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214 Cards in this Set
- Front
- Back
What is the most common location of salivary gland tumors?
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partoid gland - they are usually benign
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types of tumors in the salivary glands
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pleomorphic adenoma - mots common tumor; painless, movable mass; benign with high rate of recurrence
Warthin's tumor - benign; heterotopic salivary gland tissue trapped in a lymph node, surrounded by lymphatic tissue mucoepidermoid carcinoma - most common maligant tumor |
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what is the most common malignant tumor in the salivary gland?
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mucoepidermoid carcinoma
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what is the most common tumor in the salivary glands?
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pleomorphic adenoma - painless, movable mass; benign with high rate of recurrance
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warthin's tumor
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benign tumor of the salivary glands; heterotopic salivary gland tissue trapped in lymph node, surrounded by lymphatic tissue
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Failure to relax the lower esophageal sphincter results in what?
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Achlasia - bird beak sign on swallowing study
loss of myenteric (auerbach's plexus) - in muscularis externa layer * high LES opening pressure and uncoordinated peristalsis |
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Achalasia is associated with what?
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an increased risk of esophageal carcinoma
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What other things can cause achlasia besides failure of LES to relax?
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Chagas disease (infection with trypanosoma cruzi in south america) - can cause secondary achalasia
- CREST syndrome (scleroderma) is associated with esophageal dismotility involving low pressure proximal to LES |
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heartburn and regurgitation upon lying down what is it? What else can it present with?
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gastroesophageal reflux disease - can also present with a nocturnal cough and dyspnea - risk of developing barrett's esophagus and adenocarcinoma
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To check
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Revisar
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Mallory Weiss syndrome
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tear in the esophagus - from retching - painful mucosal lacerations at the GE junction - leads to hematemesis - usually found in alcoholics and bulimics
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what disease is commonly found in alcoholics and bulimics?
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Mallory Weiss syndrome
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Boerhaave syndrome
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transmural esophageal rupture due to violet retching
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Esophageal strictures
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associated with lye ingestion and acid reflux
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What is associated with lye ingestion and acid reflux?
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esophageal stricture
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What is associated with esophagitis?
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reflux, infection (HSV-1, CMV, Canidida), or chemical ingestion
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Plummer-Vinson syndrome
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1. glossitis
2. dysphasia (Esophageal webs) 3. Iron deficiency anemia |
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What changes in barrett's esophagus?
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glandular metplasia - replacement of nonkeratinized (stratified) squamous epitheium with intestinal (columnar) epithelium in the distal esophagus
* caused by chronic GERD - increased risk of adenocarcinoma |
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Progressive dysphagia (solids - liquids) - weight loss
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esophageal cancer
worldwide - squamous cell carcinoma is most common in the US adenocarcinoma and squamous cell carcinoma occur at equal frequencies |
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Where does squamous cell carcinoma and adenocarcinoma affect in the esophagus?
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upper and middle 1/3 - squamous cell carcinoma of esophagus
lower 1/3 adenocarcinoma of the esophagus (barrett's) |
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Most common type of esophageal cancer world wide?
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squamous cell carcinoma - affecting upper and middle 1/3 of esophagus
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most common type of esophageal cancer in US?
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adenocarcinoma and squamous cell carcinoma occur at equal frequencies in the US
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risk factors for esophageal carcinoma?
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ABCDEF
Alcohol/achlasia Barrett's esophagus Cigarettes Diverticuli (zenker's) Esophageal web/Esophagitis Familial |
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what are symptoms of malabsorption syndromes?
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diarrhea, steatorrhea, weight loss, weakness
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Celiac sprue
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autoantibodies to gluten (gliadin) in wheat and other grains. Proximal small bowel primarily - microscopically - blunting of small intestine villi
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Tropical sprue
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probably infectious - responds to antibiotics. Similar to celiac sprue but can affect entire small bowel
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What disease is common to celiac sprue - but can affect the entire small bowel?
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Tropical sprue - probably infectious cause
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PAS- positive macrophages in laminia propria, mesenteric nodes - what disease?
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Whipple's disease - caused by tropheryma whippelii (gram +) - symptoms include: arthralgias, cardiac and neurologic symptoms
*most common in older men |
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what is the most common disaccharidase deficiency?
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lactase deficiency (brush border enzyme) - milk intolerance. Normal appearing villi. osmotic diarrhea
*since lactase is at the tips of intestinal villi - self-limited lactase deficiency can occur following injury (viral diarrhea) |
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What causes pancreatic insufficiency?
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cystic fibrosis, obstructing cancer, and chronic pancreatitis
causes malabsorption of fat and fat-soluble vitamins (A,D,E,K) |
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Abeta-lipoproteinemia
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hereditary inability to synthesize lipoprotein B (100 and 48) - inability to generate chylomicrons - decreased secretion of cholestrol, VLDL into bloodstream - fat accumulation in enterocytes
presents in early childhood with malabsorption (steatorrhea), night blindness, failure to thrive, ataxia, acanthosis |
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antibodies to gliadin and tissue transglutaminase
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Celiac sprue - seen in people of northern european descent
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What do you see microscopically in celiac sprue?
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blunting of the villi, lymphocytes in the lamina propria
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what are serum levels of tissue transglutaminase antibody used to screen for?
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celiac sprue
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What is celiac sprue also associated with?
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T cell lymphoma and dermatitis herpetiformis
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What causes acute gastritis?
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disruption of the mucosal barrier - inflammation (ulcers can form)
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What are some causes of acute gastritis?
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NSAID use - in people with rheumatoid arthritis (inhibit prostaglandins - which decrease gastric mucosa production), alcohol, uremia, burns (decrease plasma volume - sloughing of gastric mucosa), brain injury (vagal stimulation - increased Ach - increased H+ production from parietal cell)
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curling's ulcer
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stomach ulcer caused by burns - decrease plasma volume - increased sloughing of gastric mucosa
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Cushing's ulcer
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stomach ulcer caused by brain trauma - vagal stimulation - increased ACh - increased H+ production by parietal cells (from ACh stimulation)
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Chronic gastrits (nonerrosive)
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2 types - chronic mucosal inflammation and atropy of mucosal glands
A - (fundus/body) autoimmune B - (nonerosive) caused by H. pylori |
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type A chronic gastritis
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affects the fundus/body of the stomach - autoimmune disorder characterized by autoantibodies to parietal cells, pernicous anemia, achlorhydria - associated with other autoimmune disorders
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type B chronic gastritis
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affects the antrum of the stomach - most common type - caused by H. pylori infection. increased risk of MALT lymphoma
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Menetrier's disease
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hypertrophy of gastric rugae (looks like gyri of the brain). increased mucous cells, and atropy of parietal cells - loss of protein - increased risk of stomach cancer
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What type of cancer is stomach cancer usually?
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adenocarcinoma
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What puts someone at an increased risk of stomach cancer? Is it a good or bad cancer to have?
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Person at increased risk if they have: chronic gastritis, H. pylori infection, achlorhydria, type A blood, dietary nitrosamines (smoked food)
bad cancer - early aggressive local spread and lymph node/liver mets |
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What are 2 common features of stomach adenocarcinoma?
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acanthosis nigricans and Signet ring cells (large vacuole full of mucin displaces the nucleus of the cell to the periphery
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Linitis plastica
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term used for the stomach when it is diffusely infiltrative with adenocarcinoma (thickened, rigid appearance, "leather bottle"
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Virchow's node
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involvement of left supraclavicular node by mets from stomach cancer - on the left side b/c that is near the spot where the thoracic duct dumps into the subclavian vein
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Krukenber's tumor
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seen in stomach cancer - get bilateral mets to the ovaries (if linus plastica can see this - can also see it with breast cancer) - abundant mucus, signet ring cells
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sister mary joseph nodule
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subcutaneous periumbilical metastasis of abdominal cancer - can see if with stomach adenocarcinoma
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epigastric pain increases with meals, weight loss - what does the patient have?
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gastric ulcer
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What population does gastric ulcers usually occur in? What increases the risk of them?
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often in older patients
H. pylori infection in 70% of cases, chronic NSAID use is common in patients with them due to decreased mucosal protection against gastric acid |
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What causes gastric ulcers?
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decreased mucosal protection against gastric acid
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pain that decreases with meals - weight gain what does the patient have?
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duodenal ulcer
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What causes duodenal ulcers?
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increased gastric acid production (zollinger-ellison syndrome) or decreased mucosal protection
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What happens to brunner's glands with duodenal ulcers?
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hypertrophy - causes release of alkaline mucus to neutralize the contents coming from the stomach
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What do duodenal ulcers look like? What are potential complications?
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tend to have clean, "punched out" margins unlike the raised/irregular margins of carcinoma.
complications: bleeding, penetration into pancreas, perforation, and obstruction (not intrinsically precancerous). |
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ulcer that almost 100% of people have H.pylori infections?
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duodneal ulcer - decrease pain with meals - increased weight
- from increased gastric acid production (zollinger-ellison) or decreased mucosal protection |
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Crohn's vs. UC on possible etiology
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crohn's: disordered response to intestinal bacteria
UC: autoimmune |
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crohn's vs UC on location?
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crohns: anywhere from mouth to anus - rectal sparing though - frequently affects terminal ileum and colon - skip lesions
UC: only affects colon, always affects rectum, continuous lesions |
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crohn's vs UC for gross morphology
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crohn's: transmural inflammation, cobblestone mucosa, creeping fat, bowel wall thickening (string sign of barium study), linear ulcers, fissures, fistulas
UC: mucosal and submucosal inflammation only - friable mucosal pseudopolyps with freely hanging mesentery. loss of haustra - lead pipe appearance on imaging |
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crohn's vs UC for microscopic morphology complications
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crohn's: noncaseating granulomas and lymphoid aggregates
UC: crypt abscesses and ulcers, bleeding, no granulomas |
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crohns vs. UC for complications
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crohns: strictures, fistulas, perianal disease, malabsorption, nutritional depletion
UC: malnutrition, toxic megacolon, colorectal carcinoma |
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crohns vs. UC for intestinal manifestation
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crohns: diarrhea that may or may not be bloody
UC: bloody diarrhea |
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crohns vs. UC for extraintestinal manifestations
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crohns: migratory polyarthritis, erythema nodosum, ankylosing spondylitis, uveitis, immunologic disorders
UC: pyoderma gangrenosum, primary sclerosing colangitis |
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crohns vs. UC for treatment
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crohns: corticosteroids, infliximab
UC: ASA preparations (sulfasalazine), infliximab, colectomy |
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drug that is used to treat both UC and crohn's?
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infliximab
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sulfasalazine
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drug used for UC
ASA preparations |
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irritable bowel syndrome (IBS)
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recurrent abdominal pain associated with >2 of the following:
1. pain improves with defecation 2. change in stool frequency 3. change in appearance of stool No structural abnormalities. May be present with diarrhea, constipation or alternating. *pathophysiology is multifaceted treat symptoms |
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How do you treat IBS?
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treat the symptoms
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appendicitis
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all age groups are affected; most common indication for emergent abdominal surgery in children.
initial diffuse periumbilical pain - localized to McBurney's point (1/3 of distance from iliac crest to umbilicus) nausea, fever, may perforate - peritonitis |
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what is the most common indication for emergent abdominal surgery in children?
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appenditicis
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what is the differential for appendicitis?
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diverticulitis (elderly), ectopic pregnancy (use B-hCG to rule out)
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How do you treat appendicitis?
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surgical resection - if you leave it alone it might rupture and cause peritonitis
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blind pouch protruding from the alimentary tract that communicates with the lumen of the gut what is it?
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diverticulum
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where do most diverticulum occur?
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in the sigmoid colon
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difference between true and false diverticulum?
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true - all 3 gut wall layers outpouch
false - only mucosa and submucosa outpouch. Occur especially where vasa recta perforate muscularis externa *most common diverticulum are FALSE and acquired |
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diverticulosis
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many diverticula. common in 50% of people >60 - caused by increased intraluminal pressure and focal weakness in colonic wall. associated with low fiber diets. most common in sigmoid colon
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symptoms of diverticulosis?
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often asymptomatic or associated with vague discomfort and/or painless rectal bleeding
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diverticulitis
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inflammation of diverticula classically causing LLQ pain, fever, leukocytosis, may perforate - peritonitis, abscess formation, or bowel stenosis.
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how do you treat diverticulitis?
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antibiotics
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symptoms of diverticulitits?
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LLQ pain, fever, pain, leukocytosis
bright right rectal bleeding - may cause colovesical fistula (fistula with bladder) - pneumaturia (air in the urine) |
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left sided appendicitis?
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diverticulitis
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zenker's diverticulum
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false diverticulum. herniation of mucosal tissue at junction of pharynx and esophagus. presenting symptoms: halitosis (due to trapped food particles), dysphasia, and obstruction
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hallitosis, dysphasia, obstruction what disease?
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zenker's diverticulum - false diverticulum - herniation of mucosal tissue at junction of pharynx and esophagus
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Meckel's diverticulum
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persistance of the vitelline duct or yolk stalk. May contain ectopic acid - secreting gastric mucosa or pancreatic tissue
*most common congenital anomaly of GI tract! can cause bleeding, intussusception, volvulus, or obstruction near the terminal ileum. c |
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most common congenital anomaly of GI tract? What is it?
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persistence of vitelline duct or yolk stalk - meckel's diverticulum
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way to remember meckel's diverticulum?
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2's
2 inches long 2 feet from ileocecal valve 2% population presents in first 2 years of life may have 2 types of epithelium - gastric and pancreatic |
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Omphalomesenteric cyst
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cystic dilatation of vitelline duct - different than meckel's diverticulum
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complications of meckel's diverticulum?
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intussusception, volvulus, or obstruction near terminal ileum
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telescoping of 1 bowel segment into distal segment what is it?
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intussusception - can compromise blood supply
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intussusception is most common in who?
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young children - usually idiopathic - may be viral (adenovirus) - abdominal emergency in children
if it occurs in adults (which is unusual) it is usually because of intraluminal mass or tumor |
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Volvulus
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twisting of the bowels around mesentary - can lead to obstruction and infarction - usually occur in the elderly
can occur at cecum and sigmoid colon where there is lots of redundant bowel |
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where are common regions where volvulus can occur?
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at cecum and sigmoid colon - where there is lots of redundant bowel
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disease where failure of neural crest cell migration - increased risk in patients with down syndrome
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hirschsprung's disease - no ganglionic cells (auerbach's and meissner's plexuses) in segment on intestinal biopsy
causes congenital megacolon |
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how does hirschsprung's disease present?
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chronic constipation early in life - dilitated portion of colon proximal to aganglionic segment - involves rectum
*usually failure to pass meconium *increased risk in patients with down syndrome! |
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billious vomiting with double bubble sign on x-ray
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duodenal atresia - failure of recanalization of small bowel - associated with down syndrome
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2 GI conditions associated with down syndrome?
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Hirschsprung's disease and duodenal atresia
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meconium ileus is associated with what condition?
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cystic fibrosis - meconium plug obstructs the intestine - preventing stool passage at birth
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necrotizing entercolitis
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necrosis of intestinal mucosa and possible perforation. colon is usually involved, but can involve entire GI tract. In neonates, more common in preemies (decreased immunity)
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ischemic colitis
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reduction in intestinal blood flow causes ischemia - pain after eating - weight loss
commonly occurs at splenic flexure and distal colon. usually affects elderly |
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where does ischemic colitis usually affect?
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splenic flexure and distal colon in elderly people
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adhesion
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acute bowel obstruction, commonly from a recent surgery - can have well demarcated necrotic zones
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angiodysplasia
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tortuous dilitation of vessles - bleeding. most often found in cecum, terminal ileum and ascending colon - more common in older patients, confirmed by angiography
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colonic polyps
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masses protruding into gut lumen- sawtooth appearnce - 90% are benign - often retrosigmoid
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What polyps are precancerous?
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adenomatous - malignant risk increases with increased size, villous histology, and increased epithelial dysplasia
*the more villous the polyp the more likely it is to be malignant (VILLous = VILLainOUS) |
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hyperplastic polyp
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most common nonneoplastic polyp in colon (>50% found in retrosigmoid colon)
benign! |
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juvenile polyp
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mostly sporatic in children <5 years of age - 80% in rectum - if single - no malignant potential
Juvenile polyposis syndrome - multiple juvenile polyps in the GI tract - increased risk of adenocarcinoma |
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juvenile polyposis syndrome
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multiple polyps in GI tract - increased risk of adenocarcinoma
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Peutz Jeghers
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single polyps are not malignant
AD syndrome featuring multiple nonmalignant hamartomas throughout GI tract, along with hyperpigmented mouth, lips, hands, genitalia associated with increased risk of CRC, stomach, breast, ovary |
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What is the epidemiology for colorectal cancer?
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3 most common cancer in men and women, 3rd most deadly cancer, usually occurs in people >50 years of age, about 25% of cases have a family history
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Familial adenomatous polyposis (FAP)
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AD mutation of APC gene on chromosome 5q. Two hit hypothesis. 100% progress to CRC - must remove entire colon. thousands of polyps; pancolonic, always involves the rectum
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APC gene
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mutated in FAP - on chromosome 5q
AD inheritence |
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Hereditary nonpolyposis colorectal cancer (HNPCC/Lynch syndrome)
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AD mutation of mismatch repair gene - 80% turn into colorectal cancer - always involves proximal colon
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mismatch repair gene
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mutated in HNPCC/lynch syndrome
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lynch syndrome
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HNPCC - AD inheritance - mutated mismatch repair gene - 80% develop CRC, proximal colon is always involved
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Gardner's syndrome
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FAP + osseous and soft tissue tumors, retinal hyperplasia
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Turcot's syndrome
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FAP + malignant CNS tumor (TURcot = TURban)
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What are additional risk factors for CRC?
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IBD, strep. bovis bacteremia, tobacco use, large villous adenomas, juvenile polyposis syndrome, puetz-jeghers syndrome
|
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How does CRC present
|
proximal colo - usually no obstruction, can present with dull pain, iron deficiency anemia, fatigue
distal colon - obstruction, colicky pain, hematochezia |
|
iron deficiency anemia in older man or women post menopausal
|
iron deficiency anemia until proven otherwise
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Who do you screen for CRC?
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patients >50 with stool occult blood test and colonscopy
|
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apple core lesion on barium enema x-ray
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CRC
|
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CEA tumor marker
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for CRC
|
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what is the tumor marker for CRC?
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CEA
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What are the 2 pathways that can lead to CRC?
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1. microsatellite instability (15%): DNA mismatch repair gene mutations - sporatic and HNPCC syndrome. mutations accumulate but no defined morphologic correlates
2) APC/B-chain (chromosomal instability) pathway (85%) |
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loss of APC gene results in what?
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colon that is at risk for CRC
decreased intracellular adhesion and increased proliferation |
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K-RAS mutation results in what?
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formation of adenomas in a colon that is at risk for CRC
- unregulated intracellular signal transduction |
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loss of p53
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changes an adenoma into carcinoma
- increased tumorgenesis |
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Carcinoid tumor
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tumor of endocrine cells - 50% of small bowel tumors - most common site is in small intestine - "dense core bodies" seen on EM
often produce 5-HT (which is metbolized by the liver - so no carcinoid syndrome) if the tumor metastasizes to the liver - can no longer metabolize 5-HT - get carcinoid syndrome - wheezing, right sided heart murmers, diarrhea, flushing |
|
dense core bodies seen on EM
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carcinoid tumor of small bowel
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wheezing, right sided heart murmurs, diarrhea, flushing
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carcinoid syndrome - carcinoid tumor has metastasized to the liver (the 5-HT is not longer metabolized by the liver)
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Effects of portal hypertension
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esophageal varices - hematemesis, melena
splenomegaly - back up into spleen caput mudusae hemorrhoids ascites (increased portal pressure, decreased albumin, retention of Na+ and water) |
|
Effects of liver cell failure
|
coma, scleral icterus, fetor hepaticus (breath smells like freshly open corpse), spider nevi (increased estrogen), gynecomastia (increased estrogen - liver can't break it down), jaundice, testicular atrophy, bleeding tendency (decreased prothrombin and clotting factors), anemia, ankle edema
|
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cirrhosis
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diffuse fibrosis of liver, destroys normal artitecture, nodular regeneration
|
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micronodular cirrhosis
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nodules <3mm, uniform size. due to metabolic insult (alcohol, hemachromatosis, wilson's disease)
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macronodular cirrhosis
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nodules>3mm, vaired size. usually due to significant liver injury leading to hepatic necrosis (postinfectious, or drug induced hepatitis). increased risk of hepatocellular carcinoma
|
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What relieves portal hypertension?
|
shunt between portal and systemic circulation
|
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What is aminotransferases used to diagnose?
|
AST, ALT
viral hepatitis ALT>AST alcoholic hepatitis AST>ALT MI increased AST |
|
only an increase in AST is seen in what?
|
MI
|
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What is GGT (y-glutamyl transpeptidase) used to dignose?
|
various liver diseases; increased with heavy alcohol consumption
|
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what is alkaline phosphatase used to diagnose?
|
obstructive liver disease (hepatocellular carcinoma), bone disease, bile duct disease
|
|
what is amylase used to diagnose?
|
acute pancreatitis and mumps
|
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What is lipase used to diagnose?
|
acute pancreatitis
|
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decreased ceruloplasmin is seen in what?
|
Wilson's disease
|
|
Reye's syndrome
|
caused by giving aspirin to children with viral infections
rare, often fatal childhood hepatoencephalopathy findings: mitochondrial abrnomalities, fatty liver (microvesicular fatty change), hypoglycemia, coma associated with: viral infections (VZV, and influenza B) that has been treated with salicylates mech: aspirin metabolites decrease B-oxidation by reversible inhibition of mitochondrial enzymes DON'T GIVE ASPIRIN TO CHILDREN |
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findings in someone with reye's syndrome?
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mitochondrial abnormalities, fatty liver (microvesicular fatty change), hypoglycemia, coma
|
|
mech of reye's syndrome
|
aspirin metabolites decrease b-oxidation by reversible inhibition of mitochondrial enzymes
|
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what drug should you not give to children
|
Aspirin - can cause reye's syndrome - use acetaminophen with caution
|
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hepatic steatosis
|
seen in short-term change with moderate alcohol intake. macrovesicular fatty change that may be reversible with alcohol cessation
|
|
Alcoholic hepatitis
|
requires sustained, long-term consumption. swollen necrotic hepatocytes with neutrophilic infiltration
Mallory bodies are present (intracytoplasmic eosinophilic inclusions) (AST>ALT) |
|
mallory bodies
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intracytoplasmic eosinophilic inclusions
seen in alcholoic hepatitis in the liver |
|
alcoholic cirrhosis
|
final and irreversible damage - microdnodular irregular shrunken liver with hobnail appearnce. scelerosis around central vein (zone III). has manifestations of chronic liver disease (juandice albuminemia)
|
|
Hepatocellular carcinoma - risks factors for developing it
|
most common primary malignancy in the liver in adults
increased incidence with: hep B and C, wilson's disease, hemochromatosis, a1-antitrypsin deficiency, alcoholic cirrhosis, carcniogens (aflatoxin in peanuts) |
|
aflatoxin in peanuts can cause what?
|
hepatocellular carcinoma - it is a carcinogen
|
|
How does hepatocellular carcinoma commonly spread?
|
hematogenously
|
|
findings in hepatocellular carcinoma?
|
jaundice, tender hepatomegaly, ascites, polycythemia, hypoglycemia
|
|
what finding will you see in hepatocellular carcinoma?
|
increased alpha fetoprotein
|
|
what can hepatocellular carcionma lead to
|
budd-chiari syndrome
|
|
Nutmeg liver
|
due to back up of blood in the liver - most commonly from right sided heart failure and budd-chiari syndrome
liver appears mottled like a nutmeg if it persists centrilobar congestion and necrosis can result in cardiac cirrhosis |
|
what commonly causes nutmeg liver?
|
right sided heart failure and budd chiari syndrome
|
|
Budd-Chiari sydnrome
|
occlusion of IVC or hepatic veins - get centrilobar congestion and necrosis - leading to congestive liver disease (hepatomegely, ascites, abdominal pain, jaundice, eventually liver failure). may develop varices and have visible abdominal and back veins. Absence of JVD. Associated with polycythemia vera, pregnancy, and hepatocellular carcinoma
|
|
what is budd chiari syndrome associated with?
|
pregnancy, hepatocellular carcinoma, polycythemia vera
|
|
a1 antitrypsin deficiency
|
misfolded gene product protein accumulates in hepatocellular ER - also decreased elastic tissue in the lung causes panacinar emphysema - PAS positive globules in the liver
Codominant trait *can cause cirrhosis and liver failure |
|
physiologic neonatal jaundice
|
at birth immature UDP-glucuronyl transferase - increased unconjugated bilirubin - causes juandice/krnicterus
tx. phototherapy (converts unconjugated bilirubin into conjugated (water soluble bilirubin)) |
|
treatment for physiologic neonatal juandice?
|
phototherapy (converts water insoulable (unconjugated bilirubin) into water soluble (conjugated bilirubin)
|
|
Hepatocellular jaundice
|
increased unconjugated and conjugated bilirubin - increased urine bilirubin, normal or decreased urine urobilinogen
|
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Obstructive jaundice
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increased conjugated bilirubin, increased urine bilirubin, decreased urine urobiloinogen
|
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hemolytic jaundice
|
increased unconjugated bilirubin, no urine bilirun, increased urine urobilinogen
|
|
gilbert's syndrome
|
mildly decreaed UDP-glucouronyl transferase or decreased bilirubin uptake. Asymptomaic. Elevated unconjugated bilirubin without overt hemolysis - associated with stress
|
|
crigler-Najjar syndrome
|
absent UGT - presents early in live; patients die within a few years
tx. phlasmapheresis and phototherapy type II - less severe - can be treated with phenobarbitol (which increases synthesis of UGT) |
|
what do you treat type II Crigler-Najjar syndrome with?
|
phenobarbitol increases synthesis of UGT
|
|
How do you try to extend life expetancy in type I Crigler-Najjar?
|
phototherapy and plasmapheresis
|
|
Dubin-Jonhson syndrome
|
conjugated hyperbilirubinemia due to defective liver excretion. Grossly black Liver. Benign
|
|
Rotor's syndrome
|
similar to Dubin Johnson - but no black liver
Dubin Johnson is due to defective liver excretion of bilirubin - it is benign |
|
Wilson's syndrome
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accumulation of copper in liver because of improper hepatic copper excretion and failure of copper to enter circulation as ceruloplasmin (decreased ceruloplasmin levels)
leads to copper accumulation especially in liver, brain, cornea (kayser-fleisher rings), kidneys, and joints |
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characteristics of wilson's syndrome
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ABCD
Asterxis Basal ganglia dengernation (parkinsonism symptoms) Ceruloplasmin dcreased, cirrhosis, corneal deposits, copper accumulation, cancer Dementia hemolytic anemia |
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how do you treat wilson's disease? what is the mode of inheritance?
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Autosomal recessive inheritance
treat with penicillamine |
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hemochromatosis vs. hemosiderosis
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hemosiderosis is deposition of hermosiderin
hemochromatisis is the disease cause by iron deposition |
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Cirrhosis, Diabetes mellitus, skin pigmentation
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bronze diabetes - hemachromatosis
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risk factors of someone with hemachromatosis
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CHF and increased risk of hepatocellular carcinoma
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What causes hemochromatosis
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primary disease - autosomal recessive
secondary disease - chronic transufions therapy (B thalassemia major) |
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iron studies that you will see with hemochromatosis
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increased ferritin, increased iron, increased trnasferrin (carrier of free iron), decreased TIBC
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Person sets off metal detector at airport. What condition?
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Hemachromatosis - total body iron can reach 50g
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how do you treat hermachromatosis?
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phlebotomy, deferoxamine
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What HLA type is associated with hemachromatosis?
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HLA-A3
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Primary sclerosing colangitis
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unknown cause of concentric onion skinning of bile duct fibrosis - alternating strictures of intrahepatic and extrahepatic bile ducts on ERCP
* presents with dark urine, light stools, puritis, jaundice, hepatosplenomegley labs: increased conjugated bilirubin, increased cholestrol, increased alkaline phosphatase |
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What is primary sclerosing colangitis associated with?
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ulcerative colitis, can also see hypergammaglobulinemia (IgM) with it
can lead to secondary biliary cirrhosis |
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primary biliary cirrhosis
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autoimmune mediated - lymphocyte infiltrate and granulomas. increased serum mitochondrial antibodies
presentation: puritis, jaundice, hepatosplenomegly, dark urine, light stools labs: increased conjugated bilirubin, increased cholestrol, increased alkaline phosphatase *associated with other autoimmune conditions (CREST, RA, Celiac's) |
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jaundice, hepatosplenomegly, puritis, dark urine, light stools sign of what?
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either primary or secondary biliary cirrhosis or primary scleorsing colangitis
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what antibodies are increased in primary biliary cirrhosis?
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mitochondrial antibodies
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secondary biliary cirrhosis
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extrahepatic biliary obstruction (gallstone, biliary stricture, chronic pancretitis, carcinoma in the head of the pancreas) - increased pressure in intrahepatic ducts - injury/fibrosis and bile stasis
presentation: jaundice, puritis, dark urine, light stools, hepatosplenomegly labs: increased alk phos, increased cholestrol, increased conjugated bilirubin complicated by ascending cholangitis |
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what is cholelithiasis?
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gallstones
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risk factors for developing cholelithiasis?
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female, fat, forty, fertile
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charcots triad of colangitis
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jaundice, fever, RUQ pain
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When do gallstones form?
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when solubilizing bile acids and lecithin are overwhelmed by increased cholestrol and/or bilirubin or gallbladder stasis
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insipratory arrents on deep palpation
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positive murphy's sign - sign of cholelithiasis (gallstones)
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2 types of gallstones
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cholestrol (radiolucent with 10-20% opaque due to calcifications) 80% of stones. associated with obestity, crohn's disease, cystic fibrosis, advanced age, clofibrate, estrogens, multiparity, rapid weight loss, Native american orgin
pigment stones: radioopaque - seen in pateints wtih chronic hemolysis, alcoholic cirrhosis, advanced age, and biliary infection |
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what is the most common type of gallstones?
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cholesterol (80%)
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what can gallstones cause? How do you diagnose it? How do you treat?
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can cause ascending colangitis, acute pancreatitis, bile stasis, cholecystitis, biliary colic - can present w/o pain in diabetics
can cause fisutal between gallbladder and small intestine, if gallstone obstructs ileocecal valve - can see air in biliary tree on imaging diagnose with ultrasound tx. cholecystectomy |
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air in biliary tree what is the problem?
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gallstone has obstructed the ileocecal valve (gallstone ileus)
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cholecystitis
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inflammation of the gallbaldder - usually from gallstones; rarely ischemia or infectious (CMV). increased alk phos if bile duct becomes involved (ascending colangitis)
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what bug can cause cholecystitis?
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CMV - but usually from gallstones!
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What can cause acute pancreatitis?
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GET SMASHED
gallstones, ethanol, trauma, steroids, mumps, autoimmune diseases, scorpion sting, hypercalcemia/hyperlipidemia, ERCP, drugs (sulfa) |
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epigastric pain that radiates to the back, anorexia, nausea
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acute pancretitis
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what labs do you see for acute pancreatitis?
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elevated amylase and lipase (higher specifically)
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what can acute pancreatitis lead to?
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DIC, ARDS, diffuse fat necrosis, hypocalcemia (Ca2+ collects in pancreatic calcium soap deposits), pseudocyst formation, hemorrhage, infection, and mulitorgan failure
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Chronic pancreatitis
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can lead to pancreatic insufficiency - steatorrhea, fat soluable vitamin deficiency, diabetes mellitus
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What is chronic calcifying pancreatitis associated with?
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alcoholism and increased risk for pancreatitc cancer
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Presenting signs of pancreatic adenocarcinoma?
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abdominal pain radiating to back, weight loss, migratory thrombophlebitis (redness and tenderness on palpation of extremities) - trousseau's syndrome
obstructive jaundice with palpable gallbaldder (courvoiser's sign) |
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pancreatic adenocarcinoma
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prognosis averages 6 months - very aggressive; usually already metastasized at presentation; tumors more common in pancreatic head (obstructive jaundice). increased risk in jewish and african american males.
CEA and CA-19-9 tumor markers associated with cigarettes but not alcohol |
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CEA and CA-19-9 are tumor markers for what?
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pancreatic adenocarcinoma
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cancer that is associated with smoking but not alcohol use
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pancreatic adenocarcinoma
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which population is at increased risk for pancreatic adenocarcinoma?
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jewish and african american males
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