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USMLE STEP 1 > GI > Flashcards

GI Flashcards

1
Q

Foregut develops into

A

Pharynx to duodenum

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2
Q

Midgut develops into…

A

duodenum to transverse colon

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3
Q

Hindgut develops into…

A

Distal transverse colon to the rectum

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4
Q

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

A

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

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5
Q 
Duodenal atresia 
What is it?
Genetics?
Presentation 
XR
A

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

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6
Q

Jejunal, Ilial, or Colonic Atresia

What causes them?

A

Vascular accident (apple peel atresia)

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7
Q

Timing of midgut development

A

6th week: Midgut Herniates through umbilical ring

10th week: Returns to abdominal cavity and rotates around SMA

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8
Q

Gastroschisis
What is it?
Peritoneum?

A

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

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9
Q

Omphalocele
What is it?
Peritoneum?

A

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

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10
Q

Most common Tracheoesophageal anomaly?

A

Esophageal atresia with distal tracheoesophageal fistula (85%)

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11
Q 
Esophageal atresia with distal tracheoesophageal fistula
Presentation
XR
Cyanosis?
Clinical test?
A

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

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12
Q

H type Tracheoesophageal anomaly

A

Fistula alone

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13
Q

CXR in pure atresia type Tracheoesophageal anomaly?

A

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

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14
Q 
Congenital Pyloric Stenosis
What causes it?
Presentation?
Physical exam?
Treatment
Occurrence?
More often in...
A 
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
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15
Q

Pancreas Derived from

A

Foregut

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16
Q

Ventral Pancreatic bud contributes to

A

Pancreatic head and main pancreatic duct

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17
Q

Uncinate process of pancreas formed by the

A

Ventral bud alone

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18
Q

Dorsal pancreatic bud becomes

A

Body, tail, isthmus and accessory pancreatic duct

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19
Q

Annular Pancreas
What is it?
What may it cause?

A

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

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20
Q

Pancreas divisum

A

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

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21
Q

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

A

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

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22
Q

Do retroperitoneal structures have a mesentery?

A

No

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23
Q

Injuries to retroperitoneal structures can cause

A

Blood or gas accumulation in the retroperitoneal space

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24
Q

List of Retroperitoneal Structures

A 
"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)
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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
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``` 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
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Lactase deficiency diagnosis?
Administration of lactose produces symptoms and glucose rises < 20mg/dL
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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
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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
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``` 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)
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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)
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Curling's Ulcer
↓ plasma volume --> sloughing of gastric mucosa
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Cushing's ulcer
↑ vagal stimulation --> ↑ ACh --> ↑ H production
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Acute vs Chronic Gastritis
Erosive vs Non-Erosive
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``` 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
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``` 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
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Type A vs B Chronic Gastritis
"ABA, BAB" A is in Body and from Autoimmunity B is in Antrum and from Bacteria
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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
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Stomach Cancer: Diffuse Associated with? Histology Gross
Not associated with H pylori Signet ring cells Stomach wall grossly thickened and leathery (linitis plastica)
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Virchow's Node
Involvement of Left Supraclavicular Node by metastasis from stomach
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Krukenberg's Tumor Presents with Histology
Bilateral metastases from stomach to ovaries Presents with abundant mucus Signet ring cells
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Sister Mary Joseph's Nodule
Subcutaneous periumbilical metastasis
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``` 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
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``` 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 ```
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``` 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 ```
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Ulcer Complications
Hemorrhage or Perforation
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Location of Hemorrhage from Ulcer
Gastric or Duodenal Ulcers | Posterior > Anterior
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Location of Perforation from Ulcer
Duodenal | Anterior > Posterior
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Ruptured gastric ulcer on the lesser curvature --> bleeding from
Left Gastric Artery
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Ulcer on posterior wall of the duodenum --> bleeding from
Gastroduodenal Artery
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Inflammatory Bowel Disease
Crohn's Disease or Ulcerative Colitis
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``` 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
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Crohn's disease Histology Complications
Noncaseating granulomas and lympohid aggregates (Th1 mediated) Strictures, fistulas, perianal disease, malabsorption, nutritional depletion, colorectal cancer
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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
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Crohn's Mnemonic
"Fat Granny, and old Crone Skipping down the Cobblestone road away from the Rec"
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``` 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"
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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)
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Ulcerative Colitis Intestinal manifestations Extraintestinal manifestations Treatment
Bloody diarrhea Pyoderma gangrenosum, Primary sclerosing cholangitis, ankylosing spondylitis, uveitis ASA preparation (sulfasalazine), 6-mercaptopurine, infliximab, colectomy
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Dx criteria for IBS
Recurrent abdominal pain with ≥2 of: Pain improves with defecation Change in stool frequency Change in appearance of stool
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``` 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 ```
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``` 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
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McBurney's Point
1/3 the distance from ASIS to umbilicus
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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
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``` 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 ```
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True diverticulum
All 3 gut wall layers outpouch | Meckels
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False diverticulum AKA Definition Occur especially where...
Pseudodiverticulum Only mucosa and submucosa outpouch Occur especially where vasa recta perforate muscularis externa
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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
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Zenker's Diverticulum What kind? Where is it? Presentation
False diverticulum At Killian's Triangle Halitosis (due to trapped food particles), dysphagia, obstruction
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Killian's Triangle
Between Thyropharyngeal and Cricopharyngeal parts of the inferior pharyngeal constrictor
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``` 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
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Omphalomesenteric cyst
Cystic dilation of the vitelline duct
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``` 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
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``` 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
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``` 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
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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
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``` 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
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``` 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
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``` 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 ```
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``` 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
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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
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Hyperplastic Polyps Frequency Location
Most common non-neoplastic polyp in colon | Most (>50%) are in rectosigmoid colon
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``` 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 ```
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``` 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
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Colorectal Cancer Frequency Age of pts? Genetics
3rd most common cancer; 3rd most deadly Most pts are >50 ~25% have family history
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Genetic disorders leading to Colorectal Cancer
Familial Adenomatuous Polyposis (FAP) Gardner's Syndrome Turcot's Syndrome Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome)
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``` 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 ```
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Gardner's Syndrome
FAP + Osseous and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium
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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
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Risk factors for CRC?
IBD, tobacco, large villous adenomas, juvenile polyposis syndrome, Peutz Jeghers
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Common locations for CRC?
Rectosigmoid > Ascending > Descending
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Ascending CRC Description Presentation
Exophytic mass | Iron deficiency anemia, wt loss
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``` 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
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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
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What are they 2 molecular pathogenesises of CRC?
Microsatellite instability pathway (15%) | APC/beta catenin (chromosomal instability) pathway (85%)
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Microsatellite instability pathway for CRC pathogenesis
DNA mismatch repair gene mutations --> sporadic CRC and Lynch Syndrome Mutations accumulate but no defined morphologic correlates
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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
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``` 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
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Presentation of Portal HTN
``` Esophageal varicies --> Hematemesis + melena Peptic ulcers --> melena Splenomegaly Caput Medusae Ascites Gastropathy Hemorrhoids ```
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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
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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
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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
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Only time you can give child aspirin?
Kawasaki's Disease
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Stages of Alcoholic Liver Disease
Steatosis --> Hepatitis --> Cirrhosis
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Hepatic Steatosis What is it? Histo Reversible?
Short term change in liver with moderate alcohol intake Macrovesicular fatty change of hepatocytes Reversible with cessation
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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!"
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``` 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...)
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``` 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
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Hepatic Adenoma Danger Related to what? Prognosis
Benign liver tumor Related to oral contraceptives or steroid use Can spontaneously regress
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Angiosarcoma Danger? Origin? Associated with exposure to
Malignat tumor of endothelial origin | Associated with exposure to arsenic, polyvinyl chloride
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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
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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
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``` 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
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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
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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
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Physiologic Neonatal Jaundice What causes it Treatment
Immature UDP Glucuronyl transferase --> unconjugated hyperbilirubinemia --> jaundice/kernicterus Phototherapy (converts UCB to water soluble form)
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Names of Hereditary Hyperbilirubinemias
Gilbert's Syndrome Crigler-Najjar Syndrome Type 1 Dubin Johnson Syndrome
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``` 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 ```
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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
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Rotor's Syndrome
Mild form of Dubin Johnson Syndrome that does not cause black liver
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``` 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

USMLE STEP 1 (25 decks)

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