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172 Cards in this Set
- Front
- Back
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What is the gender difference of Hirschprung disease?
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M:F = 4:1
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Hirschprung disease:
__1__ of the colon proximally to an __2__ segment of the rectum |
1. Dilation
2. aganglionic |
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Why does Hirschprung disease occur?
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faulty migration of precursors of intestinal ganglionic cells
- develop from the neural crest and migrate into fetal intestine |
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What is there a lack of in Hirschprung disease?
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Auerbach and Meissner plexuses
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How is Hirschprung disease diagnosed?
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Rectal biopsy looking for absence of ganglion cells
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How does Hirschprung disease present clinically?
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- delayed passage of meconium
- chronic constipation in a young child |
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What gene mutation is associated with Hirschsprung disease 50% of the time?
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RET gene or RET ligand gene
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What syndrome is Hirschsprung disease associated with?
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Down syndrome
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What other defects is Hirschsprung disease associated with?
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VSD
Hydrocephalus Meckel diverticulum |
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What can be a cause of acquired Hirschsprung disease?
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Chagas' disease and destruction of ganglion cells by leishmania
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What is Meckel Diverticulum a remnant of?
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Vitelline duct (Omphalomesenteric)
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Describe the Four 2's in Meckel Diverticulum
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- 2% of the normal population
- 2 feet from ileocecal valve - 2 cm in length - 2% symptomatic - 2% of ectopic ulcers |
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Decreased blood flow and ischemia of the bowel secondary to Atherosclerosis with thrombosis, thromboembolism, or reduced cardiac output from shock
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Ischemic Bowel Disease
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What group of people is Ischemic Bowel disease most common in?
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Older individuals
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What vessel supplies blood to the Small intestine?
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Superior Mesenteric Artery
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Twisting of a segment of the bowel on its vasculature mesentery, resulting in intestinal obstruction and infarction
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Volvulus
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Where does Volvulus most often occur?
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Sigmoid colon but also in the Small Intestines
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Telescoping of a proximal segment of the bowel into the distal segment
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Intussusception
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In adults what can cause Intussusception?
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Tumors
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Tortuous dilation of mucosal and submucosal blood vessels prone to rupture and bleeding
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Angiodysplasia
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What parts of the Colon are most susceptible to ischemia?
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Splenic flexure
Rectosigmoid junction |
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Where does Angiodysplasia most often occur?
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Cecum and Right Colon
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What age range is Angiodysplasia most common in?
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> 50 yoa
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What two diseases may Angiodysplasia be associated with?
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Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome) = spider veins
CREST syndrome |
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What is the clinical presentation of Angiodysplasia?
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Multiple episodes of Rectal bleeding
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Varicose dilation of anal and rectal submucosal venous plexuses
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Hemorrhoids
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What are 3 risk factors for Hemorrhoids?
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1. Constipation and prolonged straining during pooping
2. Pregnancy 3. Cirrhosis = Portal Hypertension |
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Internal Hemorrhoid:
- Covered with what mucosa? - What veins? |
1. Rectal mucosa
2. Superior Hemorrhoidal veins |
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External Hemorrhoid:
- Covered with what mucosa? - What veins? |
1. Anal squamous mucosa
2. Inferior hemorrhoidal veins |
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How do Hemorrhoid patients present clinically?
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streaks of bright red blood on hard stool
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What are 5 complications of Hemorrhoids?
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1. Bleeding
2. Thrombosis 3. Prolapse 4. Strangulation 5. Infection |
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Diarrhea: increased passage of feces including:
-frequency? -Volume? -Consistency? |
> 3-4 / day
> 250 g / day Soft, fluid, watery |
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Low volume, bloody, painful diarrhea
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Dysentery
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Describe the cause of Osmotic Diarrhea
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Caused by nonabsorbable osmotic substances in the intestinal lumen
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List some examples of things that cause Osmotic Diarrhea
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1. Mannitol or Sorbitol in Sugar-free gum
2. Magnesium salts 3. Milk with Lactase deficiency |
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Form of diarrhea where the intestinal cells secrete more water than they can absorb
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Secretory diarrhea
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List 4 causes of Secretory Diarrhea
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1. Cholera toxin
2. E. coli toxin 3. Enteropathogenic viruses - Norwalk, Rota 4. Vasoactive Intestinal Peptide |
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Describe Pancreatic cholera
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Pancreatic VIPoma secreting massive amounts of VIP causing extensive SECRETORY Diarrhea
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What are other names for Pancreatic cholera?
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VIPoma
Verner-Morrison Syndrome WDHA = watery diarrhea and resultant dehydration, hypokalemia, achlorhydria |
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Diarrhea that develops in the course of diseases that disrupt the intestinal layer or damage the mucosa
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Exudative diarrhea
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List some causes of Exudative diarrhea
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1. Invasive bacteria
- Shigella and Salmonella 2. Amoebes 3. IBD |
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Cause of viral diarrhea in infants 6-24 months
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Rotavirus
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Cause of viral diarrhea in epidemics, older children, or adults
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Calicivirus = Norwalk virus
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How does Cholera toxin work?
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activates cAMP which causes Cl- secretion into intestinal lumen = water follows
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Inadequate absorption of nutrients in the Small Intestine
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Malabsorption syndromes
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List the 3 modes of pathogenesis leading to Malabsorption syndromes
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1. Defective intraluminal digestion
2. Mucosal dysfunction 3. Transport of nutrients across the mucosa and thru the lymphatics |
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List 4 things that can cause malabsorption due to defective intraluminal digestion
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1. Pancreatic insufficiency
2. excess gastric acid production (Zollinger-Ellison Syndrome) = inactivates Pancreatic enzymes 3. Biliary obstruction 4. Bacterial overgrowth = deconjugation of bile salt |
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Excessive, large, sticky, stools that float = ?
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Steatorrhea
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Give some synonyms of Celiac disease (2)
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1. Non-tropical sprue
2. Gluten-sensitive enteropathy |
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Hypersensitivity to Gluten (and gliadin), resulting in loss of small bowel villi and malabsorption
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Celiac sprue
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What is the genetic predisposition to having Celiac Sprue?
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HLA-DQ2 (95%)
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In Celiac disease, what are people hypersensitive to?
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Gluten (gliadin)
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What pathological changes are seen in a small intestinal mucosal biopsy in Celiac disease?
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-flattening of villi
-elongation of crypts -mucosal inflammation |
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What parts of the SI are most common affected in Celiac disease?
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Proximal > Distal intestine > stomach
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What other disease is Celiac disease associated with?
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IgA-mediated Dermatitis Herpetiformis
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What gender does Celiac disease most commonly affect?
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Female (josh's girlfriend or ceCELIA)
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What antibodies are found in 90% of patients with Celiac disease?
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Anti-Endomysial Ab's
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How does a person with Celiac disease clinically present?
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Usually in childhood with Malabsorption and Steatorrhea
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Longterm-wise, what does Celiac Disease increase the incidence of?
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T-cell Lymphoma of the Intestines and Stomach
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What is Whipple disease caused by?
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Intracellular bacteria = Tropheryma whippelii
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What gender and age group does Whipple Disease usually affect?
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White males 30-60 yoa
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What is the diagnostic feature of Whipple Disease?
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Biopsy
- SI Lamina Propria contains numerous Macrophages leading to widening of the villi - Macrophage stain with PAS or E.M. and contain bacteria (Tropheryma whippelii) |
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How does a patient present clinically with Whipple disease?
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Chronic diarrhea with abdominal pain, polyarthralgia, skin pigmentation, and anemia
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Rare infectious disease involving many organs, including small intestines, joints, lung, heart, liver, spleen, and CNS
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Whipple disease
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Why does malabsorption occur in Whipple disease?
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obstruction of the lymphatics in the intestinal villi due to macrophages
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What are the 2 types of Inflammatory Bowel Disease?
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1. Ulcerative colitis
2. Crohn's disease |
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Systemic inflammatory disease of unknown etiology affecting predominantly the Colon and Terminal Ileum
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Crohn Disease
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What ethnicities/races are more often affected by Crohn's Disease?
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Whites
Ashkenazi Jews |
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What age range does Crohn's disease affect most?
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15-30 years old
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What is the distribution of Crohn's disease?
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Mouth to Anus but "discontinuous/skips"
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What is the intestinal wall like in Crohn's disease?
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Thickened and rigid = due to chronic transmural inflammation
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Describe the lumen in Crohn's disease
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Stenosis = "string sign" on barium studies
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Describe the mucosa in Crohn's disease
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Deep linear ulceration with cobble-stone like pattern
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Describe the inflammation in Crohn's disease
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Transmural chronic inflammation which leads to thickening of the intestinal wall
- covered with fat tissue under the serosa |
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List some complications of Crohn's disease
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1. Fistulas
2. Obstructions 3. Adhesions |
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Are granulomas seen in Ulcerative colitis or Crohn's?
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Crohn's disease
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How does a person with Crohn disease clinically present?
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-recurrent episodes of diarrhea
-tenesmus, cramps, blood in stool - recurrences are common, last longer, with shorter and shorter asymptomatic intervals |
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Chronic systemic inflammatory disease of unknown etiology, predimonantly limited to the mucosa of the colon
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Ulcerative colitis
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What is the distribution and location of Ulcerative Colitis?
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Ulceration begins in rectum and spreads proximally to involve the entire colon
- no skipped areas = continuous |
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Describe the inflammation in Ulcerative Colitis
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limited only to the mucosa
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Describe the gross appearance of Ulcerative Colitis
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- extensive ulceration
- pseudopolyps |
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Remnants of normal or regenerating colonic mucosa surrounded by ulcerations = ?
What are they associated with? |
Pseudopolyps in Ulcerative colitis
Ulcerative Colitis |
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Accumulation of neutrophils inside colonic crypts = ?
What are they associated with? |
Crypt abscesses in Ulcerative colitis
Ulcerative Colitis |
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What is Toxic Megacolon?
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Complication of Ulcerative Colitis
- massive dilation of the entire large intestine |
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List 5 Extraintestinal complications of IBD (Crohn's / UC)
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1. Migratory polyarthritis
2. Iridiocyclitis = inflammation of eye and iris 3. Pyoderma gangrenosum = pus coming out of skin 4. Erythema nodosum = red nodules on skin 5. Primary sclerosing cholangitis = fibrosis around the common bile duct leading to jaundice |
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IBD that has a greater risk of developing cancer
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Ulcerative colitis
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Outpouching of mucosa and/or muscularis through a defect in the intestinal wall
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Diverticulosis
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What are the 2 types of Diverticulosis and where are they most often located?
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1. Congenital = Small intestine
2. Acquired = Sigmoid colon |
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What parts of the world is Diverticulosis more common and why?
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Developed nations due to constipation related to low-fiber diets
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What is the etiology of Diverticulosis? (2)
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1. straining during defecation
2. defect in the muscle layer |
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What are 3 complications of Diverticulosis?
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1. Diverticulitis
2. Pericolitis = inflammation around the colon 3. Rupture |
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How does Diverticulitis clinically present?
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1. "Left-sided appendicitis" = Left lower quadrant pain
2. Fever 3. Leukocytosis |
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What is the cause of Diverticulitis?
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Stool impacted in the diverticulum sac
- bacteria = inflammation - ischemia and ulceration |
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When a segment of the bowel becomes imprisoned within a hernia which can lead to intestinal obstruction and infarction
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Incarcerated hernia
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Fibrous tissue between 2 loops of bowel = ?
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Adhesion
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Lack of interstinal peristalsis associated with stagnation of intestinal contents = ?
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Paralytic Ileus
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What is the most common cause of Ileus?
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Abdominal surgery
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What would be a myopathic disease causing Ileus? Neuropathic?
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Myopathic = Myasthenia
Neuropathic = Hirschsprung |
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Most common intestinal polyp
- small, dew-like, glistening nodules |
Hyperplastic polyps
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Where are Hyperplastic polyps most often found?
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Rectum or Sigmoid colon
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What are Juvenile polyps?
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-Acquired hamartomas typically found in the rectum of children <10 yoa
-Pedunculated, round, with smooth surface -Composed of mucus-filled cystic glands and edematous, inflamed stroma |
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What is the inheritance pattern of Peutz-Jeghers polyps?
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Autosomal dominance
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In Peutz-Jeghers Syndrome, what is also present besides the polyps?
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Pigmented macules on the lips and perioral skin
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Ulcerative colitis or Crohn's disease: inflammatory pseudopolyps?
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Ulcerative colitis
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List the 3 categories of Neoplastic Polyps
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1. Tubular adenoma
2. Villous adenoma 3. Tubulovilous adenoma |
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Most common neoplastic polyp
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Tubular adenoma (>90%)
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What is the distribution of Tubular Adenomas?
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equally distributed throughout the entire large intestine
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What are Tubular Adenomas composed of?
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Tubular glands lined by dysplastic columnar epithelium
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What % of Tubular Adenomas give rise to Adenocarcinoma?
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2-3%
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What is the risk of cancer in Tubular Adenomas proportional to?
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Increasing size and number of polyps
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Which neoplastic polyp has a greater tendency to transform to malignancy?
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Villous (40% vs. 2-3%)
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What is the appearance of Villous Adenomas?
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Sessile (broad-based)
Larger than Tubular adenomas Finger-like protrusions lined with Columnar epithelium |
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What do Villous adenomas secrete?
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Mucin
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What is the normal size of Tubular adenomas?
What is the size of Villous Adenomas? |
< 1 cm
~4 cm |
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What are Tubulovillous adenomas?
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Tubular adenomas that contain villous parts
-represent an intermediate step between tubular and villous adenomas |
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What is the inheritance type of Familial Adenomatous Polyposis Coli?
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Autosomal dominant
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What is the cause of Adenomatous Polyposis Coli?
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linked to the deletion of the tumor suppressor gene APC
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What chromosome is the APC gene on?
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5q21
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T or F: all patients with Familial Polyposis develop tubular adenomas and cancer
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True
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With FAP, at what age is cancer found in 100% of patients?
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by 40 yoa
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What is performed in mid-life with people with Familial Adenomatou Polyposis Coli?
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Prophylactic Colectomy
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List the genes involved in the pathway from adenomatous polyps to cancer
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1. loss of APC tumor suppressor
2. mutation of k-ras oncogene 3. loss of tumor suppressor gene p53 |
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What mutation that leads to cancer does not involve adenoma precursor lesions?
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DNA mismatch repair genes (MSH2)
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What is the most common GI malignancy
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Carcinoma of the large intestine
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Variant of FAP with multiple osteomas, fibromatosis, and epidermal inclusion cysts
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Gardner syndrome
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Variant of FAP characterized by numerous colonic adenomatous polyps and CNS tumors (Gliomas)
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Turcot Syndrome
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What are the precursor lesions of Carcinoma of the Large Intestine?
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Adenomas
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How does a person with Right-sided Colon Cancer present?
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Bleeding
1. occult blood in stool 2. Iron deficiency anemia |
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How does a person with Left-sided Colon Cancer present?
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Obstruction
1. constipation or diarrhea 2. reduced caliber stools |
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What is the appearance of Left-sided Colon cancer?
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Circumferential growth producing a "napkin-ring" configuration
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What is the appearance of Right-sided Colon Cancer?
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Exophytic or flat, broad-based lesion = Polypoid mass
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List the parts of the Large Intestine where most carcinomas develop
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1. Left-side = 55%
2. Right-side = 35% 3. Transverse = 10% |
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In a barium enema studies, what are Left-sided tumors described as looking like?
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"apple-core" like lesions
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How do Colorectal Cancer metastasize?
1. Lymphatic spread? 2. Hematogenous spread? |
1. Mesenteric lymph nodes
2. Portal vein to liver |
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What are 3 risk factors associated with Colon Cancer?
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1. high calorie intake
2. low-fiber food associated with constipation 3. high fat contect and refined sugars |
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-Glycoprotein secreted by fetal intestinal cells into the meconium
-Also secreted by Adenocarcinoma cells in the blood of patients with Colorectal Cancer |
Carcinoembryonic Antigen (CEA)
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Why is CEA not used for screening of persons at risk of developing Colon Cancer?
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CEA test lacks specificity
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What conditions are associated with elevated blood CEA? (5)
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1. Colon Adenocarcinoma
2. Adenocarcinoma of Pancreas, Gallbladder, Lung 3. Ulcerative colitis and Crohn's Disease 4. Alcohol Cirrhosis 5. Smoking |
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Define Carcinoid tumors
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Low-grade malignant tumors composed of Neuroendocrine cells often producing Serotonin
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At what size do Carcinoid tumors metastasize?
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> 2 cm
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What are the most common locations of Carcinoid tumors of the GI?
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1. Appendix = 40%
2. Rectum 3. Terminal Ileum 4. Stomach |
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How do you recognize Carcinoid tumors under EM?
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Neuroendocrine granules
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What 3 things can you stain with in Immunohistochemistry to identify Carcinoid tumors?
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1. Neuropeptide hormones
2. Chromogranin 3. Synaptophysin |
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In Carcinoid tumors, which site rarely metastisizes?
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Appendix Carcinoid tumors
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What has to happen to produce Carcinoid Syndrome?
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Metastasis to the Liver from the GI
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What are the clinical features of Carcinoid Syndrome?
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1. Facial flushing
2. Bronchospasm = wheezing 3. Cardiac fibrosis of Tricuspid and Pulmonic valve 4. Diarrhea |
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In Cardinoid tumors, what may be elevated in the urine?
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5-hydroxylindolacetic acid (5-HIAA)
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What are the most common sites of GI Lymphoma>
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1. Stomach = 55%
2. SI = 25% 3. LI = 20% |
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What type of GI Lymphoma are 90% of the cases?
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MALToma
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What are the risk factors for GI Lymphoma?
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1. H. pylori
2. Celiac disease 3. Familial Mediterranean Fever |
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What are MALTomas?
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Primary GI tumor that are low-grade malignant B-cell lymphomas originating from the Mucosa-associated Lymphoid Tissue
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What are Gastrointestinal Stromal Tumors (GIST) sensitive to?
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Gleevec
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Sarcoma of smooth muscle = ?
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Leiomyosarcoma
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Meckel Diverticulum
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What is this?
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Massive infarction leading to hemorrhaging
Obstruction of the Mesenteric Arter |
What happened here?
What was the cause? |
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Pseudomembranous colitis
C. difficile |
What is this?
What causes it? |
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Pseudomembrane in Pseudomembrane colitis
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What is this showing?
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Malabsorption Syndrome, given is the clinical finding...you tell the deficiency:
1. Growth retardation, muscle wasting, edema 2. Microcytic-hypochromic anemia 3. Macrocytic, megaloblastic anemia 4. Night blindness, Keratomalacia 5. Osteomalacia 6. Bleeding tendency 7. Tetany, paresthesia, secondary hyperparathyroidism |
1. Protein
2. Iron 3. Folate / B12 4. Vitamin A 5. Vitamin D 6. Vitamin K 7. Calcium |
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Celiac disease causes atrophy of the villi leading to malabsorption
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This is the Small Intestine...what is the pathology?
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Crohn's b/c there is a Granuloma present
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Is this Crohn's or Ulcerative Colitis? How do you know?
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Ulcerative Colitis
- pseudopolyps are present |
What is this?
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Crohn's
Ulcerative colitis |
Top = ?
Bottom = ? |
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Summary of Crohn's and Ulcerative Colitis
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-
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Toxic Megacolon
Ulcerative colitis |
What is this called?
What caused it? |
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Diverticulosis
Sigmoid colon Age Low-fiber diet |
What is this?
Most common site? Related risk factor? Presumed cause? |
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Diverticulosis
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What is this?
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Autosomal dominant disease with multiple Hamartomatous polyps in the Small Intestine + melanin pigmentation on the Oral Mucosa
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Peutz-Jeghers Syndrome
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Tubular adenoma
Benign but transforms 2-3% of the time |
What is this showing?
Benign or Malignant? |
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Villous Adenoma
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What is this?
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Familial Adenomatous Polyposis Coli
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What is this?
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Colorectal Adenocarcinoma
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What is this?
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Carcinoid tumor
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What is this?
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