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93 Cards in this Set

  • Front
  • Back
Intestinal Atresia
complete occlusion of lumen
could be from:
-fibrous diaphragm
- complete fibrosis
- complete seperation
Intestinal Stenosis
narrowing of lumen

less common
heterotopoc gastric mucosa
disccrete small nodules of gastric mucosa
- may cause obstruction, ulceration, bleeding, perforation, intussuception, pain
gastric metaplasia
involves surface epithelium only

associated with H. pylori infection and is NOT congenital
Hirschsprung's Disease
aganglionosis of a portion of the intestinal tract

The rectum is always affected with involvement of proximal colon (short segment) or entire colon (long segment).
Colon undergoes dilation and hypertrophy (Megacolon)
Clinical Features of Hirschsprung's disease
starts in neonatal period by failure to pass meconium and obstructive constipation

can lead to enterocolitis, fluid and electrolyte imbalances, and perforation leading to peritonitis
Incidence of Hirschsprung's disease
1 in 5000 births
increased frequency among siblings
predominant in males (short segment)
females have long segment more commonly

10% of cases also have Down Syndrome
Necrotizing Enterocolitis
occurs mainly in premature infants
is multifactorial but C. Difficile plays a role
intenstinal ischemia is a prerequisite condition
PAF increases mucosa permeability - now bacteria can enter gut tissue
Clinical course of NEC
bloody stools
abdominal distension
circulatory collapse
pneumatosis intestinalis - Xray showing gas in intestinal wall

involves cecum, ileum, and right colon

may become gangrenous and cause peritonitis
may culminate into sepsis and shock
usually require removal of necrotic segment - may develop post-NEC strictures
Secretory Diarrhea
more than 500 ml of fluid stool per day
isotonic with plasma
commonly caused by viral damage to mucosal epithelium

Traveller's diarrhea - ETEC, Vibrio
Laxative abuses
Pancreatic cholera syndrome - excessive VIP
Carcinoma of Thyroid - excessive calcitonin
Zollinger-Ellison syndrome
Secreting VIllius Adenoma of Rectum - Prostaglandins
Small Intestine Obstruction
Portal Hypertension w/ Sever Hypoalbuminemia
Giardiasis, Strongyloides, Amebiasis
Osmotic Diarrhea
stool has osmotic anion gap and is more than 500 ml

Inability to metabolize lactose
overuse of antacids
bile malabsorption
Exudative Diarrhea
mucosal destruction leading to bloody, purulent stools, that are continued on fasting

usually bacterial
Shigella
Salmonella
Campylobacter
E. Histolytica
Idiopathic Inflammation in Gut
Typhoid diseases in immuno suppressed
Malabsorption Diarrhea
Voluminous bulky stools, increased osmolarity, excess of fat, that is better on fasting

mucosal cell abnormalities
reduced small intestinal surface area
lymphatic obstruction
Giardiasis infection
Impaired Motility
surgical reduction of gut length
neural dysfunction - IBS
hyperthyroidism
Diabetic Neuropathy
Syndrome from Cancer

small intestinal diverticula
bacterial overgrowth of SI
Clinical Evaluation of Diarrhea
History and Physical
Stool Exam
- stain for pus
- stain for fat (chronic - pancreatic insufficiency, acute - giardia/travellers diarrhea)
- check for blood
- check for eggs or parasites
- measure volume
Infectious Enterocolitis
acute - self limiting diarrhea
major cause of morbidity in children

usually viral, but also bacterial

bacterial infections - damage to surface epithelium, PMN infiltrate

Shigella and Salmonella
Antibiotic Associated Colitis
acute condition from inflammatory cells and debris overlying sites of mucosal injury - makes a "pseudomembrane"
- leads to megacolon, sepsis, and shock

caused by toxin A: potent enterotoxin, affects cytoskeleton and is chemotactic to PMNs, which release prostaglandins and leukotrienes

toxin B: cytotoxin
Cause of AAC
alteration of normal flora
toxigenic C difficile colonizes
- associated with broad spectrum AB's including C diff.
- long hospital stays and old age are also risk factors
Diagnosis of C. difficile
pressence of inflammatory cells in stool
bloody diarrhea
endoscopy shows colitis or psuedomembranous colitis
(not 100% reliable)

histology: epithelial necrosis with fibrin overlying. PMN's

test for toxin A or B
Diversion Colitis
inflammatory mucosal lesion of surgically isolated segments of colon

enterocytes normally use short chain fatty acids for their calorie supply
removal of ileum may deprive nutrition from colin

can be mild or resemble ulcerative colitis

restore fecal flow or supply fat enema
Malabsorption
diarrhea, weight loss, malnutrition, bulky stools, greasy

fecal fat is the cornerstone of diagnosis - do fat test
small intestine biopsy can look for inflammatory cells, villus abnormalities, lymphomas and whipples disease, sprue
Pathogenesis Celiac Disease
chronic disease, lesions in SI, impaired absorption, which improves with wheat restriction

celiac sprue, GSE (also names)

fundamentally sensitive to gluten which is a water insoluble protein

T cell mediated chronic inflammation with autoimmune intolerance to gluten
Morphology of Celiac Disease
flattened mucosa in small intestine
diffuse enteritis
ATROPHY of villi
vascular degeneration
increase lymphocytes
increased mitotic activity in crypts
Clinical Features of Celiac Disease
flattened or scalloped appearance of intestine on endoscopy

flatulence and weight loss
blistering dermatitis herpetiformis
Diagnosis of Celiac Disease
documentation of malabsorption
visualization of lesions in bowel, and relief of symptoms with wheat removal
-IgA TTG and IgA EMA

(TTG is also seen in Sjogrens)
Tropical Sprue
Celiac-like disease seen in the far east, Caribbean
overgrowth of toxigenic gram negative bacteria in JEJUNUM

Bacteroides is absent
B21, folate, and fat are malabsorbed

shortening and thickening of villi with increased infiltrate
Whipple Disease
A rare disease caused by Tropheryma Whippeli

can involve any organ, but mainly intestine and brain

G+, proliferates in macros, no immune reaction

SI with distended macros, PAS positive
causes ssteatorrhea with abd pain, arthritis, and neurochanges
FATAL
Inflammatory Bowel Disease
CD - can affect any portion of the tract
most often distal SI
UC - limited to colon and rectum

extraintestinal inflammatory manifestations
common in developed countries

exaggerated response to commensal microorganisms, and have defects in barrier function
T cell responses of IBS
CD4+ T cells damage tissue

CD - delayed type hypersensitivity by inductions of INF producing Th1 cells - produces granulomas
Diagnosis of IBD
no single test

pANCA - positive often in patients with UC

ASCA antibody is high in CD patients
Crohns Disease
females more often, whites more often and JEWS, Smoking is a risk factor

usually distal ileum, sometimes with involvement of colon

dull gray serosa, creeping fat, rubber intestinal wall, hypertrophy of muscularis propria, narrow lumen
sharp demarcation between diseased and non-diseased segments
aphthous ulcers, linear ulcers, narrow fissures between mucosa folds - can lead to fistulas
Histological features of Crohn's Disease
mucosal inflammation, with bottow heavy appearance
chronic damage to mucosa
ulceration
transmural inflammation affecting all layers
non-caseating granulomas
mural changes
Clinical Manifestations of Crohn's Disease
fatigue
prolonged diarrhea w/ pain
weight loss and fever
poor growth in children

long standing CD leads to increased cancer incidence
Ulcerative Colitis
only affects mucosa and submucosa
no granulomas

more common in blacks, and females
peaks between 20 and 25

pancolitis, and in severe cases can cause backwash ileitis
friable and bleeds easily, ulcerations starting in distal colon, and extending from there
pseudopolyps
tunnels through mucosa
NO serpentine ulcers
flattened mucosal surface
Histology of UC
mononuclear infiltrate in LP
crypt abscesses = aggregates of neutrophils
NO granulomas
sometimes muscularis propria will be exposed
submucosal fibrosis is a sign of regeneration
Basal lymphoplasmacytosis - indicator of chronic inflammation
Dysplasia and UC
dysplasia in multiple sites w/ inflammation masking signs of carcinoma
infiltrative carcinoma with masses - important for early diagnosis

nuclear atypia and cytoplasmic differentiation - plaque like lesions, edemoas, or invasive carcinomas
Clinical Features of UC
relapsing disorder with attacks of bloody diarrhea containing stringy mucus
lower abd pain relieved by defecation
precipitated by emotional or physical stress
toxic Megacolon
Most severe cases of UC
damage to muscularis propria and neural plexus
colon swells and becomes gangrenous
Collagenous and Lymphocytic Colitis
"kinder and gentler"
3 - 20 non bloody watery bowel movements per day
cramping abdominal pain
unremarkable X rays

CC - patches of band like collagen under surface of epithelium (middle aged older women)
LC - intraepithelial infiltrate of lymphocytes, strong associated with autoimmune diseases (equal males and females)
Bowel Ischemia
Acute occlusion of one of 3 major branches may lead to meters of infarcted intestine
end arteries can become occluded and cause small infarcts
severity of Ischemia
transmural - all layers
mural infarction - mucosa and submucosa
mucosal
Morphology of Transmural Infarction
sudden and total occlusion of arterial blood flow
greatest risk at splenic flexure (right between sup. and inf. mesenterics)

infarcts appear hemorrhagic because of blood into damaged areas
wall becomes thickend and rubbery
sharp demarcation in arterial occlusions
venous has dusky areas blended into normal tissue

edema, hemorrhage, necrosis sloughing into lumen, becomes gangrenous and can perforate
Morphology of Mucosal and Mural Infarcts
dark red luminal hemorrhage
inflammatory cells and exudate absent from serosal surface
superficial ulceration may be present
Mild Ischemia
epithelium or villi may be necrotic or slough off
inflammation is absent
mild vascular dilation
deep crypts are intact
may lead to enterotoxic bacterial overgrowth - psuedomembranous colitis
Chronic Ischemia
chronic vascular insufficiency

may mimic enterocolitis or IBD

becomes fibrotic -> stricture
Clinical Features of Ischemia
high death rate
older individuals
more common in people with premature bowel disease

severe abdominal pain and tenderness with nausea, vomiting, bloody diarrhea or melanotic stool

decreased bowl sounds, muscular rigidity
diagnosed often delayed due to more common causes

may lead to sepsis
Angiodysplasia
non-neoplastic lesion of vascular dilation and malformation
tortuous dilations of submucosal and mucosal blood vessels
Congenital Diverticula
all three layers of bowel
Meckel Diverticulum - persistence of vitelline duct - causes outpouching of antimesenteric border of ileum - just proximal to ileocecal valve - may cause bleeding from peptic ulceration
Acquired diverticula
left side of the colon/sigmoid colon
only outpouching of mucosa and submucosa
usually are multiple and become infected
morphology of Diverticula
small flask like or spherical outpouchings

disect into the fat (epiploic appendices) and may be missed

flattened or atrophic mucosa with absent muscularis mucosa

hypertrophy of muscularis propria in bowel segment
taeniae coli are prominent
Diverticulitis
Obstruction and perforation of diverticula - inflammatory changes

eventually becomes fibrotic - can resemble colon cancer
Causes of Mechanical Obstruction
Adhesions
Hernias
Volvulus
Tumors
Strictures
Gallstones
Meconium
Causes Pseudo Obstruction
paralytic ileus
bowel infarction
myopathies and neuropathies
Hernias
Weakness or defect in peritoneal wall
anterior at inguinal and femoral canals, umbilicus, and surgery sites
viscera can protrude into sac and become trapped - infarct
Adhesions
caused by surgical procedures, infection and endometriosis
can cause peritonitis

as peritonitis heals, adhesions may develop
Intussusception
one segment of intestine constricted by peristalsis - telescoped into distal segment of intestine - pulls mesentery in with it

patient is otherwise healthy

sometimes associated with rotavirus - inflammation serves as traction poiint
Volvulus
complete twisting of bowel
sigmoid, cecum, stomach
Polyps
tumorous mass, protruding into lumen
Sessile: lacks a definable stalk
Pedunculated: has astalk

nonneoplastic - result of abnormal mucosal maturation
adenomatous - epithelial polyps arise as result of dysplasia - neoplastic precursor
Inflammatory Polyps
non-neoplastic
psuedopolyps
islands of inflammed, regenerating mucosa - patients with severe IBD
Lymphoid Polyps
essential normal variant of mucosa
Hyperplastic polyps
most common and are small
well formed glands and crypts w/ feathery epithelial profile
- may progress to adenocarcinoma
Juvenile Polyps
malformations of mucosal epithelium and lamina propria
large, rounded, smooth

lamina propria makes up most of bulksurface is congested or ulcerated

single - no malignant potential

-- AD disorder juvenile polyposis syndrome -- there are multiple polyps in the GI tract -- does have risk for transformation
Peutz-Jeghers polyps
involve epithelium, lamina propria, muscularis mucosa

rare AD condition
melanotic mucosa and cutaneous pigmentation around the lips, genitalia, and palms
increased risk for intussesception
patients have increased risk of developing other carcinomas (sex cord tumors)

mutation of STK11 on Chromosome 19
-- serine/threonine kinase activity
Cowden syndrome
AD syndrome - multiple harmatomas of all three germ layers

predisposes to breast and thyroid cancers

PTEN gene on Chromosome 10
Cronkhite-Canada syndrome
GI hamartomas and ectodermal abnormalities (nail atrophy and skin pigmentation)

eitiology is unknown
Adenomas
intraepithelial neoplasms
tubular adenomas - most common
villous adenomas - least common
tubulovillous adenomas

low - high grade
precursor lesions to colorectal adenocarcinomas
large villous are the most worrisome
small tubulars are the most innocent

can be assymptomatic, cause anemia or occult bleedint
villous are most likely to bleed
- can also secrete mucus - leads to hypoproteinemia or hypokalemia
Serrated Adenoma
resemble benign hyperplastic polyps
have dysmaturational crypts
High grade dysplasia (CIS)
little or no metasstatic potential because of lack of lymphatic channels

when is penetrates muscularis mucosa and the submucosal space - termed adenocarcinoma

can remove via endoscope provided the lesions does not have lymphatic or blood supply, is well differentiated, and superficial
Colorectal Carcinoma
occur sporadically
-directly proportional to amount of adenomas
- peak incidence pf polyps precedes peak incidence of carcinoma
- carcinomas are often surrounded by adenomas
- complete recision of adenoma reduces carcinoma prevalence
Molecular Carcinoma
APC/B cadherin - chromosomal instability - increased mutations of oncogenes and tumor supressors
- follows adenoma/carcinoma pathway

loss of APC
-5q21
- first event in formation of adenomas

mutation of KRAS
- most frequent oncogene in cancer and adenomas

loss of p53
- last step in carcinogenesis

Microsatellite instability pathway
- damage to mismatch repair genes
Morphology of Colorectal carcinoma
most common in rectum/sigmoid
right sided carcinomas have increase microsatellite instability

rarely familial
Patterns of Colorectal Carcinoma
Proximal Colon:
polyploid, exophytic mass
rarely cause obstruction

Distal Colon:
annular, encircling lesions
sides are firm, center is ulcerated
Histology of Colorectal Carcinoma
tall columnar - anaplastic
strong desmoplastic stromal response
produce mucin - worse prognosis
Clinical Features of Colorectal Carcinoma
assymptomatic for years
insidous onset
cecal and right colon lead to fatigue weakness, iron def. anemia
left sided lesions lead to crampy lower left quad pain, and occult bleeding

(if an elderly man has iron def. anemia is colorectal cancer until proven otherwise)

met. to liver, lungs and bones
Familial Polyposis Syndrome
AD disorder
high propensity for malignant transformation

Peutz-Jaeghers - hamartomatous polyp, increased risk for cancer

juvenile polyposis syndrome

Cowden syndrome

FAP - many andenomatous polyps - always progresses to ccarcinoma

HNPCC - colorectal cancer, endometrial carcinoma, and ureter/pelvis
Clinical features of FPS
unusually young children
multiple tumors in single organ
positive family history
Familila Adenomatous Polyposis Syndrome
chromosome 5q21

Classic:
500-2500 adenomas
include stomoach and SI
Tubular adenomas usually, occasional villous
early detectionis important in cancer prevention

Attenuated:
fewer polyps, mainly in colon
still high risk for cancer

Gardner's:
FAP with ostomas, epidermal cysts, fibromatosis

Turcot:
polyps and CNS tumors
APC gene mutation
Hereditary Nonpolyposis Colorectal Cancer Syndrome (HNPCC)
Lynch Syndrome
increased risk of colorectal and extraintestinal cancer
low number of adenomas, but occur and an earlier age

-microsatellite repair problems from DNA repair gene mutations
Carcinoid Tumors
from mucosal endocrine cells
predominantly in the GI
peak incidence in people over 60
Histology of Carcinoid Tumors
form islands, sheets, ect.
scant pink cytoplasm
round nucleous
have membrane bound secretory granules

stain positive for chromogranin A, Synaptophysin, and enolase granules
Clinical Features of Carcinoid Tumors
rarely produce local symptoms
bleeding may occur, but most are assymptomatic

the secretory products can lead to Zollinger-Ellison, Cushings, ect.
Carcinoid Syndrome
excess elaboration of serotonin

vasculomotor disturbances
cutanous flushes
cyanosis
imtenstinal hypermotility
diarrhea
cramps/nausea
bronchospasms
hepatomegaly
nodular liver
pulmonic and tricuspid stenosis
pericardial fibrosis
MALT lymphomas
spordaic Bcell lymphoma
most common in West Hemis.

in early stages act like focal tumor - ameanable to surgery
relapses may occur
t(11:18) is common mutation
CD 10 and 5 NEGATIVE

arise anywhere in gut
immunoproliferative small intestinal disease (IPSID)
mediterranean lymphoma
B cell lymphoma with diffuse mucosal plasmacytosis - secrete lots of IgAlpha heavy chain

malabsorption and weight loss prior
Intestinal T cell Lymphoma
long standing malabsorption syndrome
diagnosed in younger adults with long history of malabsorption

mostly in proximal SI
Morphology if GI lymphomas
plaque like expansion of mucosa and submucosa
produce full mural thickening
can protrude into lumen, and ulcerate
can invade muscular layer and decrease motility
Mesenchymal Tumors
Lipomas - in submucosa
Lipomatous hypertrophy at ileocecal valve

Leiomyomas, and leiomyosarcomas - GIST's
Gastrointestinal Stromal Tumors (GIST's)
mostly in stomach but sometimes in SI
Tumors of the Anal Canal
basaloid pattern, SCC, ad adenocarcinoma

Basaloid - sporatic and uniform

SCC - HPV infection, linked to immunosuppression
Acute Appendicitis
inflammation associated with obstruction - fecalith
-causes collapse of venous drainage
- ischemic injury
- bacterial proliferation
Morphology and Histology of Acute Appenidicitis
neutrophillic exudate throughout all three layers
subserosal vessels are congested with perivascular neutrophil presence

changes appearance of serosa from glistening to dull
(early appendicitis)

fibrinopurulent exudate and abscesses form on wall
- suppurative necrosis

green-black gangrenous necrosis through wall which is likely to rupture and cause peritonitis

histological diagnosis is PMN's in muscularis propria
Carcinoid Tumor of Appendix
most common
discovered at time of surgery or exam of resected appendix

usually in distal tip - solid bolous swelling
Mucocele
globular enlargement of appendix by mucus, usually as a result of fecalith obstruction
mucin secreting cells atrophy

may occasionally spill into peritoneal cavity - infection

may also be from mucin secreting adenoma or adenocarcinoma - pseudomyxoma peritoneii