Gastroenterology Flashcards
Causes of dysphagia
Neuromuscular motility disorders - solids and liquids
- Achalasia
- Scleroderma
Esophageal obstruction - solids
- Strictures - d/t esophageal reflux, alkali ingestion, or radiation to chest
- Cancer
- Esophageal webs or rings
Plummer-Vinson Syndrome
Esophageal webs
Dysphagia
iron deficiency anemia
Diagnostic testing for dysphagia
EGD - most common
Barium swallow - when diverticula/risk of perf is high
Manometry if EGD unrevealing and/or esophageal motility disorder suspected
Radiologic studies for anatomic structures of GI tract
barium swallow - esophagus, LES, stomach
Gastric emptying study - stomach, pyloric sphincter, duodenum - eval gastroparesis
Small bowel follow-through (SBFT) - stomach to terminal ileum
Barium enema - rectum to appendix
Feeding methods when unable to eat by mouth and complications/risks associated
NG tube - complication: worsening GERD, pressure necrosis
Percutaneous endoscopic gastrostomy (PEG) tube
TPN - risk sepsis, infections including fungal with central line; increased risk bile stasis and calculus cholecystitis
Causes of pseudoachalasia
chagas dz
neoplasm
scleroderma
Achalasia
Impaired peristalsis, decreased LES relaxation d/t intramural neuron dysfunction
Clinical features
- Progressive dysphagia of solids and liquids
- Regurgitation, cough, aspiration, heartburn
- wt loss - from poor intake
Diagnostics:
EGD r/o neoplasm
Manometry - increased LES pressure, incomplete LES relaxation, decreased peristalsis
“Birds beak” on barium swallow
Tx:
Pneumatic dilation and myotomy - risk GERD or perf
-botox
-nitrates and/or dihydropyridine CCBs - cardiac effects
Diffuse esophageal spasm
disorder, non peristaltic contractions of lower esophagus
CP and dysphagia of liquids and solids
Dx:
barium swallow - corkscrew pattern
Manometry - non peristaltic, uncoordinated esophageal contractions
Endoscopy - r/o structural disorders
Tx: CCB, TCAs
Nitrates relieve pain but worsen reflux
Esophageal diverticula and location
Zenker diverticulum - immediately before upper sphincter
Traction diverticulum - mid esophagus
Epiphrenic diverticulum - immediately above LES
Zenker diverticulum
Outpouching in upper posterior esophagus d/t striated muscle weakness
halitosis difficulting instating swallowing regurgitation of food several days after eating it Dysphagia Aspiration
Dx: barium swallow - EGD dangerous - risk perf
Tx: cricopharyngeal myotomy and diverticulectomy
Complications: aspiration pna, squamous cell carcinoma
GERD
transient relaxation of LES -> reflux of gastric content into esophagus
Risk: obesity, hiatal hernia, pregnancy, scleroderma
Burning CP 30-90 min after eating sour taste regurgitation Nausea Cough Aggravating factors: etoh, fatty foods, tobacco, lying down
Dx: - clinical
EGD with tx failure/red flags (bleeding, wt loss, dysphagia, odynophagia, protracted vomiting)
Ambulatory pH monitoring to verify GERD
Manometry assess other causes of dysphagia
Tx: refractory: nissen fundoplication
Complications of GERD
ulceration -> bleeding stricture Barrett's esophagus Adenocarcinoma Reflux-induced asthma laryngeal disorders
Squamous cell carcinoma of esophagus
MC worldwide
Risk: alcohol and tobacco use
Adenocarcinoma of esophagus
more common in US
Risk: obesity, tobacco use, barrett’s esophagus
Esophageal cancer - clinical features, Dx, Tx
Features: progressive dysphagia Wt loss Odynophagia, reflux GI bleeding Vomiting, weakness, cough, hoarseness
Dx:
barium swallow - esophageal narrowing and mass
Test of choice: EGD - bx
MRI, CT, PET scan - determine extension and metastases
Tx:
Total esophagectomy for early disease
Radiation and chemo in advanced or as neoadjuvant therapy
Poor prognosis
Mallory weiss tear vs boerhaave syndrome
MW: longitudinal mucosa laceration - MC distal esophagus/proximal stomach
BS: esophageal perforation or rupture - MC distal; life threatening
Hiatal hernia - types and tx
Sliding - MC
- GE junction and stomach displaced through diaphragm
- Tx with PPIs
Paraesophageal
- stomach protrudes through diaphragm, GE junction remains in normal location
- risk incarceration, ischemia
- Tx: surgical repair, especially w/ sxs
Dx: barium swallow
Gastritis
Causes:
H.pylori, NSAIDs, etOH, smoking, severe illness, autoimmune dz, Crohn dz, radiation
Clinical features: epigastric pain with tenderness N/V loss of appetitie early satiety wt loss
DX:
EGD
H. pylori - urea breath test, antral bx, serum Ab (+ if ever exposed), stool antigen
Tx:
Stop offending meds
H. pylori negative: PPI, H2 blockers
H. pylori positive: PPIs, Amox (metronidazole if pcn allergic), clarithromycin x2 weeks
Gastric vs duodenal ulcer Age - Cause- % of cases Pain occurs - Gastric acid - Gastrin level -
Gastric: Age - older Cause- H. pylori, NSAIDs % of cases - 25% Pain occurs - soon after eating -> N/V Gastric acid - normal or low Gastrin level - high
Duodenal: Age - younger Cause- H pylori >90% % of cases - 75% Pain occurs - 2-5 hours after eating; eating initially improves, worse later Gastric acid - high Gastrin level - low
Curling ulcer
Severe burns -> duodenal ulcer
reduced plasma volume -> ischemia and cell necrosis
Cushing ulcer
Increased ICP -> stimulation of vagal nuclei
-> increased gastric acid secretion
What do you check if GERD refractory to treatment?
gastrin level
R/o Zollinger ellison sn
PUD
Sx: GERD epigastric pain N/V bleeding
Dx:
Abd XR - perf, free air under diaphragm
Barium study - collection of barium in ulcer pits
EGD - most effective - bx - r/o cancer, test for H. pylori
Tx: Control active bleeding Acid suppression Protect mucosa Eradicate H. pylori Severe dz: parietal cell vagotomy or antrectomy
Types of gastric cancer
Adenocarcinoma - MC
squamous cell carcinoma
Linitis plastica - all layers of stomach - leather bottle - poor prognosis
Gastric cancer - risk, features, markers, dx, tx
Risk:
H. pylori, FHx, tobacco use, etoh use, consumption of nitrosamine (preserved foods), Men > women
Features:
Wt loss, anorexia, early satiety, vomiting/dysphagia, epigastric pain
Virchow node - left supraclavicular LN
Sister Mary Joseph node - periumbilical node
Markers:
CEA - increased in 50%
CA 19-9 increased in 20%
Tx:
distal 1/3 - subtotal gastrectomy
Middle or upper - total gastrectomy (or invasive lesions)
Chemo and radiation
Early detection - 70% cure rate
Most late = poor, less than 15% 5 yr survival
Nutritional deficiency associated with surgical therapy for obesity
Iron, vit B12, folate, thiamine, vit D
Most common causes of SBO
A - adhesions - 70%
B - bulge - incarcerated hernias 10%
C - Cancer - colon, ovarian mets 15%
Less common causes of SBO
Bowel wall inflammation - Crohn disease, appendicitis, diverticulitis
Bowel wall hematoma d/t trauma
Strictures - Crohn dz, radiation enteritis, prior surgery
Gallstone ileus
Bezoar
Intussusception
Volvulus - large > small intestine
S/S of SBO
N/V
Diffuse abdominal pain and distension
No BM or flatus
dehydration -> orthostatic hypotension, tenting of skin
Bowel sounds high pitched and hyperactive early - “tinkly”
Later hypoactive
Abd XR: distended loops of bowel, multiple air-fluid levels
Management of SBO
NPO - bowel rest
IVF
Correct electrolyte derangements - esp vomiting
NG tube - low intermittent suction to decompress
Hospital observation with frequent assessments
Avoid opioids - worsen disease
surgery - laparotomy and lysis of adhesions
Whipple disease
Malabsorptive dz - Tropheryma whipplei infection
White males
Abd pain Diarrhea Wt loss JOINT PAIN Neurologic problems - dementia, cerebellar ataxia
Dx: intestinal bx:
- blunting of villi
- lamina propria filled with fat droplets and PAS+ foamy macrophages (bright pink) contain T. whipplei
Tx:
IV ceftriaxone x2 weeks
TMP-SMX x 12 mo to prevent relapse
Features of malabsorption
Wt loss edema - protein malabsorption Diarrhea - osmotic load Steatorrhea Glossitis - B vit def Dermatitis - niacin def, zinc def
Site of ethanol absorption
stomach
Vit B12 site of absorption
terminal ileum
Tropical sprue
Caribbean, India, SE Asia
Steatorrhea, Chronic D - fat malabsorption
Megaloblastic anemia - B12/folate malabsorption
Abdominal distention - sugar malabsorption
Pedal edema - protein malabsorption
Bx:
blunting villi
Inflammatory cells in lamina propria
Dx of exclusion
Tx: tetracycline
Folic acid 3-6 mo
B12 shots
Celiac sprue
Sensitive to gluten - gliadin
Northern European ancestry
Bulky, foul-smelling diarrhea Steatorrhea Wt loss Iron def anemia Osteopenia Dermatitis herpetiformis - elbows, knees
Serology:
IgA anti tissue transglutaminase Ab - not rec anymore
Anti-endomysial Ab
Bx:
Blunting of villi
Hypertrophy of crypts
Tx: GF diet
Dapsone for dermatitis herpetiformis
Diagnostic testing for inflammatory diarrhea
Occult blood
fecal leukocytes
Diagnostic testing for steatorrhea
sudan stain (qualitative) Quantitative fecal fat - 72 hr stool collection
D-xylose test
Carbohydrate malabsorption
tests for passive absorption of carbs, normal suggests pancreatic insufficiency
Lactose breath hydrogen test
Carbohydrate malabsorption
measure H content inn reach following oral lactose challenge - high breath H suggests lactase deficiency
Lactose absorption test
Carbohydrate malabsorption
measure blood glucose following oral lactose challenge - failure of blood glucose to rise suggests lactase deficiency
Stool pH test
Carbohydrate malabsorption
lactase deficiency - not reliable
Formula and interpretation of stool osmotic gap
290 - 2 (Na+stool + K+stool)
normal 50-100
> 125 - osmotic diarrhea - extra osmoles in stool
less than 50 - secretory diarrhea
Causes of osmotic diarrhea and definition
watery diarrhea - water drawn into lumen by undigested solutes
-better when stop eating
lacunose, milk of magnesia
Carbohydrate malabsorption - celiac, Whipple dz
Fat malabsorption - pancreatic insufficiency
Causes and definition of secretory diarrhea
High volume stool output even when fasting
Carcinoid syndrome
VIPoma, gastrinoma
Cholera
ETEC
Viral gastroenteritis - adults vs kids
Adults: norovirus MC, rotavirus, adenovirus, astrovirus
Kids: rotavirus
Diarrhea, N/V, abdominal pain
Low grade fever
Blood or mucus in stool rare
labs - normal
Tx: hydrate
Rotavirus
very common in kids profuse diarrhea - bright green/yellow, foul smelling dehydration winter months ELISA or PCR
Norovirus
Vomiting prominent
diarrhea
Winter months
Bacillus cereus
Fried rice
Sxs within hours of eating
Tx: hydration
Campylobacter jejuni
Poultry
2nd MC foodborne bacterial GI infection
can have Bloody D, often watery
Abdominal pain
Fever
Rare association w/ Guillian-Barre 2-3 weeks after; reactive arthritis
Tx: hydration
severe cases - fluoroquinolone (cipro, levo) or azithromycin
Clostridium botulinum
Honey and home canned foods - preformed toxin = quick onset
N/V/D
B/l symmetric DESCENDING weakness starting with b/l CN neuropathies
Tx: monitor closely
Intubate if needed
Botulinum antitoxin with PCN G
Infant botulism
honey
colonize GI tract - release toxin in vivo
Clostridium difficile
Superinfection begins after use of broad-spectrum abx - Clindamycin
watery or blood diarrhea
can develop pseudomembranous colitis
Tx: metronidazole or vancomycin PO
-recurrent - adjunct tx - cholestyramine - binds toxin
Enterotoxigenic Escherichia coli
contaminated food and water - foreign travel
watery diarrhea, V, fever
Tx: hydration
Enterohemorrhagic Escherichia coli type O157:H7
undercooked ground beef
Bloody diarrhea, vomiting, fever, abdominal pain
Hemolytic uremic syndrome
Treatment - hydration, supportive
Abx worsens d/t toxin release
Hemolytic uremic syndrome (HUS)
Thrombocytopenia
hemolytic anemia
Acute renal failure -> risk death
Staphylococcus aureus
poultry, dairy, eggs, produce (room temp) - picnic salads
early sxs d/t toxin - Vomiting within hours, D later
tx: hydration
Salmonella spp.
MC: food borne bacterial GI infection
Raw meat, poultry, fresh produce
bloody diarrhea, fever, vomiting
Tx: hydration
Immunocompromised or severely ill: fluoroquinolone - cipro/levo
Shigella spp.
food and waterborne - overcrowding population
“Bacterial dysentery”
Fever N/V Severe bloody diarrhea Abdominal pain HUS
Tx: hydration, fluoroquinolone (cipro), TMP-SMX
Vibrio cholerae
“rice water diarrhea”
seafood
Copious watery diarrhea
Severe electrolyte imbalances
death
Tx: aggressive hydration
Tetracycline, doxycycline decrease duration
Vibrio parahaemolyticus
seafood - oysters
Tx: hydration
Yersinia enterocolitica
pork, produce, puppy feces
Diarrhea
20% pharyngitis
Pseudoappendicitis
Tx: hydration
Giardia lamblia
surface water, streams
greasy, foul-smelling diarrhea
abdominal pian
malaise
dx: cysts and trophozoites in stool
Tx: metronidazole
Entamoeba histolytica
water/streams
“Amoebic dysentery”
Bloody diarrhea - mild to severe
abdominal pain
Severe disease -> liver abscess
Tx: metronidazole, paromomycin
Cryptosporidium parvum
food or waterborne
Immunocompromised - HIV
Watery D
abdominal pain
malaise
acid-fast stain shows parasites
Tx: nitazoxanide
Trichinella spiralis
worm
undercooked pork
fever myalgia PERIORBITAL EDEMA eosinophilia CNS sxs Cardiac sxs
Tx: albendazole, mebendazole
Taenia solium
worm
undercooked pork
mild diarrhea
CNS symptoms
Taeniasis - tapeworm in gut only
Cysticercosis - cysts in muscle
Neurocysticercosis - cysts in brain
Tx: praziquantel for gut infection; albendazole for CNS infection +/- corticosteroids
Approach to acute diarrhea
Non - severe: self limiting
Severe: hypovolemia, bloody stools, fever, >6 stools per day, duration >1 week, severe abdominal pain, older age and immunocompromised
Testing:
Fecal leukocytes, stool cx, +/- ova and parasites if immunocompromised, +/- Giardia and E. histolytica if assoc with surface water; c. diff if recent abx
Empiric abx: fluoroquinolone for 3-5 days
-bloody diarrhea, fever, severe dehydration, immunocompromised state
Irritable bowel syndrome
Dx of exclusion
Sxs: chronic abdominal pain, D and/or C, bloating, N, mild abd tenderness, frequent/urgent urinary sxs, fibromyalgia sxs
Rome criteria - recurrent abd pain/discomfort at least 3 days/mo in last 3 mo associated with at least 2:
- relief with defecation
- Onset assoc w/ change in frequency of stool
- Onset assoc with change in form of stool
Not consistent with IBS: anorexia, wt loss, malnutrition, progressively worsening pain, pain that prevents sleep, rectal bleeding
-electrolyte disturbances, anemia, increasing inflammatory markers
Non pharm tx:
High fiber diet, avoid gas producing foods, avoid lactose/gluten controversial
Psychotherapy to relieve stress
Pharma:
Constipation predominant: psyllium, polyethylene glycol, lubiprostone, linaclotide
Diarrhea predominant: loperamide (imodium), eluxadoline
Abd pain and bloating: antispasmodics - diclycomine, hyoscyamine
Antidepressants - SSRI for pain
Crohn disease
Reaction to intestinal flora
Transmural inflammation - anyway mouth to anus
Spares rectum - skip lesions
Barium study: “String sign”, cobblestone pattern
Presentation:
strictures, D, malabsorption, wt loss, rare bloody stools, fistulas
Arthritis (HLA-B27), immunologic problems, dermatologic - erythema nodosum
Increased colon cancer risk - not as much as UC
Tx: Mesalamine, sulfasalazine - 5-ASAs Azothioprine, mercaptopurine, methotrexate Infliximab, adalimumab - anti-TNF Steroids Abx
Ulcerative colitis
Autoimmune reaction
Inflammation limited to colon
Continuous inflammation - always affects rectum
Inflammation limited to mucosa and submucosa
Barium study - “Lead pipe” obliterated haustra
Bx: crypt abscess, ulcerations
Presentation:
bloody D, malnutrition, increased risk of colon CA
PSC, pyoderma gangrenosum
HLA-B27 - sacroilitis, uveitis
Tx: Sulfasalazine 6-mercaptopurine infliximab colon resection - curative
Ileus
Disruption of normal propulsive ability of bowel d/t decreased peristalsis
MC: recent surgery
infection, opiates, DM
Vague abd pain N/V bloating No BM Decreased bowel sounds mild abdominal tenderness No rebound Unable to tolerate meals
Abd XR: mild distension of affective bowel and air-fluid levels
Barium enema - r/o true obstruction
Tx: erythromycin, neostigmine, or metoclopramide
Decrease or stop opioids
IVF
NPO +/- NG tube
Serial abdominal exams to monitor worsening
Volvulus
elderly, children
Air-filled loop of sigmoid colon twists about mesentery
Severe -> obstruct, impair blood supply
Slowly progressive abd pain, N/C, late V
+/- double bubble on abd XR
+/- “bird beak” on barium enema
CT scan - dx
Tx: self limited
No signs of perf -> flex sig
If signs of perf -> laparotomy
detorsion followed by sigmoid resection - high recurrence
Intestinal ischemia
elderly
Mesenteric ischemia - small intestine - SMA
Ischemic colitis - large intestine - SMA/IMA
Watershed area highest risk - splenic flexure
Causes: Splanchnic vasoconstriction acute arterial occlusion Venous thrombosis or hypo perfusion chronic form d/t atherosclerosis - pain after eating
Abd pain out of proportion to exam
Elevated WBC - severe dz
XR: dilated loops of bowel, bowel wall thickening
Dx: CT angio
Tx:
Venous thrombosis - anticoagulate chronically
Arterial embolism - surgical laparotomy with embolectomy
Non-occulsive vasoconstriction - stop drugs causing vasoconstriction
Bowel necrosis - surgical resection
Appendicitis
Obstruction - fecalith, calculi, lymphoid hyperplasia, tumors
Infection
Periumbilical pain -> RLQ
McBurney’s point
Psoas sign - RLQ with passive hip extension
Rovsing sign - RLQ pain with LLQ palpation
WBC elevated with left shift - >17K assoc with perf
CT scan with IV/PO contrast - enlarged appendix, wall thickening, periappendical fat stranding
U/S in kids
Tx: IVF, correct electrolyte abnormalities
Non-perf: single dose cefoxatin, ampicillin/sulbactum, or cefazolin plus metronidazole
Perf: pip/tazo, ticarcillin/clavulanate or ceftriaxone plus metronidazole
Appendectomy
Carcinoid tumor
Neuroendocrine tumor - secretes serotonin
MC site ileum; lungs, rectum, appendix (MC appendix tumor)
Asx if not mets; +/- abd pain
Carcinoid syndrome: d/t large quantity of serotonin
“Be FDR” - Bronchoconstriction (wheezing), Flushing, Diarrhea, Right-sided valve dz (restrictive CM - serotonin induced fibrosis of valvular endocardium)
Dx:
Incidental in asx
24 hr urine - 5-HIAA if sxs
Tx: Non-metastatic - remove tumor Carcinoid syndrome: octreotide or depot lanreotide - suppress gut hormones, decrease motility Bronchoconstriction: b2-agonists Diarrhea: loperamide
Anorectal abscess
infection of anal crypt or hemorrhoid, hair follicle
throbbing rectal pain
fever
tenderness on DRE
Tx: abx and drain
Pilonidal disease
1 or more cutaneous sinus tracts in superior midline gluteal cleft
obstruction -> cysts with drainage, abscesses
painful
Tx: I&D, surgical closure of sinus tracts
Diverticulosis
MC sigmoid colon
MC cause of acute lower GI bleeding over 40 yo
Risk:
Low fiber/high fat diet, older age
Cramping, bloating, flatuence, irregular defecation
painless rectal bleeding
Dx:
barium enema
Colonoscopy - sclerose vessel
Tx:
high-fiber diet/low fat - control bleeding
Angiography - embolization
Resect if unable to control bleeding
Presentation of diverticulutis
mean age 63 yo
LLQ pain
constipation
fever
elevated WBC with left shift or normal
Perf: CXR - free air
CT - oral and IV contrast - localized bowel wall thickening, diverticula
Colonoscopy no role, risk of perf
Outpatient vs inpatient treatment for diverticulitis
Out pt: - mild
bowel rest, PO abx
Fluoroquinolone (cipro) + metronidazole
Amox-clav
TMP-SMX + Metronidazole
Inpatient: elderly, immunocompromised, significant co-morbiditities
High fever
Significant leukocytosis
Unable to maintain hydration
IVF
Amp-sulbactam
pip-taco
Ticarcillin-clav
S/S of acute abd -> surgical exploration via midline incision
Hemorrhoids
Internal - superior rectal v., above pectinate (dentate) line, painless
External: inferior rectal v. , below pectinate line, painful
Dx:
Sigmoidoscopy
Colonoscopy
Anoscope
Tx: sitz baths high fiber diet avoid straining Rubber banding surgical excision - significant post op pain
Anal fissures
posterior wall of anus
painful
2/2 trauma - defecation, anal sex
-> spasm of rectal sphincter
Tx:
1st line: stool softeners, adequate hydration, typical nitroglycerin
botox in sphincter muscle - prevent spasm
Partial spincterotomy -> 10-30% risk of fecal incontinence
Rectal fistulas (fistula in ano)
tract between rectum and other structure - MC skin
2/2 inflammation
easily infected
S/S:
pain with defecation
visible draining tract - pus or fluid
Tx:
Fistulotomy
If Crohns - abx, immunosuppressants
Complications of diverticulitis
colonic abscesses
fistulas
sepsis
Major risk factors for adenocarcinoma of pancreas
exocrine tumor
FHx Chronic pancreatitis DM Tobacco use High fat diet obesity physical inactivity
Pancreatic adenocarcinoma - presentation, dx, tx, complications
Presentation:
abd pain - gradual, gnawing, epigastric, may radiate to back
Wt loss - d/t anorexia, malabsorption; n/v
Jaundice - d/t biliary obstruction
+/- palpable abd mass
+/- palpable contender gallbladder (Courvoisier sign)
Dx: High bilirubin and alk phos CA 19-9 Mass visible on US or CT Bx via endoscopic US, ERCP, or percutaneous needle bx
Tx: Whipple procedure (no mets) - pancreaticoduodenectomy plus chemo +/- radiation
Advanced dz: palliative care - biliary stunting, duodenal stunting, pancreatic enzyme supplements
Complications: trousseau syndrome - migratory thrombophlebitis
Zollinger-Ellison syndrome (gastronoma) - presentation, dx, tx
Gastrin secreting tumor
More common in duodenum
MC type of functional pancreatic neuroendocrine tumor
Presentation:
PUD and D
Common in MEN type 1
Frequently malignant
Dx: Best test: fasting gastrin usually >1000 Secretin stimulation test: stimulates gastrin secretion in gastrinoma (usually suppresses gastrin) Imaging to locate tumor: -CT -MRI -Octreotide scan (somatostatin receptor scintigraphy, SRS) -Endoscopic U/S - can do FNA
Tx:
High dose PPIs or octreotide to suppress acid secretion
Surgical resection - if resettable
Insulinoma - presentation, dx, tx
insulin-secreting tumor -> hypoglycemia
Dx:
Labs: hypoglycemia, elevated fasting insulin level, elevated C peptide
CT or abd U/S to locate tumor
Tx:
Surgical resection if resectable
If not - diazoxide (prevents insulin release) or octreotide (shuts down production of insulin)
Glucagonoma- presentation, dx, tx
Glucagon-secreting tumor
Presentation:
Hyperglycemia and refractory DM
Necrolytic migratory erythema - painful, pruritic, migratory rash on face, perineum, sometimes extremities
Dx:
Labs: hyperglycemia, elevated glucagon level
CT, MRI, or octreotide scan to locate tumor
Tx:
Octreotide to suppress glucagon secretion
Surgical resection if possible
Frequently malignant and often widely metastatic at dx -> poor prognosis
VIPoma - presentation, dx, tx
Islet cell tumor secretes vasoactive intestinal peptide -> increased secretion of water into lumen and increased motility
Presentation: high volume water diarrhea
Dx:
Labs: elevated serum VIP, low stool osmotic gap (secretory)
CT, MRI, octreotide scan to locate tumor
Tx:
hydration and electrolyte replacement -esp K+
Octreotide to suppress VIP secretion
Surgical resection but usually mets at dx
major risk factors for cholelithiasis
Fat
Fertile
Females
over 40
OCPs and HRT
FHx
Prolonged TPN
Rapid weight loss
Cholelithiasis - presentation, dx, tx
Often asx
Postprandial RUQ abd pain - “biliary colic”, worse after fatty meals
Possible N/V, abdominal fullness
PE generally negative
Dx:
Labs normal
RUQ U/S shows hyperlucent gallstones
Tx: cholecystectomy - if symptomatic
Complications: acute cholecystitis, cholangitis, acute pancreatitis
Acute cholecystitis- causes, presentation, dx, tx
Causes:
gallstone obstruction
Acalculous - critically ill (ischemia/necrosis) or TPN
Features: Severe RUQ abd - may radiate to back or right shoulder Fever Possible N/V, anorexia Abd tenderness and guarding Positive Murphy sign
Dx:
Labs: leukocytosis with left shift; serum bilirubin and alk phos usually not elevated
U/S: gallstones, thickening of gallbladder wall; sonographic murphy sign
HIDA scan - gallbladder fails to fill normally
Tx: cholecystectomy for mild to moderate disease
Abx and biliary stenting for critically ill who can’t tolerate surgery (organ failure)
Acute cholangitis- presentation, dx, tx
Bile duct obstruction (MC gallstones, stricture, cancer of pancreatic or biliary tree) -> infection of proximal biliary tree
Features:
Charcot’s triad - fever, jaundice, RUQ pain
Reynolds pentad - AMS, hypotension (shock)
Dx:
Leukocytosis with left shift
Elevated conjugated bilirubin and alk phos
+/- elevated AST/ALT (hepatocyte damage)
Check blood cx - E.coli, Klebsiella, Enterococci
Best radiologic test: ERCP
Tx:
Broad spectrum abx x7-10 days - pip-taco, levofloxacin
Endoscopic biliary drainage
Delayed cholecystectomy once cooled off
Gallbladder cancer - presentation, dx, tx
90% adenocarcinoma - aggressive
Risk factors - mainly gallstones
Presentation:
Often asx - may cause abd pain, jaundice, wt loss, V
Palpable gallbladder
Dx:
Labs: elevated bili and alk phos
endoscopic U/S: gallbladder mass, thickening of wall, possibly gallbladder calcification (porcelain gallbladder 10-30% CA)
Tx:
Cholecystectomy and LN dissection and local hepatic resection (gallbladder fossa)
Post-op or palliative chemo/radiation
Primary Biliary Cholangitis- description, risk, presentation, dx, tx
Autoimmune destruction of intrahepatic bile ductules -> cholestasis -> cirrhosis (late)
Risk: Women 30-65 yo; assoc with Sjogren’s, CREST, RA
Features:
frequently asx - incidental finding
Sxs: fatigue and pruritis w/ excoriations
-pruritis often starting during pregnancy but not received postpartum
Skin changes: hyper pigmentation, xerosis, dermatographism
Xanthomas and xanthelasms
Hepatomegaly progressively worsens +/- splenomegaly
Malabsorption and steatorrhea from less bile acid secretion
Cirrhosis, jaundice, ascites, edema, and portal htn late
Dx:
Elevated alk phos, bili (late), cholesterol
ANTI-MITOCHONDRIAL Ab
ANA
Tx:
URSODEOXYCHOLID ACID - changes bile composition, delays progression, not curative
Liver transplant
Primary sclerosis cholangitis description, risk, presentation, dx, tx
Progressive inflammation, fibrosis, and sclerosis of intra AND extra hepatic bile ducts
Risk: males, 40 yo
Strong association with UC
Features:
Frequently asx
Fatigue, pruritis w/ excoriations, jaundice, hepatomegaly
Dx: Elevated alk phos +/- elevated bili ANA negative p-ANCA positive ERCP - irregular biliary stricturing and dilation "beads on string"
Tx: Liver treatment - no medical tx available
Complication: risk of cholangiocarcinoma
Causes of secondary sclerosis cholangitis
Intraductal biliary stones Surgical trauma or blunt abdominal trauma to biliary tree Drugs -IV chemo Recurrent pancreatitis Autoimmune pancreatitis AIDS cholangiopathy
Causes of unconjugated hyperbilirubinemia
Increased bilirubin production:
- hemolytic anemia
- hematoma breakdown
Impaired bilirubin uptake and storage:
- viral hepatitis
- Drugs - rifampin
Decreased UDPGT activity
- neonatal physiologic jaundice
- Gilbert syndrome
- Crigler Najjar Syndrome type 1 and 2
Causes of conjugated hyperbilirubinemia
Impaired bilirubin transport - can’t excrete
- Dubin Johnson Sn - black
- Rotor Sn
Biliary epithelial damage
- hepatitis
- cirrhosis
- liver failure
Intrahepatic biliary obstruction
- PBC
- PSC
- Drugs - chlorpromazine, arsenic
Extrahepatic biliary obstruction
- pancreatic neoplasm
- pancreatitis
- cholangiocarcinoma
- choledocholithiasis
- biliary strictures
Gilbert Syndrome
AR
Mild UDPGT deficiency
Mild jaundice following exercise, stress, fasting
Labs: mild elevated indirect bili (less than 3)
Tx: none
Crigler-Najjar type 1
AR
Severe UDPGT deficiency
Persistent neonatal jaundice at 1-2 days old
Kernicterus
labs: markedly elevated indirect bili (>5)
Tx:
Phototherapy
Plasmapheresis
Liver transplant as adolescent
Crigler-Najjar type 2
Mild UDPGT deficiency
Jaundice begins in childhood or adolescence
Labs: mildy elevated indirect bili
Tx:
Phenobarbital to induce UDPGT
Hepatitis symptoms
asx or…
malaise arthralgia fatigue N/V RUQ pain Scleral icteris HSM LAD
Rare -> fulminant failure -> coagulopathy, ascites
Hepatits A
Transmission: Fecal-oral, poor sanitation
Risk: international travel - S. and Central America
Labs:
Hep A IgM during illness
Hep A IgG after resolution or vaccine
Tx: supportive - self limited
Vaccine available - given 1 yr, 2 yr
Hepatitis E
Southeast Asia
Fecal/oral, contaminated water
More likely to cause fulminant hepatic failure in pregnancy
Labs: PCR, Hep E IgM
Tx: supportive
Hepatitis B
perinatal, sex (MC), blood
Chronic infection: 90% prenatal transmission; adults less than 5%
Extrahepatic: polyarteritis nodosa, nephropathy, aplastic anemia
Increased risk of HCC - U/S q6mo, +/- AFP
Significance of HBsAg
active disease
Significance of HBsAb
recovery from active infection or immunization
Significance of HBcAb
History of infection - IgM early, IgG late
Significance of HBeAg
Active viral replication, high transmissibility
Significance of HBeAb
low transmissibility
Significance of HBV DNA
active viral réplication, treatment indicated when high
Hepatitis D
Delta virus - defective pathogen requiring Hep B confection
Blood, sex
Highest mortality - 20%
Tx: pegylated IFN-a x 1 yr - causes flu like sxs
Hepatitis C
50-85% remain chronically infected
Increased risk of HCC - U/S q6 mo
Extrahepatic manifestations: membranoproliferazive glomerulonephritis, essential mixed cryoglobulinemia, lymphoma, thyroiditis, polyarteritis nods, porphyria cutaneous trade, lichen plans, DM
Blood and sex (rare)
Dx: Hep C Ab, RNA
Tx: Ledipasvir-sofosbuvir; ombitasvir-paritaprevir-ritonavir + dasabuvir
Types of alcoholic liver disease
Alcoholic fatty liver disease (steatosis)
- 90% of heavy drinkers >30 g etOH/day
- Fat droplets within hepatocytes
- rapidly reversible with abstinence
Alcoholic hepatitis (steatohepatitis)
- 33% of steatosis who continue to drink
- Fat infiltration plus neutrophil inflammation; hepatocyte swelling and necrosis; some fibrosis
Cirrhosis
- 50% of steatohepatitis
- progressive fibrosis surrounds nodules of regenerative hepatocytes
- becomes irreversible -> liver failure
- increased risk of HCC
Nonalcoholic steatohepatitis (NASH)
Causes: obesity, DM, hld, insulin resistance
Hepatic insulin resistance -> excess lipid accumulation in liver
->cirrhosis and HCC
Dx:
Chronically elevated LFTs
Liver U/S, CT, MRI: steatosis but cannot detect inflammation/fibrosis
Fibrosure
Tx: Avoid all etoh Wt loss Keep HbA1c under 7 TZD (pioglitazone) - improve LFTs, possible histology improvement
Alcoholic liver disease
Long asx period >20 yr
S/s: jaundice, anorexia, N/V, RUQ pain, abd distention, ascites, HSM, liver tenderness, muscle wasting, encephalopathy
Dx: Elevated AST/ALT 2:1, elevated alk phos, bili low albumin Prolonged PT/PTT Leukocytosis (neutrophil predominance) Macrocytosis (folate/B12 deficiency) Thrombocytopenia
Tx:
Stop etoh use
Diet mod
Liver transplant if abstinent for at least 6 mo
Findings consistent with hepatocyte failure in cirrhosis
Jaundice - impaired bilirubin conjugation
bleeding/bruising - impaired synthesis of coagulation factors (elevated PT/PTT)
Edema and ascites - impaired synthesis of albumin
Hepatic encephalopathy and asterisks - impaired ammonia metabolism
Impaired sex hormone metabolism -> testicular atrophy, gynecomastia, spider telangiectasis, palmar erythema
Treatment of hepatic encephalopathy
lactulose - lowers colonic pH, prevents ammonia reabsorption in GI tract, facilitates fecal ammonia excretion
Rifaximan - kills enteric bacteria that generate ammonia
Causes of portal hypertension
Prehepatic: portal vein thombosis
Intrahepatic:
- cirrhosis
- Hepatic parenchymal disease (alcoholic liver dz, PBC, PSC, amyloidosis)
- Granulomatous dz (sarcoidosis)
- Schistosomiasis (outside US)
Posthepatic
- Right sided heart failure
- Budd-Chiari syndrome
- Thrombosis of hepatic v.
Budd-Chiari Syndrome
thrombosis and occlusion of hepatic v or intrahepatic/suprahepatic portion of IVC
Presentation:
Acute: acute RUQ, hepatomegaly, rapid jaundice and ascites
Subacute/chronic: gradual ascites, LE edema, cirrhosis, portal htn over few months
Eventual liver failure and hepatic encephalopathy
Dx:
U/S - best test
Hepatic venography gold standard
Tx:
- thrombolytics
- Diuretics and anticoagulation
- Angioplasty
- Shunt
Clinical presentation of portal hypertension
HSM ascites portosystemic anastomoses (gut, butt, caput) -esophageal varices -Anorectal varices -Caput medusae -Renal varices -Paravertebral varices
Serum-ascites albumin gradient (SAAG)
Serum albumin - ascites albumin
> or equal to 1.1 - portal htn - low albumin
- Cirrhosis
- alcoholic hepatitis
- heart failure/constrictive pericarditis
- massive hepatic mets
- Budd-Chiari syndrome
less than 1.1 - ascites not due to portal htn High albumin ascites: -peritoneal carcinomatosis (ovarian CA) -Peritoneal TB -Pancreatitis -Serositis Low serum albumin -Nephrotic syndrome
High albumin + high LDH r/o cancer
Spontaneous bacterial peritonitis
Infection of ascitic fluid
Fever
+/- abd pain/tenderness
AMS common
Diarrhea 2/2 overgrowth of enteric bacteria (esp E. coli)
Dx: SAAG >1.1 Ascites gram stain and cx Ascites neutrophil count >250 Low ascites glucose High ascites LDH - lysis of neutrophils
Tx:
Cefotaxime, ceftriaxone x5 day - cover E.coli, Klebsiella, Enterococcus, Staph and strep
Albumin - maintains plasma volume -> preserves renal function -> reduces renal impairment and mortality
Treatment of portal hypertension
Ascites: daily morning dose of spironolactone + furosemide
Esophageal varices:
- propranolol or nadalol to prevent hemorrhage
- Bleeding: octreotide or terlipressin to reduce splanchnic blood flow; endoscopic ligation/sclerotherapy
Hepatic shunting or trans jugular intrahepatic portosystemic shunt (TIPS)
-increased risk of hepatic encephalopathy (no filtering through liver)
Hereditary hemochromatosis
AR
Hepatomegaly, abd pain, cirrhosis
DM
Skin hyper pigmentation
Hypogonadism (anterior pituitary) -> testicular atrophy, impotence, amenorrhea
Restrictive or dilated (MC) CM
Arthralgia and arthropathy - squaring off of end of bones, hook like osteophytes of 2nd-3rd MCP joints
Dx:
Elevated AST/ALT +/- hyperglycemia
Elevated serum iron, ferritin, and transferrin
Genetic testing
Liver bx - blue dots (iron granules) inside hepatocytes
Tx:
Phlebotomy
Rarely used - deferoxamine or other iron chelation
Avoid etoh
Risk HCC
Wilson disease
AR
Mean age 12-23
Hepatomegaly, hepatic steatosis, cirrosis -> liver failure
Neuro: dysarthria, dystonia, tremor, parkinsonism
Psych: depression, personality changes, psychosis
Kayser-Fleischer rings
Dx: Elevated LFTs LOW SERUM CERULOPLASM increased urinary copper genetic testing Liver bx - brown, steatosis, fibrosis
Tx:
Copper chelation - trientine (fewer s/e) or penicillamine
Zinc supplements (interferes with Cu absorption)
Restrict dietary copper (shellfish, organ meats, chocolates, nuts, mushrooms)
Liver transplant may be necessary
Alpha 1 antitrypsin deficiency
Panacinar emphysema d/t elastin destruction in lungs
mutated a1 antitrypsin accumulates in hepatocytes
Autosomal co-dominant
Increased risk of HCC
Dx:
Genetic testing
Liver bx
Tx: liver and lung transplant
Autoimmune hepatitis
Women > Men
Asx or subclinical can -> acute liver failure or cirrhosis
Dx:
Type 1 (classic): ANA and Anti-Smooth Muscle Abs
Type 2: Abs against Liver-Kidney Microsomal (LKM) Ag or liver cytosol antigens
Tx: glucocorticoids +/- azathioprine
Hepatic adenoma
Benign
Women 20-44 - OCP
Anabolic steroids
Glycogen storage disease type I and III
usually asx
+/- RUQ pain or fullness
Malignant transformation in 10%
Tx:
DC OCPs
Monitor with serial imaging and serial AFT
+/- resection if >5 cm
Hepatocellular carcinoma
Risk:
HepB/C
Cirrhosis
Aflatoxin - aspergillus contaminated corn, peanuts, soybeans
Presentation:
Decompensation of chronic liver disease
Wt loss, hepatomegaly, RUQ pain, hepatic bruit (highly vascular tumor)
Paraneoplastic syndromes
Dx:
elevated LFTs, elevated AFP
U/S - solid tumor; CT/MRI - malignant features
Core needle bx only if dx unclear on imaging - risk of bleeding
Tx:
Small: resect, chemo
Unresectable: liver transplant, radio frequency ablation, chemoembolization
Aggressive - high mortality
Paraneoplastic syndromes associated with HCC
Polycythemia - secretion of expo
Hypercalcemia - secretion of PTH-rP (like squamous cell lung CA)
Watery diarrhea - secretion of VIP and other factors
Hypoglycemia - high metabolic needs
Skin lesions
Cancers association with polycythemia as a paraneoplastic syndrome
Pheo
RCC
HCC
Hemangioblastoma
