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Flashcards in GI Deck (320):
1

Foregut develops into

Pharynx to duodenum

2

Midgut develops into...

duodenum to transverse colon

3

Hindgut develops into...

Distal transverse colon to the rectum

4

Developmental Defect of Anterior Abdominal Wall Due to Failure of
Rostal Fold Closure:
Lateral Fold Closure:
Caudal Fold Closure:

Rostal Fold Closure: Sternal Defects
Lateral Fold Closure: Omphalocele, Gastroschisis
Caudal Fold Closure: Bladder Exstrophy

5

Duodenal atresia
What is it?
Genetics?
Presentation
XR

Failure to Recanalize
Trisomy 21 (Down Syndrome)
Early bilious vomiting with proximal stomach distention
"Double Bubble" on XR

6

Jejunal, Ilial, or Colonic Atresia
What causes them?

Vascular accident (apple peel atresia)

7

Timing of midgut development

6th week: Midgut Herniates through umbilical ring
10th week: Returns to abdominal cavity and rotates around SMA

8

Gastroschisis
What is it?
Peritoneum?

Extrusion of the abdominal contents through the abdominal folds; not covered by peritoneum

9

Omphalocele
What is it?
Peritoneum?

Persistance of herniation of abdominal contents into umbilical cord; covered by peritoneum

10

Most common Tracheoesophageal anomaly?

Esophageal atresia with distal tracheoesophageal fistula (85%)

11

Esophageal atresia with distal tracheoesophageal fistula
Presentation
XR
Cyanosis?
Clinical test?

Drooling, choking, vomiting with first feeding
Air in stomach visible on XR (TEF allows air into stomach)
Cyanosis secondary to laryngospasms (to avoid reflux-related aspiration)
Clinical test: failure to pass NG tube into stomach

12

H type Tracheoesophageal anomaly

Fistula alone

13

CXR in pure atresia type Tracheoesophageal anomaly?

In pure atresia (esophageal atresia only) CXR shows gasless abdomen

14

Congenital Pyloric Stenosis
What causes it?
Presentation?
Physical exam?
Treatment
Occurrence?
More often in...

Hypertrophy of pylorus
Nonbilious projectile vomiting at 2 weeks of age
Palpable olive mass in epigastric region
Surgical incision
Occurs 1/600 live births
More often in first born males

15

Pancreas Derived from

Foregut

16

Ventral Pancreatic bud contributes to

Pancreatic head and main pancreatic duct

17

Uncinate process of pancreas formed by the

Ventral bud alone

18

Dorsal pancreatic bud becomes

Body, tail, isthmus and accessory pancreatic duct

19

Annular Pancreas
What is it?
What may it cause?

Ventral pancreatic bud abnormally encircles 2nd part of duodenum
May cause duodenal narrowing

20

Pancreas divisum

Ventral and dorsal parts of pancreas fail to fuse at 8 weeks

21

Where does the spleen arise from?
What kind of tissue is this?
Where does it get its blood supply from?

Arises in mesentery of stomach
Mesodermal tissue
Supplied by foregut (celiac artery)

22

Do retroperitoneal structures have a mesentery?

No

23

Injuries to retroperitoneal structures can cause

Blood or gas accumulation in the retroperitoneal space

24

List of Retroperitoneal Structures

"SAD PUCKER"
Suprarenal gland (adrenal)
Aorta and IVC
Duodenum (2nd and 3rd parts)
Pancreas (except the tail)
Ureters
Colon (descending and ascending)
Kidneys
Esophagus (lower 2/3)
Rectum (lower 2/3)

25

Falciform Ligament
Connects
Structures Contained
Derivative of

Connects liver to abdominal wall
Contains ligamentum teres hepatis (from fetal umbilical vein)
Derivative of ventral mesentery

26

Hepatoduodenal Ligament
Connects
Structures Contained

Connects liver to duodenum
Contains portal triad

27

Portal Triad

Hepatic Artery, Portal Vein, Common Bild Duct

28

Omental Foramen
Name
What is it?
What is inside of it?

Epiploic Foramen of Winslow
Connects Greater and Lesser Sacs
Hepatoduodenal Ligament is inside of it

29

Pringle Maneuver

Compression of Hepatoduodenal ligament in omental foramen to control bleeding

30

Gastrohepatic Ligament
Connects?
Structures contained?
Separates?
Surgery?

Connects Liver to lesser curvature of the Stomach
Contains gastric arteries
Separates greater and lesser sacs on the Right
May be cut during surgery to access lesser sac

31

Gastrocolic Ligament
Connects?
Structures contained?
Part of?

Connects greater curvature of stomach to transverse colon
Contains gastroepiploic arteries
Part of greater omentum

32

Gastrosplenic Ligament
Connects?
Structures contained?
Function?

Connects greater curvature of stomach to the spleen
Contains short gastrics and Left gastroepiploic vessels
Separates greater and lesser sacs on the left

33

Splenorenal Ligament
Connects?
Structures contained?

Connects Spleen to Posterior Abdominal Wall
Splenic artery and vein. Tail of pancreas

34

Layers of Gut Wall

From Inside to Outside: "MSMS"
Mucosa, Submucosa, Muscularis Externa, Serosa (when intraperitoneal)/Adventitial (when retroperitoneal)

35

Layers of Gut Mucosa and function of each layer

Epithelium (absorption), Lamina Propria (support), Muscularis Mucosa (motility)

36

What is included inside the submucosa?

Submucosal Nerve Plexus (Meissner's)
Glands

37

What is included inside the Muscularis externa?

Myenteric Nerve Plexus (Auerbach's)

38

Ulcers extend into

submucosa, inner or outer muscular layers

39

Erosions extend into

Mucosa only

40

Frequency of basal electric rhythm?
Stomach
Duodenum
Ileum

Stomach: 3 waves/min
Duodenum: 12 waves/min
Ileum: 8-9 waves/min

41

Histology of the Esophagus

Non-Keratinized Stratified Squamous Epithelium

42

Histology of the Stomach

Gastric Glands

43

Histology of the Duodenum

Villi and Microvilli
Brunner's Glands (in submucosa)
Crypts of Lieberkuhn

44

Histology of the Jejunum

Plicae Circulares
Crypts of Lieberkuhn

45

Histology of the Ileum

Peyer's Patches (lamina propria, submucosa)
Plicae Circulares (proximal ileum)
Crypts of Lieberkuhn
Largest # of goblet cells in SI

46

Histology of the Colon

Crypts by no villi
Numerous goblet cells

47

Branches of the abdominal Aorta that supply GI structures branch in which direction?

Anteriorly

48

Branches of the abdominal Aorta that supply non-GI structures branch in which direction?

Laterally

49

SMA Syndrome

Transverse portion (3rd part) of Duodenum entrapped between SMA and Aorta --> Intestinal Obstruction

50

Level of Celiac Trunk

T12

51

Level of SMA

L1

52

Level of Left Renal Artery

L1

53

Level of IMA

L3

54

Bifurcation of Abdominal Aorta occurs at what level?

L4

55

Foregut
Artery
Parasympathetic Innervation
Vertebral Level
Structure supplied

Celiac
Vagus
T12/L1
Stomach to proximal duodenum, Liver, Gallbladder, Pancreas, Spleen (mesoderm)

56

Midgut
Artery
Parasympathetic Innervation
Vertebral Level
Structure supplied

SMA
Vagus
L1
Distal duodenum to proximal 2/3 of transverse colon

57

Hindgut
Artery
Parasympathetic Innervation
Vertebral Level
Structure supplied

IMA
Pelvic
L3
Distal 1/3 of transverse colon to upper portion of rectum

58

Splenic flexure

Bend between transverse and descending colon
Watershed region

59

Branches of Celiac Trunk

Common Hepatic, Splenic, Left Gastric

60

Strong anastomoses in stomach blood supply

L and R Gastroepiploics
L and R Gastrics

61

Poor anastomoses in stomach blood supply

Short Gastrics (if splenic artery is blocked)

62

Collateral circulation if abdominal aorta is blocked?

Internal Thoracic (mammary) --> Superior epigastric ↔ Inferior epigastric --> External iliac
Celiac Trunk --> Superior pancreaticoduodenal ↔ Inferior pancreaticoduodenal -- SMA
SMA --> Middle Colic ↔ Left Colic --> IMA
IMA --> Superior Rectal ↔ Middle and Inferior Rectal --> Internal Iliac

63

Portosystemc Anastomoses

L Gastric Vein ↔ Esophageal Vein --> Azygos
Paraumbilical Vein ↔ Superficial and Inferior Epigastric (below umbilicus) and Superior Epigastric and Lateral Thoracic (above umbilicus)
Superior Rectal ↔ Middle and Inferior Rectal

64

SMV and IMV drain into

Portal Vein

65

Varices of Portal HTN

Varices of "Gut, Butt, and Caput"
Esophageal varices, Internal hemorrhoids, Caput medusae

66

Surgical treatment of Portal HTN

"TIPS" Transjugular Intrahepatic Portosystemic Shunt between Portal Vein and Hepatic Vein percutaneously

67

What is the Pectinate (Dentate) Line

Where endoderm (hindgut) meets ectoderm

68

Above the pectinate line
What kind of hemorrhoids?
What kind of cancer?
Arterial Supply
Venous drainage

Internal Hemorrhoids
Adenocarcinoma
Superior Rectal Artery from IMA
Superior Rectal Vein --> IMV --> Portal Vein

69

Below the pectinate line
What kind of hemorrhoids?
What kind of cancer?
Arterial Supply
Venous drainage

External Hemorrhoids
Squamous Cell Carcinoma
Inferior Rectal Artery from Internal Pudendal
Inferior Rectal Vein --> Internal Pudendal Vein --> Internal Iliac --> IVC

70

Internal Hemorrhoids
Innervation?
Pain?
Lymphatic drainage?

Visceral Innervation, therefore NOT painful
Drained by Deep Nodes

71

External Hemorrhoids
Innervation?
Pain?
Lymphatic drainage?

Somatic Innervation (inferior rectal branch of pudendal nerve) and therefore Painful
Drained by Superficial Inguinal Lymph Nodes

72

Apical Surface of hepatocytes face

Bile Canaliculi

73

Basolateral Surface of hepatocytes face

Sinusoids

74

Zones of Liver
What is each one vulnerable to?

I: periportal --> Affected 1st by viral hepatitis
II: intermediate
III: pericentral vein (centrilobular)
Affected 1st by ischemia, Contains P450 system, most sensitive to toxin injury, site of alcoholic hepatitis

75

Common Hepatic Duct
Formed from
Goes to

R and L Hepatic Ducts
Joins Cystic duct to form Common Bile Duct

76

Common Bile Duct
Formed from
Goes to

Cystic Duct + Common Hepatic Duct
Joins Main Pancreatic Duct at Ampulla of Vater in Duodenum

77

Ampulla of Vater

Where the Main Pacreatic Duct joins the Common Bile Duct in the 2nd part of the Duodenum

78

Sphincter of Oddi

Sphincter around ampulla of vater

79

Gallstones lodged in ampulla of Vater block

Both bile and pancreatic ducts

80

Tumors that arise near the head of the pancreas near the duodenum can cause

Obstruction of the common bile duct

81

Organization of Vessels in Femoral Region

Lateral to Medial to find your "NAVEL"
Nerve, Artery, Vein, Empty space, Lymphatics

82

Femoral Triangle contains

Femoral Vein, Artery, and Nerve

83

Femoral Sheath
Location
Contents

3-4cm below inguinal ligament
Contains femoral vein, artery, and canal (deep inguinal lymph nodes) but not the femoral nerve

84

Relation between IVC and the Aorta

IVC is to the R of Aorta in MRI/CT

85

Contents of Inguinal Canal

Ilioinguinal nerve
Male: Spermatic Cord
Female: Round Ligament

86

Diaphragmatic Hernia
Definition
Think what kind of pt?
Most common kind of DH?

Abdominal structures enter the thorax
May occur in infants a a result of defective development of pleuroperitoneal membrane
Most commonly a Hiatal Hernia (stomach herniates through esophageal hiatus of diaphragm)

87

Sliding Hiatal Hernia
Frequency?
Results in what?

Most common hiatal hernia
GE junction is displaced upwards resulting in a hourglass stomach

88

Paraesophageal Hernia

GE junction is normal
Fundus protrudes into the thorax

89

Indirect Inguinal Hernia
Goes through
Location
Occurs in what kind of pt?
Follows path of?
What is it covered by?

Goes through internal (deep) inguinal ring, external (superficial) inguinal ring and into the scrotum.
Enters internal inguinal ring lateral to the inferior epigastric artery
Occurs in male infants owing to failure of processus vaginalis to close (from hydrocele)
Follows path of descent of the testes
Covered by all 3 layers of spermatic fascia

90

Direct Inguinal Hernia
Definition
Location
Occurs in what kind of pt?
Goes through
What is it covered by?

Protrudes through inguinal (Hesselbach's) Triangle
Bulges directly through abdominal wall medial to inferior epigastric artery
Older men
Goes through external (superficial) inguinal ring only
Covered by external spermatic fascia

91

Location of Direct vs Indirect Inguinal Hernias?

"MDs don't LIe"
Medial to inferior epigastric = Direct
Lateral to inferior epigastric = Indirect

92

Femoral Hernia
Location
Goes through
Most common in
Leading cause of

Protrudes below inguinal ligament
Goes through Femoral Canal below and lateral to pubic tubercle
Most common in Women
Leading cause of bowel incarceration

93

Hesselbach's Triangle

Inferior epigastric vessels
Lateral border of rectus abdominis
Inguinal ligament

94

Gastrin
Source
Location of Source
Action

G Cells in Antrum of stomach
↑ Gastric H secretion (through ECL cells that release Hist)
↑ Growth of gastric mucosa
↑ Gastric motility

95

Gastrin
↑ by
↓ by
What syndrome produces ↑ Gastrin secretion?

↑ by stomach distention, alkalinization, AA (esp Phenylalanine and Tryptophan), peptides, vagal stimulation
↓ by stomach ph < 1.5
↑↑ in Zollinger-Ellison Syndrome

96

Chronic Proton Pump Inhibitors (PPI) lead to

↑ Gastrin production

97

Cholecytokinin
Source
Location of Source
Action
Regulation

I cells in the duodenum and jejunum
↑ pancreatic secretion (via muscarinic pathways) and gallbladder contraction
↓ gastric emptying
Relaxes sphincter of Oddi
CCK secreted in response to ↑ FA and AA in duodenum

98

Secretin
Source
Location of Source
Action
Regulation

S cells in duodenum
↑ pancreatic bicarb secretion, bile secretion
↓ gastric acid secretion
Secretion ↑ w/ acid and FA in duodenum

99

Pancreatic enzymes function at what pH

Basic pHs

100

Somatostatin
Source
Location of Source
Action
Regulation
Affects Re Growth?

D cells in pancreatic islets and GI mucosa
↓ gastric acid and pepsinogen secretion, pancreatic and small intestine fluid secretion, gallbladder contraction, insulin and glucagon release
Secretion is ↑ by acid
Secretion is ↓ by vagal stimulation
Antigrowth hormone (inhibits digestion and absorption of substances needed for growth)

101

Glucose Dependent Insulinotropic Peptide
AKA
Source
Location of Source
Exocrine
Endocrine
Regulation

Gastric Inhibitory Peptide (GIP)
K cells in duodenum and jejunum
Exocrine: ↓ Gastric H secretion
Endocrine: ↑ insulin release
Secretion is ↑ by FA, AA, and oral glucose

102

Vasoactive Intestinal Polypeptide
Source
Location of Source
Action
Regulation

Parasympathetic ganglia in sphincters, gallbladder and SI
↑ intestinal water and electrolyte secretion and ↑ relaxation of intestinal smooth muscle and sphincters
Secretion is ↑ by distention and vagal stimulation
Secretion is ↓ by adrenergic input

103

VIPoma
Kind of cells
Presentation

non-α, non-β islet pancreatic tumors secrete VIP
"WDHA"
Copious Watery Diarrhea, HypoK, and Achlorhydria (no gastric acid produced)

104

Nitric Oxide
Actions in GI tract
Especially present in
Implicated in what disorder

Smooth muscles relaxation
Especially in lower esophageal sphincter
Loss of NO secretion is implicated in achalasia

105

Motilin
Location of Source
Action
Regulation
Agonists? Uses of agonists?

Small Intestine
Produced migrating motor complexes
Secretion ↑ in fasting state
Agonists like erythromycin used to stimulate intestinal peristalsis

106

Intrinsic Factor
Source
Location of Source
Action

Parietal Cells in Stomach (Body)
Vit B12 binding protein

107

Where is Vit B12 absorbed?

Bound to IF in terminal ileum along with bile acids

108

Autoimmune destruction of parietal cells leads to

Chronic gastritis and pernicious anemia

109

Gastric Acid
Source
Location of Source
Action
Regulation

Parietal Cells in Stomach
↓ stomach pH
Secretion ↑ by Hist, ACh, Gastrin
Secretion ↓ Somatostatin, GIP, prostaglandins, secretin

110

Gastrinoma

Gastrin secreting tumor that causes high levels of acid secretion and ulcers

111

Pepsin
Source
Location of Source
Action
Regulation

Chief Cells in Stomach (Body)
Protein digestion
Secretion is ↑ by vagal stimulation and local acid

112

Activation of pepsinogen

Converted to pepsin in presence of H+

113

Bicarb
Source
Location of Source
Action
Regulation

Mucosal cells of stomach, duodenum, salivary glands, pancreas and Brunner's Glands (in the duodenum)
Neutralizes acid
Secretion is ↑ from pancreatic and biliary secretion with secretin

114

Mucus that covers the gastric epithelium traps what?

Traps bicarb

115

Saliva
Secreted from
Stimulated by

Parotid, Submandibular and Sublingual glands
Supplied by sympathetic (β --> cAMP) and parasympathetic activity (M --> IP3)

116

Components of Saliva with function

Amylase digests starch
Bicarb neutralizes bacterial acids
Mucin lubricates food

117

Tonicity of Saliva

Normally hypotonic because of absorption but more isotonic with higher flow rates (less time for absorption)

118

How would Atropine affect parietal cells vs. G cells

Atropine --/ parietal cells
Atropine leaves G cells unaffected because the Vagus nerve releases GRP, not ACh to activate them

119

Brunner's Glands
Location
Function
Hypertrophied in...

Duodenal submucosa
Secrete Alkaline mucus
Hypertrophied in peptic ulcer disease

120

Receptors on Parietal Cells

ACh --> M3 --> Gq --> IP3 --> ATPase
Gastrin --> CCKB --> Gq --> IP3 --> ATPase
Hist --> H2 --> cAMP --> ATPase
Prostaglandins/misoprostol Receptors --> Gi --/ cAMP
Somatostatin Receptors --> Gi --/ cAMP

121

Pancreatic Secretions
Tonicity of Fluid
How does [electrolyte] change with flow?

Isotonic fluid
Low flow --> High [Cl]
High flow --> High [HCO3]

122

Pancreatic Secretions
Names and Roles

α amylase --> Starch digestion (secreted in active form)
Lipase. Phospholipase A, Colipase --> fat digestion
Proteases
Trypsinogen --> Activation of proenzymes (including trypsinogen)

123

Pancreatic Proteases
Names
Secreted as...

Trypsin, Chymotrypsin, Elastase, Carboxypeptidase
Secreted as proenzymes (zymogens)

124

What converts trypsinogen into trypsin

Enterokinase/enteropeptidase and then trypsin itself

125

Where is enterokinase/enteropeptidase secreted from?

Duodenal mucosa

126

Salivary Amylase
Role
MoA
Yields

Starts digestion
Hydrolyzes α(1-4) linkages to yield disaccharides (maltose and α-limited dextrins)

127

Pancreatic Amylase
Concentrated in
MoA

Highest concentration in duodenal lumen
Hydrolyzes starch to oligosaccharides and disaccharides

128

Oligosaccharide Hydrolase
Location
Role
MoA

At brush border of intestines
Rate limiting step in carbohydrate digestion
Produces monosaccharides from oligo- and disaccharides

129

Carbohydrate absorption
What kind of carbs?
By what cells?
What enzymes remove them from lumen?
What enzymes transport them to the blood?

Only monosaccharides (glucose, galactose, and fructose are absorbed by enterocytes
Glucose and Galactose are taken up by SGLT1 (Na dependent)
Frucose taken up by facilitated diffusion through GLUT5
All sugars enter blood via GLUT2

130

How to distinguish GI mucosal damage from other causes of malabsorption?

D-xylose absorption test

131

Iron absorption
Ionic state?
Location

As Fe2+ in duodenum

132

Folate absorption
Location

Jejunum

133

Peyer's Patches
What are they?
Where are they?
Contain what kind of specialized cells?

Unencapsulated lymphoid tissue in LP and submucosa of ileum
Contains specialized M cells that take up antigen

134

Peyer's Patches
What happens in germinal centers? Where do cells from germinal centers go? What do they do?

In germinal centers, B cells are stimulated to differentiate into IgA secreting plasma cells
IgA cells migrate to LP
IgA receives protective secretory component and is then transported across the epithelium to the gut to deal with intraluminal antigen

135

Antibody in the gut?

Secretory IgA
"Intra Gut Antibody"

136

Composition of Bile

Bild salts (bile acids conjugated to glycine and taurine to be made water soluble), Phospholipids, Cholesterol, Bilirubin, Water, Ions

137

Rate limiting step in bile production

Cholesterol 7α hydroxylase

138

Functions of Bile

Digestion and absorption of lipids and fat soluble vitamins
Cholesterol excretion (the body's only means)
Antimicrobial activity (via membrane disruption)

139

Bilirubin
Product of
Removed from blood by
Conjugated with
Excreted in

Product of heme metabolism
Removed from blood by liver
Conjugated with glucuronate
Excreted in bile

140

Direct Bilirubin

Conjugated with glucuronic acid
Water soluble

141

Indirect Bilirubin

Unconjugated
Water insoluble

142

How is unconjugated bilirubin transported in the blood

Bound to albumin

143

What enzyme conjugates bilirubin

UDP glucuronsyl transferase

144

What happens to conjugated bilirubin in the gut?

Gut bacterial break it down into urobilinogen
80% of excreted in feces as stercobilin to give stool its brown color
20% is reabsorbed

145

What happens to the reabsorbed urobilinogen?

10% goes to kidneys and excreted in urine as urobilin (gives urine yellow color)
90% enters enterohepatic circulation

146

Salivary Gland Tumor
Dangerous?
Common Location?

Generally benign and occurs in the parotid gland

147

Salivary Gland Pleomorphic Adenoma
Dangerous?
Kind of tumor? Composition?
Frequency?
Presentation
Recurrence?

Benign
Mixed tumor of cartilage and epithelium
Most common salivary gland tumor
Painless and mobile mass
Frequently recurs

148

Warthin's Tumor
Kind of tumor?
Descriptive name?
Dangerous?
Description

Salivary Gland Tumor
Papillary Cystadenoma Lymphomatosum
Benign
Cystic tumor with germinal centers

149

Achalasia
What is it?
Presentation
Diagnosis
Increased Risk of...

Failure of the lower esophageal sphincter to relax due to loss of myenteric plexus
Progressive dysphagia to solids and liquids
Barium swallow shows dilated esophagus with area of distal stenosis (Birds Beak)
Increased risk of squamous cell carcinoma

150

Secondary Achalasia may arise from...

Chagas disease

151

Scleroderma and Esophagus?

Associated with esophageal dysmotility involving low pressure proximal to LES

152

DDx for progressive dysphagia to solids and liquids?

Achalasia and Scleroderma

153

GERD
Common Presentation
May also present with
Pathology

Heartburn and regurgitation upon lying down
May also present with nocturnal cough, dyspnea, adult onset asthma
Decrease in LES tone

154

Esophageal varices
What are they?
Secondary to?

Painless bleeding of dilated submucosal veins in the lower 1/3 of the esophagus
Secondary to portal HTN

155

DDx for Esophagitis

Reflux, Infection, chemical ingestion

156

What organisms cause infectious Esophagitis?

Candida (white pseudomembrane), HSV1 (punched out ulcers), CMV (linear ulcers)

157

Mallory-Weiss Syndrome
What is it?
What causes it?
What does it lead to?
Usually found in what kind of pt?

Painful Mucosal laceration at the GE junction
Due to severe vomiting
Leads to hematemesis
Alcoholics and bulimics

158

Boerhaave Syndrome

"Been Heaving Syndrome"
Transmural esophageal rupture due to violent retching

159

Esophageal Strictures are associated with...

Lye ingestion and acid reflux

160

Plummer Vinson Syndrome

Triad of: Dysphagia (due to esophageal webs), Glossitis, Iron Deficiency Anemia

161

Barrett's Esophagus
What is it?
Due to
Associated with

Glandular metaplasia: replacement of stratified squamous epithelium with intestinal (non-ciliated columnar with goblet cells) epithelium in the distal esophagus
Due to GERD
Associated with esophagitis, esophageal ulcers, and increased risk for esophageal adenocarcinoma

162

SCJ or Z line?

Squamocolumnar Junction at LES

163

Esophageal Cancer
What kinds of cancer?
Presentation
Prognosis

Squamous Cell Carcinoma or Adenocarcinoma Presents with progressive dysphagia (first solids, then liquids) and weight loss
Prognosis is poor

164

Risk factors for Esophageal Cancer

"AABCDEFFGH"
Achalaisa, Alcohol (squamous), Barrett's (adeno), Cigarettes, Diverticula (Zenker's) (squamous), Esophageal webs (squamous), Familial, Fat (adeno), GERD (adeno), Hot liquids (squamous)

165

Most common Esophageal cancer
Worldwide?
US?

Worldwide: squamous
US: adenocarcinoma

166

Esophageal Cancers
Location of Squamous vs Adenocarcinoma?

Squamous: upper 2/3
Adenocarcinoma: lower 1/3

167

Malabsorption Syndromes can cause...

Diarrhea, Steatorrhea, Wt loss, Weakness, Vitamin and Mineral deficiencies

168

Malabsorption Syndromes Names

"These Will Cause Devastating Absorption Problems"
Tropical Sprue, Whipple's Disease, Celiac Sprue, Disaccharidase deficiency, Abetalipoproteinemia, Pancreatic insufficiency

169

Tropical Sprue
Cause
Responds to
Similar to
Can affect

Unknown cause
Responds to antibiotics
Similar to Celiac Sprue
Can affect entire small bowel

170

Whipple's Disease
What is it?
Histology
Presentation
Most often in what kind of pt?

"Foamy Whipped cream in a CAN"
Infection with Tropheryma whipplei (gram +)
PAS+ fomay macrophages in the intestinal LP and mesenteric nodes
Cardiac Symptoms, Arthralgias, and Neurologic Symptoms
Most often in older men

171

Celiac Sprue
Pathology
Stool?
Ethnicity?
Genetics
What part of GI tract?

AutoAbs to gluten (gliadin) in wheat and other grains
Leads to steatorrhea
Northern European descent
HLA-DQ2 and 8
Primarily affects distal duodenum or proximal jejunum

172

Disaccharidase Deficiency
Most common kind?
Histology
Diarrhea?
Self limiting kind?

Most common is lactase deficiency
Normal appearing villi
Osmotic diarrhea
Lactase normally at tips of villi, so self limiting kind can occur following infection

173

Lactase deficiency diagnosis?

Administration of lactose produces symptoms and glucose rises < 20mg/dL

174

Abetalipoproteinemia
PathoPhys?
Presentation?

↓ synthesis of apolipoprotein B --> inability to generate chylomicrons --> ↓ secretion of choesterol, VLDL into blood and fat accumulates in enterocytes
Presents in early childhood with malabsorption and neurologic manifestations

175

Pancreatic Insufficiency
What can cause it?
What does it lead to
Stool?

Caused by CF, Obstructing Cancer, Chronic Pancreatitis
Causes malabsorption of fat and fat soluble vitamins (A, D, E, K)
↑ neutral fat in stool

176

Celiac Sprue
Histology
Ab findings?
Screening
Associated with
Increased risk for

Blunting of villi. Crypt hyperplasia. Lymphocytes in LP
Anti-endomysial, anti-tissue transglutaminase, and anti-gliadin Abs
Serum levels of tissue transglutaminase Abs used for screening
Associated with dermatitis herpetiformis
Moderately increased risk for malignancy (T cell lymphoma)

177

Acute Gastritis
MoA
What can cause it?
Especially common among

Disruption of mucosal barrier --> inflammation
"U want a SNAC?"
Caused by stress, NSAIDs (↓ PGE --> ↓ gasric mucosa protection), Alcohol, Uremia, Burns (Curling's ulcer), Brain Injury (Cushing's ulcer)
Especially common among alcoholics and NSAID users (RA pts)

178

Curling's Ulcer

↓ plasma volume --> sloughing of gastric mucosa

179

Cushing's ulcer

↑ vagal stimulation --> ↑ ACh --> ↑ H production

180

Acute vs Chronic Gastritis

Erosive vs Non-Erosive

181

Type A Chronic Gastritis
What part of stomach?
Pathology
Produces what?
Associated with what?

Fundus and Body
AutoAbs to parietal cells
Produces pernicious Anemia and Achlorhydria
Associated with other immune disorders

182

Type B Chronic Gastritis
What part of stomach?
Frequency
Pathology
Increased risk of what?

Antrum
Most common type of chronic gastritis
Caused by H pylori infection
Increased risk of MALT lymphoma

183

Type A vs B Chronic Gastritis

"ABA, BAB"
A is in Body and from Autoimmunity
B is in Antrum and from Bacteria

184

Menetrier's Disease
What is it?
Cancerous?
Description

Gastric hypertrophy with protein loss, Parietal cell atrophy, ↑ mucous cells
Precancerous
Rugae of stomach are so hypertrophied that they look like brain gyri

185

Stomach Cancer
What kind of cancer?
Does it spread?
Often presents with

Almost always adenocarcinoma
Early aggressive local spread and node/liver metastases
Often presents with acanthosis nigricans

186

Stomach Cancer: Diffuse
Associated with?
Histology
Gross

Not associated with H pylori
Signet ring cells
Stomach wall grossly thickened and leathery (linitis plastica)

187

Virchow's Node

Involvement of Left Supraclavicular Node by metastasis from stomach

188

Krukenberg's Tumor
Presents with
Histology

Bilateral metastases from stomach to ovaries
Presents with abundant mucus
Signet ring cells

189

Sister Mary Joseph's Nodule

Subcutaneous periumbilical metastasis

190

Intestinal Stomach Cancer
What causes it
Associated with
Common location
Looks like

Caused by H pylori infection, dietary nitrosamines (smoked foods), achlorhydria, chronic gastritis,
Associated with Type A blood
Commonly on lesser curvature
Looks like ulcer with raised margins

191

Peptic Ulcer Disease

Gastric Ulcers
Duodenal Ulcers

192

Gastric Ulcer
Pain
Weight
H pylori
Causes
Risk
Often occurs in what kind of pt?

Pain increases with meals
Weight loss
H pylori in 70%
↓ mucosal protection against gastric acid; NSAID use
Risk of Carcinoma
Often occurs in older patients

193

Duodenal Ulcer
Pain
Weight
H pylori
Causes
Risk
Histological changes

Pain decreases with meals
Weight gain
H pylori in 100%
↓ mucosal protection or ↑ gastric acid secretion
Generally benign
Hypertrophy of Brunner's glands

194

Ulcer Complications

Hemorrhage or Perforation

195

Location of Hemorrhage from Ulcer

Gastric or Duodenal Ulcers
Posterior > Anterior

196

Location of Perforation from Ulcer

Duodenal
Anterior > Posterior

197

Ruptured gastric ulcer on the lesser curvature --> bleeding from

Left Gastric Artery

198

Ulcer on posterior wall of the duodenum --> bleeding from

Gastroduodenal Artery

199

Inflammatory Bowel Disease

Crohn's Disease or Ulcerative Colitis

200

Crohn's Disease
Etiology
Distribution
Location
Gross
XR

Disordered response to intestinal bacteria (Th1 mediated)
Any portion of the GI tract; usually terminal ileum and colon; rectal sparing
Skip Lesions
Transmural inflammation; Cobblestone mucosa, creeping Fat, linear ulcers, fissures, fistulas
Bowel wall thickening --> "string sign" on barrium swallow x ray

201

Crohn's disease
Histology
Complications

Noncaseating granulomas and lympohid aggregates (Th1 mediated)
Strictures, fistulas, perianal disease, malabsorption, nutritional depletion, colorectal cancer

202

Crohn's disease
Intestinal manifestations
Extraintestinal manifestations
Treatment

Diarrhea (w/ or w/o blood)
"Just got crushed by a stone --> red eyes, mouth hurts, and an aching back"
Migratory polyarthritis, erythema nodosum, ankylosing spondylitis, uveitis, kidney stones (Uric Acid from dehydration and CaOxylate from acidic urine), aphthous ulcers
Corticosteroids, azathioprine, methotrexate, infiximab, adalimumab

203

Crohn's Mnemonic

"Fat Granny, and old Crone Skipping down the Cobblestone road away from the Rec"

204

Ulcerative Colitis
Etiology
Location
Distribution
Gross

Autoimmune (Th2 mediated)
Colon; always rectal involvement
Continuous lesion
Friable mucosal pseudopolyps with freely hanging mesentery; Loss of haustra --> "lead pipe appearance"

205

Ulcerative Colitis
Histo
Cell mediating the reaction?
Complications

Mucosal and submucosal inflammation only, crypt abscesses and ulcers, bleeding, no granulomas (Th2 mediated)
Malnutrition, sclerosing cholangitis, toxic megacolon, colorectal carcinoma (worse with right sided colitis or pancolitis)

206

Ulcerative Colitis
Intestinal manifestations
Extraintestinal manifestations
Treatment

Bloody diarrhea
Pyoderma gangrenosum, Primary sclerosing cholangitis, ankylosing spondylitis, uveitis
ASA preparation (sulfasalazine), 6-mercaptopurine, infliximab, colectomy

207

Dx criteria for IBS

Recurrent abdominal pain with ≥2 of:
Pain improves with defecation
Change in stool frequency
Change in appearance of stool

208

IBS
Structural changes
Classic pt?
Timeline of symptoms
Presentation
Pathophysiology
Treatment

No structural changes
Most common in middle aged women
Chronic timeline
May present with diarrhea, constipation or alternating
Multifaceted pathophysiology
Treat the symptoms

209

Appendicitis
What is it?
What causes it?
Presentation
If perforates...
DDx
Treatment

Acute inflammation of the appendix
Obstruction by fecalith (adults) or lymphoid hyperplasia (children)
Initial diffuse periumbilical pain migrates to McBurney's point. Nausea. Fever.
If perforates --> peritonitis
DDx: Diverticulitis (elderly), ectopic pregnancy,
Treatment: appendectomy

210

McBurney's Point

1/3 the distance from ASIS to umbilicus

211

Diverticulum
What is it?
Most are...
Occurs most often in

Blind pouch protruding from alimentary tract that communicates with lumen of the gut
Most (esophagus, stomach, duodenum, colon) are acquired and are false (lack or have attenuated muscularis externa)
Occurs most often in sigmoid colon

212

Diverticulosis
What is it?
Frequency?
Caused by
Associated with
Most often located in

Many false diverticula
Common (~50% in pts >60)
Caused by Increased intraluminal pressure and focal weakness in colonic wall
Associated with low fiber diet
Most often located in sigmoid colon

213

True diverticulum

All 3 gut wall layers outpouch
Meckels

214

False diverticulum
AKA
Definition
Occur especially where...

Pseudodiverticulum
Only mucosa and submucosa outpouch
Occur especially where vasa recta perforate muscularis externa

215

Diverticulosis
Presentation
Common cause of
Complications

Often asymptomatic or associated with vague discomfort
Common cause of hematochezia
Can lead to diverticulitis and fistula

216

Diverticulitis
What is it?
Classic presentation
May lead to
If perforates
Treatment

Inflammation of diverticula
LLQ pain, fever, leukocytosis, Stool occult blood +/- hematochezia
May lead to colovesical fistula which would cause pneumaturia
Perforation --> peritonitis, abscess formation, or bowel stenosis
Treat with antibiotics

217

Zenker's Diverticulum
What kind?
Where is it?
Presentation

False diverticulum
At Killian's Triangle
Halitosis (due to trapped food particles), dysphagia, obstruction

218

Killian's Triangle

Between Thyropharyngeal and Cricopharyngeal parts of the inferior pharyngeal constrictor

219

Meckel's diverticulum
What kind?
Caused by?
May contain
Frequency
Presentation
What can it do to the GI tract?
Diagnostic test

"5 2s: 2in long, 2ft from ileocecal valve, 2% of pop, First 2 years of life, 2 types of tissue"
True diverticulum
Caused by persistence of the vitelline duct
May contain ectopic acid-secreting gastric mucosa +/or pancreatic tissue
Most common congenital anomaly of the GI tract
Melena, RLQ pain
Can cause intussusception, volvulus or obstruction near terminal ileum
Pertechnetate study for ectopic uptake

220

Omphalomesenteric cyst

Cystic dilation of the vitelline duct

221

Intussusception
What is it?
Common location
Presentation
Can lead to
Frequency and Cause
Urgency

Telescoping of 1 bowel segment into distal segment
Commonly at ileocecal junction
Currant jelly stools
Can compromise blood supply
Unusual in adults (intraluminal mass or tumor). Majority in children (idiopathic, adenovirus)
abdominal emergency in children

222

Volvus
What is it?
Can lead to
Common locations
What kind of pt?

Twisting of portion of bowel around its mesentery
Can lead to obstruction and infarction
May occur at cecum and sigmoid colon (redundant mesentery)
Usually in elderly pt

223

Hirschsprung's Disease
What is it?
PathoPhys
Presentation
Gross
Location
Risk Increases with
Diagnosed with
Treatment

Congenital megacolon
Failure of neural crest cells --> lack of ganglion cells/enteric nervous plexus (Auerback's + Meissner's)
Chronic constipation in early life. Failure to pass meconium
Grossly dilated portion of the colon proximal to the aganglionic segment, resulting in a transition zone
Involves rectum
Risk Increases with Down Syndrome
Diagnosed with suction biopsy
Treatment: resection

224

Source of Inferior and Superior Epigastric Arteries?

Inferior: External Iliac Artery
Superior: Internal Thoracic Artery

225

Meconium Ileus

Seen in CF
Meconium plug obstructs intestine preventing passage of stool at birth

226

Necrotizing enterocolitis
What is it?
Risk of...
Where is it usually?
Classic pt?

Necrosis of intestinal mucosa
Risk of perforation
Usually in colon but can involve entire tract
Neonates, more common in preemies

227

Ischemic colitis
What is it?
Presentation
Common location
Classic pt?

Reduction in intestinal blood flow --> ischemia
Pain out of proportion with physical findings. Pain after eating --> wt loss
Splenic flexure and distal colon
Elderly

228

Adhesion
What is it?
When does it happen?
Frequency?
Gross

Fibrous band of scar tissue
Commonly forms after surgery
Most common cause of small bowel obstruction
Well demarcated necrotic zones

229

Angiodysplasia
What is it?
Presentation
Common location
What kind of pt?
Diagnostic test

Tortuous dilation of vessels
Hematochezia
Terminal ileum, Cecum, Ascending Colon
Older pt.
Confirmed by angiography

230

Colonic Polyps
What are they?
Cancer?
Gross
Types Re Histology?

Masses protruding into gut lumen
90% are non-neoplastic
Sawtooth appearance
Tubular Adenoma: Small, rounded, more likely to be benign
Villous Adenoma: Long, finger like projections

231

Adenomatous Polyps
What are they?
Risk Increases w/...
Presentation

Precancerous polyps that are precursors to Colorectal cancer
Malignant risk associated with increased size, villous histology (villous = villainous), increased epithelial dysplasia
Often asymptomatic. Lower GI bleed, partial obstruction, secretory diarrhea

232

Hyperplastic Polyps
Frequency
Location

Most common non-neoplastic polyp in colon
Most (>50%) are in rectosigmoid colon

233

Juvenile Polyps
What are they?
Who gets them?
Where are they?
Malignant?

Sporadic lesion
Children <5
80% in rectum
Single: no malignant potential
Multiple: Increased risk for adenocarcinoma

234

Peutz-Jeghers
Genetics
Features
Presentation
Risk

Autosomal Dominant syndrome
Multiple non malignant harmatomas throughout GI tract
Hyperpigmented mouth, lips, hands and genitalia
Increased risk for colorectal cancer and other visceral malignancies

235

Colorectal Cancer
Frequency
Age of pts?
Genetics

3rd most common cancer; 3rd most deadly
Most pts are >50
~25% have family history

236

Genetic disorders leading to Colorectal Cancer

Familial Adenomatuous Polyposis (FAP)
Gardner's Syndrome
Turcot's Syndrome
Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome)

237

Familial Adenomatuous Polyposis
Dominance?
Mutation w/ chromosome
Risk of CRC
Gross?
Location

Autosomal dominant
Mutation of APC gene on chromosome 5q (2 hit hypothesis)
100% progress to CRC
Thousands of polyps; Pancolonic
Always involves rectum

238

Gardner's Syndrome

FAP + Osseous and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium

239

Turcot's Syndrome

"Turcot = Turban"
FAP + malignant CNS tumors

240

Lynch Syndrome
Dominance
Mutation
Risk of CRC
Location

Autosomal Dominant
Mutation in DNA mismatch repair genes
~80% progress to CRC
Proximal colon always involved

241

Risk factors for CRC?

IBD, tobacco, large villous adenomas, juvenile polyposis syndrome, Peutz Jeghers

242

Common locations for CRC?

Rectosigmoid > Ascending > Descending

243

Ascending CRC
Description
Presentation

Exophytic mass
Iron deficiency anemia, wt loss

244

Descending CRC
Description
What does it produce?
Presentation
Rarely, but can, present as...

Infiltrating mass
Produces partial obstruction
Colicky pain, hematochezia
Rarely, but can, present as Streptococcus bovis bacteremia

245

Diagnosis of CRC
What raises suspicion in a pt?
Screening: Initial and Recurrence
XR

Iron Deficiency Anemia in males >50 and postmenopausal females
Screen for pts >50 with colonoscopy or stool occult blood test
CEA tumor marker is a screen for recurrence
Apple core lesion on barium enema XR

246

What are they 2 molecular pathogenesises of CRC?

Microsatellite instability pathway (15%)
APC/beta catenin (chromosomal instability) pathway (85%)

247

Microsatellite instability pathway for CRC pathogenesis

DNA mismatch repair gene mutations --> sporadic CRC and Lynch Syndrome
Mutations accumulate but no defined morphologic correlates

248

Chromosomal instability pathway for CRC pathogenesis

"AK-53"
Normal Colon --> loss of APC gene --> decreased intracellular adhesion and increased proliferation --> Colon at risk --> K-RAS mutation --> unregulated intracellular signal transduction --> Adenoma --> Loss of p53 --> Increased tumorigensis --> Carcinoma

249

Carcinoid Tumor
What kind of tumor?
Frequency?
Location
EM
Produce
Presentation
Symptoms only observed if...
Treatment

NeuroEndocrine tumor
50% of small bowel tumors
Appendix, ileum, and rectum
EM: Dense core bodies
Produces 5HT
"CARC" --> Cutaneous flushing, Asthmatic wheezing, R heart murmurs, Cramps (diarrhea)
Symptoms only observed if metastases exist beyond liver because liver will degrade 5HT
Resection, Octreotide, Somatostatin

250

Liver Cirrhosis
Description
Gross
Histo
Increased risk for
Etiologies

Diffuse fibrosis w/ nodular regeneration --> destruction of normal architecture of liver
Macronodules
Regenerative nodules and bridging fibrosis
Increased risk for hepatocellular carcinoma
Alcohol (60-70%), viral hepattis, biliary disease, hemochromatosis

251

Presentation of Portal HTN

Esophageal varicies --> Hematemesis + melena
Peptic ulcers --> melena
Splenomegaly
Caput Medusae
Ascites
Gastropathy
Hemorrhoids

252

Presentation of Liver Cell Failure

Coma, Scleral icterus, Fetor Hepaticus, Spider Nevi, Gynecomastia, Jaundice, Testicular atrophy, Asterixis, Bleeding tendency, Anemia, Ankle Edema

253

Aminotransferases
Names
Marker for
Ratio Re Different Diseases

AST and ALT are Liver Enzymes
Marker for Liver Pathology
ALT > AST --> Viral Hepatitis
AST > ALT --> Alcoholic Hepatitis

254

Alkaline Phosphatase is a marker for

ALP
Hepatocellular carcinoma, bone disease, bile duct disease

255

Gamma Glutamyl Transpeptidase (GGT)

Elevated in liver and biliary disease but not in bone disease

256

Amylase is a marker for

Acute Pancreatitis or Mumps

257

Lipase is a marker for

Acute Pancreatitis

258

Ceruloplasmin is a marker for

Decreases in Wilson's Disease

259

Reyes Syndrome
What is it?
Frequency
Classic pt
Cellular Findings
Presentation
Mechanism

Fatal Childhood Hepatoencephalopathy
Rare
Child given aspirin for viral infection (especially VZV and Influenza B)
Mitochondrial abnormalities, Fatty Liver (microvesicular fatty change)
Hypoglycemia, vomiting, hepatomegaly, coma
Aspirin metabolites --> decreased beta oxidation by reversible inhibition of mitochondrial enzymes

260

Only time you can give child aspirin?

Kawasaki's Disease

261

Stages of Alcoholic Liver Disease

Steatosis --> Hepatitis --> Cirrhosis

262

Hepatic Steatosis
What is it?
Histo
Reversible?

Short term change in liver with moderate alcohol intake
Macrovesicular fatty change of hepatocytes
Reversible with cessation

263

Alcoholic Hepatitis
In order to develop, one needs...
Histology
AST/ALT

In order to develop, one needs sustained, long term consumption
Swollen, necrotic hepatocytes with neutrophilic infiltration. Mallory Bodies (intracytoplasmic eosinophilic inclusions)
AST > ALT (usually AST/ALT > 1.5) "make a toAST with alcohol!"

264

Alcoholic Cirrhosis
Reversible?
Gross
Histology
How does it manifest itself?

Irreversible
Micronodular, irregular, shrunken liver with "hobnail" appearance
Sclerosis around central vein (zone III)
Manifests as chronic liver disease (jaundice, hypoalbuminemia...)

265

Hepatocellular Carcinoma (Hepatoma)
Frequency
Increased risk w/...
Presentation
Serum markers?
How does it spread
May lead to...

Most common primary malignancy of the liver in adults
Increased risk w/ Hepatitis B and C, Wilson's, Hemochromatosis, Alpha 1 antitrypsin deficiency, Alcoholic cirrhosis and carcinogens (e.g. aflatoxin from Aspergillus)
Jaundice, Tender Hepatomegaly, Ascities, Polycythemia, Hypoglycemia
Increased Alpha Fetoprotein
Spreads hematogenously
May lead to Budd Chiari Syndrome

266

Cavernous hemangioma
Frequency
Danger
Typical pt
Biopsy?

Common, benign liver tumor
Age 30-50
Biopsy contraindicated because of risk of hemorrhage

267

Hepatic Adenoma
Danger
Related to what?
Prognosis

Benign liver tumor
Related to oral contraceptives or steroid use
Can spontaneously regress

268

Angiosarcoma
Danger?
Origin?
Associated with exposure to

Malignat tumor of endothelial origin
Associated with exposure to arsenic, polyvinyl chloride

269

Nutmeg Liver
What causes it?
Can result in?

Mottled appearance because of backup of blood into liver
R sided heart failure or Budd Chiari
Can result in centriolobular congestion and necrosis which will lead to cardiac cirrhosis

270

Budd Chiari Syndrome
What is it?
Presentation
Associations?

Occlusion of IVC or hepatic vein --> centriolobular congestion and necrosis --> congestive liver disease
Hepatomegaly, ascites, abdominal pain, varices, visible abdominal and back veins. No JVD
Associated with hypercoagulable state, polycythemia vera, pregnancy, hepatocellular carcinoma

271

Alpha 1 AntiTrypsin Deficiency
What is it?
Histo
Affects on other organs
Genetics

Misfolded gene products aggregate in hepatocellular ER --> cirrhosis
PAS + globules in liver
Panacinar emphysema because of decreased elastic tissue in lung
Codominant trait

272

Jaundice
What is it?
What literally causes it
What conditions or processes can lead to it?

Yellow skin +/or sclera resulting from elevated bilirubin
Caused by Direct hepatocellular injury, Obstruction of bile flow, Hemolysis

273

Hepatocellular Jaundice
Hyperbilirubinemia
Urine Bilirubin
Urine Urobilinogen

Direct/Indirect
Increased
Normal or Decreased

274

Obstructive Jaundice
Hyperbilirubinemia
Urine Bilirubin
Urine Urobilinogen

Direct
Increased
Decreased

275

Hemolytic Jaundice
Hyperbilirubinemia
Urine Bilirubin
Urine Urobilinogen

Indirect
Absent (acholuria)
Increased

276

Physiologic Neonatal Jaundice
What causes it
Treatment

Immature UDP Glucuronyl transferase --> unconjugated hyperbilirubinemia --> jaundice/kernicterus
Phototherapy (converts UCB to water soluble form)

277

Names of Hereditary Hyperbilirubinemias

Gilbert's Syndrome
Crigler-Najjar Syndrome Type 1
Dubin Johnson Syndrome

278

Gilbert Syndrome
What happens?
Presentation
Labs
Aggravation
Clinical Consequences?

Mildly decreased UDP-glucuronyl transferase --> decreased bilirubin uptake
Asymptomatic
Elevated unconjugated bilirubin w/o overt hemolysis
Bilirubin increases with fasting and stress
No clinical consequences

279

Crigler-Najjar Syndrome Type 1
What is it?
When does it present
Prognosis
Findings
Labs
Treatment

Absent UDP-glucuronyl transferase
Presents in early life
Patients die within a few years
Jaundice, kernicterus (bilirubin deposition in brain)
Increased unconjugated bilirubin
Plasmapheresis and phototherapy

280

Crigler Najjar Syndrome Type II
Severity
Treatment

Less severe form
Responds to phenobarbital which increases liver enzyme synthesis

281

Dubin Johnson Syndrome
What is it?
What is it due to?
Gross
Danger

Conjugated hyperbilirubinemia due to defective liver excretion
Grossly black liver
Benign

282

Rotor's Syndrome

Mild form of Dubin Johnson Syndrome that does not cause black liver

283

Wilson's Disease
Mnemonic
AKA
MoA
What accumulates where?
Characterized by...
Treatment
Genetics

"Copper is Hella BAD"
Hepatolenticular degeneration
Inadequate hepatic copper excretion and failure of copper to enter circulation as ceruloplasmin
Accumulates in Liver, Brain, Cornea, Kidneys, and Joints
Ceruloplasmin decreased, Cirrhosis, Corneal deposits (Kayser-Fleischer rings), Copper accumulation, Carcinoma (hepatocellular), Hemolytic anemia, Basal ganglia degeneration (parkinsonism), Asterixis, Dementia, Dyskinesia, Dysarthria
Penicillamine, Zn
Autsoomal recessive (chromosome 13)

284

How is copper normally excreted?

ATPase (ATP7B gene) transports copper from hepatocytes into bile

285

Hemochromatosis
Inheritance
What is it?
Presentation
Can result in
Etiology
Labs
Treatment

Autosomal recessive
Disease caused by iron deposition
Micronodular Cirrhosis, Diabetes Mellitus, Skin pigmentation (bronze diabetes)
Can result in CHF, testicular atrophy, hepatocellular carcinoma
Primary: autosomal recessive mutation of HFE gene (associated with HLA-A3)
Secondary: chronic transfusion therapy (e.g. beta thalassemia major)
Increased ferritin and iron. Decreased TIBC --> Increased transferrin saturation
Repeated phlebotomy, deferasirox, deferoxamine

286

Secondary Biliary Cirrhosis
PathoPhys
Presentation
Labs
Complication

Extrahepatic biliary obstruction (gallstone, biliary stricture, chronic pancreatitis, carcinoma of the pancreatic head) --> Increased pressure in intrahepatic ducts --> Injury, Fibrosis, and Bile Stasis
Pruritus, jaundice, dark urine, light stool, hepatosplenomegaly
Increased conjugated bilirubin, cholesterol, and ALP
Complicated by ascending cholangitis

287

Primary Biliary Cirrhosis
PathoPhys
Histo
Presentation
Labs
Associations

Autoimmune reaction --> lymphocytic infiltrate + granulomas
Pruritus, jaundice, dark urine, light stool, hepatosplenomegaly
Increased conjugated bilirubin, cholesterol, and ALP
Also increased serum mitochondrial Abs including IgM
Associated with other autoimmune diseases (CREST, RH, Celiac)

288

Primary Sclerosing Cholangitis
PathoPhys
Imaging
Presentation
Labs
Associations
What can in lead to?

Unknown cause of concentric "onion skinning" bile duct fibrosis --> alternating strictures and dilation with beading of intra and extrahepatic bile ductrs on ERCP
Pruritus, jaundice, dark urine, light stool, hepatosplenomegaly
Increased conjugated bilirubin, cholesterol, and ALP
Hypergammaglobinemia (IgM)
Associated with UC
Can lead to secondary biliary cirrhosis

289

Cholelithiasis
What causes them?
Risk factors?
Types

Increased cholesterol +/or bilirubin, Decreased bile salts, gallbladder stasis
4Fs: Fat, Female, Fertile (pregnant), and Forty
Cholesterol stones and Pigment stones

290

Cholesterol stones
Frequency
Imaging?
Associated with

80% of stones
Radiolucent (10-20% opaque due to calcification)
Associated with Obesity, Crohn's, CF, Age, Clofibrate, Estrogen, Multiparity, Rapid Wt Loss, Native American origin

291

Pigment Gallstones
Frequency
Imaging
Seen in patients with...
Color Re Etiology

20%
Radiopaque
Seen in patients with chronic hemolysis, alcoholic cirrhosis, advanced age, biliary infection
Black - hemolysis
Brown - infection

292

Cholelithiasis
What can it lead to?
Presentation?
When might the presentation be different?
Diagnosis
Treatment

Cholecystitis, Ascending Cholangitis, Acute Pancreatitis, Bile Stasis, Biliary Colic (neurohormonal activation like CCK trigger contraction of gallbladder which forces stone into cystic duct)
Charcot's Triad of Jaundice, Fever, RUQ Pain with a + Murphy's sign (Inspiratory arrest on deep RUQ palpation)
Painless in diabetics
Diagnose with US, Radionuclide Biliary Scan
Cholecystectomy

293

Possible Complication of Cholelithiasis
Image of this complication

Fistula between gallbladder and SI --> air in biliary tree
If gallstone obstructs ileocecal valve, air can be seen on the biliary tree on imaging

294

Cholecystitis
What is it?
What causes it?
Labs

Inflammation of gallbladder
Usually from gallstones but can be caused by ischemia or infection (CMV)
Increased ALP if bile duct becomes involved (ascending cholangitis)

295

Acute Pancreatitis
What is it?
What causes it?
Presentation
Labs

Autodigestion of pancreas by pancreatic enzymes
"GET SMASHED"
Gallstones, Ethanol, Trauma, Steroids, Mumps, Autoimmune, Scorpion sting, Hypercalcemia, Hypertriglyceridemia, ERCP, Drugs (Sulfa)
Epigastric pain radiating to the back, anorexia, nausea
Elevated amylase, lipase

296

Acute Pancreatitis
What can it lead to?
Complications

DIC, ARDS, Diffuse fat necrosis, Low Ca (collects in pancreatic soap deposits), pseudocysts, hemorrhage, infection, multiorgan failure
Pancreatic pseudocyst (lined by granulation tissue, not epithelium) which can rupture and hemorrhage

297

Chronic Pancreatitis
What is it?
What causes it?
What can it lead to?
Labs

Chronic inflammation, atrophy, and calcification of the pancreas
EtOH or idiopathic
Can lead to pancreatic insufficiency, DM, and adenocarcinoma
Amylase and Lipase are increased but less than in acute pancreatitis

298

Pancreatic Adenocarcinoma
Prognosis
Arises from
Serum markers
Risk factors
Presentation
Treatment

Poor prognosis
Arises from ducts in pancreatic head
C-19-9 and CEA (less specific)
Tobacco, Chronic Pancreatitis, Age (>50), Jewish or Black
Abdominal pain radiating to back, Weight loss (malabsorption or anorexia), Trousseau's Syndrome, Obstructive jaundice, Courvoisier's sign
Whipple procedure, Chemo, Radiation

299

Trousseau's Sign

Migrating thrombophlebitis --> redness and tenderness to palpation of extremities

300

Courvoisier's Sign

Palpable, nontender gallbladder

301

H2 Blockers
Names
MoA
Use
Tox

Cimetidine, ranitidine, famotidine, nizatidine
"Take H2 when you need a table for 2 before you DINE"
--/ H2 resulting in decreased secretion of H by parietal cells
Peptic ulcer disease, gastritis, mild esophageal reflux
C --/ P450 (multiple drug interactions) and anti androgenic (prolactin release, gynecomastia, impotence, decreased libido in males). Crosses BBB (leading to confusion, dizziness, headache) and placenta
R and C decrease renal excretion of Cr

302

PPI
Names
MoA
Use
Tox

Omeprazole, lansoprazole, esomeprazole, pantoprazole, dexlansoprazole
Irreversible inhibition of H/K ATPase in Parietal cells
Peptic ulcer disease, gastritis, esophageal reflux, Zollinger Ellison Syndrome
Increased risk for C diff and pneumonia. Hip fractures, decreased serum Mg with long term use

303

Bismuth, Sucralfate
MoA
Use

Binds to ulcer base, provides physical protection and allowing HCO3 secretion to reestablish pH gradient in mucous layer
Ulcer healing, travelers diarrhea

304

Misoprostol
MoA
Use
Tox

PGE analog --> increased production and secretion of gastric mucous barrier + decreased acid production
Prevention of NSAID-induced peptic ulcers; maintenance of ductus arteriosus; labor induction (ripens cervix)
Diarrhea, Abortifacient

305

Octreotide
MoA
Use
Tox

Somatostatin analog
Acute variceal bleeds, acromegaly, VIPoma, carcinoid tumor
Nausea, cramps, steatorrhea

306

Antacids
Names
Use
Tox

Aluminum Hydroxide, Magnesium Hydroxide, Calcium Carbonate
Can affect absorption, bioavailability, or urinary excretion or other drugs by altering gastric and urinary pH or delaying gastric emptying
All: HypoK
Al: Constipation (aluMINIMUM amount of feces), HypoPhosphatemia, proximal muscle weakness, osteodystrophy, seizures
Mg: Diarrhea (Must Go to Bathroom), Hypoflexia, Hypotension, Cardiac Arrest
Ca: HyperCa, Rebound acid increase, can chelate other drugs (tetracycline)

307

Osmotic Laxatives
Names
MoA
Use
Tox

Mg Hydroxide, Mg Citrate, Polyethylene Glycol, Lactulose
Osmotic load to draw out water
L can be used to treat hepatic encephalopathy since gut flora degreate it into metabolites (lactic acid and acetic acid) that promote nitrogen excretion as NH4
Use: Constipation
Tox: Diarrhea, dehydration, Abuse by bulimics

308

Inflximab
MoA
Use
Tox

Anti TNF Ab
CD, UC, RA
Infection (TB), fever, hypotension

309

Sulfasalazine
MoA
Activation
Use
Tox

Combination of sulfapyridine (antibacterial), 5-aminosalicylic acid (anti-inflammatory)
Activated by colonic bacteria
UC, CD
Malaise, nausea, Sulfonamide tox, reversible oligospermia

310

Ondansetron
MoA
Use
Tox

5HT antagonist = powerful central acting antiemetic "Keep on dancing"
Control vomiting post-op and during chemo
Headache, constipation

311

Metoclopramide
MoA
Use
Tox
Contraindications

D2 antagonist, Increases resting tone, contractility, LES tone, motility. Does not influence colon transit time
Diabetic and post-surgery gastroparesis, antiemetic
Increased Parkinsonian effect, restlessness, drowsiness, depression, nausea, diarrhea, Interaction with digoxin and diabetic agents.
Contraindicated in pts with small bowel obstruction and PD

312

Iron Transport, Storage and Regulation
Ferritin
Transferrin
Transferrin Receptor
Regulation

Ferritin stores Iron in cells (esp liver and kidney). Has IRE in 5' UTR
Transferrin transports iron in the blood. Has IRE in 3' UTR
Transferrin Receptor allows iron to enter cells
Regulated at level of translation
Low Fe --> IRP binds UTRs and F is blocked and T is stabilized
High Fe --> IRP cannot bind and F is translated and T is destabilized

313

Muscles That Make Up the Upper Esophageal Sphincter

Inferior Constrictor and Cricopharyngeus

314

Protein absorption in small intestine
What can move across apical surface? Via what transporters?
What can move across basolateral surface?

AA, dipeptides and tripeptides can move across apical surface.
AA with Na and dis and tris with H+
Only AA can move across basolateral surface

315

Electrolyte transport in Jejunum
Na?
H?
HCO3?

Na absorbed with sugars and AA and in exchange for H
Bicarb transported out of cell on basolateral surface into blood

316

Electrolyte transport in Ileum
Na?
H?
HCO3?
Cl?

Na absorbed with sugars and AA and in exchange for H
Bicarb transported out of cell on apical surface into lumen in exchange for Cl

317

Electrolyte transport in Colon epithelium
Na
K
Regulation

Na channels absorb Na
K channels secrete K
Aldosterone upregulates both channels and the Na/K ATPase

318

Electrolyte transport in Colon crypts
What happens net?
Basolateral transporters
Apical transporters
Regulation
Pathology

Net secretion
Na/K ATPase and NaK2Cl cotransporter basolaterally
Cl channel apically
VIP, ACh and other hormones --> cAMP --> Cl Channels
Cholera toxin --> α of G protein --> cAMP --> Cl channels inappropriately open --> Na and water follow

319

How does EtOH cause acute pancreatitis?

EtOH --> Ca --> trypsin

320

Hereditary Hemochromatosis Type 1 PathoPhys

HFE (chromosome 6) regulates hepcidin
Hepcidin is released by the liver when Iron is high to stop absorption of Fe (blocks Ferroportin channel which allows Fe to enter circulation)
In HHT1, Hepcidin is not produced