
This autoimmune cholangiopathy is ANCA+
Primary sclerosing cholangitis
This autoimmune cholangiopathy confers an increased risk of cancer
PSC
This autoimmune cholangiopathy has a strong association with Sjogrens syndrome
PBC
This liver disease presents with an enlarged cirrhotic liver with florid duct lesions. What is its prognosis
PBC – treatable with oral ursodeoxycholic acid!
Which disease is treataed with oral ursodeoxycholic acid?
PBC
String of beads morphology
PSC
Onion skin fibrosis with tombstone scars
PSC
8 year old female with PCKD or Caroli disease presents with RUQ pain and jaundice
Choledochal cyst (dilation of common bile duct)
Caroli syndrome
a baby liver absolutely riddled with cysts
Von Meyenberg complexes
bile duct hamartomas
Peliosis hepatitis
Dilation of sinusoids due to impaired efflux of hepatic blood; related to bartonella in AIDS patients
Vanishing bile duct syndrome is associated with
host-versus-graft disease
Single hepatic mass lesion with a stellate central scar (fibromuscular hyperplasia) in a healthy adult
Focal nodular hyperplasia
Liver covered in nodules but no evidence of fibrosis; associated with transplants and systemic disease
Nodular regenerative hyperplasia
Which has a worse prognosis (and what is the prognosis): focal nodular hyperplasia vs nodular regenerative hyperplasia
Nodular regenerative hyperplasia –> portal HTN
Most common benign neoplasm of the liver
Cavernous hemangioma
HNF-1A
Hepatocellular adenoma from birth control
Null levels of LFABP
Hepatocellular adenoma from birth control (HNF-1A)
Related to MODY-3 diabetes
Hepatocellular adenoma from birth control (HNF-1A)
Rank the hepatocellular adenomas in terms of their risk of malignant transformation
B-catenin > inflammatory > HNF-1A birth control
hepatocellular adenoma with telangiectasia
Inflammatory
Glutamine synthetase + indicates this genetic mutation
B catenin
FAP and Beckwith Wiedemann predispose to this liver disease
Hepatoblastoma
2 most common genes associated with HCC
B-catenin activation P53 inactivation (esp with aflatoxin)
Which is more highly correlated with aflatoxin: p53 or B catenin?
p53
HNF4-alpha
inflammatory HCC
AFP+
HCC
Primary metastasis site from the liver
lung
Oncocytic tumor
HCC
Warthin
Klatskin tumor
Cholangiocarcinoma arising in the perihilar region – the most common CCA!
ABCG8 gene (ATP cassette transporter)
hereditary gallstones
Ascending cholangitis causes this kind of stone
pigment
Bouveret syndrome
gallstone ileus
Bacteria most commonly implicated in acute cholecystitis
Salmonella typhi
Rokitansky-Aschoff sinus
pocket of gallbladder poking through muscular wall; caused by reactive hyperplasia secondary to chronic cholecystitis
Xanthoma cell
foamy bilious macrophage
Which is more common: extrahepatic CCA or gallbladder CA?
Gallbladder CA
ERBB2 (Her-2/neu) mutation
Gallbladder cancer
Most common metastasis site for gallbladder CA
liver
PDX1
pancreatic agenesis
SPINK1
protein that prevents the healthy pancreas from digesting itself; mutated in AR pancreatitis
2 most common causes of pancreatitis
Alcohol; gallstones
Gender preference; pancreatitis
male
pathology made worse by hypercalcemia
pancreatitis
which is the only pancreatic enzyme that is normally activated inside the pancreas?
lipase
Which virus can randomly cause pancreatitis?
mumps (paramyxo)
PRSS1
AD pancreatitis; gain of function in trypsinogen gene (“press the on button”)
How many patients with hereditary pancreatitis will develop pancreatic CA?
40%
Morphologic difference between acute interstitial & acute necrotizing pancreatitis
Interstitial = necrosis of fats Necrotizing = necrosis of pancreatic cells
Which is elevated first in pancreatitis: amylase or lipase?
amylase
How many cases of chronic pancreatitis are hereditary?
25%
Acinar cell dropout indicates
chronic pancreatitis
Chronic pancreatitis with large numbers of protein plugs indicates this etiology
Alcoholism
Why can opiate abuse lead to pancreatitis?
increased tone of sphincter of Oddi
Severe chronic pain is a consequence of this disease
chronic pancreatitis
AD-PCKD and VHL predispose to this condition
congenital pancreatic cyst
Which pancreatic cystic neoplasm is located at the head of the pancreas and commonly involves the pancreatic duct?
IPMN (intraductal papillary mucinous neoplasm)
Which pancreatic cystic neoplasm is more common in males?
IPMN (intraductal papillary mucinous neoplasm)
Which pancreatic cystic neoplasm is common in young women?
solid pseudopapillary neoplasm
Rank the cystic pancreatic neoplasms by malignant potential
- solid pseudopapillary neoplasm
- mucinous cystic = IPMN
- serous cystic
This pancreatic cystic neoplasm is associated with a VHL mutation
serous cystic
These pancreatic cystic neoplasms are associated with a KRAS mutation
mucinous cystic; IPMN
This pancreatic cystic neoplasm is associated with a WNT mutation
solid pseudopapillary neoplasm
A precursor lesion to which cancer is delineated by marked telomere shortening?
PanIN (pancreatic CA)
This cancer is differentiated by:
1) tumor suppressor mutation - CDKN2A
2) oncogene activation – KRAS
Pancreatic CA
CDKN2A methylation has been linked to both this GI cancer and melanoma
Pancreatic CA
Most common site for pancreatic CA metastasis
liver
Cancer of which organ is famous for having a very thick, dense fibrous capsule (large desmoplastic reaction)?
Pancreas
+ Carcinoembryonic Ag
Pancreatic CA
This rare cancer presents with secretion of active pancreatic enzymes and metastatic fat necrosis
Acinar cell carcinoma
This rare cancer has a good prognosis, occurs in children, and has mixed acinar/squamous cells
Pancreatoblastoma
Where are curling ulcers located?
Stomach
Lesions related to PPI’s and FAP
Fundic gland polyps
Stomach polyps made of intestinal cells
Gastric adenoma
PRE MALIGNANT IF LARGE
