Gastrointestinal Flashcards

1
Q
Drooling
Choking
Vomiting with first feed
Cyanosis (laryngospasm)
Air in stomach on XR
Failure to pass NG tube
A

ESOPHAGEAL ATRESIA WITH DISTAL TEF

Note - CXR shows gasless abdomen in pure EA

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2
Q

Mechanism of jejunal and ileal atresia - “apple peel” sign on XR

A

Disruption of mesenteric vessels leading to ischemic necrosis

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3
Q

Nonbilious projectile vomiting (2-6 wks)
Palpable olive
Visible epigastric peristaltic waves
Hypokalemic hypochloremic metabolic alkalosis

Associated with Macrolide use

A

HYPERTROPHIC PYLORIC STENOSIS

Treat with pyloromyotomy

Note - Bilious emesis occurs if occlusion is past 2nd part of duodenum

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4
Q

Two causes of early bilious vomiting and double bubble sign on XR

A
Duodenal atresia (recanalization defect)
Annular pancreas (ventral bud)
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5
Q

Embryologic origin of spleen

A

Mesentery (mesodermal)

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6
Q

Retroperitoneal structures

“SAD PUCKER”

A
Suprarenal glands
Aorta and IVC
Duodenum
Pancreas
Ureters
Colon (ascending and descending)
Kidneys
Esophagus
Rectum

Note - First part of duodenum and tail of pancreas are peritoneal

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7
Q

Contents of hepatoduodenal ligament

Note - Target of the Pringle maneuver

A

Portal triad:
Proper hepatic artery
Hepatic vein
Bile duct

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8
Q

Contents of gastrohepatic ligament

Note - Cut to access lesser sack

A

Gastric vessels

Note - Gastrohepatic and Hepatoduodenal ligaments form the lesser omentum

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9
Q

Contents of…
Gastrocolic
Gastrosplenic
Splenorenal

A

Gastroepiploic arteries
Short gastrics
Splenic artery and vein

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10
Q

Layers of gut wall (4)

A

Mucosa (epithelium, lamina propria, muscularis mucosa)
Submucosa (Meissner plexus, connective tissue)
Muscularis externa (Auerbach/Myenteric plexus)
Serosa (intraperitoneal)/Adventitia (extraperitoneal)

Note - Ulcers to submucosa, erosions to mucosa

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11
Q

Fastest and slowest basal electrical rhythms of GI tract

A
Fastest = Duodenum
Slowest = Stomach
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12
Q

Histology of esophagus

A

Nonkeratinized stratified squamous epithelium

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13
Q

Duodenal glands secreting HCO3-

A

Brunner glands

Note - May become hypertrophied with duodenal ulcers

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14
Q

GI glands enterocytes, goblet cells, Paneth cells, and stem cells

A

Crypts of Lieberkuhn

Note - Goblet cell number increases as you approach rectum

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15
Q

Location of Peyer’s patches (GALT)

A

Lamina propria of Ileum

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16
Q

Location of plicae circulares - protrusions of villi

A

Jejunum

Proximal ileum

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17
Q

Dieting/underweight patient with postprandial pain due to intermittent intestinal obstruction

A

SUPERIOR MESENTERIC SYNDROME

Transverse (3rd) portion of duodenum compressed between SMA/aorta

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18
Q

Blood supply and parasympathetic innervation to…
Foregut (T12/L1)
Midgut (L1)
Hindgut (L3)

A

Celiac/Vagus
SMA/Vagus
IMA/Pelvic

Note - Foregut includes lower esophagus, stomach, proximal duodenum, liver, gallbladder, pancreas, and spleen

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19
Q

Branches of common hepatic artery

A

Proper hepatic artery
Right gastric
Gastroduodenal (supraduodenal, superior pancreaticoduodenal, right gastroepiploic)

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20
Q

Portosystemic anastomoses at..
Esophagus
Umbilicus
Rectum

A

L gastric-Azygous
Paraumbilical-Epigastric
Superior rectal (IMA)-Middle/Inferior rectal (internal pudendal)

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21
Q

Lymphatic drainage above and below the pectinate line

A

Above…
Inferior mesenteric (superior rectal)
Internal iliac nodes (middle rectal)

Below
Inguinal nodes

Note - Superficial inguinal nodes drain most of the cutaneous lymph from the umbilicus down

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22
Q

Orientation of hepatocytes in liver

A

Apical surface faces bile canaliculi

Basolateral surface faces sinusoids (fenestrated endothelium) draining to central vein

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23
Q

Location and function of Kupffer cells and Stellate (Ito) cells

A

Kupffer cells = Sinusoidal, macrophages

Stellate (Ito) cells = Space of Disse between hepatocytes and sinusoids, store Vit A (quiescent) and produce ECM (activated)

Note - Bridging fibrosis in cirrhosis via Stellate cells

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24
Q

Zone of liver affected earliest by viral hepatitis and ingested toxins

A

ZONE I

Zone near portal triad - blood flows away and bile flows towards

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25
Q

Zone of liver affected earliest by…
Ischemia
Metabolic toxins
Alcoholic hepatitis

Note - Contains Cytochrome P-450 which is involved in damage by metabolic toxins (e.g. CCl4 oxidative damage)

A

ZONE III

Zone near central vein - farthest from portal triad

Note - Zone II affected by yellow fever

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26
Q

Causes (2) of double-duct sign

A

Choledocolithasis (cholangitis, pancreatitis)

Ductal adenocarcinoma in head of pancreas (painless jaundice)

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27
Q

Path of inguinal canal

A

Spermatic cord through transversalis fascia
Through deep ring (transversus abdominis)
Through internal oblique
Through superficial ring (external oblique aponeurosis)
Into scrotum

Note - Internal spermatic fascia from transversalis fascia, cremasteric muscle from internal oblique, and external spermatic fascia from external oblique aponeurosis

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28
Q

Mechanism of indirect inguinal hernia

Note - Lateral to inferior epigastrics

A

Failure of processus vaginalis to close (can form hydrocele)

Note - Path follows descent of testes and covered by all 3 layers of spermatic fascia

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29
Q

Mechanism of direct inguinal hernia

Note - Medial to inferior epigastrics

A
Protrudes through transversalis fascia of Hesselbach triangle...
Inferior epigastric (superior)
Inguinal ligament (inferior)
Rectus abdominus (medial)

Note - Path is through superficial ring and only contains external spermatic fascia from external oblique aponeurosis

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30
Q

Most common hernia to incarcerate (non-reducible) and strangulate (ischemia)

A

FEMORAL HERNIAS

More common in women - Occurs just lateral to pubic tubercle (medial to femoral vein)

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31
Q

Action and regulation of Parietal cells (under epithelium, pink oxyntic glands) - located in body

A

GASTRIC ACID

Increased - Histamine, Ach (vagal M3), Gastrin
Decreased - Somatostatin, Secretin, GIP, Prostaglandin

Also produces IF which binds Vit B12 for absorption in terminal ileum

Note - Autoimmune destruction of parietal cells results in chronic gastritis and pernicious anemia

Note - Atropine blocks vagal Ach (cephalic phase) stimulation of M3 receptor

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32
Q

Action and regulation of Chief cells (deep, basophilic oxyntic glands) - located in body

A

PEPSIN PROTEASE

Increased - Vagal stimulation

Note - HCl required for conversion of pepsinogen to pepsin

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33
Q

Action and regulation of G cells - located in antrum and duodenum

A

GASTRIN

Enters systemic circulation…
Increases Histamine/Parietal HCl secretion
Growth of gastric mucosa
Gastric motility

Increased - Distention, Alkalinization, GRP (vagal), Protein
Decreased - pH < 1.5

Note - Also increased by chronic PPI use, H. pylori (chronic atrophic gastritis), and Zollinger-Ellison gastrinoma

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34
Q

Action and regulation of Brunner cells (in submucosa of duodenum)

A

BICARBONATE

Increased - Secretin ( S cells in crypts of lieberkuhn, pancreas then prompted to make bicarb rich fluid )

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35
Q

Action and regulation of D cells - located in atrum, pancreatic islets

A

SOMATOSTATIN

Decreases all gastrointestinal hormones, pancreatic secretions, gastric emptying, and insulin/glucagon release

Increased - Gastric acid
Decreased - Vagal stimulation

Note - Octreotide is a synthetic somatostatin

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36
Q

Action and regulation of I cells - located in duodenum, jejunum

A

CHOLECYSTOKININ

Increases pancreatic enzyme and HCO3 secretion
Increases gallbladder contraction
Relaxes sphincter of oddi
Decreases gastric emptying

Increased - Fatty acids, Amino acid

Note - Acts on muscarinic pathways in pancreas

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37
Q

Action and regulation of S cells - located in duodenum

A

SECRETIN

Increases pancreatic HCO3 secretion
Increases bile secretion
Decreases gastric acid secretion

Increased - Fatty acids, Gastric acid in duodenum

Note - Required for functioning of pancreatic enzymes

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38
Q

Action and regulation of K cells - located in duodenum, jejunum

A

GLUCOSE-DEPENDENT INSULINOTROPIC PEPTIDE (GIP)

Decreases gastric acid secretion
Increases insulin release (increased beta sensitivity)

Increased - Fatty acids, Amino acids, Glucose (oral)

Note - With GLP-1 (incretins) responsible for increased insulin release with oral glucose compared to IV glucose

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39
Q

Action and regulation of Motilin - secreted in small intestine

A

Produces migrating motor complexes

Increased - Fasting state

Note - Acted on by Erythromycin

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40
Q

Action and regulation of vasoactive intestinal polypeptide (VIP)

Note - Secreted in parasympathetic ganglia in sphincters, gallbladder, and small intestine

A

Increased pancreatic HCO3 and Cl secretion
Increases water and electrolyte secretion
Relaxes intestinal smooth muscle and sphincters
Inhibits Gastrin action on parietal cells

Increased - Distension, Vagal stimulation
Decreased - Adrenergic stimulation

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41
Q

Watery secretory diarrhea
Hypokalemia
Achlorhydria

Note - Persists with fasting

A

VIPoma

Pancreatic islet tumor (non-a, non-b)

Treat with Octreotide

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42
Q

Action and regulation of Ghrelin - secreted in stomach

A

Increases appetite

Increased - Fasting
Decreased - Food

Note - Increased in Prader-Willi

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43
Q

Action of ECL cells - located in systemic circulation

A

Produce Histamine to increase parietal cell HCl secretion - Direct Gastrin action on parietal cells is minimal

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44
Q

Cl and HCO3 concentration in pancreatic secretions relative to flow rate

A

High flow = High HCO3

Low flow = High Cl

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45
Q

Mechanism of zymogen activation in pancreatic secretions

A

Enterokinase/enteropeptidase on brush-border cells (duodenum, jejunum) converts Trypsinogen to Trypsin

Trypsin activates other proteases (Chymotrypsin, Elastase, Carboxypeptidase) and also more Trypsin (positive feedback)

Note - SPINK1 and Trypsin also inactivate Trypsin to prevent inappropriate cascading

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46
Q

Intestinal channels responsible for glucose absorption

A

Apical:
Na-dependent SGLT1 - Glucose, Galactose
GLUT-5 - Fructose

Basolateral:
GLUT-2 - All carbohydrates

Note - Na-dependent SGLT1 use secondary active transport, while all other GLUTs are carried-mediated (facilitated diffusion)

Note - D-xylose absorption test distinguishes GI mucosal damage from pancreatic insufficiency

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47
Q

Location of absorption of…
Iron
Folate
B12

A
Duodenum
Small bowel
Terminal ileum (with bile)
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48
Q

Function of Peyer patches

A

Contain M cells which sample antigens and lead to B cell stimulation - leads to plasma cell IgA secretion with protective secretory component for transfer to epithelium

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49
Q

Rate limiting enzyme in bile synthesis

A

Cholesterol 7a-hydroxylase

Note - Secretion into bile is the only method available for cholesterol excretion

Notes - Fibrates inhibit this enzyme and thus decrease the cholesterol:bile acid ratio leading to gallstone formation

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50
Q

Mechanism of bilirubin excretion

A

Splenic heme oxygenase converts Heme to Biliverdin
Biliverdin reduced to indirect Bilirubin (water insoluble)
Secreted from Macrophages into Blood (binds albumin)
Hepatic sinusoids to space of Disse to hepatocytes
UDPGT conjugates with Glucuronic acid
To bile canaliculi as direct Bilirubin (water soluble)
Gut bacteria convert to Urobilinogen

Note - Majority is secreted as feces (brown), what is not secreted is mostly reabsorbed into the liver while a small minority is excreted into the kidney (yellow)

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51
Q

Neoplasm responsible for painless mass/swelling of the parotid

A

PLEOMORPHIC ADENOMA

Recurs if incompletely excised or ruptures

Note - Pain indicates CN VII involvement by malignant Mucoepidermoid carcinoma

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52
Q

Benign cystic tumor of parotid with germinal center

A

WARTHIN TUMOR

Papillary cystadenoma lymphomatosum

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53
Q

Progressive dysphagia to solids and liquids - may result in esophageal SCC

A

ACHALASIA

Loss of myenteric Auerbach plexus (inhibitory ganglion cells) decreases NO producing neurons at LES and causes incomplete relaxation, and uncoordinated or absent peristalsis

Note - May also be due to Chagas

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54
Q

Distal esophageal pathology resulting in air surrounding the aorta on CT

A

BOERHAAVE PNEUMOMEDIASTINUM

Hamman’s sign (crunching) on chest auscultation

Note - Mallory-Weiss tears at junction of GE (more distal)

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55
Q

Mechanism of eosinophilic esophagitis

A

Atopy leads to dysphagia and food impaction - results in esophageal rings and linear furrows

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56
Q

CMV vs HSV-1 esophagitis

A
CMV = Linear ulcers
HSV-1 = Punched-out ulcers
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57
Q

Causes of chronic cough

A

Postnasal drip
GERD
Asthma

Note - Odynophagia and failure of previously effective therapy in GERD indicates progression to erosive esophagitis/ulcer

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58
Q

Dysphagia
Iron deficiency anemia
Esophageal webs
May result in esophageal SCC

A

PLUMMER-VINSON SYNDROME

esophageal webs are mucosal folds that cause solid food dysphagia in mid to lower esophagus

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59
Q

Mechanism of scleroderma esophageal dysmotility

A

Esophageal smooth muscle atrophy leading to decreased LES pressure and dysmotility - results in acid reflux (strictures, Barrett’s) and dysphagia (aspiration)

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60
Q

Dysphagia to solids - may progress to liquids eventually

A

ESOPHAGEAL CARCINOMA

SCC (upper 2/3) - Alcohol, Smoking, Achalasia, Hot liquids, Caustic strictures

Adeno (lower 1/3) - Smoking, Achalasia, Chronic GERD, Barrett’s, Obesity

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61
Q

Causes (3) of acute gastritis

A

Decreased PGE2 (e.g. NSAIDs) removes inhibition of parietal cells

Burn induced hypovolemia results in mucosal ischemia (Curling’s ulcer)

Brain injury increases vagal stimulation, increasing Ach stimulation of parietal cells (Cushing ulcer)

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62
Q

Mechanism and causes (2) of chronic gastritis

A

Mucosal inflammation leads to atrophy
Hypochlorhydria and compensatory hypergastrinemia
Eventually intestinal metaplasia/gastric cancer

H. Pylori - Reduction in D cells with Antral predominant gastritis increasing duodenal ulceration

Pernicious anemia - Autoantibodies to parietal cells and IF affects Body/Fundus (Antral sparing)

Note - H. Pylori also causes gastric ulcers (decreased mucosal protection) and MALT lymphoma (increased immune response)

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63
Q

Hypertrophic rugae
Excess mucus
Protein loss
Parietal cell atrophy

A

MENETRIER DISEASE

Gastric hyperplasia of mucosa - precancerous

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64
Q

KIT+ tumor of interstitial cells of Cajal (pacemaker cells)

A

GIST

Mesenchymal neoplasm with low malignancy

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65
Q

Weight loss
Early satiety
Acanthosis nigricans
Leser-Trelat (explosive seborrheic keratosis)

A

GASTRIC CANCER

Most commonly adenocarcinoma - may also be lymphoma, GIST, carcinoid

Note - Metastasis to lymph nodes and liver

66
Q
Gastric cancer associated with...
H. pylori
Nitrosamines (smoked foods)
Smoking
Achlorhydria (chronic gastritis)
A

INTESTINAL

Lesser curvature ulcer with raised margins

67
Q

Histology of linitis plastica (diffuse) gastric cancer

A

SIGNET RINGS

Mucin-filled cells with peripheral nuclei

68
Q

Involvement of left supraclavicular node from metastasis

A

VIRCHOW NODE

Metastasis from gastric cancer

69
Q

Bilateral signet ring cells in ovaries

A

KRUKENBERG TUMOR

Metastasis from gastric cancer

70
Q

Subcutaneous periumbilical metastasis

A

SISTER MARY JOSEPH NODULE

Metastasis from gastric cancer

71
Q

Mechanism and causes of gastric/duodenal ulcers

A

Gastric - Decreased mucosal protection secondary to H. pylori or NSAIDs

Duodenal - Increased acid secretion or decreased mucosal protection secondary to H. pylori or Zollinger-Ellison syndrome

Note - Gastric ulcers should be biopsied to rule out malignancy, but duodenal ulcers are more often benign

72
Q

Most common location of ulcer hemorrhage and perforation

A
Hemorrhage = Posterior > Anterior
Perforation = Anterior > Posterior

Note - Perforation only likely with duodenal ulcers

73
Q

Stain for fecal fat

A

Sudan stain

74
Q

Small intestine biopsy showing…
Villous atrophy (blunting)
Crypt hyperplasia
Increased Intraepithelial lymphocytosis

A

CELIAC DISEASES

HLA-DQ2/DQ8

IgA anti-tissue transglutaminase, anti-endomysial, anti-deaminated gliadin peptide

Note - Avoid wheat, barley, rye

75
Q

Disease associated with dermatitis herpetiformis

A

CELIAC DISEASE

Anti-epithelial transglutaminase

76
Q

Cramping and flatulence
Osmotic diarrhea with acidic stool
Normal biopsy

A

LACTOSE INTOLERANCE

Confirm with hydrogen breath test > 20 ppm

Note - Secondary lactose deficiency common after infectious (e.g. Giardia) or inflammatory (e.g. Celiac) disease

77
Q

Decreased duodenal pH (decreased HCO3)

Decreased fecal elastase

A

PANCREATIC INSUFFICIENCY

Deficiency in Vit A, D, E, K, B12

78
Q
Histology associated with...
Diarrhea
Mesenteric nodes
Arthralgia
Neurologic abnormalities
Endocarditis
A

WHIPPLE DISEASE

Intracellular Gram+ Tropheryma whipplei
PAS+ foamy macrophages in interstitial lamina propria

79
Q

Diseases appearing with…

Cobblestone mucosa
Creeping fat
Bowel wall thickening ("string sign") that can result in bowel obstruction
Linear ulcers and fissures
Anorectal findings (tags, fissures)

Continuous mucosal and submucosal inflammation including the rectum
Friable mucosa with pseudopolyps
Freely hanging mesentery
Loss of haustra (lead pipe)

A

Crohn’s - Transmural skip lesions with rectal sparing often presenting with RLQ pain and occult blood

UC - Continuous mucosal and submucosal inflammation including the rectum often presenting with LLQ pain and hematochezia

80
Q

Diseases appearing with…

Crypt abscesses and ulcers
Bleeding
No granulomas

Noncaseating granulomas
Lymphoid aggregates

A

UC

Crohn’s

81
Q

Diseases resulting in…

Fistulas (e.g. pneumaturia)
Abscess
Strictures/Obstruction

Colorectal cancer (non-polyploid, multifocal)
Toxic megacolon/Perforation
A

Crohn’s

UC

82
Q

Diseases associated with…

Anterior uveitis
Migratory monoarticular arthritis
Erythema nodosum
Ankylosing spondylitis

Pyoderma gangrenosum
Primary sclerosing cholangitis (p-ANCA)

Aphthous ulcers
Gallstones (decreased bile resorption)
Calcium oxalate stones (increased oxalate absorption)
Malabsorption (e.g. ADEK deficiency)

A

Both
UC
Crohn’s

Note - Crohn’s more likely to result in malabsorption due to terminal ileal involvement

83
Q

Treatment of UC and Crohn’s

A
UC:
5-ASA (Mesalamine)
6-Mercaptopurine
Infliximab
Colectomy
Crohn's:
Corticosteroids
Azathioprine
Metronidazole/Ciprofloxacin
Infliximab or Adalimumab (anti TNF-a)

Note - Smoking decreases UC (but increases Crohn’s)

84
Q
Recurrent abdominal pain
Improvement with defecation
Change in stool frequency
Change in appearance of stool
Normal biopsy
A

IRRITABLE BOWEL SYNDROME

85
Q

Differential for appendicitis

A

Elderly men = Diverticulitis

Women = Ectopic pregnancy (b-hCG)

86
Q

Difference between true and false diverticulum

A

True = All 3 gut layers outpouch, including muscularis externa (e.g. Meckel)

False = Only mucosa and submucosa outpouch, often near vasa recta perforation of muscularis externa due to increased intraluminal pressure (e.g. constipation)

87
Q

Treatment of…
Painless brisk hematochezia
LLQ pain
Fever

A

DIVERTICULITIS

Antibiotics - If complicated (abscess, fistula, obstruction, perforation) then percutaneous drainage or surgery

Note - Painless, low-volume bleeding is likely venous instead (e.g. AVM)

88
Q
Dysphagia
Obstruction
Gurgling
Aspiration
Halitosis
Neck mass
A

ZENKER (FALSE) DIVERTICULUM

Esophageal dysmotility results in herniation at Killian triangle at inferior pharyngeal constrictor

motor abnormality of the esophagus

89
Q

Toddler with…
Melana
RLQ pain

Associated with terminal ileal…
Intussusception (red currant jelly stool)
Volvulus
Obstruction

A

MECKEL (TRUE) DIVERTICULUM

Persistence of vitelline-duct - may contain ectopic acid-secreting gastric mucosa (Pertechnetate study+) leading to peptic ulceration (melena)

heterotopic gastric mucosa or pancreatic tissue may be seen in the midst of normal intestinal mucosa

Note - Related to Omphalomesenteric cyst which is a cystic dilation of the vitelline duct

Note - “Rule of 2s”

90
Q

Bilious emesis
Abdominal distention
Failure to pass meconium
Failure of internal sphincter to relax

Associated with RET mutation

A

HIRSCHSPRUNG’S DISEASE

Failure of neural crest cell migration - increased risk in Down’s syndrome (+duodenal atresia)

Aganglionic region is narrow and results in dilatation of proximal colon (rectum/anus always involved)

Note - Diagnose with rectal suction biopsy

91
Q

Associated with Ladd bands - fibrous band between cecum and RLQ compressing duodenum

A

MALROTATION

Failure of counterclockwise rotation

Leads to midgut volvulus as narrow mesentery predisposes to twisting around SMA - omega sign on XR, bird’s beak on barium enema, “corkscrew” small bowel on barium swallow

Note - Sigmoid volvulus is more common in the elderly

92
Q

Lead points for intussusception

A

Meckel diverticulum
Peyer patch hypertrophy (adenovirus infection)
Tumor (adults)

Note - Target-sign on US

93
Q

Difference between acute mesenteric ischemia, chronic mesenteric ischemia, and colonic ischemia

A

Acute mesenteric = Pain out of proportion to exam with currant jelly stool

Chronic mesenteric = Postprandial bowel angina

Colonic = Crampy abdominal pain followed by hematochezia in elderly or surgery patients

94
Q

Premature, formula-fed infant with…
Feeding intolerance
Abdominal distension
Abdominal erythema

May result in…
Pneumatosis intestinalis
Portal venous gas
Free air under diaphragm

A

NECROTIZING ENTEROCOLITIS

95
Q

Polyp resulting from…
Chromosomal instability pathway
Mutations in APC/KRAS

Note - Most sporadic cases of CRC

A

ADENOMATOUS

Villous more malignant than tubular

96
Q

Polyp resulting from…
CpG hypermethylation pathway
Microsatellite instability/BRAF mutations

A

SERRATED

“Saw-tooth” crypts on histology

97
Q

Mutation/chromosome associated with familial adenomatous polyposis

Note - May also present with hepatoblastoma

A

Autosomal dominant mutation of APC tumor suppressor gene - Chromosome 5q

Note - 2-hit hypothesis

98
Q

Familial adenomatous polyposis
Osseous and soft tissue tumors
Congenital hypertrophy of retinal pigment epithelium
Impacted/supernumerary teeth

A

GARDNER SYNDROME

99
Q

Familial adenomatous polyposis

Malignant CNS tumor

A

TURCOT SYNDROME

100
Q

GI hamartomas
Hyperpigmentation
Increased risk of GI/breast cancer

A

PEUTZ-JEGHERS SYNDROME

Autosomal dominant

Hamartomas - Generally non-neoplastic solitary lesions of normal colonic tissue with distorted architecture

101
Q

Child with hamartomas throughout the GI tract - associated with increased risk of CRC

A

JUVENILE POLYPOSIS SYNDROME

Autosomal dominant

102
Q

Colon cancer
Endometrial cancer
Ovarian cancer
Skin cancer

A

LYNCH SYNDROME (HNPCC)

“CEOS of Lynch inc.”

Autosomal dominant mutation of DNA mismatch repair genes with subsequent microsatellite instability of cell DNA (MSH2,MSH6, MLH1,PMS2)

103
Q

Difference between presentation of rectosigmoid and ascending CRC

A

Ascending:
Exophytic mass
Iron deficiency anemia
Weight loss

Rectosigmoid:
Infiltrating mass/encircling
Partial obstruction ("apple core" on barium enema)
Colicky pain
Hematochezia

Note - IDA in males and postmenopausal women means colonoscopy!

Note - Can follow with CEA but cannot diagnose with it

104
Q

Mechanism of chromosomal instability pathway for CRC

A

Loss of APC gene
Decreased adhesion, increased proliferation
KRAS mutation
Unregulated intracellular signaling leads to adenoma
Loss of tumor suppressor (p53)
Carcinoma

Note - In colitis associated CRC p53 mutations occur early and APC mutations late

105
Q

Causes (3) of portal hypertension

A

Cirrhosis
Budd-Chiari (hepatic vein thrombosis)
Schistosomiasis

106
Q

Ratio between ALT and AST in alcoholic hepatitis

A

AST/ALT > 2

Note - In non-alcoholic liver pathology ALT > AST as it is more specific to the liver

107
Q

Differentiating bone disease and biliary disease in elevated ALP

A

Elevated GGT in biliary disease

Note - Often will see pale stool and dark urine

108
Q

Mechanism of thrombocytopenia in liver pathology

A

Decreased liver thrombopoietin production

Splenomegaly from portal HTN related splenomegaly

109
Q
Child with VZV or influenza with...
Vomiting
Coma
Hypoglycemia
Hepatomegaly
Microvesicular fatty change
A

REYE HEPATIC ENCEPHALOPATHY

ASA metabolites decreased b-oxidation in mitochondria

110
Q

Liver with…
Swollen necrotic hepatocytes
Neutrophilic infiltration
Mallory bodies - intracytoplasmic eosinophilic inclusions of damaged keratin fibers

A

ALCOHOLIC HEPATITIS

Hepatic steatosis precedes this and presents as macrovesicular fatty change - depleted NAD+ inhibits b-oxidation and inducing TG formation

111
Q

Histologic appearance of cirrhosis

A

Sclerosis around central vein

Note - Irreversible

112
Q

Cellular ballooning and eventual necrosis with ALT > AST

A

NON-ALCOHOLIC FATTY LIVER DISEASE

May lead to cirrhosis and HCC independent of alcohol use

113
Q

Mechanism of treatment for hepatic encephalopathy

A

Lactulose increases bacterial conversion of NH3 to NH4 trapping it in the gut

Rifaximin/Neomycin decreases NH3 producing gut bacteria

Note - Diuresis/TIPS (like renal failure) will impede clearance of NH3 from the blood

114
Q
Jaundice
Ascites
Anorexia
Hepatomegaly (may be tender)
Polycythemia (EPO production)
Elevated AFP
A

HEPATOCELLULAR CARCINOMA

Associated with HBV integration into genome

Note - In cirrhosis shrunken liver instead

115
Q

Liver tumors where biopsy is contraindicated due to hemorrhage risk

A

Cavernous hemangioma: Common benign liver tumor made of cavernous blood-filled spaces

Hepatic adenoma: Rare benign liver tumor associated with OCP and anabolic steroid use (generally right lobe) - appears glandular

116
Q

Malignant tumor associated with Arsenic/Vinyl Chloride exposure

A

ANGIOSARCOMA

Endothelial origin

117
Q

Centrilobular congestion
Grossly dark spots on tan background (“nutmeg” liver)
Absent JVD

A

CONGESTIVE HEPATOPATHY (BUDD-CHIARI)

Unlike cardiac cirrhosis, JVD and hepatojugular reflex are not present

118
Q

Cirrhosis with PAS+ globules in liver

A

A1AT DEFICIENCY

Misfolded protein aggregates in hepatocellular ER - decreased functioning protein (elastase) in lungs leads to emphysema

119
Q

Asymptomatic or mild jaundice after fasting or stress

Indirect hyperbilirubinemia

A

GILBERT SYNDROME

Autosomal Recessive mildly decreased UDPGT

120
Q

Early onset jaundice and kernicterus

Indirect hyperbilirubinemia

A

CRIGLER-NAJJAR TYPE I

Autosomal Recessive absent UDPGT

Note - Type II is less fatal as it responds to Phenobarbital, increasing liver enzyme synthesis

121
Q

Grossly black liver due to epinephrine metabolites that accumulate within lysosomes
Conjugated hyperbilirubinemia
jaundice when exposed to stressor/trigger

A

DUBIN-JOHNSON SYNDROME

Autosomal Recessive defective liver excretion of bilirubin glucuronides due to mutation in canalicular membrane transport protein- benign

Note - Rotor syndrome is similar but milder and without grossly black liver (also impaired uptake)

122
Q
Young patient with...
Parkinsonism
Psychiatric disease
Fanconi syndrome
Hemolytic anemia
Cirrhosis
A

WILSON DISEASE (HEPATOLENTICULAR DEGENERATION)

Autosomal Recessive mutation in hepatocyte copper-transporting gene (ATP7B) resulting in decreased excretion in the bile - Low serum Ceruloplasmin, elevated urinary Cu

Treat with Penicillamine/Trientine chelation, oral Zinc

Note - Specifically Putamen atrophy

123
Q
Patient > 40 y/o with...
Micronodular cirrhosis
Diabetes
Skin pigmentation
Dilated cardiomyopathy (reversible)
Hypogonadism
Arthropathy (calcium pyrophosphate)

Elevated Ferritin
Elevated Iron
Decreased TIBC (elevated transferrin saturation)

A

HEMOCHROMATOSIS

Autosomal Recessive mutation in HFE - Decreased interaction with transferrin receptor results in increased apical enterocyte DMT1 and decreased hepatic Hepcidin production

Hemosiderin (iron) identified on MRI, golden-yellow granules on biopsy, or Prussian blue stain

increased risk of liver cirrhosis and HCC

124
Q
Intra- and extrahepatic ducts with...
Onion-skin bile duct fibrosis
Alternating strictures and dilation (beading)
Elevated IgM
p-ANCA+
A

PRIMARY SCLEROSING CHOLANGITIS

Can lead to secondary biliary cirrhosis - increased risk of cholangiocarcinoma and gallbladder cancer

Associated with IBD

Note - Other causes of secondary biliary cirrhosis include extrahepatic gallstones and pancreatic carcinoma

125
Q
Pruritus
Cholestasis
Hypercholesterolemia
Destruction of intrahepatic, interlobular bile ducts
Lymphocytic infiltrate
Granuloma formation
Elevated IgM
Anti-mitochondrial antibody
A

PRIMARY BILIARY CIRRHOSIS

Associated with...
Celiac sprue
Sjogren
Hashimoto thyroiditis
CREST
Rheumatic arthritis
126
Q

Causes (3) of gallstones

A

Increased cholesterol, bilirubin
Decreased bile salts/phosphatidylcholine
Gallbladder stasis

Note - Risk factors are 4 F’s (female, fat, forty, fertile/pregnant)

Note - Also associated with Crohn’s disease

127
Q

Ileus accompanied by pneumobilia

A

GALLSTONE ILEUS

Fistula between gallbladder and GI tract - gallstones in intestines, air in biliary tree

128
Q

Jaundice
RUQ pain
Fever

A

CHARCOT TRIAD (CHOLANGITIS)

129
Q
Gallstone associated with...
Crohn's disease
Alcoholic cirrhosis
Biliary infection (Ascaris, Sinensis)
Chronic hemolysis
TPN
A

PIGMENT STONES

Composed of calcium and bilirubin - soft and firable

Black = radiopaque (hemolysis)
Brown = radiolucent (infection)

Note - Infection releases b-glucuronidase which deconjugates bile, allowing it to bind Ca and form stones

130
Q
Gallstone associated with...
Crohn's disease
Obesity
Estrogen therapy (induce HMG-CoA)
Multiparity (Progesterone induced hypomobility)
TPN (hypomobility)
Fibrates
A

CHOLESTEROL STONE

Radiolucent

131
Q

Diagnosis and treatment of cholecystitis

Note - Pain from forcing of stone into cystic duct with CCK release

A

Diagnose with US - Wall thickening, pericholecystic fluid

If equivocal, consider cholescintigraphy to assess cystic duct patency

Treat with cholecystectomy only if biliary colic - Opacities in gallbladder on US may be asymptomatic

132
Q

RUQ pain
Fever
Murphy’s sign

Thickened gallbladder wall
Pericholecystic fluid
Distention

A

CHOLECYSTITIS

Stones blocking cystic duct may not appear on US - confirm with HIDA scan

Note - Emphysematous (air in wall) due to Clostridium

133
Q

Epigastric pain radiating to back
Elevated serum amylase or lipase (3x upper limit)
Edematous pancreas on imaging

A

ACUTE PANCREATITIS

Usually due to gallstones, ethanol, or trauma - may also be caused by...
Hypercalcemia/Hypertriglyceridemia
Drugs/ERCP
Autoimmune disease
Steroids
Mumps
Scorpion sting
134
Q

Fluid surrounded by granulation tissue instead of epithelium

A

PSEUDOCYST

Other complications of pancreatitis include...
Hemorrhage
Necrosis
Infection
Hypocalcemia

Note - Adipose digestion by backed up lipase leads to formation of FAs which bind Ca and precipitate as “chalk”

135
Q

Steatorrhea
Vit A, D, E, K deficiency
Diabetes
Calcification on imaging

Note - Normal amylase and lipase

A

CHRONIC PANCREATITIS

Causes include alcohol and CF

Note - May result in splenic vein thrombosis and fundal gastric varices

136
Q
Histology associated with...
Smoking
Abdominal pain radiating to back
Weight loss
Migratory thrombophlebitis
Jaundice
Palpable, nontender gallbladder (Courvoisier)
Elevated CA 19-9
A

PANCREATIC ADENOCARCINOMA

Disorganized glandular structures with cellular infiltrate

Note - Most occur in pancreatic head leading to obstructive jaundice

137
Q

Mechanism of increased blood pH following meals

A

ALKALINE TIDE

Carbonic anhydrase used to produce H+ from H2CO3 for H/K ATPase excretion into stomach - the HCO3 is secreted into the bloodstream via HCO3/Cl antiporter

138
Q

Mechanism of hepatic encephalopathy

A

Increased NH3 in Astrocytes increases Glutamate to Glutamine conversion, resulting in Astrocyte swelling and decreased Glutamine release - decreased Glutamine in surrounding neurons decreases excitatory neurotransmission (eg. Glutamate)

Increased inhibitory neurotransmission (e.g. GABA)

Causes include increased protein load (e.g. Glutamine), or GI bleeding - colonic bacteria convert hemoglobin to NH3

Note - Blood urea nitrogen (BUN) decreased unlike in uremia/hypovolemia as liver cannot convert NH3 to BUN

139
Q

Interstitial edema
Focal fat necrosis
Calcium deposits

A

ACUTE INTERSTITIAL PANCREATITIS

Eventually compromised blood flow or direct parenchymal injury causes activation of Trypsin in acinar cells - autodigestion (acute necrotic/hemorrhagic pancreatitis)

140
Q

Mechanism of cholecystitis without gallstones in a critically ill patient

A

ACALCULOUS CHOLECYSTITIS

Ischemia results in inflamed and enlarged gallbladder without stones

141
Q

Microbial causes of hepatic abscess by route of transmission…

Hepatic artery (hematogenous)

Ascension of biliary tract (e.g. cholangitis), Direct invasion, or Portal vein pyema (adjacent sources)

Penetrating injury

Foodborne

A

S. aureus

GNRs (E. coli, Klebsiella), Enterococci

Polymicrobial

E. histolytica

142
Q

Abdominal pain
Acid reflux
Distal (bulb) duodenal ulcers
Diarrhea

A

ZOLLINGER-ELLISON SYNDROME

Gastrin secreting neuroendocrine tumor (gastrinoma) in pancreas or small intestine

Diagnosed with Secretin test which shows paradoxical increase in Gastrin with Secretin administration

Note - Diarrhea from damage to intestinal epithelium preventing activation of pancreatic enzymes

143
Q

Gastrectomy patient with…
Colicky abdominal pain
Nausea
Diarrhea

A

DUMPING SYNDROME

144
Q

Supplementation given to all exclusively breastfed infants

A

Vit D
Iron (> 4 mos)

Note - All infants also given Vit K IM after birth to prevent hemorrhagic disease of the newborn

145
Q

Poor prognostic indicators in cirrhosis

A

Elevated prothrombin time
Hypoalbuminemia
Hyperbilirubinemia

146
Q

Mechanism behind periumbilical and RLQ pain in appendicitis

A

Periumbilical = Visceral distention stimulates the GVA resulting in poorly localized, dull pain at T10

RLQ pain = Eventually inflammation involves the parietal peritoneum resulting in somatic pain and rebound tenderness

147
Q

Mechanism of Crohn’s

A

Underexpression of NOD2 intracellular receptor in intestinal epithelium and macrophages

Decreased expression of NF-kB reduces cytokine production

Impaired innate immune response allows gut bacteria to produce exaggerated adaptive immune response

148
Q

Bloating
Diarrhea
Elevated Folate
Elevated vit K

A

Small intestinal bacterial overgrowth (SIBO)

149
Q
Fever
Firm, erythematous periauricular swelling
Trismus
Dysphagia
Elevated Amylase
Normal Lipase
Associated with decreased salivary flow...
Anticholinergics
Obstruction
Dehydration
Post-intubation
A

SUPPURATIVE PAROTITIS

S. aureus and Anaerobes

150
Q

Site of fat/lipid absorption

A

Jejunum

Note - Without a gallbladder bile storage does not occur but is constantly released instead so fat absorption can still occur

151
Q
Intermittent solid/liquid dysphagia
Regurgitation
Chest pain
Heartburn
Corkscrew esophagus on barium swallow
A

DIFFUSE ESOPHAGEAL SPASM

Impaired inhibitory neurotransmission from esophageal myenteric plexus

152
Q

Dysphagia with…
Basal zone hyperplasia
Elongation of lamina propria
Scattered eosinophils

A

GERD

May progress to Barrett’s

153
Q

low maternal AFP
increased nuchal translucency

failed recanalization of duodenum

A

Downs syndrome related duodenal atresia

on xray: double bubble sign and decreased distal intestinal gas

154
Q

serum sickness-like syndrome joint pain
lymphadenopathy
pruritic urticarial vasculitis rash
elevated AST and ALT

A

Hep B

prolonged PT time is poor prognosis

155
Q

what branch of the splenic artery is susceptible to ischemic injury after splenic artery blockage

A

short gastrics because no anastomosis

left gastroepiploic has anastomosis from right gastroepiploic

156
Q

apoptotic hepatocytes form round acidophilic bodies

A

councilman bodies or apoptotic bodies

acute viral hepatitis

157
Q

Abnormal sensation of a foreign body, tightness, or fullness in the throat

functional disorder of the esophagus

Not structural or motility disorder

Anxiety related

A

Globus sensation

158
Q
Dysphagia
Dysarthria
Dysphonia
impaired tongue movement
impaired facial movement
A

Psuedobulbar palsy

related to multiple sclerosis

159
Q
Cutaneous flushing
Hypotension
Secretory Diarrhea
Bronchospasm
Cardiac valvular lesions
A

carcinoid tumor that has metastasized to the liver

can also get pellagra like symptoms which may result from niacin deficiency due to depletion of tryptophan stores by carcinoid tumor as it produces excessive serotonin

tx with octreotide(somatostatin analog)

160
Q

The physical manifestations of high estrogen levels in the cirrhotic patient include

due to decreased metabolism of estrogen by the failing liver, decreased production of sex hormone binding globulin by the liver, and decreased metabolism of androgens

A
spider angiomas
palmar erythema
gynecomastia 
testicular atrophy
dupuytrens contractures
decreased body hair