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STEP 1 Pathoma > Chapter 10 > Flashcards

Chapter 10 Flashcards

1
Q

What is Behcet Syndrome?

A

Syndrome of recurrent aphthous ulcers, genital ulcers, and uveitis due to immune complex vasculitis involving small vessels (can be seen after viral infection)

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2
Q

What is squamous cell carcinoma of the mouth?

A

a MALIGNANT neoplasm of squamous cells lining the oral mucosa

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3
Q

What are the major risk factors for squamous cell carcinoma of the mouth?

A

tobacco and alcohol (floor of the mouth is the most common location)

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4
Q

What are the major precursor lesions to squamous cell carcinoma of the mouth?

A

Oral leukoplakia (cannot be scraped away; distinct from oral candidiasis and hairy leukoplakia) and erythroplakia

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5
Q

What causes hairy leukoplakia?

A

Seen in immunocompromised pts (e.g. AIDs) and is due to EBV-induced squamous cell hyperplasia

not pre-malignant

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6
Q

Erythroplakia- vascularized leukoplakia and is highly suggestive of squamous cell dysplasia

A
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7
Q

What might be some complications of mumps?

A

Bilateral inflamed parotid glands

Orchitis (risk of sterility), pancreatitis, and aspetic meningitis may also occur

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8
Q

What is the most common cause of sialadenitis (inflammation of the salivary gland)?

A

Most commonly due to sialolithiasis leading to Staph aureus infection (usually unilateral)

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9
Q

Pleomorphic adenoma- composed of stromal and epithelial tissue (most common salivary tumor)

A

Usually arises in the parotid (mobile, painless mass)

Rarely may transform into carcinoma, which presents with signs of facial nerve damage

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10
Q

What is this?

A

Esopheal webbing (most often in the upper esophagus)

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11
Q

How does Esopheal webbing present?

A

Dysphagia for poorly chewed food (increased risk of esophageal squamoua cell carcinoma)

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12
Q

What is Plummer-Vinson Syndrome?

A

Syndrome of iron deficiency anemia, esophageal webbing, and beefy-red tongue due to atrophic glossitis

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13
Q

What is this?

A

Mallory Weiss Syndrome-longitudinal laceration of mucosa at the gastroesophageal junction caused by severe vomiting (usually due to alcoholism or bulimia) and presenting with painful hematemesis

Risk of Boerhaave syndrome (can cause emphysema)

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14
Q

What are esophageal varices?

A

Dilated submucosal veins in the lower esophagus arising secondary to portal HTN (distal veins normally drain into the portal vein via the left gastric vein and in portal HTN the left gastric vein backs up into the esophageal vein, resulting in dilation)

Most common cause of death in cirrhosis

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15
Q

What is achalasia?

A

Disordered esophageal motility with inability to relax the lower esophageal sphincter (LES) due to damaged ganglion cells in the MYENTERIC PLEXUS (common with Chagas disease, Trypanosoma cruzi)

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16
Q

How does achalasia present?

A

Dysphagia for food and solids

Putrid breath

Bird Beak sign on barium swallow study

Increased risk for esophageal squamous cell carcinoma

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17
Q

What are the types of esophageal carcinoma?

A

Adenocarcinoma and squamous cell carcinoma

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18
Q

Adenocarcinoma of the esophagus

A

-Arises from preexisting Barrett esophagus; usually involves the lower 1/3rd of the esophagus

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19
Q

Squamous cell carcinoma

A

Usually arises in upper or middle third of the esophagus

Risk factors include alcohol and tobacco, hot tea, achalasia, esophageal webbing, or lye ingestion

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20
Q

What lymph nodes does the upper 1/3 of the esophageal drain to?

A

Cervical nodes (think these for spread of squamous cell carcinoma)

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21
Q

What lymph nodes does the middle 1/3 of the esophageal drain to?

A

Mediastinal or tacheaobronchial nodes

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22
Q

What lymph nodes does the lower 1/3 of the esophageal drain to?

A

celiac and gastric nodes

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23
Q

What causes gastroschisis?

A

Congenital malformation of the anterio abdominal wall leading to exposure of abdominal contents

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24
Q

What causes omphalocele?

A

Failure of herniated intestines to return to the body during development (contents covered by peritoneum and amnion of the umbilical cord)

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25
Q

How does pyloric stenosis present?

A

This is a congenital hypertrophy of pyloric smooth muscle (more common in males) classically presents as:

Projectile NONBILIOUS vomit

Visible peristalsis

Olive-like abdominal mass

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26
Q
A

Myotomy

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27
Q

What is acute gastritis?

A

Acidic damage to the stomach mucosa due to imbalance between mucosal defenses (which include the mucin layer produced by foveolar cells, bicarb secretion by surface epithelium, and normal blood supply) and the acidic environment

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28
Q

What are the risk factors for acute gastritis?

A

Severe burn (Curling ulcer)-hypovolemia leads to decreased blood supply

NSAIDS

Heavy EtOH consumption

Chemotherapy

Increased intracranial pressure (Cushing ulcer)- increased stimulation of the vagus nerve leads to increased acid production

Shock

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29
Q

What are main types of chronic gastritis?

A

chronic autoimmune gastritis and chronic H. pylori gastritis

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30
Q

Describe chronic autoimmune gastritis

A

Due to autoimmune destruction of gastric parietal cells, which are located in the stomach body and fundus (type IV rxn)

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31
Q

What are the clinical features of chronic autoimmune gastritis?

A
  1. Atrophy of mucosa with intestinal metaplasia
  2. Aclorhydria with increased gastrin levels and antral G-cell hyperplasia
  3. Megaloblastic (perincious) anemia due to lack of intrinsic factor
  4. Increased risk for intestinal type gastri adenocarcinoma
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32
Q

Where does H. pylori most commonly infect?

A

the stomach ANTRUM

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33
Q

How does H. pylori cause infection?

A

It uses ureases and proteases along with inflammation to weaken mucosal defenses to cause epigastric abdominal pain and increased risk for ulceration

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34
Q

H. Pylori increases the risk for what diseases?

A

MALT lymphoma and intestinal type gastric adenocarcinoma

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35
Q

How is H. pylori tx?

A

Triple therapy with a confirmation negative urea breath test and lack of stool antigen to confirm eradication

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36
Q

What is peptic ulcer disease?

A

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

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37
Q

Duodenal ulcers are almost ALWAYS due to ______

A

H. pylori (95+%) and rarely due to ZE syndrome

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38
Q

How might a duodenal ulcer present?

A

Epigastric pain that improves with meals and diagnostic endoscopic biopsy shows ulcer with hypertrophy of Brunner glands

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39
Q

A major complication of duodenal ulcers is _________

A

If they arise in the posterioe duodenum (usually anterior), they can rupture leading to bleeds from the gastroduodenal artery

40
Q

Gastric ulcers are most commonly due to _________

A

H. pylori (75%), NSAIDs and bile reflux

41
Q

How might a gastric ulcer present?

A

Epigastric pain that worsens with meals (ulcer is usually along the lesser curvature of the antrum)

NOTE: Rupture carries risk of bleeding from the left gastric artery

42
Q

Keep in mind to always keep carcinoma on the Ddx for ulcers

A

Duodenal ulcers are almost nerve malignany but gastric ulcers can be caused by gastric carcinoma (intestinal subtype) so note these differences:

benign ulcers tend to be small (<3cm), and sharply demarcated, while malignany ulcers tend to be large and irregular. Biopsy required

43
Q

What are the types of gastric carcinoma (malignant proliferation of the surface epithelial cells-adenocarcinoma)?

A

Diffuse type and intestinal

44
Q

Describe intestinal type gastric carcinoma (more common)

A

Presents as large, irrgular ulcers with heaper up margins, most commonly along the lesser curvature of the antrum (similar to gastric ulcers)

45
Q

What are the main risk factors for intestinal type gastric carcinoma?

A

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

46
Q

Describe diffuse type gastric carcinoma

A

Marked by SIGNET ring cells (front) that diffusely infiltrate the gastric wall where desmoplasia results in thickening of the stomach wall to produce linitis plastica

Note that diffuse type gastric carcinoma is NOT associated with H. pylori, intestinal metaplasia, or nitrosamines

47
Q

How might gastric carcinoma present?

A

Presents late with weight loss, abdominal pain, anemia, and early satiety

rarely presents as acenthosis nigricans or Leser-Trelet sign

48
Q

What is Leser-Trelet sign?

A

The sign of Leser-Trélat may be more precisely defined as the abrupt appearance of multiple seborrheic keratoses caused by an associated cancer and the rapid increase in their size and number. This definition is parallel to that of malignant acanthosis nigricans; both conditions are obligate paraneoplastic syndromes

49
Q

Spread of gastric cancer is classically to where?

A

the left supraclavicular node (Virchow node)

periumbilical region (Sister Mary Joseph nodule)- seen with intestinal type

Bilateral ovaries (Krukenberg tumor)- seen with diffuse type

most commonly to the liver

50
Q

Duodenal atresia, or failure of the dudenum to canalize, is associated with ___________

A

Down syndrome

51
Q

What are the clinical features of Duodenal atresia?

A

Polyhydramnioes

Distension of the stomach and blind-looped duodenum (‘double bubble sign)

BILIOUS vomiting

52
Q

What is Meckel diverticulum?

A

An outpouching of all three layers of the bowel wall (true diverticulum) arising due to failure of the vitelline duct to involute

Seen in 2% of population, 2” long, and within 2 ft of the ileocecal valve

53
Q

How might a Meckel diverticulum present?

A

Within the first 2 yrs of life with bleeding due to heterotrophic gastric mucosa, volvulus, intussusception, or obstruction mimicing appendicitis (most cases are asymtpomatic though)

54
Q

What are the most common locations for volvuls (twisting of the bowel causing obstruction)?

A

Sigmoid colon in elderly and cecum in young adults

55
Q

What is the most common cause intussusception in children?

A

lymphoid hyperplasia (e.g. due to rotavirus), which usually arisies in the terminal ileum, leading to intersusception into the ccum

In adults, the most common cause is tumor

56
Q

Note that the small bowel is HIGHLY susceptible to ischemic injury and some causes include transmural infarction with thrombosis/embolism of the SMQ or thrombosis of the mesenteric vein

Will present with abdominal pain, bloody diarrhea, and decreased bowel sounds

A
57
Q

Describe lactose intolerance

A

Decreased function of the lactase enzyme found in the brush border of enterocytes (lactose normally breaks down to glucose and galactose) causing a presentation of abdominal distenstion with diarrhea upon diary consumption

58
Q

What is Celiac disease?

A

Immune-mediated damage of the small bowel villi due to gluten exposure (associated with HLA-DQ2/8)

59
Q

What is gluten?

A

Present in wheat and grains (its most pathogenic component is gliadin)

Once absorbed gliadin is deamidated by tissue transglutaminase (tTG) and is then presented via MHC II to helper T cells

60
Q

How does celiac disease present?

A

Children classically present with abdominal distension, diarrhea, and failure to thrive, while adults present with chronic diarrhea and bloating

61
Q

What is a classic association with Celiac Disease?

A

Dermatitis herpeiformis- small, herpes like vesicles due to IgA depositoon at the tips of dermal papillae (resolves with gluten free diet)

62
Q

What are the lab findings for celiac disease?

A

IgA ABs agaiant endomysium, tTG, or gliadin; IgG Abs are also present and are useful for diagnosis individuals with IgA deficiency

Duodenal biopsy reveals flattening of villi, crypt hyperplasia, and increased intraepithelial lymphocytes with damage mostly centered in the duodenum

63
Q

Complications of Celiac disease?

A

Swall bowel carcinoma and T-cell lymphoma

64
Q

What is tropical sprue?

A

Damage to the SMALL BOWEL villi due to an unknown organism resulting in malabsorption which is similar to celiac disease except:

  1. Occurs in tropical regions
  2. Arises after infectious diarrhea and responds to ABX
  3. Damage mostly involves the JEJUNUM and ILEUM with complications of secondary vtiB12 deficiency
65
Q

What is Whipple Disease?

A

Systemic tissue damage marked by macrophages loaded with Tropheryma whippelii organisms (positive for PAS)

66
Q

Where does Whipple disease classically affect most?

A

Small bowel lamina propria where macrophages compress lacteals and chylomicrons cannot be transferred from enterocytes to lymphatics causing fat malabsorption and steatorrhea

other sites include the synovium of joints (arthritis), cardiac valves, lymph nodes, and the CNS

67
Q

What is abetalioproteinemia?

A

AR deficiency of apolipoprotein B-48 and B-100 causing malabsorption due to defective chylomcicron formation (requires B48) and absent plasma VLDL and LDL (require B100)

68
Q

What is a carcinoid tumor?

A

A malignant proliferation of neuroendocrine cells (low-grade malignacy)- positive for chromogranin

69
Q

What is the most common site of a carcinoid tumor?

A

Can arise anywhere along the gut, but the small bowel is the most common site where it goes as a submucosal polyp-like nodule

70
Q

Carcinoid tumors often secrete ______

A

Serotonin which is released in blood and metabolized by the liver MAO into 5-HIAA which is excreted in urine

NOTE: METS to the liver allows serotonin to bypass liver metabolism, which is then released into the hepatic vein into systemic circulation resulting in CARCINOID SYNDROME

71
Q

How might carcinoid syndrome present?

A

Bronchospasm, diarrhea, and flushing of skin

72
Q

How might carcinoid heart disease present?

A

RIGHT-SIDED valvular fibrosis (increased collagen) leading to tricuspid regurg and pulmonary valve stenosis (left sided lesions are not seen due to the presence of MAO in the lung)

73
Q

Describe Hirschsprung Disease

A

Defective relaxation and peristalsis of the rectum and distal sigmoid colon (associated with Down syndrome) due to congenital failure of ganglion cells (neural crest derived) to descend into myenteric and submucosal plexus

Tx involves resection of the involved bowel

74
Q

Describe Ulcerative Colitis

A

Wall involvement: Mucosal and submucosal ulcers

Location: Begins in rectum and can extend proximally up to the cecum (involvement is CONTINUOUS)

Symptoms: Left lower quadrant pain With bloody diarrhea

75
Q

More on Ulcerative Colitis

A

Inflammation: Crypt abscesses with neutrophils

Gross appearance: Psudeopolyps with loss of hastrua (Lead pipe appearance)

Complications: Toxic megacolon and carcinoma (generally only a concern after 10 yrs)

Smoking: protects against UC

76
Q

Describe Crohn Disease

A

Wall Involvement: Full-thickness inflammation with knife-like fissures

Location: Anywhere from mouth to anus with skip lesions; terminal ileum is the most common site, rectum is least common

Symptoms: Right lower quadrant pain (ileum) with NON-bloody diarrhea

77
Q

More on Crohn Disease

A

Inflammation: lymphoid aggregates with GRANULOMAS

Gross appearance: Cobblestone mucosa, creeping fat, and structures (string sign on imaging)

Complications: Malabsorption with nutritional deficiency, calcium oxlate nephrolithiasis, fistula formation, and carcinoma

Smoking: Increased risk for CD

78
Q

What are some possible extraintestinal manifestations of CD or UC?

A

Arthritis, uveitis, erythema nodosum, pyoderma grangreosum, p-ANCA, and primary sclerosing cholangitis

79
Q

Describe colonic diverticula

A

Outpouchings of mucosa and submucosa through the muscularis propria (false diverticulum) related to wall stress and arising where the vasa recta traverse the muscularis propria (sigmoid colon most common)

Usually asymptomatic but can present with heamtochezia, diverticulitis like symptoms

80
Q

What is angiodysplasia?

A

An acquired malformation of mucosal and submucosal cpaillary beds usually arising in the cecum and right colon due to high wall tension

Rupture classically presents as hematochezia in an older adult

81
Q

What is hereditary hemorrhagic telangiectasia?

A

AD disease resulting in thin-walled blood vessels, esp. in the mouth and GI tract

82
Q

What is ischemic colitis?

A

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

Atherosclerosis of the SMA is the most common cause

Presents with postprandial pain and weight loss; infarction results in pain and bloody diarrhea

83
Q

What are the most common types of colonic polyps?

A

Hyperplastic and adenomatous

84
Q

Describe Hyperplastic polyps

A

Due to hyperplasia of glands, classically showing a serrated appearance on microscopy and usually arising in the left colon (rectosigmoid)- benign with no malignant potential

85
Q

What is the adenoma-carcinoma sequence describing the molecular progression from normal colonic mucosa to adenomatous poylp to carcinoma?

A
  1. APC mutations
  2. Kras mutation leads to formation of polyp
  3. p53 mutation and increased expression of COX allow for proression carcinoma (aspirin impedes progression)
86
Q

What is Familial adenomatous polyposis (FAP)?

A

AD disorder of 100s-1000s of adenomatous colonic polyps due to inherited APC gene mutation on chromosome 5

Tx: Colon and rectum removed prophyalctically

87
Q

What is Gardner syndrome?

A

FAP + fibromatosis and osteomas

88
Q

What is Turcot syndrome?

A

FAP + CNS tumors (medulloblastoma and glial tumors)

89
Q

What is a juvenile polyp?

A

Sporadic, hamartomatous (benign) polyp that arises in children under 5 yo and usually presents as a solitary rectal polyp that prolapses and blleds

90
Q

What is Peutz-Jeghers Syndrome?

A

hamartomatous (benign) polyps throughout the GI tract and mucocutaneous hyperpigmentation (freckle-like spots) on lips, oral mucosa, and genital skin

Increased risk for colorectal, breast, and urinary cancers

91
Q

CRC most commonly arises from what?

A

The adenoma-carcinoma sequence but other cause include microsatellite instability (MSI)

92
Q

Describe Hereditary Nonpolyposis colorectal carcinoma (HNPCC)?

A

Due to inhertied mutations in DNA mismatch repair enzymes

93
Q

Colonic carcinoma is associated with an increased risk of _________ endocarditis

A

Strep bovis

94
Q

Staging of CRC

A

T- depth of invasionl tumors limited to mucosa generally do NOT spread due to lack of lymphatics in the mucosa

N- spread to regional nodes

M- most commonly to the liver

95
Q

_____ is a serum tumor marker that is useful for assessing tx response and detecting recurrence (not useful for screening)

A

CEA

STEP 1 Pathoma (18 decks)

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