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193 Cards in this Set

  • Front
  • Back
Most common tumor of the salivary gland


Most common malignant tumor of the salivary gland
pleomorphic adenoma

mucoepidermoid carcinoma
Patient presents with a painless, moveable mass on their salivary gland

histologically: polygonal and epithelial spindle shaped myoepithelial cells in a variable stroma

dx?

course?
pleomorphic adenoma

benign with high rate of recurrence
Salivary gland tumor

Patient has salivary gland tissue trapped in a LN, surrounded by germinal centers (lymphatic tissue)

dx and course
Warthin's tumor, benign
Child presents with a painless, slow growing, hard mass on salivary gland

histo: see squamous cells, mucus-secreting cells, intermediate cells

dx? course?
mucoepidermoid carcinoma

malignant
Patient has progressive dysphagia to solids and liquids

-on barium swallow, you see dilated esophagus with an area of distal stenosis (bird's beak)

dx?
what is the cause?
increases risk of what?
achalasia

loss of myenteric (aurebach's) plexus --> failure of LES to relax --> high LES opening pressure and uncoordinated peristalsis

increased risk of esophageal carcinoma
Patient with Chagas' disease develops dysphagia to solids and liquids
-see stenosis on barium swallow

dx?
achalasia secondary to chagas disease
Patient has esophageal dysmotility involving low pressure proximal to the LES

one disease association
scleroderma (CREST)
Patient presents with
-heartburn
-regurgitation upon lying down
-nocturnal cough and dyspnea

dx?
GERD
Patient presents with painless bleeding of submucosal veins in lower 1/3 of esophagus

histo: can see sclerosed area with esophageal mucosa normal

dx?
esophageal varices
3 conditions associated with esophagitis
Reflux
Infection (HSV, CMV, Candida)
Chemical ingestion
Patient is an alcoholic/bulemic who presents with hematemesis

on endoscopy, you see mucosal lacerations at GE junction

dx?

what caused it?
Mallory-weiss

severe vomiting
patient has been violently retching --> transmural esophageal rupture

dx
BoerHaave Syndrome

Been Heaving Syndrome
Esophageal strictures

associated with 2 conditions
lye ingestion

acid reflux
Patient is a post-menopausal woman, comes in with triad of
-dysphagia (esophageal webs)
-glossitis
-iron deficiency anemia
plummer vinson syndrome
What happens in Barret's esophagus

associated with what three conditions
Chronic acid reflux (GERD) --> replacement of stratified squamous epithelium with intestinal (columnar) epithelium in distal esophagus

esophagitis, esophageal cancers, esophageal ulcers
Risk factors for esophageal cancer (mnemonic)
ABCDEF
Alcohol/achalasia
Barrett's
Cigarettes
Diverticuli
Esophageal web (plummer vinson), Esophagitis
Familial
Patient who smokes, drinks, has Barrett's and a family history of esophageal cancer comes in with progressive dysphagia (solids --> liquids) --> weight loss

most common type in world and where is the lesion?

most common type in US and where is the lesion
Esophageal cancer

world = sq cell - upper and middle 1/3

US = adenocarcinoma - lower 1/3
Patient has diarrhea, steatorrhea, weight loss, weakness

You suspect she cannot absorb her food

6 possible diagnoses
Tropical sprue
Whipple's
Celiac sprue
Disaccharidase deficiency
Abeta-lipoproteinemia
Pancreatic insufficiency

These Will Cause Devestating Absorption Problems
How is tropical sprue different from celiac sprue (2 ways)
1. tropical is infectious, celiac is autoimmune

2. tropical affects whole small bowel, celiac just the proximal (jejunum)
How do you treat tropical sprue
Antibiotics
An older man gets diarrhea, steatorrhea, weight loss, and weakness

-also has arthralgias, cardiac, neuro symptoms

you recover a gram positive organism with PAS-positive macrophages in the intestinal lamina propria and mesenteric nodes

dx? organism
Whipple's disease (malabsorption)

Tropheryme whippelii
Celiac sprue

cause
autoantibodies to gluten (gliadin) in wheat and other grains
Following an injury (like viral diarrhea) to the intestinal vili, a patient drinks milk, develops osmotic diarrhea

what happened
lactase is located in tips of villi, so if these are damaged, you experience self-limited disaccaridase deficiency
Most common disaccharidase deficiency

how do the villi look
lactase deficiency --> milk intolerance

normal appearing villi
Malabsorption syndromes

Young child presents with malabsorption and neuro smptoms

-on histo, you see fat accumulation in enterocytes

dx? what 's going on
abeta lipoproteinemia

decreased synth of apo B --> cannot generate chylomicros --> cannot secrete cholesterol, VLDL into blood stream --> accumulates in enterocytes
Pancreatic insufficiency

3 causes
Cystic fibrosis

obstructing cancer

chronic pancreatitis
Patient gets malabsorption of fat and fat-soluble vitamins (A, D, E, K)

patient also has chronic pancreatitis

dx?
pancreatic insufficiency
Patient is of N. European descent, gets steatorrhea

-find antibodies to gliadin and tissue transglutaminiase

histo: villous blunting, crypt hyperplasia, lymphocytes in lamina propria

dx?
associated with what conditions?
Celiac sprue = autoimmune intolerance of gliadin, decreases mucosal absorption in jejunum

assoc with dermatitis herpetiformis, increases risk of malignancy (T-cell lymphoma from lymphocyte infiltrate)
How do NSAIDS --> acute gastritis
NSAIDs reduce PGE2 --> decreases gastric mucosa protection
How do burns lead to acute gastritis
burns --> decreased plasma volume --> sloughing of gastric mucosa (Curling's ulcer)

BURNed by a CURLING iron
How does brain injury lead to acute gastritis
Increased ICP --> vagal stimulation --> increased ACh --> increased H production (Cushing's ulcer)

"alway's CUSHION the brain"
Patient with an autoimmune disorder gets
-autoantibodies to parietal cells
-pernicious anemia
-achlorhydria

dx?
Chronic gastritis type A (fundus/body)
Most common type of chronic gastritis?

cause?

increases what?
Type B (antrum)

H. pylori

increases risk of MALT
Patient gets gastric hypertrophy with protein loss, parietal cell atrophy, and increased mucous cells

also see that rugae of stomach are hypertrophied, look like brain gyri

dx?
course?
menetrier's disease

precancerous
stomach cancer
a. common type
b. course
c. common features
a. adenocarcinoma
b. early, aggressive local spread and node/liver mets
c. signet ring cells, acanthosis nigricans
Dietary nitrosamines (smoked fod), achlorhydria, chronic gastritis, type A blood

associated with what condition
stomach cancer
what is linitis plastica
stomach cancer that is diffusely infiltrative (thickened, rigid appearance, "leather bottle")
Patient has stomach cancer, metastasizes to left supraclavicular node

what is the node called
Virchaow's node
Patient has stomach cancer that progresses to bilateral ovaries

there are abundant mucus and signet ring cells

dx?
Krukenberg's tumor
Patient has stomach cancer, metastasizes to subcutaneous periumbilical region

dx?
sister mary joseph nodule
Patient comes in with abdominal pain that gets worse with meals, weight loss
-patient is older
-they take NSAIDS
-have a positive urease test

dx?
cause?
Gastric ulcer

H. Pylori in 70%
Due to decrease in protection against gastric acid
Difference in pain sequelae between a gastric and a duodenal ulcer
gastric = pain worse with meals --> wt. loss

duodenal = pain better with meals --> wt. gain
Duodenal ulcer

cause
nearly 100% have H. pylori infection


gastric acid secretion (eg. ZE syndrome) or decrease in mucosal protection
duodenal ulcer

what happens to brunner's glands
hypertrophy
stomach cancer
a. common type
b. course
c. common features
a. adenocarcinoma
b. early, aggressive local spread and node/liver mets
c. signet ring cells, acanthosis nigricans
Dietary nitrosamines (smoked fod), achlorhydria, chronic gastritis, type A blood

associated with what condition
stomach cancer
what is linitis plastica
stomach cancer that is diffusely infiltrative (thickened, rigid appearance, "leather bottle")
Patient has stomach cancer, metastasizes to left supraclavicular node

what is the node called
Virchaow's node
Patient has stomach cancer that progresses to bilateral ovaries

there are abundant mucus and signet ring cells

dx?
Krukenberg's tumor
Patient has stomach cancer, metastasizes to subcutaneous periumbilical region

dx?
sister mary joseph nodule
Patient comes in with abdominal pain that gets worse with meals, weight loss
-patient is older
-they take NSAIDS
-have a positive urease test

dx?
cause?
Gastric ulcer

H. Pylori in 70%
Due to decrease in protection against gastric acid
Difference in pain sequelae between a gastric and a duodenal ulcer
gastric = pain worse with meals --> wt. loss

duodenal = pain better with meals --> wt. gain
Duodenal ulcer

cause
nearly 100% have H. pylori infection


gastric acid secretion (eg. ZE syndrome) or decrease in mucosal protection
duodenal ulcer

what happens to brunner's glands
hypertrophy
How are the margins of a duodenal ulcer different from those of a carcinoma
ulcer = clean, "punched out" margins

carcinoma = raised/irregular margins
4 complications of duodenal ulcer

Is it cancerous?
bleeding, penetration to pancreas. perforation, obstruction

No
IBD: Crohn's vs. UC

etiology
C = disordered response to intestinal bacteria

UC = autoimmune
IBD: Crohn's vs. UC

location?
Skipping?
Rectum?
C = any portion of GI (often terminal ileum and colon), skip lesions, rectal sparing

UC = colon, continuous, involves rectum
IBD: Crohn's vs. UC

a. barium swallow x-ray image
C = string sign from bowel wall thickening

UC = lead pipe from loss of haustra
IBD: Crohn's vs. UC

layers of inflammation
C = transmural

UC = mucosal and submucosal
IBD: Crohn's vs. UC

gross appearance of mucosa and surrounding
C = cobblestone mucosa with creeping fat + linear ulcers, fissures, fistulas

UC = friable mucosal pseudopolyps with freely hanging mesentary
IBD: Crohn's vs. UC

Micro: nonccaseating granulomas and lymphoid aggregates
Crohns
IBD: Crohn's vs. UC

Micro: crypt abscesses and ulcers, bleeding, NO granulomas
UC
IBD: Crohn's vs. UC

Complications include strictures, fistulas, perianal disease, malabsorption, nutritional depletion, colorectal cancer
Crohn's
IBD: Crohn's vs. UC

complications include malnutrition, toxic megacolon, colorectal carcinoma
UC
IBD: Crohn's vs. UC

Bloody diarrhea +
-pyoderma gangrenosum
-primary sclerosing cholangitis

treat?
UC

ASA prep (sulfasalazine), 6-MP, infliximab, colectomy
IBD: Crohn's vs. UC

Diarrhea (may be bloody) +
-migratory polyarthritis
-erythema nodosum
-ankylosing spondylitis
-uveitis
-immunologic disorders

treat?
Crohn's

corticosteroids, infilximab
Patient has recurrent, chronic abdominal pain with alternating diarrhea and constipation

also has 2 or more of the following
1. pain improves with defecation
2. change in stool frequency
3. change in stool appearance

dx?
Treat?
Irritable Bowel syndrome

treat symptoms
- SSRI's, TCA's, loperamide
Most common indication of emergent abdominal surgery in kids
appendicitis
Patient presents with diffuse periumbilical pain --> localizes to area between iliac crest and umbilicus

also has nausea, fever

possible cause in kids?
2 causes in adults?
appendicitis

kids - lymphoid hyperplasia after viral infection

adults - obstruction, fecalith
Patient presents with diffuse periumbilical pain --> localizes to area between iliac crest and umbilicus

What must you rule out if patient is elderly? If a woman?
Appendicitis symptoms

elderly = diverticulitis

woman = ectopic pregnancy (use B-hCG)
patient has appendicitis

what is one complication you fear
perforation --> periotonitis
Where do diverticula most often affect?
sigmoid colon
What is a diverticulum?

difference between true and real?
blind pouch in gut

true = all three layers outpouch (Meckel's)

false = divertculum or pseudodiverticulum - only mucosa and submucosa outpoutch (occurs especially where vasa recta perforate muscularis externa)
Patient with vague discomfort and/or painless rectal bleeding eats a low fiber diet, is over 60 yo

what is he at risk for?

cause?
diverticulosis = many diverticula, most often in sigmoid colon

high intraluminal pressure --> focal weakness in colonic wall
Patient has
-bright red rectal bleeding
-bubbles in pee (pneumaturia) due to colovesical fistula (colon and bladder fistula)
-LLQ pain, fever, leukocytosis

dx?
what is happening?
what do you fear?

treat?
diverticulitis = inflammation of diverticula (macrophages)

fear perforation --> peritonitis, abscess formation, or bowel stenosis

give antibiotics
Patient presents with halitosis, dysphagia, obstruction

on barium swallow, you see herniation of mucosal tissue at junction of pharynx and esophagus

dx?
Zenker's diverticulum (false)
Most common congenital anomaly of GI tract

what is the congenital problem
Meckel's diverticulum

persistence of the vitelline duct or yolk stalk
Patient has
-bleeding
-intussusception (part of bowel invaginated on another)
-volvulus (obstruction do to abnormal bowel twisting) near terminal ileum

pectenate scan finds ectopic acid-secreting gastric mucosa and pancreatic tissue

dx?
5 "rule of 2's"?
Meckel's diverticulum (true)

2 inches long
2 feet from ileocecal valve
2% of population
2 year-olds or younger
2 types of epithelium (gastric/pancreatic)
What is an omphalomesenteric cyst

what can it be confused with
cystic dilation of vitelline duct

Meckel's diverticulum = persistence of vitelline duct or yolk stalk
Patient is a kid that gets 'telescoping' of 1 bowel segment into a distal segment

dx?
cause in kids?
cause in adults?
treat?
intussusception - can compromise blood supply

kids - idiopathic, adenovirus
adults (rare) - intraluminal mass

treat: surgery
Patient gets twisting of portion of bowel around it's mesentary --> obstruction, infarction

who does this affect mostly?

where does it affect mostly?
elderly

cecum and sigmoid colon area (redundant mesentary)
Baby fails to pass meconium

Presents with chronic constipation early in life

On intestinal biopsy, you see no meissner's or aurebach's plexuses

Baby also has Down Syndrome

Dx?
pathology/embryological problem
Hirschsprung's disease (risk increases with Down's)

Failure of neural crest cell migration --> lack of enteric nerve plexus --> constricted aganglionic segment with megacolon proximally
Kid with Down's syndrome has early bidious vomiting and proximal stomach distention

you see closed off small bowel

dx?

what happened?
duodenal atresia

failure of recanalization of small bowel
Baby born with CF cannot poop

dx?
what happens?
meconium ileus

meconium plug obstructs intestine --> no POO
What is the condition

necrosis of intestinal mucosa, possible perforation, often involving colon, can involve whole GI

more often seen in preemies

dx?
necrotizing enterocolitis
Elderly person has pain after eating --> weight loss

caused by low intestinal blood flow

what is happening? where?
ischemic colitis

splenic flexure and distal colon
what is an adhesion
acute bowel obstruction, often from recent surgery --> well-demarcated necrotic zones
older patient presents with bleeding in intestine

angiography shows tortuous dilation of vessels in cecum, terminal ileum, and ascending colon

dx?
angiodysplasia
2 types of colonic polyps

what do they look like and how dangerous are they
tubular adenomas = small, rounded, less malignant potential

villous adenomas = long, finger-like, more malignant potential
What are colonic polyps

where do they often occur

are the cancerous
masses protruding into gut lumen, giving a sawtooth appearance

often rectosigmoid

90% non-neoplastic
type of polyps that are precancerous

factors affecting malignancy
adenomatous

increasing size, villous histology, increased epithelial dysplasia --> colorectal cancer
What is the most common non-neoplastic polyp in the colon and where are they found
hyperplastic

>50% at rectosigmoid
mostly sporadic polyps in children < 5

where?

what if they are single?
what if multiple?
juvenile

rectum

if single, no malignant potential

If multiple, indicates juvenile polyposis syndrome -> increases risk of adenocarcinoma
Autosomal dominant syndrome featuring nonmalignant hamartomas in GI +
-hyperpigmented mouth, lips, hands, genitalia

dx? increases risk of what
Peutz-Jeghers


increases risk of CRC and visceral malignancies
3rd most common cancer, 3rd most deadly in USA, mostly affects men >50, 25% have a family history
colorectal cancer
Patient has thousands of polyps all over colon, always involves rectum

what is the genetic defect?
Familial adenomatous polyposis

autosomal dominant - APC gene (5q)

Requires two hits --> CRC
Familial adenomatous polyposis + osseous and soft tissue tumors, retinal hyperplasia
Gardner's syndrome
Familial adenomatous polyposis + malignant CNS tumor
Turcot's syndrome

TURcot's = TURban
HNPCC
a. genetics
b. how many progress to CRC
c. what is always involved
a. autosomal dominant mutation in DNA mismatch repair genes

b. 80%

c. proximal colon
IBD
Strep bovis bacteremia
tobacco use
large villous adenoma
Juvenile polyposis syndrome
Peutz-Jeghers syndrome

risk factors for what
Colorectal cancer
Where is the colorectal cancer

obstruction, colicky pain, hematochezia
distal colon
Where is the colorectal cancer

dull pain
iron deficiency anemia
fatigue
proximal colon
Patient is an older male with iron deficiency anemia
-stool occult blood test positive
-see CEA tumor marker
-apple core lesion on barium xray

dx?
colorectal cancer
2 molecular pathways to colorectal cancer
1. microsattelite instability (15%): DNA mismatch repair gene mutations --> sporadic, HNPCC syndrome
-mutations accumulate, but no morphology

2. APC/b-catenin (85%)
What is the order of genetic mutations that lead from a normal colon to carcinoma
1. loss of APC --> decreased intercellular adhesion, increased proliferation

2. K-RAS mutation - unregulated intracellular signal transduction --> adenoma

3. p53 --> tumorigenesis --> carcinoma
Carcinoid tumor

tumor of what kind of cells?
3 most common sites?
where is it most malignant?
neuroendocrine cells (secrete 5-HT)

appendix, ileum, rectum

small intestine
Carcinoid tumor

what do you see on EM
dense core bodies
What determines if a carcinoid tumor causes carcinoid syndrome
If tumor is confined to GI, no syndrome (liver metabolizes 5-HT)

if metastasizes (usually to liver) --> syndrome
patient has
-wheezing
-right sided heart murmur
-diarrhea
-flushing
-high 5-HT in blood

dx?
carcinoid tumor, metastasized outside GI
-Esophageal varices (hematemesis)
-peptic ulcer --> melena
-splenomegaly
-caput medusae, ascites
-gastropathy
-hemorrhoids

what is the condition
portal HTN
-coma
-sleral icterus
-breath smells like freshly opened corpse
-spider nevi
-gynecomastia
-jaundice
-testicular atrophy
-asterixis
-decreased prothrombin and clotting factors (bleeding up)
-anemia
-ankle edema

effects of what
liver cell failure
what is cirrhosis

how does the liver respond
fibrosis of liver

nodular regeneration
Metabolic insults --> Alcohol, hemochromatosis, Wilson's disease

what type of hepatocellular regeneration
micronodular (<3mm), uniform size
injury causing necrosis (post-infectus, drug induced)

what type of hepatocellular regeneration

increases risk of what
macronodular (>3mm), variable size


increases risk of hepatocellular carcinoma
what is bridging fibrosis
hepatocellular regeneration

regenerative lesions connected by fibrous bands
How can you relieve portal HTN
shunt between portal and systemic circulation
What is the probable diagnosis in GI

ALT > AST
viral hepatitis
What is the probable diagnosis in GI

AST > ALT
alcoholic hepatitis
What is the probable diagnosis in GI

AST up
Myocardial infarction
What is the probable diagnosis in GI

GGT (gamma glutamyl transpeptidase)
Liver diseases, heavy alcohol consumption
What is the probable diagnosis in GI

alkaline phosphatase up (3)
Obstructive liver disease (hepatocellular carcinoma)

Bone disease

Bile duct disease
What is the probable diagnosis in GI

high amylase (2)
acute pancreatitis, mumps
What is the probable diagnosis in GI

lipase
acute pancreatitis
What is the probable diagnosis in GI

ceruloplasmin down
wilson's disease
A child has a VZV or influenza B infection

after treatment, gets mitochondiral abnormalities, fatty liver (microvesicular), hypoglycemia, coma

what was he treated with?

what is the MOA of disease
Reye's syndrome -child given aspirin

aspirin metabolites inhibit beta oxidation by reversible inhibition of mitochondrial enzyme
Patient with moderate alcohol intake has abundant fat-filled vacuoles w/o inflammation in liver

hepatomegaly

dx?
treat?
hepatic steatosis

reversible --> stop alcohol!
Patient with long term alcohol use has
-AST>ALT (1.5:1)
-swollen and necrotic hepatocytes with PMN infiltrate
-See intracytoplasmic eosinophilic inclusions that look like twisted ropes

dx?
Alcoholic hepatitis
(see mallory bodies)

you're toASTed with alcoholic hepatitis (remember AST > ALT)
Patient with long-term alcohol use has
-jaundice
-hypoalbuminemia
-histo: see sclerosis around central vein (zone III)
-gross: shrunken liver with large nodules

dx?
alcoholic cirrhosis

final and irreversible
Most common primary malignant tumor of liver in adults
hepatocellular carcinoma
hepatitis B and C
Wilson's
hemochromatosis
a1-antitrypsin deficiency
alcoholic cirrhosis
aflatoxin (peanuts)

increase risk of what
hepatocellular carcinoma
patient has
-jaundice
-tender hepatomegaly
-ascites
-polycythemia
-hypoglycemia
-a-fetoprotein increases

dx?
may lead to what?
how is it spread
hepatocellular carcinoma

budd-chiari (occlusion of hepatic veins)

hematogenous
nutmeg liver

pathologically, what is happening? what can it lead to?

2 causes?
backup of blood into liver, can lead to centrilobular congestion and necrosis --> cirrhosis

RH failure, budd-chiari
Patient has congestive liver disease
-hepatomegaly
-ascites
-abdominal pain
-can cause liver failure
-varices, visible abdominal and back veins

-no JVD

dx?

what is happening?
Budd - Chiari

occlusion of IVC or hepatic veins --> cetnrilobular congestion and necrosis
Hypercoaguable state
polycythemia vera
pregnancy
hepatocellular carcinoma

associated with what condition that causes congestive liver disease?
budd-chiari = occlusion of IVC or hepatic veins
Patient gets panacinar emphysema due to decrease in elastic tissue in lungs

also see PAS-positive globules in liver

dx?
path?
genetics?
a1-antitrypsin deficiency

misfolded gene product accumulates in hepatocellular ER, lungs lose elastic tissue

codominant
What is physiologic neonatal jaundice

How do you treat
immature UDP-glucoronyl transferase --> unconjugated bilirubinemia --> jaundice, kernicterus

phototherapy (converts UCB to water-soluble form)
2 types of bilirubin

which is water soluble? How is it excreted?
conjugated and unconjugated

conjugated (direct) is soluble --> excreted in urine, goes to bile to make urobilinogen
What are 2 process by which urobilinogen is made
direct bilirubin put into bile, converted by gut bacteria to urobilinogen

heme metabolism
Patient has jaundice...what type

-high conjugated and unconjugated bilirubin

-high urine bilirubin

-normal to low urobilinogen
hepatocellular
Patient has jaundice...what type


-high conjugated bilirubin
-high urine bilirubin
-low urine urobilinogen
obstructive (interrupted drainage of bile in biliary system)
Patient has jaundice...what type

-high unconjugated bilirubin
-acholuria (no bilirubin in urine)
-high urine urobilinogen
hemolytic
Hereditary hyperbilirubinemias

Patient has unconjugated hyperbilirubinemia with no clinical consequences
-no hemolysis
-increased bilirubin with fasting and stress

dx? path?
Gilbert's

Mildly decreased UDP-glucoronyl transferase or decreased bilirubin uptake
Hereditary hyperbilirubinemias

Early in life, patient presents with
-jaundice
-kernicterus (bilirubin in brain)
-high unconjugated bilirubin

dx? path?
Crigler-Najjar syndrome type I

absent UDP-glucoronyl transferase --> patients DIE within a few years
Crigler-Najjar syndrome

how is type II different than type I
disease = absence of UDP glucoronyl transferase

type II is less severe responds to phenobarbitol, which increases liver enzyme synthesis
Hereditary hyperbilirubinemias

patient has conjugated bilirubinema

grossly black liver

dx?
course?
Dubin-Johnson = impaired excretion of conjugated bilirubin

benign
How does Dubin-Johnson compare to Rotor's syndrome
Both are caused by defect in excretion of conjugated bilirubin

Rotor's is less severe, does not cause black liver
Patient has
-Asterexis
-Basal ganglia degeneration (parkinsonism)
-Ceruloplasmin low, Cirrhosis, Corneal deposits (brown ring around iris)
-Dementia, Dyskinesia, Dysarthria
-hemolytic anemia

dx?
path?
genetics?
treat?
wilson's (symptoms = ABCD)
(Kayser fleisher rings)

inadequate hepatic copper excretion (doesn't enter circulation as ceruloplasmin) --> copper accumulation in liver, brain, cornea, kidneys, joints

autosomal recessive

penicllamine
Patient sets off metal detector at airport. He goes to security, where they see he has

-Cirrhosis
-Diabetes
-Bronze skin color

dx? path
hemochromatosis (symptoms = Hemachromatosis Can Delay Boarding)

Deposition of hemosiderin (iron) in liver, other tissues

50g to set off metal detectors
On histology with prussian blue stain, you see
-hemosiderin in liver cells

dx?
increases risk of what 2 conditions?
Hemochromatosis

CHF, hepatocellular carcinoma
Hemochromatosis

what should the levels be of
a. ferritin
b. iron
c. TIBC
d. transferrin saturation
a. high
b. high
c. low
d. high
2 causes of hemochromatosis
primary (autosomal recessive) (assoc. with HLA A3)

secondary to chronic transfusion
Hemochromatosis

2 treatments
1. phlebotomy

2. deferoxamine (chelates Fe)
Patient presents with
-pruritis, jaundice, dark urine, light stool, hepatosplenomegaly

labs: high conjugated bilirubin, high cholesterol, high alk phos

dx? 3 types
biliary tract disease

secondary biliary cirrhosis (obstruction), primary (autoimmune), primary sclerosing cholangitis
Pathophys of secondary biliary cirrhosis

How can it be complicated
gallstone, biliary stricture, chronic pancreatitis, pancreatic head cancer --> obstruct biliary duct --> increases pressure in intraheptatic ducts --> injury fibrosis and bile stasis

complicated by ascending cholangitis
patient presents with biliary tract disease + serum mitochondrial antibodies (including IgM)

-patient also has CREST, RA, Celiac (another autoimmune disease)

dx?

path
primary biliary cirrhosis

autoimmune --> lymphocytic infiltrate + granulomas
Patient has biliary tract disease symptoms +
-hyper IgM
-also has Ulcerative colitis

ERCP = you see beading of intra and extrahepatic bile ducts

dx? path?
primary sclerosing cholangitis

unknown cause --> onion skin fibrosis of bile duct --> alternating strictures and dilation --> beading of intra and extrahepatic bile ducts
3 causes of gallstones
increased cholesterol/bilirubin, decrease in bile salts, and gallbladder stasis
2 types of gallstones

how do they look on radiography?
1. cholesterol stones (mostly radiolucent) = 80% of stones

2. pigment stones (radioopaque)
Obesity
Crohn's
CF
advanced age
clofibrate
estrogens
multiparity
rapid wt. loss
Native American origin

associated with what
cholesterol gallstones
chronic hemolysis
alcoholic cirrhosis
advanced age
biliary infection

associated with what
pigment stone
patient has jaundice, fever, RUQ pain

-Murphy's positive

dx?

4 risk factors
cholangitis

Female
Fat
Fertile
Forty
cholecystitis
ascending cholangitis
acute pancreatitis
bile stasis
biliary colic (bile duct contraction)

caused by what?
cholelithiasis
Gallstones

what happens if a fistula grows between gall bladder and small intestine
air in biliary tree
if you see air in biliary tree on imaging, where is the gallstone
obstructing ileocecal valve (gallstone ileus)
How do you diagnose gallstones?

How do you treat?
ultrasound

cholecystectomy
Cholecystitis

common causes

what do you see if bile duct becomes involved
gallstones, rarely ischemia or infection (CMV)

see increased alk phos
Gallstones
Ethanol
Trauma
Steroids
Mumps
Autoimmune disease
Scorpion sting
Hypercalcemia/Hyperlipidemia
ERCP
Drugs (sulfas)

all of these cause what?
Acute pancreatitis = autodigestion of pancreas by pancreatic enzymes

GET SMASHED
Patient has epigastric abdominal pain that radiates to back, anorexia, nausea

labs: elevated amylase, lipase

dx?
acute pancreatitis
DIC
Acute Respiratory Distress Syndrome

diffuse fat necrosis
hypocalcemia
pseudocyst formation
hemorrhage
infection
multiorgan failure

possible results of what condition?
acute pancreatitis

(Ca collects in pancreatic calcium soap deposits --> hypocalcemia)
patient has steatorrhea, fat-soluble vitamin deficiency, diabetes mellitus

dx?
chronic pancreatitis --> pancreatic insufficiency
Choronic calcifying pancreatitis

strongly associated with what 2 factors?

what does it cause?
alcoholism, smoking

pancreatic cancer
Patient presents with
1. abdominal pain radiating to back
2. wt loss (malabsorption and anorexia)
3. migratory thrombophlebitis = red, tender palpation of extremities
4. obstructive jaundice with palpable gallbladder

Labs: CEA and CA-19-9

Dx? prognosis?

Associated factors?
pancreatic adenocarcinoma (usually in pancreatic head)

very aggressive, 6 months survival

associated with cigarettes and chronic pancreatitis (not alcohol)
Cimetidine, ranitidine, famotidine, nizatidine

class?

MOA?
H2 blockers (DINE for 2)

reversible block of H2 receptors on parietal cell --> decreases H secretion
Treatment for peptic ulcer, gastritis, mild esophageal reflux
H2 blockers: cimetidine, ranitidine, famotidine, nizatidine
1. inhibits P450
2. antiandrogenic (PRL release, gynecomastia, imotence, low libido in males)
3. cross BBB (confusion, dizzy headache)
4. cross placenta
5. decreases renal excretion of creatine

side effects of what GI drug
Cimetidine (H2 blocker)
Negative effect common to cimetidine and ranitidine
decreases renal excretion of creatinine
Omeprazole, lansoprazole

class?

MOA
PPI

irreversible inhibit H/K ATPase in stomach parietal cells
Treatment for Peptic ulcer, gastritis, esophageal reflux, ZE syndrome
PPI - omeprazole, lansoprazole
Drug used to increase ulcer healing, traveler's diarrhea

MOA
Bismuth, scuralfate

Binds to ulcer base --> physical protection, allows HCO3 secretion to reestablish pH gradient in mucous layer
5 drugs to treat peptic ulcer from h.pylori
PPI
Metronidazole
Amoxicillin
Tetracycline
Bismuth

Please MAke my Tummy Better
Drug used to prevent NSAID-induced peptic ulcers, maintain patent ductus arteriosus, used to induce labor

MOA

Tox
Misoprostol

PGE1 analog --> increases production and secretion of gastric mucous barrier, decreases acid production

Diarrhea, abortifacent (don't give to women of childbearing age)
Pirezepine, Propantheline

MOA

Tox
Antimuscarinics = block M1 receptors on ECL cells (low histamine secretion) and M3 receptors on parietal cells (low H secretion)

Tachycardia, dry mouth, difficulty focusing eyes
Drug for acute variceal bleeds, acromegaly, VIPoma, carcinoid tumors

MOA

tox
Octreotide

Somatostatin analog

nausea, cramps, steatorrhea
antacids

how can they affect absorption, bioavailability or urinary excretion of other drugs
alters gastric and urinary pH, delay gastric emptying
Antacid use - what's the drug?

overuse -->
-constipation
-hypophosphatemia
-proximal muscle weakness
-osteodystrophy
-seizures
aluminium hydroxide

aluMINIMUM amount of feces
Antacid use - what's the drug?

overuse --> diarrhea, hyporeflexia, hypotension, cardiac arrest
Magnesium hydroxide

Mg = Must Go to the bathroom
Antacid use - what's the drug?

overuse --> hypercalcemia, rebound acid increase
Calcium carbonate
Common side effect to all antacids
hypokalemia
Drug to treat Crohn's disease, RA

MOA

tox
Infilximab

monoclonal Ab to TNF (proinflammatory cytokine)


Respiratory infection (reactivation of TB), fever, hypotension
Drug used for UC, Crohn's

MOA

Tox
Sulfasalazine

combination of sulfapyridine (antibact) and 5-aminosalicylic acid (anti-inflamm) --> activated by colonic bact

malaise, nausea, sulfonamide tox, reversible oligospermia
Drug to control vomiting post-op and in patients undergoing cancer chemo

MOA
Tox
5-HT3 antagonist --> central acting emetic

Headache, constipation
Drug to treat diabetic and post-surgery gastroparesis

MOA

tox
metoclopramide

D2 receptor, 5-HT3 antagonist --> increases resting tone, contractility, LES tone, motility
(Not colon transport time)


-Parkinson effects
-Restlessness, drowsiness, fatigue, depression, nausea, diarrhea
-Drug int. with digoxin, diabetic agents
-don't use if small bowel obstruction