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Flashcards in CH10 - Gastrointestinal Pathology Deck (345)
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1

What are some diseases that affect the oral cavity?

1) cleft up and palate 2) aphthous ulcer 3) Behcet syndrome 4) Oral herpes 5) squamous cell carcinoma

2

What is cleft up and palate?

Full-thickness defect of lip or palate

3

What is cleft up and palate due to?

failure of facial prominences to fuse

4

How is face formed?

During early pregnancy, facial prominences (one from superior, two from the sides, and two from inferior) grow and fuse together to form the face

5

Does the cleft lip and palate usually occur together or separate?

Usually together, isolated cleft lip or palate is less common.

6

What is aphthous ulcer?

Painful, superficial ulceration of the oral mucosa

7

When does aphthous ulcer arise?

in relation to stress and resolves spontaneously, but often recurs

8

What is aphthous ulcer characterized by?

a grayish base surrounded by erythema

9

What is behcet syndrome?

Recurrent aphthous ulcers, genital ulcers, and uveitis

10

What is behcet syndrome due to?

immune complex vasculitis involving small vessels

11

When might you see behcet syndrome?

after viral infection, but etiology is unknown

12

What is oral herpes?

Vesicles involving oral mucosa that rupture, resulting in shallow, painful, red ulcers

13

What is oral herpes usually due to?

HSV-1

14

For oral herpes when does the primary infection occur?

in childhood; lesions heal, but virus remains dormant in ganglia of the trigeminal nerve.

15

For oral herpes what might cause reactivation of the virus?

Stress and sunlight, leading to vesicles that often arise on the lips (cold sore)

16

What is squamous cell carcinoma of the oral cavity?

Malignant neoplasm of squamous cells lining the oral mucosa

17

For oral squamous cell carcinoma what are the major risk factors?

Tobacco and alcohol

18

What is the most common location for oral squamous cell carcinoma?

Floor of mouth

19

Regarding oral squamous cell carcinoma what are the precursor lesions

Oral leukoplakia and erythroplakia

20

What is leukoplakia?

It is a white plaque that cannot be scraped away; often represents squamous cell dysplasia

21

What is oral candidiasis?

it is a white deposit on the tongue, which is easily scraped away; usually seen in immunocompromised states

22

What is hairy leukoplakia?

it is a white, rough (hairy) patch that arises on the lateral tongue. It is usually seen in immunocompromised individuals (AIDS) and is due to EBV-induced squamous cell hyperplasia; not pre-malignant

23

What is erythroplakia?

(red plaque) represents vascularized leukoplakia and is highly suggestive of squamous cell dysplasia,

24

Would you biopsy erythroplakia and leukoplakia?

they are often biopsied to rule out carcinoma

25

What are salivary glands?

they are exocrine glands that secrete saliva.

26

What are salivary glands divided into?

major (parotid, submandibular, and sublingual glands and minor glands (hundreds of microscopic glands distributed throughout the oral mucosa)

27

What is the mumps?

Infection with mumps virus resulting in bilateral inflamed parotid glands

28

In mumps, what other infections in addition to the mumps virus may be present?

Orchitis, pancreatitis, and aseptic meningitis may also be present.

29

Why is serum amylase increased?

due to salivary gland or pancreatic involvement

30

What is there a risk of with orchitis?

carries risk of sterility, especially in teenagers.

31

What is sialadenitis?

Inflammation of the salivary gland

32

What is sialadenitis most commonly due to?

an obstructing stone (sialolithiasis) leading to Staphylococcus aureus infection; usually unilateral

33

What is pleomorphic adenoma?

Benign tumor composed of stromal (e.g., cartilage) and epithelial tissue;

34

What is the most common tumor of the salivary gland?

Pleomorphic adenoma

35

Where does pleomorphic adenoma usually arise?

in parotid

36

What does pleomorphic adenoma present as?

a mobile, painless, circumscribed mass at the angle of the jaw

37

What is the rate of recurrence in pleomorphic adenoma?

High rate of recurrence;

38

In pleomorphic adenoma what often leads to incomplete resection?

extension of small islands of tumor through tumor capsule

39

What does pleomorphic adenoma present as?

Rarely may transform into carcinoma, which presents with signs of facial nerve damage (facial nerve runs through parotid gland)

40

What is a warthin tumor?

Benign cystic tumor with abundant lymphocytes and germinal centers (lymph node-like stroma);

41

What is the 2nd most common tumor of the salivary gland?

Warthin tumor

42

Where deos the warthin tumor almost always arise?

in the parotid

43

What is a mucoepidermoid carcinoma?

Malignant tumor composed of mucinous and squamous cells

44

What is the most common malignant tumor of the salivary gland?

Mucoepidermoid carcinoma

45

Where does mucoepidermoid carcinoma usually arise?

in the parotid

46

What does mucoepidermoid carcinoma commonly involve?

the facial nerve

47

What is a tracheoesophageal fistula?

Congenital detect resulting in a connection between the esophagus and trachea

48

What is the most common variant of tracheoesophageal fistula?

consists of proximal esophageal atresia with the distal esophagus arising from the trachea

49

How does a tracheoesophageal fistula present?

with vomiting, polyhydramnios, abdominal distension, and aspiration

50

What is esophageal web?

Thin protrusion of esophageal mucosa, most often in the upper esophagus

51

What does esophageal web present with?

dysphagia for poorly chewed food

52

What is there an increased risk for with esophageal web?

esophageal squamous cell carcinoma

53

What is Plummer-Vinson syndrome characterized by?

severe iron deficiency anemia, esophageal web, and beefy-red tongue due to atrophic glossitis.

54

What is zenker diverticulum?

An outpouching of pharyngeal mucosa through an acquired defect in the muscular wall (false diverticulum)

55

Where does the zenker diverticulum arise?

above the upper esophageal sphincter at the junction of the esophagus and pharynx

56

What does Zenker Diverticulum present with?

dysphagia, obstruction, and halitosis (bad breath)

57

What is Mallory weiss syndrome?

Longitudinal laceration of mucosa at the gastroesophageal (GE) junction

58

What is Mallory weiss syndrome caused by?

severe vomiting, usually due to alcoholism or bulimia

59

What does Mallory weiss syndrome present with?

painful hematemesis

60

With Mallory weiss syndrome, what is there a risk of?

Boerhaave syndrome

61

What is Boerhaave syndrome?

rupture of esophagus leading to air in the mediastinum and subcutaneous emphysema

62

What is esophageal varices?

Dilated submucosal veins in the lower esophagus

63

What does esophageal varices arise with?

It arises secondary to portal hypertension

64

What does the distal esophageal vein normally drain into?

the portal vein via the left gastric vein

65

What is seen in portal hypertension?

the left gastric vein backs up into the esophageal vein, resulting in dilation (varices)

66

What are the symptoms for esophageal varices?

Asymptomatic, but risk of rupture exists

67

What does esophageal varices present with?

painless hematemesis

68

What is the most common cause of death in cirrhosis?

Esophageal varices

69

What is achalasia?

Disordered esophageal motility with inability to relax the lower esophageal sphincter (LES)

70

What is achalasia due to?

damaged ganglion cells in the myenteric plexus

71

Where are the ganglion cells of the myenteric plexus located?

between the inner circular and outer longitudinal layers of the muscularis propria

72

What are the ganglion cells of the myenteric plexus important for?

regulating bowel motility and relaxing the LES.

73

What causes damage to ganglion cells [of the myenteric plexus]?

can be idiopathic or secondary to a known insult (e.g., Trypanosoma cruzi infection in Chagas disease)

74

What are the clinical features for achalasia?

1. Dysphagia for solids and liquids 2. Putrid breath 3. High LES pressure on esophageal manometry 4. Bird-beak sign on barium swallow study 5. Increased risk for esophageal squamous cell carcinoma

75

What is there an increased risk for with achalsia?

Esophageal squamous cell carcinoma

76

What is GERD?

Gastroesophageal reflux disease - Reflux of acid from the stomach due to reduced LES tone

77

What are the risk factors for GERD?

include alcohol, tobacco, obesity, fat-rich diet, caffeine, and hiatal hernia.

78

What are the clinical features for GERD?

1. Heartburn (mimics cardiac chest pain) 2. Asthma (adult-onset) and cough 3. Damage to enamel of teeth 4. Ulceration with stricture and Barrett esophagus are late complications.

79

What is barrett esophagus?

Metaplasia of the lower esophageal mucosa from stratified squamous epithelium to nonciliated columnar epithelium with goblet cells seen in

80

What % of patients with GERD present with Barrett Esophagus?

10%

81

Barrett Esophagus is a response of what to what stimuli?

Response of lower esophageal stem cells to acidic stress

82

What might Barrett Esophagus progress to?

May progress to dysplasia and adenocarcinoma

83

What is esophageal carcinoma?

Subclassified as adenocarcinoma or squamous cell carcinoma

84

What is adenocarcinoma?

a malignant proliferation of glands;

85

What is the most common type of esophageal carcinoma in the West?

Adenocarcinoma

86

From what does adenocarcinoma arise?

from preexisting Barrett esophagus; usually involves the lower one-third of the esophagus

87

What is squamous cell carcinoma?

it is a malignant proliferation of squamous cells

88

What is the most common esophageal cancer worldwide?

Squamous cell carcinoma

89

Where does squamous cell carcinoma usually arise?

in upper or middle third of the esophagus;

90

What are the major risk factors for squamous cell carcinoma?

1) Alcohol and tobacco (most common causes) 2) Very hot tea 3). Achalasia 4) Esophageal web (e.g., Plummer-Vinson syndrome) 5) Esophageal injury (e.g., lye ingestion)

91

What is the prognosis for esophageal carcinoma?

Poor prognosis due to late presentation

92

What are the symptoms for esophageal carcinoma?

progressive dysphagia (solids to liquids), weight loss, pain, and hematemesis.

93

In addition to the symptoms for esophageal carcinoma what may squamous cell carcinoma additionally present with?

hoarse voice (recurrent laryngeal nerve involvement) and cough (tracheal involvement).

94

In esophageal carcinoma what determines the location of lymph node spread?

it depends on the level of the esophagus that is involved

95

For esophageal carcinoma involvement of the upper 1/3 of the esophagus results in what lymph node spread?

cervical nodes

96

For esophageal carcinoma involvement of the middle 1/3 of the esophagus results in what lymph node spread?

mediastinal or tracheobronchial nodes

97

For esophageal carcinoma involvement of the lower 1/3 of the esophagus results in what lymph node spread?

celiac and gastric nodes

98

What is gastroschisis?

congenital malformation of the anterior abdominal wall leading to exposure of abdominal contents

99

What is omphalocele?

Persistent herniation of bowel into umbilical cord

100

What is omphalocele due to?

failure of herniated intestines to return to the body cavity during development, the contents are covered by peritoneum and amnion of the umbilical cord

101

What is pyloric stenosis?

congenital hypertrophy of pyloric smooth muscle; more common in males

102

How does pyloric stenosis classically present?

two weeks after birth as; 1. Projectile nonbilious vomiting 2. Visible peristalsis 3. Olive-like mass in the abdomen

103

What is the treatment for pyloric stenosis?

myotomy

104

What is acute gastritis?

Acidic damage to the stomach mucosa

105

What is acute gastritis due to?

imbalance between mucosal defenses and acidic environment

106

What do the defenses of acute gastritis include?

mucin layer produced by foveolar cells, bicarbonate secretion by surface epithelium, and normal blood supply (provides nutrients and picks up leaked acid).

107

How is a severe burn a risk factor for acute gastritis?

(Curling ulcer) Hypovolemia leads to decreased blood supply.

108

How are NSAIDs a risk factor for acute gastritis?

decreased PGE

109

How is increased intracranial pressure a risk factor for acute gastritis?

(Cushing ulcer) Increased stimulation of vagus nerve leads to increased acid production

110

What is the relationship between shock and acute gastritis?

Multiple (stress) ulcers may be seen in ICU patients

111

What are the risk factors for acute gastritis?

1. Severe burn 2. NSAIDs 3. Heavy alcohol consumption 4. Chemotherapy 5. Increased intracranial pressure 6. Shock.

112

In acute gastritis what does acid damage result in?

superficial inflammation, erosion (loss of superficial epithelium), or ulcer (loss of mucosal layer)

113

What is chronic gastritis?

Chronic Inflammation of stomach mucosa

114

What is chronic gastritis divided into?

two types based on underlying etiology: chronic autoimmune gastritis and chronic H pylori gastritis

115

What is chronic autoimmune gastritis due to?

autoimmune destruction of gastric parietal cells

116

Where are the gastric parietal cells located?

in the stomach body and fundus.

117

What is chronic autoimmune gastritis associated with?

antibodies against parietal cells and/or intrinsic factor; useful for diagnosis, but pathogenesis is mediated by T cells (type IV hypersensitivity)

118

What are the clinical features for chronic autoimmune gastritis?

1) Atrophy of mucosa with intestinal metaplasia 2) Achlorhydria with increased gastrin levels and antral G-cell hyperplasia 3) Megaloblastic (pernicious) anemia due to lack of intrinsic factor 4) Increased risk tor gastric adenocarcinoma (intestinal type)

119

What is chronic H pylori gastritis due to?

H pylori-induced acute and chronic inflammation

120

What is the most common form of gastritis?

(90%) chronic H pylori gastritis

121

How does H pylori lead to gastritis?

H pylori ureases and proteases along with inflammation weaken mucosal defenses; antrum is the most common site

122

How does H. Pylori gastritis present?

with epigastric abdominal pain; increased risk for ulceration (peptic ulcer disease), gastric adenocarcinoma (intestinal type), and MALT lymphoma

123

What is the treatment for H Pylori gastritis?

involves triple therapy.

124

What is triple therapy?

Proton pump inhibitors and antibiotics

125

What does triple therapy treatment for H pylori gastritis result in?

1) Resolves gastritis/ulcer and reverses intestinal metaplasia 2) Negative urea breath test and lack of stool antigen confirm eradication of H pylori.

126

What is peptic ulcer disease?

Solitary mucosal ulcer involving proximal duodenum (90%) or distal stomach (10%)

127

What is the duodenal ulcer due to?

almost always due to H pylori (> 95%); rarely, may be due to Zollinger Ellison (ZE) syndrome

128

What does a duodenal ulcer present with?

epigastric pain that improves with meals

129

What does biopsy of a duodenal ulcer show?

Diagnostic endoscopic biopsy shows ulcer with hypertrophy of Brunner glands.

130

What might happen to a duodenal ulcer?

May rupture leading to bleeding from the gastroduodenal artery (anterior ulcer) or acute pancreatitis (posterior ulcer)

131

What is gastric ulcer usually due to?

H pylori (75%); other causes include NSAlDs and bile reflux.

132

What doses gastric ulcer present with?

epigastric pain that worsens with meals

133

Where is the gastric ulcer usually located?

on the lesser curvature of the antrum.

134

What does rupture of gastric ulcer result in?

carries a risk of bleeding from left gastric artery

135

What is the differential diagnosis of ulcers?

includes carcinoma.

136

Might duodenal ulcers lead to carcinoma?

Duodenal ulcers are almost never malignant (duodenal carcinoma is extremely rare).

137

Is there a relationship between gastric ulcers and carcinoma?

Gastric ulcers can be caused by gastric carcinoma (intestinal subtype)

138

How do benign peptic ulcers usually appear?

small (< 3 cm), sharply demarcated (punched-out), and surrounded by radiating folds of mucosa

139

How do malignant ulcers appear?

they are large and irregular with heaped up margins

140

What is required for definitive diagnosis regarding gastric ulcers?

biopsy

141

What is gastric carcinoma?

Malignant proliferation of surface epithelial cells (adenocarcinoma)

142

How is gastric carcinoma subclassified?

into intestinal and diffuse types

143

Which subclassification of gastric carcinoma is more common?

Intestinal type

144

How does the intestinal type of gastric carcinoma present?

presents as a large, irregular ulcer with heaped up margins;

145

What does the intestinal type of gastric carcinoma most commonly?

involves the lesser curvature of the antrum

146

How is gastric carcinoma similar to gastric ulcer?

They both involve the lesser curvature of the antrum

147

What are risk factor for the intestinal type of gastric carcinoma?

they include intestinal metaplasia (e.g., due to H pylori and autoimmune gastritis), nitrosamines in smoked foods (Japan), and blood type A.

148

How is the diffuse type of gastric carcinoma characterized?

by signet ring cells that diffusely infiltrate the gastric wall, desmoplasia resulting in thickening of stomach wall (linitis plasties)

149

What is the diffuse type of gastric carcinoma not associated with

its not associated with H pylori, intestinal metaplasia, or nitrosamines

150

What does gastric carcinoma present with?

It presents late with weight loss, abdominal pain, anemia, and early satiety; rarely presents as acanthosis nigricans or Leser-Trelat sign

151

What might gastric carcinoma spread to?

lymph nodes can involve the left supraclavicular node (Virchow node).

152

For gastric carcinoma what does distant metastasis most commonly involve?

Liver

153

For the intestinal type of gastric carcinoma what does distant metastasis involve?

Periumbilical region (Sister Mary Joseph nodule)

154

For the diffuse type of gastric carcinoma what does distant metastasis involve?

Bilateral ovaries (Krukenberg tumor)

155

What is duodenal atresia?

Congenital failure of duodenum to canalize;

156

What is duodenal atresia associated with?

Down syndrome

157

What are the clinical features for duodenal atresia?

1. Polyhydramnios 2. Distension of stomach and blind loop of duodenum (double bubble) 3. Bilious vomiting

158

What is meckel diverticulum?

Outpouching of all three layers of the bowel wall (true diverticulum)

159

Why does meckel diverticulum arise?

due to failure of the vitelline duct to involute

160

For meckel diverticulum what is the rule of 2s?

1) Seen in 2% of the population 2) 2 inches long and located in the small bowel within 2 feet of the ileocecal valve 3) during the first 2 years of life with bleeding, volvulus, intussusception, or obstruction (mimics appendicitis); however, most cases are asymptomatic

161

What is the most common congenital anomaly of the GI tract?

Meckel diverticulum

162

How long is the meckel diverticulum?

2 inches long

163

Where is the meckel diverticulum located?

in the small bowel within 2 feet of the ileocecal valve

164

How can meckel diverticulum present?

during the first 2 years of life with bleeding, volvulus, intussusception, or obstruction (mimics appendicitis); however, most cases are asymptomatic

165

Why is there bleeding during the presentation of meckel diverticulum?

Its due to heterotopic gastric mucosa

166

What is volvulus?

Twisting of bowel along its mesentery

167

What does volvulus result in?

obstruction and disruption of the blood supply with infarction

168

What are the most common locations for a volvulus?

sigmoid colon in the elderly and cecum in young adults

169

What is intussusception?

Telescoping of proximal segment of bowel forward into distal segment

170

How does intussusception work?

Telescoped segment is pulled forward by peristalsis, resulting in obstruction and disruption of blood supply with infarction.

171

What is intussusception associated with?

a leading edge (focus of traction)

172

What is the most common cause of intussusception in children?

lymphoid hyperplasia (e.g., due to rotavirus) which usually arises in the terminal ileum, leading to intussusception into the cecum

173

What is the most common cause of intussusception in adults?

tumor.

174

What is small bowel infarction?

Small bowel is highly susceptible to ischemic injury.

175

When does transmural infarction occur?

with thrombosis/embolism of the superior mesenteric artery or thrombosis of the mesenteric vein.

176

When does mucosal infarction occur?

with marked hypotension

177

What are the clinical features for small bowel infarction?

they include abdominal pain, bloody diarrhea, and decreased bowel sounds,

178

What is lactose intolerance?

Decreased function of the lactase enzyme found in the brush border of enterocytes

179

What is the normal function of lactase?

It normally breaks down lactose into glucose and galactose.

180

How does lactose intolerance present?

with abdominal distension and diarrhea upon consumption of milk products; undigested lactose is osmotically active.

181

How does lactose deficiency develop?

it may be congenital (rare autosomal recessive disorder) or acquired (often develops in late childhood);

182

How is lactose deficiency related to small bowel infarction?

temporary deficiency is seen after small bowel infection (lactase is highly susceptible to injury).

183

What is celiac disease?

Immune-mediated damage of small bowel villi due to gluten exposure

184

What is celiac disease associated with?

HLA-DQ2 and DQ8

185

In what is gluten present?

in wheat and grains

186

What is the most pathogenic component of gluten?

gliadin

187

What happens to gliadin once absorbed?

gliadin is deamidated by tissue transglutaminase (tTG).

188

What happens to deamidated gliadin?

it is presented by antigen presenting cells via MHC class II where the helper T cells mediate tissue damage.

189

What is the clinical presentation for celiac disease in herpes?

Children classically present with abdominal distension, diarrhea, and failure to thrive

190

What is the clinical presentation for celiac disease in adults?

It classically presents with chronic diarrhea and bloating

191

What is seen on the skin in celiac disease?

Small, herpes-like vesicles may arise on skin (dermatitis herpetiformis).

192

What is dermatitis herpetiformis in celiac diseae due to and how does it resolve?

IgA deposition at the lips of dermal papillae it resolves with gluten-free diet

193

What are the laboratory findings for celiac disease?

1) IgA antibodies against endomysium, tTG (tissue transglutaminase), or gliadin

194

What lab finding for celiac disease is also used in IgA deficiency?

IgG antibodies are also present and are useful for diagnosis in individuals with IgA deficiency

195

What is the relationship between IgA deficiency and celiac disease?

increased incidence of IgA deficiency is seen in celiac disease

196

What does duodenal biopsy reveal in celiac disease?

flattening of villi, hyperplasia of crypts, and increased intraepithelial lymphocyte

197

In celiac disease where is damage most prominent?

in the duodenum (jejunum and ileum are less involved)

198

When would the symptoms of celiac disease resolve?

with gluten-free diet.

199

What are late complications that present as refractory disease despite good dietary control?

Small bowel carcinoma and T-cell lymphoma

200

What is tropical sprue?

Damage to small bowel villi due to an unknown organism resulting in malabsorption

201

How does tropical spure compare to celiac disease?

Its similar to celiac disease except 1. Occurs in tropical regions (e.g., Caribbean) 2. Arises after infectious diarrhea and responds to antibiotics

202

In tropical spure, where is damage most prominent?

in jejunum and ileum (secondary vitamin B12 or folate deficiency may ensue); duodenum is less commonly involved.

203

What is whipple disease?

Systemic tissue damage characterized by macrophages loaded with Tropheryma whippelii organisms; partially destroyed organisms are present in macrophage lysosomes (positive for PAS).

204

What is the classic site of involvement in whipple disease?

the small bowel lamina propria

205

What happens in whipple disease?

1. Macrophages compress lacteals. 2. Chylomicrons cannot be transferred from enterocytes to lymphatics. 3. Results in fat malabsorption and steatorrhea

206

What are the other common sites of involvement for whipple disease?

they include synovium of joints (arthritis), cardiac valves, lymph nodes, and CNS.

207

What is abetalipoproteinemia?

Autosomal recessive deficiency of apolipoprotein B-48 and B-100

208

What are the clinical features for abetalipoproteinemia?

Malabsorption due to defective chylomicron formation (requires B-48), Absent plasma VLDL and LDL (requires B-100)

209

What is carcinoid tumor?

Malignant proliferation of neuroendocrine cells; low-grade malignancy

210

What do the tumor cells in carcinoid tumor contain?

neurosecretory granules that are positive for chromogranin

211

Where can carcinoid tumor cells arise?

Can arise anywhere along the gut; small bowel is the most common site

212

What does carcinoid tumor grow as?

It grows as a submucosal polyp-like nodule

213

What is the relation between carcinoid tumor and serotonin?

The neurosecretory granules often secrete serotonin

214

What is serotonin released into?

the portal circulation

215

Where is serotonin metabolized?

Its metabolized by liver monoamine oxidase (MAO) into 5-HIAA (5-hydroxyindoleacetic acid)

216

What happens to 5-HIAA?

it is excreted in the urine.

217

What does metastasis of carcinoid tumor to the liver result in?

it allows serotonin to bypass liver metabolism.

218

What is carcinoid syndrome / heart disease?

Serotonin is released into the hepatic vein and leaks into systemic circulation via hepato-systemic shunts, resulting in carcinoid syndrome and carcinoid heart disease.

219

What is carcinoid syndrome characterized by?

bronchospasm, diarrhea, and Hushing of skin;

220

What might trigger symptoms of carcinoid syndrome?

it can be triggered by alcohol or emotional stress, which stimulate serotonin release from the tumor,

221

What characterizes carcinoid heart disease?

It is characterized by right-sided valvular fibrosis

222

In carcinoid heart disease why is there right sided valvular fibrosis?

increased collagen

223

What does the right sided valvular fibrosis seen in carcinoid heart disease lead to?

tricuspid regurgitation and pulmonary valve stenosis

224

In carcinoid heart diseae are there left-sided valvular lesions?

No, they are not seen due to presence of monoamine oxidase which metabolizes serotonin in the lung,

225

What is acute appendicitis?

Acute inflammation of the appendix; most common cause of acute abdomen

226

What is the most common cause of acute abdomen?

Acute appendicitis

227

What is acute appendicitis related to?

obstruction of the appendix by lymphoid hyperplasia (children) or a tecalith (adults)

228

What is McBurney point?

It is where the pain for acute appendicitis localizes to lower right quadrant

229

What are the clinical features in acute appendicitis?

1. Periumbilical pain, fever, and nausea; pain eventually localizes to right lower quadrant (McBurney point). 2. Rupture results in peritonitis that presents with guarding and rebound tenderness.

230

What is a common complication for acute appendicitis?

Periappendiceal abscess is a common complication

231

What is inflammatory bowel disease?

Chronic, relapsing inflammation of bowel

232

What is inflammatory bowel disease possibly due to?

abnormal immune response to enteric flora

233

How does inflammatory bowel disease classically present?

in young women (teens to 30s) as recurrent bouts of bloody diarrhea and abdominal pain

234

Where is inflammatory bowel disease more prevalent?

in the West, particularly in Caucasians and Eastern European Jews

235

How do you diagnose inflammatory bowel disease?

It?s a diagnosis of exclusion whose symptoms mimic other causes of bowel inflammation

236

What is inflammatory bowel disease subclassified as?

ulcerative colitis or Crohn?s disease

237

What is the wall involvement for ulcerative colitis?

Mucosal or submucosal ulcers

238

Where does ulcerative colitis begin?

In the rectum

239

What does ulcerative colitis involve after the rectum?

It can extend proximally up to the cecum, the remainder of the GI tract is unaffected

240

What are the symptoms of ulcerative colitis?

Left lower quadrant pain (rectum) with bloody diarrhea

241

What is involved in inflammation with ulcerative colitis?

Crypt abscesses with neutrophils

242

What is the gross appearance of ulcerative colitis?

Pseudopolyps, loss of haustra ? lead pipe sign on imaging

243

What are the complications for ulcerative colitis?

Toxic megacolon and carcinoma

244

For ulcerative colitis what is the risk based on?

Its based on extent of colonic involvement and duration of disease (generally not a concern until >10 years of disease)

245

What are the associations for ulcerative colitis?

Primary sclerosing cholangitis and p-ANCA positivity

246

What is the effect of smoking on ulcerative colitis?

Smoking protects against it

247

What is the wall involvement for crohns disease?

Full thickness inflammation with knife like fissures

248

What is the location for Chrons disease?

Anywhere from mouth to anus with skip lesions

249

What is the most common and least common site for chrons disease?

Most common is the terminal ileum and the least common is the rectum

250

What are the symptoms for chron?s disease?

Right lower quadrant pain (ileum) with non bloody diarrhea

251

What is involved with the inflammation in Crohn?s disease?

Lymphoid aggregates with granulomas (40% of cases)

252

What is the gross appearance of Crohn Disease?

Cobblestone mucosa, creeping fat, and strictures (string sign on imaging)

253

What are the complications for Crohns Disease?

Malabsorption with nutritional deficiency, calcium oxalate nephrolithiasis, fistula formation, and carcinoma if colonic disease is present

254

What are the associations for Crohns disease?

Ankylosing spondylitis, sacroiliitis, migratory polyarthritis, erythema nodosum and uveitis

255

What effect does smoking have on Crohns disease?

It increases the risk for developing Crohns disease

256

What is hirschsprung disease?

Defective relaxation and peristalsis of rectum and distal sigmoid colon

257

What is hirschsprung disease associated with?

Down syndrome

258

What is hirschsprung disease due to?

congenital failure of ganglion cells which are neural crest-derived, to descend into myenteric and submucosal plexus

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Where is the myenteric (Auerbach) plexus located?

between the inner circular and outer longitudinal muscle layers of the muscularis propria and regulates motility

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Where is the submucosal (Meissner) plexus located?

in the submucosa and regulates blood flow, secretions, and absorption.

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What are the clinical features for hirschsprung disease based on?

Obstruction

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What are the clinical features for hirschsprung disease?

1. Failure to pass meconium 2. Empty rectal vault on digital rectal exam 3. Massive dilatation (megacolon) of bowel proximal to obstruction with risk for rupture

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In hirschsprung disease what does rectal suction biopsy reveal?

lack of ganglion cells.

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What is the treatment for hirschsprung disease?

involves resection of the involved bowel; ganglion cells are present in the bowel proximal to the diseased segment.

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What is colonic diverticula?

Outpouchings of mucosa and submucosa through the muscularis propria (false diverticulum)

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What is colonic diverticula related to?

wall stress

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What is colonic diverticula associated with?

constipation, straining, and low-fiber diet; commonly seen in older adults (risk increases with age)

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From where does the colonic diverticula arise?

where the vasa recta traverse the muscularis propria (weak point in colonic wall)

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What is the most common location for colonic diverticula?

sigmoid colon

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What are usually the symptoms for colonic diverticula?

Its usually asymptomatic

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What are the complications for colonic diverticula?

1. Rectal bleeding (hematochezia) 2. Diverticulitis 3. Fistula

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What is diverticulitis due to?

obstructing fecal material

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How does diverticulitis present?

with appendicitis-like symptoms in the left lower quadrant

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What is the relationship between colonic diverticula and a fistula?

Inflamed diverticulum ruptures and attaches to a local structure.

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What does the colovesicular fistula present with?

air (or stool) in urine

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What is angiodysplasia?

Acquired malformation of mucosal and submucosal capillary beds

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How does angiodysplasia usually arise?

in the cecum and right colon due to high wall tension

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How does rupture in angiodysplasia classically present?

as hematochezia in an older adult.

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What is hereditary hemorrhagic telangiectasia?

Autosomal dominant disorder resulting in thin-walled blood vessels, especially in the mouth and GI tract

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What does rupture in hereditary hemorrhagic telangiectasia present as?

presents as bleeding.

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What is ischemic colitis?

Ischemic damage to the colon, usually at the splenic flexure, watershed area of superior mesenteric artery (SMA)

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What does ischemic cholitis present with?

postprandial pain and weight loss; infarction results in pain and bloody diarrhea.

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What is irritable bowel syndrome?

Relapsing abdominal pain with bloating, flatulence, and change in bowel habits (diarrhea or constipation) that improves with defecation

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What is irritable bowel syndrome classically seen in?

middleaged females

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What is irritable bowel syndrome related to?

disturbed intestinal motility; no identifiable pathologic changes

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What may improve the symptoms of irritable bowel syndrome?

Increased dietary fiber may improve symptoms.

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What are colonic polyps?

Raised protrusions of colonic mucosa

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What are the most common types of colonic polyps?

hyperplastic and adenomatous polyps

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What are hyperplastic polyps due to?

hyperplasia of glands; classically show a serrated appearance on microscopy

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What is the most common type of polyp and where does it usually arise?

Hyperplastic polyps and it usually arises in the left colon (rectosigmoid)

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Can hyperplastic polyps result in cancer?

No its benign, with no malignant potential

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What are adenomatous polyps due to?

neoplastic proliferation of glands

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What is the 2nd most common type of colonic polyp?

Adenomatous polyps

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Can andenomatous polyps lead to cancer?

Benign, but premalignant, it may progress to adenocarcinoma via the adenoma-carcinoma sequence

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What is the adenoma-carcinoma sequence?

it describes the molecular progression from normal colonic mucosa to adenomatous polyp to carcinoma.

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What is APC?

APC (adenomatous polyposis coli gene)

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What do mutations in the APC lead to?

either sporadic or germline mutations increase risk for the formation of polyp.

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Mutations of what leads to increased risk for polyp progression to carcinoma?

APC, k-ras and p53

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How does k-ras mutation affect polyps?

leads to formation of polyp

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What is the effect of p53 mutation and polyps?

p53 mutation and increased expression of COX allow for progression to carcinoma; aspirin impedes progression from adenoma to carcinoma.

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How is screening for polyps performed?

by colonoscopy and testing for fecal occult blood

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How do polyps present clinically?

they are usually clinically silent, but can bleed.

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What is the goal in term of polyps?

it is to remove adenomatous polyps before progression to carcinoma

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On colonoscopy can you tell the difference between hyperplastic and adenomatous polyps?

No they look identical. Hence, all polyps are removed and examined microscopically

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With polyps what is the risk for progression from adenoma to carcinoma related to?

size > 2 cm, sessile growth, and villous histology.

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What is familial adenomatous polyposis?

(FAP)Autosomal dominant disorder characterized by 100s to 1000s of adenomatous colonic polyps

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What is familial adenomatous polyposis (FAP) due to?

inherited APC mutation (chromosome 5); increases propensity to develop adenomatous polyps throughout colon and rectum

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How is familial adenomatous polyposis treated?

Colon and rectum are removed prophylacticly ; otherwise, almost all patients develop carcinoma by 40 years of age.

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What is gardner syndrome?

it is FAP with fibromatosis and osteomas

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What is fibromatosis?

it is a non-neoplastic proliferation of fibroblasts

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Where does fibromatosis arise?

in retroperitoneum (desmoid) and locally destroys tissue

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What is Osteoma?

it is a benign tumor of bone that usually arises in the skull

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What is Turcot syndrome?

it is FAP with CNS tumors (medulloblastoma and glial tumors)

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What is a juvenile polyp?

Sporadic, hamartomatous (benign) polyp that arises in children (< 5 years)

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How does juvenile polyp usually present?

as a solitary rectal polyp that prolapses and bleeds

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What is juvenile polyposis characterized by?

multiple juvenile polyps in the stomach and colon; large numbers of juvenile polyps increase the risk of progression to carcinoma.

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What is peutz-jeghers syndrome?

Hamartomatous (benign) polyp throughout GI tract and mucocutaneous hyperpigmentation (freckle-like spots) on lips, oral mucosa, and genital skin; autosomal dominant disorder

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In Peutz-Jeghers Syndrome what is there an increased risk for?

colorectal, breast, and gynecologic cancer

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What is colorectoal carcinoma?

Carcinoma arising from colonic or rectal mucosa

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What is the 3rd most common site of cancer?

Colorectal carcinoma

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What is the 3rd most common cause of cancer-related death?

Colorectal carcinoma

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What is the peak incidence for colorectal carcinoma?

it is 60-70 years of age

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What does colorectal carcinoma most commonly arise from?

adenoma-carcinoma sequence

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For colorectal carcinoma what is a second important molecular pathway?

the microsatellite instability (MSI) pathway

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What are microsatellites in the MSI pathway?

they are repeating sequences of noncoding DNA; integrity of the sequence (stability) is maintained during cell division.

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What does instability in the microsatellite instability pathway indicate?

defective DNA copy mechanisms (eg. DNA mismatch repair enzymes).

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What is HNPCC

Hereditary non polyposis colorectal carcinoma

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What is HNPCC due to?

Inherited mutations in DNA mismatch repair enzymes.

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With HNPCC what is there an increased risk for?

colorectal, ovarian, and endometrial carcinoma

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Where does colorectal carcinoma arise?

de novo (not from adenomatous polyps) at a relatively early age; usually right-sided

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How does the screening for colorectal carcinoma occur?

via endoscopy and fecal occult blood testing

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When does screening for colorectal carcinoma begin?

at 50 years of age

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What is the goal for screening for colorectal carcinoma?

To remove adenomatous polyps before carcinoma develops and to detect cancer early (before clinical symptoms arise).

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In colorectal carcinoma where can carcinoma develop?

anywhere along entire the length of the colon

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In colorectal carcinoma how does left-sided carcinoma usually grow?

as a napkin-ring lesion;

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In colorectal carcinoma how does left-sided carcinoma present?

With decreased stool caliber, left lower quadrant pain, and blood-streaked stool

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In colorectal carcinoma how does right-sided carcinoma usually grow?

as a raised lesion;

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In colorectal carcinoma how does right-sided carcinoma usually present?

with iron deficiency anemia (occult bleeding) and vague pain. All older adults with iron deficiency anemia has colorectal carcinoma until proven otherwise.

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What infection is colonic carcinoma associated with?

an increased risk for Streptococcus bovis endocarditis.

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What is the T stage for colorectal carcinoma based on?

depth of invasion;

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Do tumors limited to the mucosa generally spread?

No they do not spread due to lack of lymphatics in the mucosa

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What does the N stage for colorectal carcinoma represent?

spread to regional lymph nodes

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What does the M stage for colorectal carcinoma represent?

distant spread

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What does the M stage for colorectal carcinoma most commonly involve?

the liver

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What is CEA?

it is a serum tumor marker that is useful for assessing treatment response and detecting recurrence; not useful for screening