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GASTROENTEROLOGY
KAPLAN

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tYv 2001 Ka
P'an Medical (80) 533-8850 or (949) 476-6282
GASTROENTEROLOGY:
DISEASES OF THE ESOPHAGUS
Pathogenesis: The esophagus serves as a food conduit from the oropharynx to the
stomach. Food
passes down the esophagus via peristaltic contractions. The UES (upper esophage
al sphincter)
and LES (lower esophageal sphincter) serve to prevent regurgitation of food upo
n swallowing.
Any disruption in peristalsis, LES, UES or esophageal anatomy can cause dysphag
ia or
odynophagia.
Dysphagia- refers to difficulty swallowing; often present in esophageal disease
s.
Odynophagia-refers to painful swallowing; often associated with esophagitis.
Gastroesophageal Reflux Disease (GERD):
Pathogenesis: incompetence of LES
Etiology of decrease LES tone:
-pregnancy secondary to progesterone
-hiatal hernia (herniation of portion of stomach through diaphragm esophage
al hiatus).
-chocolate, peppermint, ETOH, fatty foods
-smoking
-smooth muscle relaxants- beta-adrenergics, nitrates, Ca channel blockers
Clinical presentation:
-heartburn(pyrosis)- warm/burning sensation in epigastric/substernal area;
due to
irritation of esophageal mucosa secondary to refluxed acidic material. Most
common
symptom
-atypical/angina-like chest pain(GERD is responsible for 70% of non-cardiac
chest pain).
-dysphagia-which occurs with peptic stricture
-feeling of fullness in the throat (globus)
-non-productive cough-especially when present with hoarseness
-associated with asthma( asthmatics show improvement of symptoms with GERD
therapy)
Diagnosis:
-based largely on history
-confirmed by responsiveness to therapy
-barium esophagram-only if obstructive symptoms
-EGD(esophagogastroduodenoscopy)-only indicated if patient presents with
dysphagia, weight loss, anemia, emesis, blood in stools, family h/o peptic
ulcer disease, non-cardiac chest pain unresponsive to therapy.
-24 hour pH monitoring (pH electrode placed 5cm above LES-positive if pH re
mains

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low in the esophagus).Indicated in patient with normal EGD but refractory sympto
ms;
unresponsive to proton pump inhibitors(PPIs); coughing/hoarseness or atypical ch
est pain
without any other GERD symptoms.
-Bernstein test- O.lmolar HCL is infused in the esophagus; positive test ind
icated by the
reproduction of symptoms upon infusion. Can be used in atypical chest pain cases
.
Complications:
-esophagitis-associated with 2% of GERD patients
-peptic esophageal stricture-Seen in 10% of patients; due to constriction of
esophageal lumen secondary to fibrosis; seen in patients with chronic
heartburn and dysphagia; located in lower third of esophagus
-Barrett esophagus-discussed later
-esophaeal ulcers
-bleeding
Treatment: overall goal to decrease reflux, protect esophageal lining, and impro
ve
esophageal clearance
-lifestyle modification: weight loss, small meals, sleep with head of bed el
evated, eat 3
hours before bedtime, smoking cessation, avoidance of fatty meals or sweets at b
ed time.
-antacids pm
-H2 recepter blockers such as pepcid-given BID (heals 50% of patients with e
sophagitis
seen in EGD)
-Proton pump inhibitors (i.e. omeprozole) which inbit the parietal cell H+,K
+, ATPase
pump-used in more severe cases; (heals 80% of patients with esophagitis seen in
EGD).
-prokinetic drugs (i.e. metaclopromide) taken 30 minutes before meals serve
to improve
esophageal clearance, gastric emptying and raise LES pressure. (Does not heal es
ophagitis).
-sugery-Nissen fundoplication- in which the fundus of the stomach (hence the
name
fundoplication) is wrapped around LES. Used in patients refractory to all above
medical therapy.
Must have normal esophageal peristalsis (as evidenced in motility study) in orde
r to have this
surgery otherwise iatrogenic achalasia can occcur. (can be done laparoscopically
).
-peptic strictures-treated with dilaters and acid suppression.
Achalasia;
Pathogenesis: a smooth muscle motor disorder in which LES tone is increased (it
does not
relax upon swallowing) and absence of or abnormal esophageal peristalsis. Occurs
secondary to
decrease in intramural neurons (inhibitory neurons) in smooth muscle of LES and
esophageal
body.
-most cases in U.S. are idiopathic.
-secondary causes of achalasia include:

*Chagas disease (cardiomyopathy, megacolon, megaesophagus, and achalasi

a)
seen in South America.
" gastric carcinoma or lymphoma which obstructs the esophagus.

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Clinical presentation:
-dysphagia to liquids and solids
-regurgitation hours after food ingestion
-atypical chest pain
-high aspiration risk
Diagnosis:
-barium swallow-done first; shows "bird beak sign" in which a dilated esopha
gus and
pronounced tapered narrowing at GE junction due to nonrelaxing LES.
-manometry-displays increased LES tone and abscence/abnormal
peristalsis.(See figure A)
-EGD-needed to confirm diagnosis and rule out secondary causes of
achalasia due to gastric carcinoma or lymphoma.
Treatment:
-pneumatic dilation of the LES which tears muscle fibers thereby reducing LE
S
pressure. Perforation occurs in 5%.
-botulinum toxin injected in LES-blocks cholinergic nerves in LES. Effective
in 65%.
Not long term-repeat therapy needed every 2 yrs.
-surgical myotomy of LES-circular muscle is incised;(can be done laparoscopi
cally);
complications include reflux esophagitis.
-Calcium channel blockers and nitrates provide temporary palliation.
Diffuse Esophaeeal Spasm (DES);
Pathogenesis: motor disorder of smooth muscle of the esophagus in which nonperis
taltic
spontaneous contractions occur. Secondary to degeneration of nerve processes. Un
common.
Clinical presentation:
-dysphagia
-chest pain
-symptoms often precipitated by cold and carbonated liquids and self-limited
.
Diagnosis:
-"corkscrew" pattern seen on barium swallow secondary to simultaneous
uncoordinated esophageal contractions
-confirmed by manometry-nonperistaltic uncoordinated contractions.(See
figure A)
Treatment:
-calcium channel blockers
-nitrates
-muscle relaxers

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Nutcracker Esophagus:
Pathogenesis: increased excitatory activity which results in high amplitude (hig
hpressure)
contractions.
Clinical presentation:
-chest pain
-dysphagia
Diagnosis:
-manometry- high amplitude peristaltic contractions.
Treatment:
-calcium channel blockers
-nitrates
-muscle relaxers
Anatomic Obstruction;
Pathogenesis:
-Schatski ring: thin, weblike mucosal ring located at squamocolumnar junctio
n or
proximal to LES. (See figure 1). Seen in young patiens. Hiatal hernia usually pr
esent as well.
(10% of people have asymptomatic rings)
-Plummer-Vinson syndrome: hypopharyngeal web associated with Iron deficiency
anemia. Seen in middle-aged women. Increased risk of squamous cell carcinoma.
-Peptic strictures-secondary to chronic GERD; seen in lower third of esophag
us; varies
in length.
-Esophageal carcinoma (discussed below)
Clinical presentation:(symptoms only occur when 75% of esophageal circumference
compromised)
-progressive dysphagia to solids followed by liquids in strictures and cance
r.
-intermittent dysphagia to solids and liquids in rings
-glossitis seen in Plummer-Vinson syndrome
Diagnosis:
-barium swallow-rings, webs , and peptic strictures can be visualized.
-EGD

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Treatment:
-pneumatic dilation of symptomatic lower esophageal rings and webs. (See fig
ure 2)
Amvotrophic lateral sclerosis (ALS);
Pathogenesis: Progressive degeneration of motor neurons affecting cranial nerve
nuclei and
subsequent muscle atrophy and weakness causing dysphagia. Paryngeal and laryngea
l muscles
can be affected as well. Weakness and atrophy of the tongue, soft palate and mas
seter muscles
decrease the effectiveness of chewing and swallowing food. Neuronal degeneration
secondary to
bulbar (upper motor neuron) or psuedobulbar (lower motor neuron) palsey.
Clinical presentation:
-dysphagia to solids and liquids
-choking attacks and aspiration
Diagnosis:

-clinical history
-swallowing study
Treatment:
-if severe percutaneous endoscopic gastrostomy (PEG) may be needed.
Scleroderma: (Progressive systemic sclerosis)
Pathogenesis: decrease or absence of peristalsis (like achalasia) secondary to a
trophy and
fibrosis of esophageal smooth muscle. Effects mid and distal esophagus. LES is i
ncompetent
(unlike achalasia).
Clinical presentation:
-progressive dysphagia to solids and liquids
-GERD symptoms due to decreased LES tone
Diagnosis:
-barium swallow- dilation and decreased peristalsis in mid to distal esophag
us,
-motility studies- decreased smooth muscle contraction(See figure A)
Treatment:
-anti-reflux therapy
-otherwise no effective treatment
Esophagi tis:
Etiology and Pathogenesis:

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-infectious- include viral (HSV, CMV, VZV, HIV); bacterial, Candida; seen ma
inly in
immunocompromised.
-pill induced-secondary to ingestion of pills without water; most common wit
h
doxycycline, ASA/NSAIDS, FeSO4, alendronate, and quinidine. Can be avoided by ta
king
fluids with pills in upright position.
-corrosive-caused by ingestion of caustic substances; i.e strong acids or al
kali. May cause
bleeding, perforation, and possibly death. Complicated by stricture
formation which may require dilation.
Clinical presentation:
-odynophagia
-chest pain
-dysphagia
Diagnosis:
-clinical
-EGD
Treatment:
-infectious-treat with antibiotics, antiviral (acyclovir, gancyclovir), or
antifungals respectively.
-pill induced- prevention with hydration and ingestion in upright position.
-corrosive-supportive fluids; pain medication-narcotic; dilation of strictur
es or
esophogogastrectomy in severe cases.
Barretts Esophagus:
Pathogenesis: Transformation of squamous epithelium of esophagus by columnar epi
thelium
(known as metaplasia) secondary to chronic reflux. Occurs in 10-20% GERD patient
s. 30 X

greater incidence of adenocarcinoma. Adenocarcinoma more likely if > 8cm Barrett

s , dysplasia
and smoking history present. Most prominent at gastroesophageal (GE) junction.
Clinical presentation:
-chronic GERD symptoms for > lOyrs
-increase in white males, and age over 40yrs
Diagnosis:
-EGD with biopsy displaying columnar metaplasia.
Treatment:
-antireflux therapy
-no dysplasia -surveillance EGD every 2-5 yrs.
-low-grade dysplasia- EGD every 3-6 mos.
-high dysplasia-esophageal resection.

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Esophageal Carcinoma:
Pathogenesis:
-squamous cell carcinoma- accounts for 50% of cases; proximal 2/3 of esophag
us;
associated with ETOH (especially whiskey), smoking, achalasia, diet high in nitr
ates& pickled
vegetables, Plummer Vinson syndrome, head and neck carcinoma, and lye inestion.
Also
associated with celiac sprue, pitting of palms and soles, and deficiency in vita
min A & Zinc.
More common in black males.
-adenocarcinoma-50% of cases; in Barretts esophagus; distal 1/3 of esophagus
;
incidence has risen. More common in white males.
Clinical presentation:
-progressive dysphagia for solids then liquids (seen when 60% esophageal
circumference compromised)
-odynophagia
-weight loss
-malnutrition
-chest or back pain
-regurgitation and aspiration
-hypercalcemia with squamous cell CA
Diagnosis:
-barium enema (uneven ulcerated edges seen)
-EGD with biopsy
-CT scan staging
Treatment: (poor prognosis 5% 5 yr survival)
-surgery for localized lesion
-Chemotherapy with cisplaitn + 5FU+radiation for metastasis,
-palliative care includes: endoscopic dilation and PEG placement
Zencker Diverticulum;
Pathogenesis: outpouching in upper esophagus secondary to weakness in posterior
hypopharyngeal wall.
Clinical presentation:
-halitosis
-transfer dysphagia -difficulty initiating swallowing
-regurgitation of food several days post ingestion

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Diagnosis:
-barium swallow should be done first as EGD and nasogastric intubation are
contraindicated secondary to risk of perforation through diverticulum.
Treatment:
-cricopharyngeal myotomy or diverticulectomy or both.
Mallory Weiss Syndrome;
Pathogenesis: mucosal tear due to repeated vomiting or retching. Tear located in
squamocolumnar junction
Clinical presentation:
-hemetemesis following episode of non-bloody emesis or retching.
Diagnosis:
-EGD
Treatment:
-usually self-limited
-angiographic embolization or vasopressin in refractory cases
PEARLS TO REMEMBER OF ESOPHAGEAL DISEASES:*
* GERD- lifestyle modification is first line treatment. PPI used in refractory c
ases.
*Achalsia: LES tone is increased and absence of peristalsis. Associated with Cha
gas disease.
Barium swallow shows "bird beak sign".
* Barretts Esophagus: associated w/ GERD; 30 X greater incidence of adenocarcino
ma.
Biopsy shows columnar metaplasia
* Esophageal Carcinoma- squamous cell carcinoma- proximal 2/3 of esophagus; asso
ciated
with ETOH (especially whiskey), smoking, achalasia, diet high in nitrates, Plumm
er Vinson
syndrome. Adenocarcinoma- distal 1/3; associated w/ Barretts esophagus.

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DISEASES OF THE STOMACH
Physiology: Food matter is further digested in the stomach and is the main stimu
lus for gastric
acid secretion. The stomach mixes and breaks down food further in to small parti
cles (<lmm)
wich can pass out the pylorus to the duodenum. Stomach mucosa contains parietal
cells located
in the fundus and body of the stomach. The parietal cells are responsible for se
creting HCL via a
proton pump (the final step in acid production). The parietal cell receptors are
stimulated by
gastrin (which is released by G-cells); acetylcholine (released by vagus nerve);
and histamine
(from mast cells). Gastrin is the most potent stimulant of post prandial acid se
cretion and is
stimulated by protein digestion. G-cells are located in the antnim. Coffee, wine
and beer also
increase acid production. Acid production is inhibited by negative feedback of s
ecretin and by

stomach pH <3. Secretin is released in the duodenum in response to high acidity

of contents
emptied from the stomach. Secretin will also stimulate bicarbinate release from
the pancreas to
neutralize the acid. Pepsinogen is founding mucosal cells in the stomach and is
the precursor for
pepsin. Pepsin in combination with gastric acid cause mucosal damage.
Peptic Ulcer Disease:
Pathogenesis: Lesion in mucosa of stomach or duodenum caused by corrosive effect
s of gastric
acid and pepsin.
-Helicobacter Pylori- most common cause of PUD- mainly duodenal in 80-90%. (
See
figure 3)
-NSAID induced- directly toxic to GI mucosa; occurs in stomach up to 30% of
patients
taking NSAIDS and in duodenum in up to 20%. [can cause ulcers in large bowel as
well].
-ETOH induced- directly toxic to GI mucosa; also has lipolytic activity.
-Corrosive material-directly toxic to GI mucosa; due to ingestion of acids (
HCL, sulfuric
acid) or strong alkali (lye, sodium hydroxide). Perforation, strictures and hemo
rrhage can occur.
-Stress related-secondary to mucosal injury and ischemia; up to 90% of burn
victims
(known as Curling's ulcer); s/p intracranial injury (known as Cushings ulcer);me
chanically
ventilated patients, sepsis, or coagulopathy.
Clinical presentation:
-abdominal pain
-painless GI hemorrhage (Curling's)
Diagnosis:
-Barium swallow
-EGD (positive Clo test biopsy in H. Pylori)
Treatment:
-H. Pylori- tripple antibiotic therapy
-NSAID induced- H2 receptor antagonist or proton pump inhibitors; prevention
with
misoprostol (a prostogalndin, PGE1, analogue).
-ETOH induced- H2 antagonist/ PPI
-Corrosive material- fluid hydration, antacids.
-Stress related-preventative therapy; treat cause of stress; antacids, H2
blockers.Gastrectomy in severe cases.

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NOTE: tobacco decreases healing rate of ulcers which are not secondary
to H.
Pylori. Neither corticosteroids or ETOH are themselves ulcerogenic. How
ever, if
corticosteroids are used in combination with NSAIDS they potentiate the
gastrointestinal damage NSAIDS cause. Corticosteriods cause 10 X increa
se in
NSAID induced bleeding
Non-ulcer Dyspepsia (NUD);
Pathogenesis: Abdominal pain or fullness/bloating which may be post prandial and

associated
with early satiety or nausea. Patients may also have heart burn/GERD symptoms, u
lcer-like
symptoms, or excessive flatulence. Functional in 70% of patients. NUD seen in pa
tients with
ulcer-like symptoms but without ulcer disease. Affects 20-30% of population. Pat
hogenesis is
unclear; gastric acid secretion normal; abnormal gastric/small intestine motilit
y may contribute.
Clinical presentation:
-abdominal pain, bloating, nausea
-GERD-like symptoms
-PUD-like symptoms
Diagnosis:
-clinical history
-normal EGD or normal Barium swallow
Treatment:
-GERD therapy
-Antimotility drugs
Gastrits:
-erosive: secondary to NSAID use, ETOH, corrossive materials, or stress rela
ted.
*Stress gastritis- seen in ICU setting in patients with burns,coagulopathi
es, or
intubated.
-infectious: H. pylori (as discussed below); other causes rare and include s
taphylococci,
streptococci, E.coli, Proteus.
-chronic-non-erosive (type A and Type B):
*type A: autoimmune, atrophic, pernicious anemia, achlorhyria- less
common; involves fundus and body, spares antrum. Secondary to antibodies to
parietal
cells and intrinsic factor; leads to hypergastrinemia similar to ZES patient
s (gastrin
>1000 pg/ml). In pernicious anemia the parietal cells, which also secrete in
trinsic
factor, are destroyed leading to B12 malabsorption. .Pernicious anemia assoc
iated with
increase gastric adenocarcinoma.
*type B: H.Pylori- secondary to increase gastric acid secretion; more c
ommon
cause of chronic gastritis (seen in 80%). 50% incidence in U.S. Seen primari
ly in

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Clinical presentation:
-abdominal pain
-early satiety
-nausea and vomiting
-GI bleeding
Diagnosis:
-clinical history
-EGD
*type B/h.pylori: histologic antral biopsy is gold standard. Urease tes
ts (95%

sensitive and specific)-include CLO test (antral biopsy placed on urea mediu
m. Bacteria
produce ammonia which increases pH and changes medium color) and urea breath
test
(radio labeled urea used). IgG serology indicates past exposure
Treatment:
-erosive- see treatment above under PUD due to NSAIDS, ETOH, corrosive and
stress induced.
-infectious- appropriate antibiotics
-Type A chronic-pernicious anemia- lifelong Vitamin B12.
-Type B chronic/ H.pylori- triple therapy for symptomatic patients; successf
ul
eradication in most.
Zollinger-Ellison Syndrome (ZES);
Pathogenesis: Secondary to gastrinoma which secretes gastrin and causes ulcers i
n stomach
and duodenum (1st portion) and malabsorption secondary to increase acid in duode
num.
Gastrinoma located in predominantly in head of pancreas. (can be seen in duodenu
m, stomach,
and spleen) Varies in size. 20% can be associated with MEN 1 syndrome (parathyro
id,
pituitary and pancreatic tumors). Patients 20-50 yrs of age.
Clinical Presentation:
-abdominal pain
-diarhrea/steatorrhea
Diagnosis:
-clinical history
-elevated gastrin levels (greater than 200ng/l fasting usually lOOOng/1) and
IV secretin

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-Upper GI series
-EGD- large gastric and duodenal mucosal folds.
Treatment:
-resection of tumor
-proton pump inhibitor
Carcinoid syndrome
Pathogenesis: Tumor of epithelial origin in the small intestine. Secondary to in
crease gastrin
levels. Can lead to niacin deficiency due to depletion of tryptophan (precursor
of niacin). Can
occur in appendix (non-secreting), ileum (70% secreting), bronchial (most sympto
matic).
Symptoms associated with secretion of 5-hydroxytryptophan and histamine. Associa
ted with
right-sided valvular heart disease.
Clinical presentation: (most asymptomatic)
-diarrhea
-facial flushing
-ETOH intolerance
-Tachycardia, hypotension
Diagnosis:
-24 hr urine 5-HIAA (5-hydroxyindolacetic acid)->25mg over 24hrs
Treatment:

-Surgery
-Chemotherapy
-H-2 antagonist
-Corticosteroids
-Indomethacin
-Catecholamine antagonists
-Octreatide (somatostatin analogue) therapy
Gastric Carcinoma;
Pathogenesis: Incidence of gastric cancer has decreased over last several decade
s. 90%
adenocarcinoma. 10% Non-hodgekins lymphoma, leimyosarcoma, ect. Risk factors inc
lude:
diet high in nitrates, dried, smoked and salted foods, genetic factors, Barrett
esophagus, (gastric
ulcers do not cause gastric cancer).
*MALT (mucosa associated lymphoid tissue) Lymphoma-associated with H. Pylori
infection.

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Clinical Presentation:
-early satiety
-weight loss
-nausea/vomiting if located near pylorus
-in metastasis periumbilical lymphadenopathy ("Sister Mary Joseph node"), supr
aclavicular
nodes, ovarian nodules (Krunkenberg tumor)
Diagnosis:
-clinical history
-EGD-biopsy
Treatment:
-surgery
-chemothery (adenocarcinima is radioresistant)
-tripple abx therapy for MALT Lymphoma.
Gastroparesis (Delayed Gastric Emptying);
Pathogenesis: Seen in diabetics secondary to vagal neuropathy and in patients po
st vagotomy.
Clinical Presentation:
-post prandial nausea and vomiting
-early satiety
Diagnosis:
-gastric emptying study (ingestion of radio-isotoped labeled food)
Treatment:
-Metoclopromide
Dumping Syndrome:
Pathogenesis: Post- prandial vasomotor symptoms secondary to peptic ulcer diseas
e surgery.
Due to distention of the small intestine and shift of fluids which result from t
he rapid emptying
of gastric contents into the small intestine
Clinical Presentation:
-Diaphoresis, lightheadedness, palpitations, nausea/vomiting occur in early
version-30
minutes after eating. Can also occur in later version -90 minutes post-prandial.
-Meals rich in carbohydrates (especially sucrose) elicit symptoms in late ty
pe.

Diagnosis:
-Clinical history

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Treatment:
-dietary modification-restriction of sweets
-frequent, small meals
-decrease liquid intake w/meals
Afferent Loop Svndrome;
Pathogenesis: Occurs in patients post Billroth n (gastrojejunostomy) or partial
gastrectomy. Due
to incomplete drainage of bile and pancreatic enzymes (released after eating) in
the afferent loop
as well as loop distention. In another form, bacterial overgrowth can occur (bli
nd loop
syndrome) in the afferent loop and lead to B12 and fat malabsorbtion.
Clinical presentation:
-abdominal pain and bloating 20 minutes after eating
-biliary emesis
-symptoms of B12 and fat malabsorption in blind loop syndrome
Diagnosis:
-clinical history
-B12 deficiency and fecal fat in blind loop syndrome
Treatment:
-surgical correction of afferent loop
*PEARLS for Diseases of the Stomach;
*PUD-Helicobacter Pylori- most common cause of PUD;duodenal 80-90%. NSAID more
common in stomach. Diagnose w/ Clo test. IgG serology indicates past exposure. H
.Pylorimost common cause of chronic antral gastritis. MALT (mucosa associated lymphoid
tissue)
Lymphoma-associated with H. Pylori infection.
* tobacco decreases healing rate of ulcers. Corticosteroids and ETOH are not ulc
erogenic.
Corticosteroids + NSAIDS= worse NSAIDS induced damage. Corticosteriods cause 10
X
increase in NSAID induced bleeding
* Gastric Carcinoma- 90% adenocarcinoma. Gastric ulcers do not cause gastric can
cer
* Carcinoid- associated w/ right-sided valvular heart disease ; diagnose by urin
e 5-HIAA.
DISEASES OF THE INTESTINE:
Inflammatory Bowel Disease;
Crohn's Disease;

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Pathogenesis: More common in white patients than asian or black. 6X more common
in
Ashkinazi Jews. Prevelence males=females. Peak in patients 15-35 yrs of age and
second peak at

60 yrs of age. There is genetic predisposition to disease. Thought to be immune

mediated.
Inflammation is discontinuous with "skip lesions" and is transmural. Transmural
inflammation may lead to formation of fistula and abscess. Can involve small int
estine and
colon: 30% small intestine (terminal ileum); 30% colonic; 40% both (ileocolic).
Rectal
sparing. Smokers more likely to develop Crohn's.
Clinical presentation:
-blood in stool (less common than in UC)
-abdominal pain
-fever
-ASCA +(anti-Saccharomyces cereviciae antibodies) in colonic involvement
-elevated sedimentation rate
-weight loss
-iron deficiency anemia
-B 12 deficiency
Extraintestinal manifestations:
-arthritis
-iritis/uveitis episcleritis
-erythema nodosum (more common in colonic disease)
-Pyoderma gangrenosum (more common in colonic disease)
-apthous ulcers
Complications:
-fistula formation
-obstruction
-urinary oxalate stones
Diagnosis:
-sigmoidoscopy/colonoscopy- discontinuous areas of inflammation; pseudopolyp
s;
ulcerations; cobblestone appearance; granulomas seen on histology. (See figures
4 and 5)
-Small bowel series (caution use in severe colitis)- string sign in terminal
ileum; as well
as mucosal irregularities listed above
Treatment:
-sulfaslalazine- sulfonamide( sulfapyridine) + mesalamine (5-ASA). 5 ASA is
active
component. Sulfasalazine is split in colon; not effective for small bowel diseas
e. 5-ASA used for
small bowel disease. Sulfapyridine can cauuse reversible infertility in men and
leukopenia.
-corticosteroids
-immunosuppressive therapy (steroid sparing): 6 mercaptopurine, azathyroprin
e;
infliximab
-metronidazole-perirectal disease and fistulas

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-B 12 supplementation
-surgery- for complications; high rate of disease recurrence( 50-75% over 5
yrs) in
resected site; ileal resection can lead to secretory diarrhea (see below)
Ulcerative Colitis (UC);
Pathogenesis: Prevalence of UC higher than Crohn's. More common in white patient

s than
asian or black. 6X more common in Jewish patients. Peak in patients 15-35 yrs of
age and 60 yrs
of age.. There is genetic predisposition to disease. Thought to be immune mediat
ed.
Inflammation involves colon and is continuous. High incidence of rectal involvem
ent (95%).
85% mild/moderate disease; 15% fulminant disease. Risk of cancer > Crohn's and i
n patients
with pancolitis >10yrs. Risk of cancer 20% at 20yrs of disease. Smoking cessatio
n exacerbates
disease.
Clinical presentation:
-Bloody diarrhea and pus
-abdominal pain
-fever
-P- ANCA positive (perinuclear antineutrophil cytoplasmic autoantibodies)
-elevated sedimentation rate
-weight loss
-iron deficiency anemia
Complications
-toxic megacolon- inflammation leading to dilation and perforation
Extraintestinal manifestations:
-arthritis
-iritis/uveitis episcleritis
-erythema nodosum
-pyoderma gangrenosum
-sclerosing cholangitis (suspect if increase in alkaline phosphatase)
-cholangiocarcinoma
*most relapse within 1 year of initial flare.
Diagnosis:
-signioidoscopy/colonoscopy- continuous inflammation; friable mucosa; loss of
mucosal
vascular pattern,; exudate; pseudopolyps; ulcerations; small crypt abscesses see
n on microscopy.
(See figure 6)
-barium enema (caution use in severe colitis)
Treatment:
-sulfaslalazine- sulfonamide( sulfapyridine) + salicylate (5ASA). 5 ASA is a
ctive

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component
-corticosteroid- more responsive than Crohn's patients
-enemas- with mesalamine or steroids for proctosigmoiditis
-immunosuppressive therapy (steroid sparing)- 6 mercaptopurine and azathyrop
rine;
cyclosporine
-20-30% require colectomy- curative; recommended if dysplasia present and in
tractable
disease.
Diarrhea
Pathogenesis: Approximately 9 liters of fluid passes to the GI tract. 8L are abs
orbed in the small
intestines. 1 liter enters the colon where SOOcc are absorbed leading to 200cc o

f stool a day. Stool

becomes more formed in the colon. Stool normally weighs 200g/d. Diarrhea- stool
weight >
200g/d and increase in frequency. Etiology includes infectious, antibiotic induc
ed,
malabsorption, inflammatory bowel diseease (IBS), and irritable bowel disease (I
BS). See
sections on malabsorption, IBD, IBS, and antibiotic induced diarrhea. Acute diar
rhea < 14 days;
Chronic diarrhea >2-3 weeks. Diarrhea can also be divided into secretory and osm
otic diarrhea.
Infectious diarrhea:
-enterotoxigenic- cholera, E. coli (travelers diarrhea)
-invasive- Campy lobarcter -most common; raw poultry (found in up to 50% of raw chick
en!),
mountain stream water.
-Shigella- transmitted by food or water. Common in day care centers
-Salmonella-raw eggs or dairy products
-Yersinia-joint pain and rash in addition to abdominal pain. May resemble ap
pendicitis.
Can be associated with Reiter's syndrome (arthritis, conjunctivitis, urethritis)
-E. coli 0157- undercooked beef, unpasteurized milk. Found in North west U.S
.
Associated with hemolytic uremic symdrome (HUS) and thrombotic thrombocytopenic
purpura
(TIP)
-Clostridia- see abx associated diarrhea.
-Giardia- amebic diarrhea. Most common from=asymptomatic carrier
-Cryptosporidia-most common in immunocompromised.
Secretory diarrhea- large volume of diarrhea> 1 liter. Hormone mediated: carcino
id
syndrome, Zollinger-Ellison syndrome, Vasoactive intestinal polypeptide (VIP) se
creting
pancreatic adenomas. Irritative laxatives-phenolpthalein can also cause this.
Can also occur post small bowel terminal ileum resection:
< 100cm terminal ileum removed, diarrhea secondary to bile salt
accumulation and treated with cholestyramine.
>100cm terminal ileum causes decreased bile acid leading to
malabsorption; treated with low fat diet and medium chain triglycerides
.

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Osmotic diarrhea- secondary to undigested solute pulling fluid in intestinal lum
en. Solutes
include- lactase deficiency is most common , carbohydrates (fructose and sorbita
l). Other
causes are nutrient malabsorption (secondary to Celiac sprue, pancreatic insuffi
ciency, and
bacterial overgrowth) and Mg laxatives.
Clinical presentation:
-increase in stool amount and frequency
-fever with invasive diarrhea
-abdominal pain and bloating
-nausea/vomiting

-bloody stools in most invasive diarrhea

Diagnosis:
-stool electrolytes-secretory vs. osmotic: 2[Na +K] = 290mosm/L (serum osmol
ality) in
secretory diarrhea. Serum osmolality- 2[Na + K] > 50 in osmotic diarrhea.
-fecal leukocytes- positive in all invasive diarrhea except Giardia and
-Cryptosporidia. Also positive in IBD.
-stool culture and sensitivity; ova and parasite
-string test and stool Giardia antigen for Giardia.
-Colonoscopy-biopsy
-NaOH test- turns stool red in phenolphthalein use.
Treatment: most infectious diarrhea self-limited
-Antibiotics- TMX/SMX (Bactrim)-most invasive diarrhea; erythromycincampylobacter; metronidazole-Giardia. Quinolones can also be used.
-IVF hydration
-avoid anticholinergic drugs in invasive diarrhea.
Irritable Bowel Syndrome (IBS):
Pathogenesis: Most common gastrointestinal disease seen in outpatients. Characte
rized by
altering bowel habits (constipation or diarrhea), abdominal pain and distention
relieved by
defecation. Symptoms intermittent but must be present for at least 3 months. Sli
ght female
predominance. Absence of nocturnal symptoms. Associated with domestic violence a
nd child
abuse. May be associated with depression.
Clinical Presentation:
-Rome criteria: 3 months of recurrent abdominal pain relieved by defecation
with
change of bowel habits; with at least 2 of following: altered consistency, frequ
ency and passage

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of stool, abdominal distention, or mucus in stool.
Diagmosis:
-rule out lactose intolerance, IBD, hypo/hyperthyroid; flex sig in patients
>40yrs old.
-clinical history- Rome criteria as above
Treatment: depends on presentation
-reassurance
-dietary modification including fiber supplements
-antidiarrheal agents
-anticholinergic agents
-tricyclic anti-depressents
-prokinetics
Malabsorption:
Pathogenesis: Disruption in absorption of digested food particles across intesti
nal wall. Villi and
microvilli on brush border of intestinal wall provide large surface area for abs
orption. Bowel
motility allows for adequate exposure of food particles to intestinal villi. Dis
ruption of
absorption can be secondary to Celiac sprue, tropical sprue, bacterial overgrowt
h, and pancreatic
insufficiency.
*Celiac Sprue- malabsorption secondary to atrophy of small bowel caused by intol

erance to
gluten (protein in wheat). Gluten sensitivity thought to be immune mediated. The
orydeficiency in mucosal peptidase required for gluten metabolism. Associated with
dermatitis
herpatiformis and lymphoma. Women account for 70% of cases.
*Tropical Sprue- malabsorption of unknown etiology. Acquired in tropics (onset c
an be years
after exposure); thought to be secondary to microorganism and toxin.
*Bacterial overgrowth- bacterial growth limited by normal peristalsis. Thereby a
ny disorder
leading to bowel stasis can promote bacterial growth. Commonly seen after ileoce
cal resection,
subtotal gastrectomy, Crohn's disease, scleroderma, and diabetes.
*Pancreatic Insufficiency- secondary to "burnt out pancreas" decrease pancreatic e
nzyme
production secondary to chronic pancreatitis, pancreatic cancer or cystic fibros
is.
Clinical presentation:
-Steatorrhea- > 14gms/dl fecal fat; >40gm/d in pancreatic insufficiency
-Iron deficiency (small bowel disease)
-Fat soluble vitamin deficiency
-weight loss

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-abdominal bloating
Diagnosis:
-sudan stain- for fecal fat is gold standard
-D-xylose test: 25gm oral xylose given. Normal->5gm xylose on urine or >20mg
/dl in
serum. Normal in pancreatic insufficiency.
-Abnormal -< 5gm in urine or <20mg/dl in serum. Test abnormal in small
bowel pathology (celiac/tropical sprue, bacterial overgrowth)
-antiendomysial and antigliadin antibody- celiac sprue
-small bowel biopsy- celiac/tropical sprue (flattening of mucosal villi and
mononuclear infiltrate in celiac sprue; thickened villi in tropical sprue)
-breath test- radio-labeled xylose or lactulose: bacterial overgrowth
-low trypsin level and secretin test (see chronic pancreatitis)-pancreatic
insufficiency.
Treatment:
-celiac sprue- gluten free diet
-tropical sprue- TMP/SMX (Bactrim) or tetracycline for 3-6 mos and folic aci
d
-bacterial overgrowth- empiric abx
-pancreatic insufficiency- pancreatic enzymes
-vitamin replacement
Whipple Disease;
Pathogenesis: caused by Tropherema whippeli a gram positive actinomycete. Lympha
tic
obstruction occurs and leads to symptomatology. Common in middle aged men
Clinical Presentation:
-Diarrhea- severe malabsorption
-hypoalbunemia
-fever/chills
-arthralgia

-central nervous system involvement

-lymphadenopathy
-weight loss
Diagnosis:
-small bowel biopsy- foamy macrophages on periodic acid Schiff (PAS) stain.
Treatment:
-antibiotics- TMX/SMX (Bactrim) or tetracycline for 6 mos to 1 yr.

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Diverticular Disease of the Colon:
Diverticulosis:
Pathogenesis-outpouchings of colonic mucosa through the muscularis up to artery.
Thought to
occur secondary to increase colonic intramluminal pressure. May be secondary to
decrease fiber
in diet. Most common in sigmoid colon. Increase incidence with age. Prevelence 5
0% of adults
over age 50.
Clinical presentation:
-most patients asymptomatic
-painless rectal bleeding- hematochezia
Diagnosis:
-Colonoscopy, bleeding scan, or angiograpgy if active diverticular bleeding
-Barium enema
Treatment:
-diverticular bleeding usually self limited
-colonoscopic therapy or
-surgery if diverticular bleeding severe
Pathogenesis: complication of diverticulosis; microperforation of diverticulum s
econdary to
undigested food of fecalith trapped in diverticula; abscess may by present.
Clinical presentation:
-Fever
-left lower quadrant abdominal pain
-palpable sigmoid mass
Diagnosis:
-US or CT scan
-Leukocytosis and positive blood cultures
Treatment:
-bowel rest
-abx therapy
-(avoid colonoscopy in acute diverticulitis). Flex sig after acute episode i
n patients > 50
to r/o perforating colon cancer.

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Antibiotic Associated Colitis/Peudomembranous Colitis;
Pathogenesis: pseudomembranous colitis secondary to antibiotic usage. Can be see
n as early as
1 week of starting ABX to 6 weeks after stopping ABX use. Can be seen in 20% asy

mptomatic
healthy carriers. Toxic megacolon is a complication.
Clinical presentation:
-diarrhea post abx therapy
-abdominal pain
Diagnosis:
-fecal leukocytes
-stool toxin assay or enzyme immunoassay (EIA). Toxin positive in up to 80%
after 3
specimens.
-colonoscopy- pseudomembrane, plaques, and exudate.
Treatment:
-stop inciting abx
-metronidazole x 10-14 days; Up to 20% relapse rate; If infection recurs rep
eat
same treatment (can also use oral vancomycin).
Pathogenesis: Decrease in stool frequency < 3 stools per week. Can result from a
bnormal
colonic transit time or anorectal disfunction. Can be secondary to medications (
anticholinergics,
narcotics, Calcium channel bolckers and iron most common and laxative abuse), pr
egnancy,
low fiber diet, prolonged physical inactivity, and colonic neoplasm. Can occur s
econdary to
systemic diseases: hypothyroidism, diabetes, collagen vascular disease (systemic
sclerosis),
CNS disease (CVA, Parkinsonism, Hirschsprung disease), Chagas disease.
*Chagas' disease- caused by damage of myenteric plexis by parasite Trypanoso
ma
cruzi. Parasite also causes achalasia and cardiomyopathy. Seen in South America.
*Hirschsprung disease- aganglionic megacolon resulting from absence of myete
ric
neurons in distal colon proximal to anus leading to contraction and of colon caus
ing
obstruction and proximal dilation The internal anal sphincter fails to relax wit
h rectal distention.
Seen usually in infants- diagnossd by 6 mos of age.
Clinical presentation:
-< 3 stools per week
-abdominal pain
-hard stools and straining with defecation
Diagnosis:
-Clinical history
-Abdominal X-ray

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-flexible sigmoidoscopy
-barium enema
Treatment:
-increase dietary fiber ( 20-30g/day)
-exercise
-bulk forming, osmotic, and stimulant laxatives
-surgery-for Hirschsprung disease.
Ileus/functional obstruction;
Pathogenesis: Obstruction is most likely intestinal and caused by post operative

adhesions.
Other causes include carcinoma, volvulus, diverticuitis. Intestinal distention o
ccurs secondary to
the build up of gas and fluid which occurs in the bowel most proximal to the obs
truction.
Clinical presentation:
-abdominal pain and distention
-high pitched bowel sounds
-nausea/vomiting
-constipation and obstipation
Diagnosis:
-X- ray-air/fluid levels
Treatment:
-Surgical for complete obstruction or non-resolving partial obstruction,
-nasogastric tube placement and electrolyte replacement
Intestinal Cancer:
Colon Cancer;
Pathogenesis: Arises from adenomatous polyps (mucosal protrusion) > 2cm in size.
Polyps
villous or tubulovillous in histology (villous has highest potential of carcinom
a). Sessile ( flat
based) polyps more malignant potential then pedunculated polyps. Progression of
adenomatous
polyp to cancer is 5-10 yrs. Patients age > 50. Hyperplastic polyps are not mali
gnant. Risk
factors include: family history, genetics (APC gene, p54), inflammatory bowel di
sease, low
fiber/high animal fat diet, tobacco smoke, and Streptococcus bovis bacteremia. D
ecreased
risk with 20+yr ingestion of asprin.
Familial polvposis;
Pathogenesis: all are autosomal dominant
-Familial colonic polyposis: thousands of colonic polyps (100% malignancy po
tential)
-Gardner syndrome: colonic and small bowel polyps with soft tissue and bony
tumors

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(osteomas)
-Turcot's syndrome: colonic polyps and central nervous system tumors.
-Peutz-Jeghers syndrome: Hamartomatous polyps in large and small intestines;
freckles seen on lips and buccal mucosa. Intussusception is a complication. Smal
l risk of cancer.
-Juvenile polyuposis syndrome: Hamartomatous polyps in small and large intes
tine.
NO MALIGNANT POTENTIAL.
Hereditary non-polvposis syndrome (HNPCC):
Pathogenesis: Cancer arises from normal mucosa. Cancer history in at least 3 fir
st degree
relatives; seen in at least 2 generations; occurring in at least 1 family member
< 50 yrs of age.
Cancer proximal in location. Strong association with ovarian and endometrial can
cer in
women.
Clinical presentation:

-occult bleeding
-frank bleeding (hematochezia)- advanced rectosigmoid lesions,
-weight loss
-Iron deficiency anemia (in any male or post menopausal women is GI cancer u
ntil
proven otherwise!)
Diagnosis:
-colonoscopy-biopsy
-physical exam and X-ray (polyposis syndromes)
-Carcinoembryonic antigen (CEA) levels- not specific for diagnosis but can b
e
followed to detect recurrence post operatively.
-Metastasis most common in liver via portal circulation (seen on CT scan); E
xception is
Rectal carcinomas that bypass portal circulation and will metastasize to other r
egions.
*Screening:
-Annual digital rectal exam starting at age 40
-Annual hemoccult testing of stool starting at age 50
-Colonoscopy every 3-5 years recently recommended for screening asymptomatic
patients starting at age 50 and found to be more accurate then flex sig and bari
um enema (N Engl
JMed 20000;343:162-8).
Classification- Duke's:
-class A: mucosa and submucosa; 95% 5 yr survival
-class B: muscularis(Bl) into or through serosa (B2); 70-85% 5 yr survival.
-class C: regional lymph nodes; 35-65% 5 yr survival.
-class D: distant metastasis; 5% 5 yr survival.
Treatment:

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-Surgery (total colectomy in familial colonic polyposis); Dukes A and B.
-Chemotherapy- 5FU and leucovorin; combined with surgery stage C.
-Radiation- rectal carcinoma
Appendicitis;
pathogenesis: inflammation of appendix secondary to obstruction and ulceration i
n lumen of
appendix caused by fecalith.. Occurs equally in males and females predominantly
20-30 yrs of
age.
Clinical presentation:
-abdominal pain- periumbilical region shifting to right lower quadrant with
rebound
tenderness on exam.
-Anorexia
-Nausea/ vomiting
-Fever
-Leukocytosis
Diagnosis:
-Clinical history and physical
-CT scan
Treatment:
-surgery-appendectomy
Intestinal Ischemia;
Pathogenesis: can be colonic or mesenteric.

*Ischemic colitis: affects areas supplied by inferior mesenteric artery (spl

enic flexure,
descending, sigmoid colon). Common in elderly secondary to vascular disease. Ass
ociated with
hypercoagulable low flow states. Non-occlussive
*Mesenteric ischemia: known as intestinal angina, occlusive type- disease se
condary
to arterial thrombus or embolis of celiac or mesenteric vessels. Common in elder
ly patients with
atrial fibrillation or valvular heart disease, peripheral vascular disease. Nonocclusive typeexact cause unknown; may be due to arrhythmia, hypotension, dehydration. Associa
ted with
tobacco use.
Clinical presentation:
-ischemic colitis:
*LLQ abdominal pain followed by defecation

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* gross rectal bleeding
-mesenteric ischemia:
*dull abdominal pain shortly after eating; pain usually worse than abdo
minal
exam
*mild occult bleeding
Diagnosis:
-ischemic colitis:
^abdominal X-ray and barium enema- "thumb printing"
*colonoscopy procedure of choice -hemmorhage, ulcertions and friable mu
cosa
-mesenteric ischemia:
*angiography
Treatment:
-ischemic colitis- most self limited
*bowel rest
*IVF,
*abx
*surgery if severe.
-mesenteric ischemia*angiography and embolectomy
Gastrointestinal Bleeding;
Upper GI bleeding:
Pathogenesis: Site proximal to ligament of Treitz. Sources of bleeding include g
astric erosions,
peptic ulcer disease,, varices from portal hypertension. Mallory-Weiss tear, eso
phagitis, and
gastric tumor.
Clinical presentation:
-hemetemesis-red in color if immediately after bleeding; coffee-ground in co
lor due to
acid breakdown of blood.
-Melena- black tarry stools; requires at least 60cc blood loss. Can last for
7 days after
bleed. Color secondary to breakdown of blood to hematin by hydrochloric acid.
-Orthostatic hypotension- indicates 20% reduction in blood volume

Diagnosis:
-clinical history
-nasogastric lavage-positive for blood usually
-EGD
-Occult bleeding- in slower bleed
-anemia- note initial hematocrit may not reflect brisk bleeding. Hematocrit
may become
hemodiluted with fluid hydration.

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-BUN may be elevated due to production of urea from bacterial breakdown of b
lood. (
BUN out of proportion to creatinine)
Treatment:
-EGD therapy- bipolar electrocoagulation, injection therapy, sclerotherapy a
nd band
ligation for varices.
-IV omeprazole (proton pump inhibitor) recently been found to reduce risk of
recurrent
bleeding from peptic ulcers (N Engl J Med 2000;343:310-6).
-IV fluid hydration; blood transfusion if necessary.
-Surgery
-Angiographic therapy
Lower GI Bleeding;
Pathogenesis: Distal to ligamnent of Treitz. Sources of bleeding: diverticular m
ost common in
the elderly; hemorrhoids most common in the young. Other sources include angiody
splasia,
infectious (Shigella, Salmonella, Clostridium difficile, E. Coli 0157), inflamma
tory bowel
disease, carcinoma, mesenteric ischemia, Meckle's diverticulum and Dieulafoy les
ion.
*Angiodysplasia (AVM)- vascular tuft in mucosa; common source of bleeding in
elderly. AVM in right colon more likely to bleed. May be part of hereditary Osle
r-Rendu Weber
sydrome (multiple AVM on skin, mucosa, lung, brain, GI tract). Treat with endosc
opic
ingection therapy.
* Meckel's Diverticulum-most common congenital GI anomaly. Diverticula with
gastric
mucosa that may ulcerate and bleed. Most common location is distal ileum. Seen i
n 2% of
children and young adults. Diagnose with increase uptake of technetium (Meckel's
scan).
Clinical presentation:
-hematochezia-bright red blood per rectum
-orthostatic hypotension, tachyardia
-lightheadedness, syncope, diaphoresis.
Diagnosis:
-clinical history
-anoscopy, flexible sigmoidoscopy, or colonoscopy
-Bleeding scan
-nasogastric lavage negative
-occult bleeding in slower bleed
-anemia- note initial hematocrit may noe reflect brisk bleeding. Hematocrit

may become
hemodiluted with fluid hydration.
Treatment:
-Endoscopic therapy- bipolar electrocoagulation, ingection therapy
-Angiography

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-Surgery
PEARLS for Diseases of the Intestine:
* Crohn's Disease: skip lesions discontinuous areas of inflammation;cobblestone
appearance;
granulomas seen on histology. Small bowel series-string sign in terminal ileum.
* Ulcerative Colitis: continuous inflammation and friable mucosa. 95% rectal inv
olvement.
Higher cancer risk. Associated with primary sclerosing cholangitis.
*Campylobacter-most common cause of infectious diarrhea.
* Fecal leukocytes- positive in all invasive diarrhea except Giardia and Cryptos
poridia. Also
positive in IBD.
* Secretory vs Osmotic diarrhea: stool electrolytes-secretory vs. osmotic: 2[Na
+K] =
290mosm/L (serum osmolality) in secretory diarrhea. Serum osmolality- 2[Na + K]
> 50 in
osmotic diarrhea.
*Celiac Sprue- Associated with dermatitis herpatiformis and lymphoma.
*Malabsorption-steatorrhea: > 14gms/dl fecal fat; >40gm/d in pancreatic insuffic
iency;
sudan stain- for fecal fat is gold standard. D-xylose test: Normal in pancreatic
insufficiency
and abnormal in small bowel pathology (celiac/tropical sprue, bacterial overgrow
th).
*Diverticulitis- avoid colonoscopy in acute diverticulitis; Flex sig after acute
episode in patients
> 50 to r/o perforating colon cancer.
* Whipple disease: Tropherema whippeli. Small bowel biopsy- foamy macrophages on
periodic acid Schiff (PAS) stain.
* Lower GI Bleeding: diverticular most common in the elderly; hemorrhoids most c
ommon
in the young.
* Ischemic colitis: abdominal X-ray and barium enema- "thumb printing"
*Colon Cancer: arises from adenomatous polyps> 2cm in size. Polyps villous or
tubulovillous in histology; villous and sessile polyps have highest potential of
carcinoma).
Hyperplastic polyps are not malignant. Iron deficiency in any male or post menop
ausal
women is GI cancer until proven otherwise! Carcinoembryonic antigen (CEA) levels
- not
specific for diagnosis but detects recurrence.
DISEASES OF THE PANCREAS:
Pancreatitis:
Pathogenesis: Pancreas prduces up to SOOOc of alkaline fluid containing enzymes
which aid in
digestion. Pancreatic bicarbinate secretion provides proper pH for enzymes to wo
rk. Secretin,
cholecystokinin (CCK), and bile salts stimulate pancreatic secretion. Inflammati

on of pancreas
thought to be secondary to autodigestion by proteolytic enzymes; ETOH (most comm
on in
U.S.) or gallstones are predominant causes. Other etiologies include: hvpertrigl
yceridemia
(>1000mg/dl), hypercalcemia, trauma, infectious, microlithiasis, medications (DD
I, Bactrim,
tetracycline pentamidine, oral hypoglycemics, furosamide/thiazide diuretics), tr
auma, pancreatic
divism, pancreatic cancer. 10% mortality rate.

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*note: ETOH is most common cause of pancreatitis, but the majority of alcoho
lics do not
develop pancreatitis.
*Splenic vein thrombosis- complication of acute and chronic pancreatitis; pr
esence of
gastric varices in absence of esophageal varices.
Clinical presentation:
-epigastric abdominal pain radiating to back worse after food ingestion.
-nausea/ vomiting
-fever, tachycardia
-decreased bowel sounds
-palpable pseudocyst
-Cullen's sign- occurs in necrotizing pancreatitis; blue discoloration in um
bilicus
secondary to hemoperitineum
-Turner's sign- occurs in necrotizing pancreatitis; blue discoloration of fl
anks secondary
to break down of hemoglobin in tissues
Prognosis: Ranson's criteria: (3 or more of following= higher mortality)
-On admission
*age > 55
*white blood cell count >16,000
*LDH> 350IU/L
*glucose > 200mg/dl
*AST(SGOT)> 250 lU/liter
-At 48 hours:
*PO2< 60%
*calcium< 8 mg/dl (hypocalcemia not treated)
*BUN increase> 5mg/dl
*Decrease in hematocrit by 10%
*Hypoalbuminemia < 3.2g/dl
*Fluid deficit > 4 liters
Diagnosis:
-clinical history
-elevated amylase and lipase (may be normal in hypertriglyceridemia)
*amylase: 3X normal; peak 2-3 days
*lipase: 3X normal; peak 7-14 days
-leukocytosis
-ultrasound
-abdominal computed tomography (CT)-phlegmon, pancreatic necrosis, pseudocyst,
or
abscess:
*phlegmon-inflamed pancreas; 48hours of onset

*pancreatic necrosis- 2 weeks of onset

*pseudocyst- collection of pancreatic fluid; occurs at 1-4 weeks; surgical rem
oval if > 5cm
and persists for > 1 month

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*abscess- CT guided aspiration. Occurs at 4-6 weeks
-left-sided pleural effusion- can occur if fistula between pancreas and pleura
l space
develops,
Treatment: 90% self-limited
-Bowel rest-NPO
-Supportive care- IVF hydration
-analgesics
-ERCP-if stone in common bile duct
-Surgical pancreatic debridement if infected pancreatic necrosis
-CT guided darainage of abscess or pseudocyst (if > 5cm and persists for > 1
month)
Chronic Pancreatitis:
pathogenesis: results from chronic inflammation of the pancreas. 70% secondary t
o chronic
ETOH use x 10 yrs. 30% idiopathic. Associated with pancreatic divisum and cystic
fibrosis
(most common in children).
Clinical presentation:
-epigastric abdominal pain
-steatorrhea- fecal fat > 40gm/day
-malabsorption- B12
-Diabetes (when 80% pancreas not functioning)
-Weight loss
Diagnosis:
-abdominal Xray- calcifications in pancreas
-low trypsin level
-secretin stimulation test (most sensitive): IV secretin administered which
stimulates
the pancreas. Pancreatic secretions of bicarbinate in duodenum is measured. Bica
rbinate> 80
mEq/L = normal pancreatic function
-ERCP
Treatment:
-pancreatic enzymes replacement
-medium chain triglycerides in diet (digestion independent of pancreatic enz
ymes)
-decrease fat intake
-vitamin supplements
-analgesics
-oral hypoglycemics for diabetics

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*NOTE: Diabetic patients secondary to pancreatic insufficiency are more prone to

hypoglycemia because of decrease glucogon produced from non- functioning pancrea

s; Do not
strictly control glucose level in these patients!
Pancreatic Cancer;
Pathogenesis: Increase incidence in U.S. Tobacco use associated with 2X increase
in risk.
Patients usually diagnosed in advanced disease. Increase incidence in black male
s. Increase
incidence in patients with chronic pancreatitis. 70% located in the head of the
pancreas.
Clinical presentation:
-painless jaundice
-abdominal/back pain
-weight loss
-depression
Diagnosis:
-CT or MRI
- Elevated CEA and CA 19-9
-endoscopic retrograde cholangiopancreatography (ERCP)
Treatment:
-surgery ( Whipple procedure-pancreaticoduodenectomy)-currative in 10-15%
-chemotherapy- 5-FU; poor prognosis-median survival is 6 months in unresecta
ble
tumors.
*PEARLS for Diseases of the Pancreas
* Pancreatitis: ETOH (most common in U.S.) or gallstones are predominant causes.
Amylase:
3X normal; peak 2-3 days; lipase: 3X normal; peak 7-14 days. 90% self-limited
* Chronic Pancreatitis: ETOH common cause. Diabetic patients secondary to pancre
atic
insufficiency are more prone to hypoglycemia so do not strictly control glucose
levels.
*Pancreatic Cancer: Tobacco use associated with 2X increase in risk. Commonly pr
esent as
painless jaundice;Elevated CEA and CA 19-9
DISEASES OF THE LIVER;
The Liver, unlike other organs, receives a rich dual blood supply which all
ows it to
filter toxins out of circulation. The liver breaks down exogenous (drugs, ETOH)
and endogenous
(bilirubin) toxin via enzymatic oxidation, reduction and conjugation reactions.
Enzymatic

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networks such as P450 system are involved in hepatic metabolism. Hepatic degrada
tion of
arnino acids is done via transaminases: aspartate amino transferase (AST) and al
anine amino
transferase (ALT).
*Jaundice-occurs secondary to increase of serum bilirubin (>2.5 mg/dl). Incr
ease in
serum bilirubin leads to yellow discoloration of tissues receiving heavy blood s
upply. Yellow
discoloration most visible on sclera and skin on physical exam. Darkening of uri
ne occurs

secondary to increase renal excretion of bilirubin. Stools can appear chalky. Hy

perbilirubinemia
can be secondary to liver disease (conjugated) or hemolysis (unconjugated). In l
iver disease
jaudice can be secondary to: hepatocellular disease, intrahepatic obstruction ca
using cholestasis (
derease in bile acid flow) or extrahepatic biliary obstruction.
*Cirrhosis- ETOH most common cause in U.S. Can also occur secondary to PBC,
PSC,
drugs/toxins, hemochromatosis, Wilson's disease, alpha 1-antitrypsin deficiency,
and
schistosomiasis as discussed below. Cirrhosis occurs secondary to irreversible l
iver injury.
Fibrosis, necrosis, and nodular regeneration are seen on pathology. Hepatocytes
loose functioning
and jaundice, coagulopathy, edema, ascites, gynecomastia, and encephalopathy res
ult. Portal
hypertension, subsequent esophageal varices (See figure 7), and splenomegaly can
develop.
Sclerotherapy and banding are used to treat varices. Propanolol prophylactically
decrease
incidence of rebleeding.
*Ascites- diagnosed by parecentesis. Calculation of Serum-ascites gradient (
SAAG)
helps to determine etiology. SAAG>l.lg/dl with a low ascitic total protein sugge
sts portal
hypertension (cirrhosis, liver failure) or myxedema. SAAG>1 and acsitic protein
high= Right
heart failure. SAAG <1 and high ascitic protein =nephrotic syndrome, pancreatiti
s, carcinoma,
and tuberculosis. Spontaneous bacterial peritonits (SBP) is more likely to devel
op when SAAG>
1 in portal hypertensive patients. Ascites is treated by fluid restriction, para
centeses, and diuresis.
Diurese < or =1 liter a day because this excedes the capacity of normal peritone
al resorbtion of
fluid and can lead to excess volume depletion. The increase in abdominal pressur
e caused by
ascites can decrease venous retun from the leg and lead to lower leg edema.
Cholelithiasis:
PatHogenesis: Cholelithiasis is a common disease with increasing worlwide preval
ence. 2-3X
more common in women. Prevalence of disease increases with age. More prevalent a
mong
Native Americans. Gallstones can originate from the gallbladder (cholesterol sto
nes) or
common bile duct (calcium bilirubinate/pigmented stones). 80% stones are cholest
erol and
gallbladder in origin. Other associated factors include: rapid weight loss, fami
ly hisory of
gallstones, pregnency, obesity, total parenteral nutrition, estrogen replacement
therapy, ileal
disease (Crohn's), hypertriglyceridemia, and older oral contraceptives. Pigmente
d stones are
associated with alcohol abuse, hemolytic anemia, clonorchis (biliary trematode)
and cirrhosis.
Impaired gallbladder motility and biliary sludge can also lead to gallstone form
ation.
Complications include acute cholecystitis, ascending cholangitis, and pancreatit
is. Also

associated with carcinoma of the gallbladder. Calcification of gallbladder sugge

sts carcinoma.

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Clinical presentation:
-2/3 asymptomatic
-right upper quadrant and epigastric pain lasting 30 minutes (often nocturna
l)
-common bile duct stones-persistant RUQ pain after cholecystectomy
-Charcot's triad in cholangitis: fever, jaundice and RUQ pain
Diagnosis:
-clinical history
-ultrasound (90% sensitive)
-ERCP for common bile duct stones
Treatment:
-asymptomatic- watchful waiting
-cholecystectomy-70% recurrence of pain within 2 years
-ERCP with sphincterotomy (for common bile duct stones)
-oral bile acids (UDCA)- effective in non-calcified cholesterol stones <5mm
-antibiotics for cholangitis
Primary Biliary Cirrhosis (PBC);
Pathogenesis: Autoimmune disease of chronic cholestasis(decrease in bile acid fl
ow) leading to
destruction of intrahepatic bile ducts. PBC is characterized by accumulation in
serum of
cholesterol, bilirubin, and bile acids, which are normally secreted in bile. Pre
dominantly found
in woman. Associated with autoimmune diseases such as Sjoegren's syndrome, rheum
atoid
arthritis, or sclerdoma. Approximately 30% of patients with PBC have thyroid dis
orders.
Predominantly found in middle aged women. Unknown etiology: environmental as wel
l as
genetic factors have been theorized to be involved in pathogenesis. Disease ulti
mately progresses
to cirrhosis. Median survival 12 years without therapy.
Clinical presentation:
-1/3 patients asymptomatic
-fatigue (most common)
-pruritus
-skin pigmentation
-hepatomegaly
-vitamin D dependent osteomalacia and progressive osteoperosis
-jaundice (in later stages)
-steatorrhea and malabsorption (late stage)
-30% associated hypo/hyperthyroid symptoms
-xanthomas and xxanthelasmias secondary to hypercholesterolemia)
Diagnosis:
-elevation of alkaline phostatase (AP) and (gammaglutamyl transpeptidase (GG
T) 2-5X

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476-628?
normal, with or without mild elevation of AST and ALT
-+ antimitochondrial antibody AMA (type M2) > 1:40.
-normal extrahepatic bile ducts on ultrasound
-Liver histology indicating inflammatory destruction of intralobular bile du
ctsand portal
granuloma (pathognomonic)
-Hypercholesterolemia (no increased risk of coronary artery disease because
HDL also
elevated)
-Increased ceruloplasmin
Treatment:
-Ursodeoxycholic acid (UDCA)-most promising; improves LFT's and symptoms
-Liver transplantation (in end stage disease)
-Calcium, Vitamin D replacement (in advanced stages)
-Cholestyramine, UV light, Opiate antagonists for pruritus
-dietary modification: medium chain triglycerides and decrease dietary fat
Primary Sclerosing Cholangitis (PSC);
Pathogenesis: Focal disease of cholestasis involving progressive destruction of
both intra and
extrahepatic bile ducts. Bile ducts have characteristic pearl-like appearance wh
ich is due to
strictures and dilations of the bile ducts secondary to fibrosis and inflammatio
n. The disease
ultimately progresses to cirrhosis. 2/3 patients male. Median age at diagnosis i
s 36-39 yrs of
age. Associated with Inflammatory bowel disease, predominantly Ulcerative Coliti
s (UC) in 8090% of UC patients (conversely 15% of UC patients have PSC). Also linked to smok
ing
behavior. Etiology unknown; genetic and immune factors thought to be involved. U
p to 15 % of
pateitns develop cholangjocarcinoma.
Clinical presentation:
-20-30% asymptomatic
-pruritus
-fatigue
-right upper quadrant pain
-jaundice
-steatorrhea and malapsorption
-osteoperosis
-signs of portal hypertension (later stages)
-associated with UC
Diagnosis:
-elevated AP and GGT (2x normal) with slight elevaton of AST and ALT
-characteristic stricturing, beading and irregularity of intra and extrahepa
tic bile ducts
seen on ERCP
-compatible liver biopsy revealing inflammation and fibrosis of hepatic duct
s
-increase in ceruloplasmin and bilirubin in later stages

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Treatment:

-Ursodeoxycholic acid (UDCA)-improves LFT's and symptoms

-Endoscopic dilation ofmajor bile ducts
-liver transplantation (in later stages)
-cholestyramine, opiate antagonists, UV light for pruritus
Hemochromatosis;
Pathogenesis: Disorder of iron storage secondary to increase intestinal absorpti
on of iron.
Excess iron deposit in tissues and organs. Iron predominantly deposited in liver
, pancreas,
pituitary, and heart. Ultimately progresses to fibrosis and failure of organs it
involves.
Etiology includes hereditary/genetic as well as iron overload disorders (siderob
lastic anemia
or thalassemia). Malfunction leading to increased intestinal absorption unkown.
Autosomal
recessive in origin. 5-10x more common in men 40-60 yrs of age. Up to 30% incide
nce of
hepatocellular cancer.
Clinical presentation: (more likely to be symptomatic in men secondary to lack o
f mensrtal
depletion of iron)
-skin pigmentation (90%)
-hepatomegaly (95%)
-diabetes mellitus (65%)
-arthropathy (40%)
-congestive heart failure (15%)
-hypogonadism
Diagnosis:
-clinical history
-elevated iron, transferrin, and ferritin
-liver biopsy: confirms diagnosis
-MRI
Treatment:
-phlebotomy-weekly or biweekly to remove excess iron
-chelating agents-deferoxamine
-liver transplantation - in liver failure
Wilsons Disease;
Pathogenesis: Genetically inherited disorder leading to impaired hepatic secreti
on of copper.
Distributes among all ethnicities, Excess copper accumulates and depostis in liv
er, brain,
cornea, kidneys. Basal ganglia dysfunction occurs with brain involvement. Predom
inantly in
adolescents -15-25 yrs of age.

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Clinical presentation:
-neurologic/psychiatric disturbance =first clinical sign. Neurologic-tremor,
chorea,
dysarthria. Psychiatric-bizarre behavior, schizophrenia, psychosis.
-Kayer-Fleischer rings in cornea pathognomonic (always accompanies
neurologic/psychiatric disturbance
-acute hepatitis-self limited; may progress to cirrhosis.
-arthritis
Diagnosis:

-low serum ceruloplasmin (in fulminant disease can be high due to liver rele
ase of
copper) and high hepatic copper
-high urine copper
-liver biopsy-confirm diagnosis
Treatment:
-penicillamine-chelation
-Liver transplantation-currative
Hepatitis; Systemic viral infection secondary to predominantly affecting the liv
er. ALT (SGPT)
and AST (SGPT) are transaminases in the liver. ALT only excreted by liver. AST-e
xcreted
anywhere in GI tract in addition to liver. In hepetitis damage predominantly aff
ects liver so
ALT> AST unlike ETOH hepatitis where opposite is true. In all hepatitis hepatic
necrosis with
mononuclear infiltrate is seen as well as cholestasis. There is also hepatic cel
l regeneration.
Cirrhosis can develop in severe chronic hepatitis. Fulminant hepatitis with mass
ive hepatic
necrosis is a risk in all viral hepatitis; however some have greater risks than
others. Pt with
fulminant hepatitis have signs and symptoms of hepatic failure: encephalopathy,
coagulopahty,
ascites, edema, etc.
Hepatitis A
Pathogenesis: single stranded RNA virus transmitted via fecal-oral route predomi
nantly;
however can also be transmitted sexually. Poor hygiene, contaminated food and sh
ell fish
increase risk of transmission. No transplacental transmission. Symptoms more com
mon in
adults. 1% risk of fulminant hepatitis. Incubation 15-50 days
Clinical presentation:
-jaundice
-low grade fever
-right upper quadrant pain
-prodromal symptoms (nausea, vomiting, fatigue, arthralgias, myalgias, anore
xia)
Diagnosis:
-hepatitis A virus (anti-HAV) IgM-indicates recent exposure

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-anti-HAV IgG-indicates previous exposure and immunity-positive forever
-elevated ALT and AST
Treatment:
-self-limited disease in most; supportive care
-inactivated vaccine recommended for patients with chronic liver disease, tr
avel to
endemic areas, and high risk behavior.
Hepatitis B
Pathogenesis: DNA virus of hepadnavirus type. Identified in all body fluids, blo
od, saliva,
synovial fluid, breast milk, ascites, cerebral spinal fluid, etc. Accounts for 5
0% cases of

fulminant hepatitis. High risk of hepatocellular carcinoama. Incubation 1-6 mont

hs. Risk of
developing chronic infection decreases with age (90% risk at birth and 5% risk i
n adulthood).
Associated with mixed cryoglobulinemia and polyarteritis nodosa.
Clinical presentation:
-jaundice
-fever
-right upper quadrant pain
-prodromal symptoms (nausea, vomiting, fatigue, arthralgias, myalgias, anore
xia)
-symptoms of fulminant hepatitis
Diagnosis:
-hepatitis surface antigen (HBsAG)=acute; eventually becomes undetectible an
d
followed by core antibody IgM; this is the "window".
-hepatitis core antibody (anti-HBc) IgM=acute; anti-HBc IgG=previous exposur
e and
persists for life.
-hepatitis B e antigen (HbeAg)- indicator of infectivity
-elevated ALT and AST; elevated prothrombin time (PT) with extensive necrosi
s
-hepatitis surface antibody (anti-HBs) alone indicates prior immunity via va
ccination. If
core antibody (anti-HBc) also present=previous infection and immunity
Treatment: check for loss of HbsAg after exposure to r/o chronic
progression.
-Supportive therapy
-Alpha interferon and ribaviron in chronic form (x 6mos -lyr)
-liver transplantation in fulmianant disease
-Hepatitis B vaccine( recombinant HBsAG) indicated for new borns, health car
e
workers, chronic liver disease; 95% protection. Hepatitis immune globulin (HBIG)
given for
post exposure prophylaxis. Hepatitis vaccine + HBIG given for known blood exposu
re; HBIG
alone for close contact w/out blood exposure.

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Hepatitis C;
Pathogenesis: Single stranded RNA virus. Transmitted predominantly via blood. Cu
rrently most
common amongst intravenous drug abusers and in prisons. Before 1990 accounted fo
r 90%
transfusion hepatitis. Risk of sexual transmission present, < 5%. Perinatal tran
smission 5%. Rate
of sexual transmission is low, 5%. Needlestick transmission 5-10%. Risk of hepat
ocellular
carcinoma 1-4% per yr in cirrhotics. Incubation 2wks-6 months. High risk of prog
ression to
chronic form, 70-80%. In U.S. gentype 1 most common. Associated with mixed
cryoglobulinemia.
Clinical presentation: only 25% symptomatic in acute setting
-jaundice
-fever

-right upper quadrant pain

-prodromal symptoms (nausea, vomiting, fatigue, arthralgias, myalgias, anore
xia)
Diagnosis:
-antibody to hepatitis C (anti-HCV)-can become undetectable after recovery;
does not
indicate immunity..
-HCV RNA- detected by PCR-most sensitive-remains detectable indefmately.
-elevated ALT and AST; elevated prothrombin time (PT) with extensive necrosi
s
Treatment: (2-4 mos post episode check for loss of HCV-RNA; persistance of
HCV-RNA= chronicity)
-Alpha interferon and ribaviron in symptomatic chronic disease x 6mos-lyr
-Liver transplanation in fulminant disease
-No vaccine available
Hepatitis D;
Pathogenesis: RNA virus. Infects either simultaneously with hepatitis B or as a
superinfection
in a person previously infected with hepatitis B. Risk of fulminant hepatitis gr
eater in
superinfection. Distribution is worldwide. Endemic in Mediterranean countries. P
redominantly
seen in patients exposed to blood (drug addicts and hemophiliacs). Does not incr
ease risk of
chronic hepatitis B infection if acutely acquired. *If anti-HBs present, then im
mune to Hepatitis
BandD
Clinical presentation:
-jaundice
-fever
-right upper quadrant pain
-prodromal symptoms (nausea, vomiting, fatigue, arthralgias, myalgias, anore
xia)

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Diagnosis:
-antibody to hepatitis D virus (anti-HDV)
-Elevated ALT and AST
Treatment:
-liver transplantation in fulminant disease
Hepatitis E: Similar to hepatitis A. Fecal/oral transmission. Seen in Africa, As
ia, Central
America. Detected by antibody to hepatitis E virus (anti-HEV IgM and IgG). No ch
ronic form.
Risk of fulminant disease high in pregnancy.
Hepatitis G: RNA virus similar to hepatitis C. Bloodborne.. Rick of acquisition
in transfusions.
Exact mode of transmission not identified. No chronic form.
Alpha 1-antitrypsin deficiency:
Pathogenesis: Autosomal recessive disorder in which patients harbor deficiency i
n serum
alpha 1-antitrypsin levels. Ultimately leads to chronic hepatitis and cirrhosis.
Emphysema often
develops in adulthood. 10% of children develop hepatitis and cirrhosis. Alpha 1antitrypsin

deficiency is responsible for 20% liver disease among neonates. In adults asympt
omatic
cirrhosis is most common presentation. Also associated with hepatocellular carci
noma.
Clinical presentation:
-asymptomatic with LFT abnormalities or symptoms of emphysema.
Diagnosis:
-electrophoresis demonstrating absence of alpha 1-antitrypsin.
-Confirmed by serum alpha 1-antitrypsin levels.
Treatmet:
-liver transplantation (not currative)
Autoimmune Hepatitis;
Pathogenesis: Chronic form of hepatitis results from cell-mediated immunologic a
ttack of
hepatocytes. Type 1 in adults and type 2 in children. More common in women. Insi
dious onset.
Associated with other autoimmune disorders (i.e. thyroiditis, rheumatoid arthrit
is) and
autoantibodies common. Can overlap with PBC and PSC rarely. Progresses to cirrho
sis.
Associated with hepatocellular carcinoma. Mortality as high as 40%.

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Clinical presentation:
-jaundice
-fatigue, malaise, arthralgias, amenorrhea
-anti-nuclear antibody (ANA)
-smooth muscle antibody (SMA)-most specific; in 80% patients.
-abnormal LFT's
Treatment: (alpha interferon worsens disease so viral hepatitis must be ruled ou
t!)
-prednisone
-Azathioprine-steroid sparing
-Liver transplantation in end stage
Hepatocellular Adenoma:
Pathogenesis: Benign tumor of the liver. Predominantly in women. Associated with
oral
contraceptive use. Usually right lobe of liver.
Clinical presentation:
-abdominal pain
-palpable mass
Diagnosis:
-Computed tomography (CT) and Magnetic resonance imaging (MRI)
Treatment:
-cessation of oral contraceptive use
-if >8-10cm surgical resection
-avoid pregnancy
Hepatocellular Carcinoma;
Pathogenesis: Among most common tumors world-wide. Common in Asia and Africa. In
crease
risk in patients with chronic hepatitis B and C. Associated with aflatoxin (pean
uts/ peanut
butter). Poor prognosis.
Clinical presentation:
-right upper quadrant abdominal pain

-abdominal mass in right upper quadrant

-bruit over liver
Diagnosis:
-CT, MRI, ultrasound
-Elevated alpha fetoprotein (AFP) levels; greater than 500mcg/l.

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-Percutaneous liver biopsy
Treatment:
-surgical resection in early detection
-chemotherapy
-liver transplantation.
Acetaminophen/tylenol induced hepatitis: Normally acitaminophen is metabolized v
ia
cytochrome P-450 system into toxic compounds which are further reduced by glutat
hione. In
tylenol overdose glutathione is depleted and results in accumulation of toxic co
mpounds which
damage the liver. In chronic ETOH ingestion/abuse and malnourished states glutat
hione is also
depleted. Consequently Acitominophen toxicity is worse in alcoholics and can lea
d to liver
transplantation. Acetominophen levels > 200 mcgm/ml ,at 4 hrs post ingestion = i
ncrease risk of
severe liver damage. Liver injury occurs 24-48 hrs post ingestion. Activated cha
rcoal and Nacetylcysteine used in treatment if acetominophen levels> 200mcg/dl.
Gilbert's Disease: Chronic unconjugated hyperbilirubinemia secondary to deficien
cy of
bilirubin glucoronyltransferase.Autosomal dominant. Patients present in their 20
's. Prevalence
3-10%. Pt often present jaudiced or with abnormal labaratory values. Jaundice wa
xes and wanes
and exacerbated by physical stress, infection, fasting, fever, surgery, or ETOH.
No therapy
indicated.
Dubin Johnson Syndrome; Autosomal Recessive disorder of conjugated hyperbilirubi
nemia
secondary to defect in biliary excretion. Pt have vague abdominal symptoms and j
aundice.
Jaundice worse during infections, pregnancy, and on oral contraceptives. Liver h
istology reveales
black pigment in hepatocytes. Rotor syndrome like dubin Johnson except is second
ary to defect
in hepatic storage and hepatocytes are without pigment.
*PEARLS for liver diseases;
* Cholelithiasis: 2-3X more common in women. 80% stones are cholesterol and gall
bladder in
origin, 10% pigmented stones. Calcification of gallbladder suggests carcinoma.Ul
trasound
90% sensitive in diagnosis. If asymptomatic- watchful waiting recommended. If sy
mptomatic,
cholecystectomy-70% recurrence of pain within 2 years. Treatment by ERCP with
sphincterotomy for common bile duct stones.
* Spontaneous bacterial peritonits (SBP): more likely to develop when SAAG> 1 in

portal
hypertensive patients.
*PBC: common in women; +AMA. Diagnosis confirmed via liver biopsy (portal granul
oma

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pathognomonic). Effects intrahepatic bile ducts. Treated w/ UDCA and liver trans
plantation.
*PSC: associated w/ Ulcerative Colitis, 80-90%; Effects predominantly extrahepat
ic bile
ducts. Increase risk of cholangiocarcinoma.. Diagnosed via ERCP and liver biopsy
. Treatment
w/ UDCA and transplantation (end stage).
*Hemochromatosis: elevated iron, transferrin, and ferritin; liver biopsy: confir
ms diagnosis.
Treatment w/phlebotomy.
*Wilson's disease: neurologic/psychiatric disturbance =first clinical sign. Kaye
r-Fleischer
rings in cornea pathognomonic; diagnosed via low serum ceruloplasmin and high he
patic
copper levels. Treat w/ penicillamine.
*Hepatitis A: single stranded RNA virus transmitted predominantly via fecal-oral
route; selflimited in most cases.
* Hepatitis B: DNA virus of hepadnavirus type. Identified in all body fluids. Ri
sk of developing
chronic infection decreases with age. Associated with mixed cryoglobulinemia and
polyarteritis nodosa. Treatment w/ alpha interferon and ribaviron in chronic for
m
* Hepatitis C: Single stranded RNA virus.Bloodborne. High risk of progression to
chronic
form, 70-80%. Associated with mixed cryoglobulinemia. Treatment w/ alpha interfe
ron and
ribaviron in symptomatic chronic disease.
* Hepatitis D: RNA virus. If occurs as superinfection in a person previously inf
ected with
hepatitis B, risk of fulminant hepatitis greater.
* Autoimmune Hepatitis:+ANA and SMA-most specific; Alpha interferon worsens dise
ase so
viral hepatitis must be ruled out. Treat with prednisone
* Hepatocellular Carcinoma: Increase risk with chronic hepatitis B and C. Associ
ated with
afl a toxin. Alpha fetoprotein (AFP)> 500mcg/l.
* Acetaminophen induced hepatitis: N-acetylcysteine used in treatment if acetomi
nophen
levels> 200mcg/dl.