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Gastrointestinal Pathology- Pathoma

Terms in this set (424)

What is a cleft lip or palate?
A full-thickness defect in the lip or palate
What is the pathogenesis of a cleft lip or palate?
Failure of the facial prominences to fuse during early pregnancy
How many facial prominences are there and what directions do they come from
5 total; 1 superior, 2 from the side, and 2 inferior
Is it more common for cleft lip and palate to occur together or isolated?
Together is more common
Patient has a painful ulceration on the inside of the lip, examination shows that the lip has a grayish base surrounded by a reddish border - what is the diagnsis
Aphthous Ulcer
The gray base of an Apthous ulcer is composed of what type of tissue and what type of collagen is in this tissue?
Granulation tissue contain Type III collagen
When is a Apthous ulcer most likely to occur?
Times of stress
What is the treatment for an Apthous ulcer?
There is none, it resolves spontaneously but can recur
Patient presents with blurred vision, floating spots, and eye pain, they also report painful mouth ulcers that recur and have a grayish base, and genital ulcers - what is the diagnosis?
Behcet Syndrome
What is the pathogenisis of Behcet Syndrome?
Small vessel immune complex vasculitis
When is Becet syndrome most likely to occur?
After a viral illness, but the etiology is unknown
Patient presents with recurring vesicular painful, red ulcers and Tzanck smear shows multinucleated giant cells - what is the diagnosis?
Oral herpes
What is the most common causal organism of Oral Herpes?
HSV-1
When does the primary infection with HSV-1 occur?
Childhood
Where does the HSV-1 infection remain dormant?
Ganglia of the Trigeminal nerve
What causes reactivation of HSV-1 infection?
Stress and sunlight
What is Squamous Cell Carcinoma of the Oral Cavity?
Malignant neoplasm of squamous cells lining the oral mucosa
What are the 2 major health risk factors for the development of squamous cell carcinoma of the oral mucosa?
Tobacco and alcohol
Where is the most common place in the mouth for squamous cell carcinoma of the oral mucosa to occur?
Floor of the mouth
What are two precursor lesions to squamous cell carcinoma of the oral mucosa?
Leukoplakia and Erythroplakia
How can leukoplakia be distinguished from oral candidiasis?
Leukoplakia cannot be scraped away and oral candidiasis can be easily scraped away
What is the distinct location of Hairy leukoplakia that distinguishes it from leukoplakia?
Hairy leukoplakia occurs on the lateral side of the tongue
What demographic is most at risk for developing oral candidiasis and Hairy leukoplakia?
Immunocompromised
Hairy leukoplakia is due to what infection?
EBV
Is Hairy leukoplakia pre-malignant?
No- it is only hyperplasia
Why is erythroplakia red?
It is well vascularized
Is erythroplakia or leukoplakia more suggestive of squamous cell dysplasia?
Erythroplakia
What should be done with erythroplakia and leukoplakia to rule out carcinoma?
Biopsy
What are the 3 majors salivary glands?
Parotid, Submandibular, and Sublingual
11 year old child presents with bilateral parotid gland enlargement - what is the most likely diagnosis?
Mumps
Other than the parotid glands what 3 locations does Mumps infect?
Testicles (Orchitis), Pancreas, Meninges (Aseptic Meningitis)
What serum marker would be elevated in a mumps infection that would indicate parotid or pancreas involvement?
Serum amylase
What is a feared complication of Mumps if it occurs in a teenager?
Sterility due to orchitis
What is Sialadenitis?
Inflammation of the Salivary gland
What is the most common cause of Sialadenitis?
Obstructing stone (sialolithiasis)
What is the most common causal organism of infection in Sialadenitis?
Staph aureus
Is Sialadenitis usually unilateral or bilateral?
Unilateral
Patient presents with a mobile, painless, well circumscribed mass at the angle of the jaw arising from the parotid gland- what is the diagnosis?
Pleomorphic Adenoma
What type of tissue composes a Pleomorphic adenoma?
It is typically a biphasic tumor consisting of stromal (e.g. cartilage) and epithelial tissue
Why does Pleomorphic adenoma have a high rate of recurrence?
It has irregular borders which that extend through the tumor capsule leading to incomplete resection
What would suggest a Pleomorphic adenoma has transformed into a carcinoma?
Signs of Facial nerve damage such as pain or palsy
Patient presents with a growing mass in the parotid gland, biopsy shows a cystic tumor with many lymphocytes and germinal center - what is the Diagnosis?
Warthin tumor
What is the most common salivary gland to have a Warthin Tumor?
It almost always arises in the parotid
Patient presents with recent onset of a growing mass on the right side of his jaw, he has experience abnormal taste sensation on the anterior 2/3 of the tongue and decreased ability to control muscles surrounding his right eye and right side of his mouth, biopsy of the mass reveals Keritin + and PAS + staining- what is the diagnosis?
Mucoepidermoid Carcinoma
Mucoepdermoid tumors consist of what type of tissue?
Mucinous (PAS +)
Squamous epithelium (Keritin +)
What cranial nerve is most often affected in Mucoepidermoid carcinoma?
Facial Nerve (VII)
Newborn presents with vomiting immediately following the first feeding, the abdomen is distended and the infant had to have the lungs suctioned due to aspiration -what is the most likely diagnosis?
Tracheoesophogeal Fistula
In the most common variant of Tracheoesophogeal Fistula there is proximal tube _____ and the distal esophagus arises from the ______
- Atresia
- Trachea
Will there be oligohydroamnios or polyhydroamnios in Tracheoesophogeal Fistula, why?
Polyhydroamnios due to decreased amniotic fluid removal by the GI tract
Patient presents with a microcytic hypochromatic anemia, on examination the the tongue appears smooth, thick and red, the patient reports difficulty swallowing especially when in a hurry to eat - what is the diagnosis?
Plummer-Vinson syndrome
Why does patient with Plummer-Vinson experience dysphagia?
Esophageal Web
An esophageal web is a protrusion of what layer of the esophagus?
Mucosa
List the 4 layers of the esophagus starting from the one that borders the lumen and work out
Mucosa
Submucosa
Muscularis Propria
Serosa
When do patients with an esophageal web experience dysphagia?
When their food is poorly chewed
Where is the most common location of an esophageal web?
Upper esophagus
Patient presents with dysphagia, obstruction, and bad breath, they state they feel like something is in the back of their throat - what is the diagnosis?
Zenker Diverticulum
What is a Zenker Diverticulum?
Outpouching of pharyngeal mucosa through a defect in the muscular wall
Is the defect in Zenker Diverticulum an acquired or congenital defect? Is the diverticulum in Zenker Diverticulum a true or false diverticulum?
Acquired; False diverticulum
Where specifically does the Zenker Diverticulum form?
At the upper esophageal sphincter at the junction between the esophagus and pharynx
29 year old female with history of bulimia presents with painful, bloody vomiting - what is the likely diagnosis?
Mallory-Weiss syndrome
What is the pathogenesis of Mallory-Weiss syndrome?
Longitudinal laceration of the mucosa at the gastroesophageal junction
What are two risk factors for the development of Mallory-Weiss syndrome?
Alcoholism and Bulimia
Mallory-Weiss syndrome puts patients at an increased risk to develop what syndrome?
Boerhaave syndrome
What is Boerhaave syndrome
Rupture of the esophagus leading to air in the mediastinum and subcutaneous emphysema
How will subcutaneous emphysema present on physical exam?
Crackling of air bubbles in the neck on palpation
A patient with a history of alcohol abuse and cirrhosis presents with painless bloody vomiting - what is the likely diagnosis?
Esophageal Varices
What are Esophageal Varices and in what part of the esophagus do they most commonly form?
Dilated submucosal veins in the lower esophagus
Trace the normal venous drainage of the upper 2/3 of the esophagus to the right atrium?
Esophageal veins to Azygous vein to Superior Vena Cava to Right Atrium
What vein drains the distal esophagus into the portal vein?
Gastric vein
What is the underlying cause for the development of esophageal varices?
Portal Hypertension
What is the most common cause of death from Cirrhosis?
Esophageal varices
Patient presents with difficulty swallowing both solids and liquids, they have bad breath, and a Barium swallow study shows a "Bird-beak" appearence - what is the diagnosis?
Achalasia
Esophageal manometry would show what finding in achalasia?
High LES pressure
Achalasia is a combination of disordered____ ____ with the inability to relax the ___ ____ ____
Esophageal motility
Lower Esophageal Sphincter
Damage to what cells leads to achalasia?
Ganglion cell of the myenteric plexus
What layer of the esophagus are the ganglion cells located?
Between the inner circular and outer longitudinal layers of the muscularis propria
Infection with what organism can result in Achalasia?
Trypanosoma cruzi in Chagas Disease
Do fluids and solids both require peristalsis?
Yes
There is an increased risk of what malignancy with Achalasia?
Esophageal squamous cell carcinoma
An 52 year old man comes to sweating and clenching is chest, a quick history reveals the man was recently started on albuterol and has had 3 cavities within the past year after having never had one before- EKG is normal -what is the most likely diagnosis?
Gastroesophageal Reflux Disease (GERD)
- Heartburn mimicking cardiac chest pain
- Adult onset asthma
- Damage to the enamel of the teeth
What is the pathogenesis of GERD?
Reflux of acid from the stomach due to reduced LES tone
What are 2 late complications of untreated GERD?
Ulceration with stricture
Barrett Esophagus
What is the pathogenesis of ulceration and stricture?
Part of mucosa and sub-mucosa are carved and the stem cells are lost, the tissue is replaced by fibrosis and the lumen narrows
Name 6 risk factors for the development of GERD?
Alcohol
Tobacco
Obesity
High-fat diet
Caffeine
Hiatal Hernia
What is a Hiatal hernia?
The cardia of the stomach moves up through the LES and into the lower esophagus
On imaging what characteristic shape with a Hiatal hernia have and why?
Hour glass shape because the part of the stomach is compressed by the LES
New born presents with difficulty breathing- assessment of the lung fields reveals bowel sounds in the lower left lung field- what is the diagnosis?
Paraesophageal Hernia
What is the pathogenesis of Paraesophageal Hernia ?
The stomach herniates through the diaphragm next to the esophagus
What growth adaptation has the lung undergone?
Lung hypoplasia
Give the exact growth adaptation of Barrett esophagus including cells types involved?
Metaplasia of the lower esophageal mucosa from nonkeritinized stratified squamous epithelium to nonciliated columnar epithelium with Goblet cells
What percent of patients with GERD go on to develop Barrett Esophagus?
10%
What is the pathogenesis of Barrett esophagus?
Stem cell metaplasia in response to lower esophageal acidic stress
If left untreated what may Barrett Esophagus progress to?
Dysplasia and adenocarcinoma
Patient presents with recent unexplained weight loss, pain, and reports several episodes of vomiting which there has been blood - the patient reports that about 6 months ago he began to have trouble swallowing solids but now also has great difficulty drinking- what is the diagnosis?
Esophageal carcinoma
If a patient presents with signs and symptoms of Esophageal carcinoma what additional sign will be present to make a diagnosis of Squamous Cell Esophageal Carcinoma more likely?
A horse voice (laryngeal nerve involvement) with a cough (tracheal involvement)
What are two subclasses of Esophageal carcinoma?
Adenocarcinoma and squamous cell carcinoma
Where is Esophageal adenocarcinoma most common?
Where is Esophageal squamous cell carcinoma most common?
- Esophageal adenocarcinoma is most common in theWest
- Esophageal squamous cell carcinoma is most common worldwide
Adenocarcinoma arises from what pre-existing pathology?
Barrett Esophagus
What part of the esophagus is usually affected in adenocarcinoma?
Lower 1/3
What part of the esophagus is usually affected in squamous cell carcinoma?
Upper 1/3 and Middle 1/3
In one word describe the risk factor for the development of Esophageal squamous cell carcinoma?
Irritation
Give 5 major risk factors for the development of Esophageal squamous cell carcinoma?
Alcohol and Tobacco
Very hot tea ( Asian and Iranian cultures)
Achalasia (Food decay)
Esophageal Web
Esophageal Injury
Give an example of a substance that can cause esophageal injury?
Lye ingestion
What is the prognosis for Esophageal Carcinoma?
Poor because it presents late
Give the lymph node drainage based on carcinoma in the following locations
Upper 1/3
Middle 1/3
Lower 1/3
Upper 1/3 - Cervical nodes
Middle 1/3 - Mediastinal or tracheobronchial nodes
Lower 1/3 - Celiac or gastric nodes
The attending shows you two newborns with their stomach contents exposed and states they have 2 different diagnoses - one has Gastroschis and one has Omphalocele - what is the distinguishing characteristic of these two diagnoses
Gastroschisis - the abdominal contents are exposed and not covered by peritoneum or amnion
Omphalocele- the abdominal contents are exposed but they are covered by both peritoneum and amnion
What is the pathogenesis of Gastroscisis?
Failure of abdominal wall closure
What is the pathogenesis of Omphalocele?
Persistent herniation of the bowel into the umbilical cord due to failure of the intestines to return to body cavity during development
A 2 week old that presents to the hospital after a 1 day history of projectile vomit, on physical exam the abdomen shows peristalsis and there is an olive size mass in the abdomen - what is the diagnosis?
Pyloric stenosis
What is the pathogenesis of Pyloric stenosis?
Hypertrophy of the pyloric smooth muscles
Is Pyloric stenosis more common in males or females?
Males
What finding in the vomit shows that the contents never reached the duodenum in Pyloric stenosis?
It is nonbilious
Is pyloric stenosis present at birth?
No, it takes a couple weeks to develop
What is the treatment for pyloric stenosis?
Myotomy
What is the pathogenesis of Acute Gastritis?
Acidic damage to the stomach mucosa due to imbalance between mucosal defenses and acidic enviornment
What cell type in the mucosal layer of the stomach produce mucin?
Foveolar cells
The surface epithelium of the stomach produces what substance to protect against an acidic environment?
Bicarbonate
How does normal blood supply act as a defense against an acidic environment?
It provides nutrients and picks up leaked acid
What patient population can develop a Curling ulcer?
Burn victims
How does a severe burn lead to acute gastritis?
Hypovolemia leads to a decreased blood supply
What enzyme in Foveolar cells is inhibited by NSAIDS, what substance decreases as a result of inhibition?
NSAIDS inhibit cylcooxygenase and decrease levels of PGE2
A decrease in PGE2 decreases what 2 stomach mucosal defenses against an acidic environment?
There will be decreased mucin production and decreased blood flow
Consumption of what beverage increases the risk for Acute Gastritis?
Alcohol
How does chemotherapy cause acute gastritis?
Chemotherapy reduces the regenerative capability of the stomach mucosa
How does a Cushing ulcer develops?
High intracranial pressure causes increased Ach release from the Vagus nerve which promotes acid secretion into the stomach
What cells in the stomach secrete acid?
Parietal cells
What 3 substances can cause the Parietal cells to secrete acid?
Ach
Histamine
Gastrin
Why are most ICU patients put on a proton pump inhibitor?
ICU patients experiencing shock are at an increased risk for stress ulcers due to decreased blood flow
Acidic damage in acute gastritis can result in what 3 forms of damage to the stomach mucosa?
Superficial inflammation
Erosion
Ulcer
What is the difference in erosion and ulcer?
Erosion- loss of superficial epithelium
Ulcer- Loss of mucosal layer
What is Chronic Gastritis?
Chronic inflammation of the stomach mucosa
What are the two main etiologies of Chronic Gastritis?
Chronic autoimmune Gastritis
Chronic H. Pylori Gastritis
In Chronic autoimmune Gastritis what cells are damaged?
Parietal cells
In what parts of the stomach are Parietal cells located?
Body and Fundus
What type of hypersensitivity is Chronic autoimmune Gastritis?
Type IV hypersensitivity
Which two antibodies are useful for diagnosis of Chronic autoimmune Gastritis?
Anti-parietal cell and Anti- Intrinsic factor
Do the antibodies mediate the damage in Chronic autoimmune Gastritis?
No, the damage is T-Cell mediated
On histology what is the classic finding in Chronic autoimmune Gastritis?
Atrophy of the mucosa with intestinal metaplasia with the presence of Goblet cells
Achlorhydria leads to an increase in what hormone level?
Gastrin
What cell undrgoes hyperplasia to produce more Gastrin?
G-cells
Where are G-cell located?
Antral of the stomach
What type of anemia is associated with Chronic autoimmune Gastritis?
Megaloblastic pernicious anemia
Why does a megaloblastic anemia occur in Chronic autoimmune Gastritis?
Destruction of parietal cells leads to low levels of Intrinsic Factor and decreased ability to absorb B12
There is an increased risk of developing what malignancy in Chronic autoimmune Gastritis?
Gastric adenocarcinoma - Intestinal type
What is the most common cause of B12 deficiency?
Pernicious anemia
What is the most common cause of chronic gastritis?
H Pylori infection
H. Pylori produces what two enzymes that weakens the mucosal surface?
Ureases and proteases
What portion of the stomach has the highest rate of Chronic Gastritis due to H. Pylori infection?
Antrum
Using the 9 quadrant grid- in what area of the abdomen does pain originate in H. Pylori Chronic Gastritis?
Epigastric region
There is an increased risk for what 2 neoplasms as a result of H. Pylori Chronic Gastritis?
Gastric adenocarcinoma ( Intestingal type) and MALT lymphoma
A MALT lymphoma will show what histology?
An increase in germinal centers with post-germinal B-cells in a marginal zone
What is required for the presence of a marginal zone?
Chronic inflammation ( Chronic Gastritis, Hoshimotos Thyroiditis, and Sjogrens)
What is the treatment for H. Pylori Chronic Gastritis?
Triple Therapy
With Triple Therapy is it possible to reverse the intestinal metaplasia seen in H. Pylori Chronic Gastritis?
Yes
What are two confirmatory tests to show that Triple Therapy has been effective?
Negative Urease breath test and lack of stool antigen
A patient presents with epigastric pain that improves with eating - what is the diagnosis?
Duodenal Ulcer
What is the cause of 95% of Duodenal Ulcer cases?
H. Pylori infection
What neoplasm often presents as a Duodenal Ulcer?
Gastrinoma as part of Zollinger-Ellison syndrome
A diagnostic endoscopy to confirm a duodenal ulcer would show what ?
Ulcer with Brunner gland hypertrophy
What is the function of Brunner glands?
Produce mucin
Where in the duodenum is the most common site of a Duodenal Ulcer?
Anterior duodenum
The presence of a Duodenal Ulcer in the posterior portion of the duodenum increases the risk of bleeding from what artery if there is rupture?
Gastroduodenal artery
What pancreatic pathology is likely if Duodenal Ulcer in the posterior part of the duodenum ruptures?
Acute pancreatitis
Patient presents epigastric pain that becomes worse with eating - what is the diagnosis?
Gastric ulcer
What is the most common cause of a Gastric ulcer?
H. Pylori
What medication increases the risk of Gastric ulcer?
NSAIDs
Where in the stomach is a Gastric ulcer most commonly located?
Lesser curvature of the antrum
If a gastric ruptures there is an increased risk of bleeding from what artery?
Gastric artery
Does a Duodenal ulcer or a Gastric ulcer carry increased risk for carcinoma?
Gastric ulcer carries an increased risk for gastric carcinoma
Describe the gross appearance of a benign gastric ulcer based on size, border, and surrounding mucosa
Less than 3 cm, sharply demarcated "punched out" border, and the surrounding mucosa has radiating folds of mucosa
Describe the gross appearance of a malignant gastric ulcer based on size, border, and surrounding mucosa?
Large, irregular border with heaped up margins
What is required for a definitive diagnosis to determine whether a gastric ulcer is benign or malignant?
Biopsy
74 year old patient presents with unexplained 12 pound weight loss, abdominal pain, hemoglobin is 8, and reports that he feels full soon after beginning to eat -examination of the axillary region reveals a darkened thickened skin - what is the diagnosis?
Gastric Adenocarcinoma
What are the two subtypes of Gastric Adenocarcinoma
Intestinal and diffuse
Which subtype of Gastric Adenocarcinoma is more common?
Intestinal
An Intestinal Gastric Adenocarcinoma will most commonly present in what area of the stomach?
Lesser curvature of the antrum
What will the gross appearance of Intestinal Gastric Adenocarcinoma show on endoscopy?
Large, irregular ulcer with heaped margins
What type of food increases the risk for Intestinal Gastric Adenocarcinoma?
Nitrosamines in smoked foods
What culture has a diet rich in smoked foods and thus has an increase risk of Intestinal Gastric Adenocarcinoma?
Japanese
What blood type carries an increased risk for the development of Intestinal Gastric Adenocarcinoma?
Blood type A
What pre-existing pathology increases the risk for Intestinal Gastric Adenocarcinoma?
Chronic Gastritis
The diffuse type of Gastric Adenocarcinoma is characterized by the presence of what cell type?
Signet ring cells
What do Signet ring cells look like on histology?
The nucleus is pushed off to the side due to high level of mucin
How does Diffuse Gastric Adenocarcinoma appear on gross exam?
It shows linitis plastica
What is linitis plastica?
Desmoplasia resulting in the thickening of the stomach wall
Is the diffuse type of Gastric Adenocarcinoma associated with the same risk factors as the Intestinal Gastric Adenocarcinoma?
No
Is the Diffuse type or Intestinal type Gastric Adenocarcinoma associated with early satiety?
Diffuse type
What is the Lesser-Trelat finding on physical exam?
Many seborrheic keratosis all over the skin
What is the most common lymph node for gastric adenocarcinoma to spread to?
Left supraclavicular node (Virchow node)
Distant metastasis most commonly involves what organ?
The liver
What is a Sister Mary Joseph nodule and what type of Gastric adenocarinoma is it associated with?
Metastasis of the intestinal type to the periumbilical region
The finiding of a Krukenberg tumor indicates metastasis to what organ?
Bilateral spread to the ovaries
Which type of Gastric Adenocarcinoma spreads to the ovaries?
Diffuse type
Infant presents with profuse vomiting and on x-ray of the abdomen there was a double bubble sign - what is the diagnosis?
Duodenal atresia
What is the pathogenesis of Duodenal atresia?
Congenital failure for the duodenum to canalize
What chromosomal abnormality puts infants at an increased risk for development of Duodenal atresia?
Down syndrome - trisomy 21
Do patients with duodenal atresia have oligohydramnios or polyhydramnios?
polyhydramnios
Why is there a double bubble sign on x-ray in Duodenal atresia?
The stomach is filled with air, and the blind loop of the duodenum is filled with air and they are separated by a pyloric sphincter
Is the vomiting in Duodenal atresia bilious or non-bilious?
Bilious
2 year old is brought in with bouts of painless rectal bleeding, the mother also states that the boy will often grab in the region of the right upper quadrant and cry as if he is in pain - what is the likely diagnosis?
Meckel Diverticulum
Is Meckel's Diverticulum a true or false diverticulum?
It is a true diverticulum- all 3 layers of bowel wall are present
Meckel diverticulum arises due to failure of what embryologic structure to involute?
Vitelline duct
When does the Vitelline duct normally form and when does it normally involute?
Form- Week 4
Involute - Week 7
What is the function of the Vitelline duct?
Allows the midgut to receive nutrients from the yolk sac
What substance can pass through a patent Vitelline duct at birth if it has completely failed to involute?
Meconium
If the Vitelline ducts has partially involuted what will the umbilicus region feel like on physical examination?
Feels solid but soft due to stool accumulation
1. What percent of the population has a Vitelline duct?
2. How long is a Vitelline duct?
3. How far away is the Vitelline duct from the ileocecal valve?
4. When does a Vitelline duct most commonly present?
2% (most common congenital anomaly of GI tract)
2 cm
2 Feet
2 years old
- These are the rules of 2
Why does a Vitelline duct present with bleeding?
Presence of heterotopic gastric mucosa
What are are 3 possible complications of a Vitelline duct?
Volvulus
Inussusception
Obstruction
If a child with a suspected Vitelline duct presents signs and symptoms of appendicitis what complication does he likely have?
Obstruction
What is volvulus?
Twisting of bowel along the mesentary
What is a complication of Volvulus?
Infarction due to obstruction and disruption of the blood supply
Where is the most common location of Volvulus in the elderly?
Sigmoid colon
Where is the most common site of volvulus in young adults?
Cecum
What is Intussusception?
Telescoping of a proximal segment of bowel into a distant one
What pulls the telescoped segment of bowel forward?
Peristalsis
What is a complication of Intussusception?
Infarction due to obstruction and disruption of blood supply
What must be present for Intussusception to occur?
A focus of traction
What is the most common cause of Intussusception in children?
Lymphoid hyperplasia
What is the most common viral cause of lymphoid hyperplasia in a child?
Rotavirus
Where is the most common segment of bowel for Intussusception to occur in a child?
terminal ileum
What is the most common cause of Intussusception in adults?
Tumor
Patient with a history of Volvulus and Intussusception presents to the ER with abdominal pain, blood diarrhea, and decreased bowl sounds - what is the diagnosis?
Small bowel infarction
On a scale of low to high how susceptible is the small bowel to ischemic injury?
Highly
What are two causes of a transmural small bowel infarct?
Superior Mesenteric Artery Thrombus
Mesenteric Vein Thrombus
Give what heart condition and one vascular condition that can cause Superior Mesenteric Artery thrombus?
Heart- Atrial fibrillation
Vascular- Polyarteritis Nodosa (vasculitis)
Give one myeloproliferative disorder and one autoimmune disorder that can cause thrombosis of the mesenteric vein?
Myeloproliferative- Polycythemia vera
Autoimmune- Lupus anticoagulant
Mucosal infartion of the small bowel is due to what?
Hypotension
13 year old presents to the office with recurrent bouts of diarrhea and bloating usually following dinner at which she often consumes skim milk - what is the diagnosis?
Lactose Intolerance
What is the pathogenesis of Lactose Intolerance?
Deficiency of the lactase enzyme at the brush border of enterocytes
What is the normal function of the lactase enzyme?
Break down lactose into glucose and galactose
Why does a lactase deficiency lead to diarrhea?
Lactose acts as an osmotic laxative
If lactose intolerance is congenital what is the genetic in
inheritance pattern?
Autosomal recessive
If lactose intolerance is acquired at what age is it most likely to present?
late childhood
A temporary lactose intolerance is likely to develop following what type of illness?
Small bowel infection
A 3 year old child and her mother come to the office complaining of similar symptoms - the child has bloating, diarrhea, and has not met growth milestones, the mother also has bloating and diarrhea- on physical exam both mother and child have many small raised vesicles on the skin - what is the diagnosis?
Celiac disease
What is the pathogenesis of Celiac disease?
Immune-mediated damage of the small bowel villi due to gluten exposure
What HLA associations are present in Celiac disease
HLA-DQ2 and HLA-DQ8
What is the most pathogenic component of gluten?
Gliadin
What enzyme deaminates gliadin in the small bowel?
Tissue Transglutaminase (tTG)
Is the damage in Celiac disease antibody driven or T-Cell driven?
T-Cell
Deaminated gliadin is presented by antigen presenting cells that contain which of the following structures - an alpha chain and beta 2 microglobulin subunit or an alpha chain and a beta chain?
Alpha chain and a beta chain- This is MHC class II
Dermatitis hermatiformis contains what immunoglobulin deposition?
IgA
Why is damage in Dermatitis hermatiformis localized to the epidermis?
The epidermis does not have blood vessel and is dependent on blood vessels in the dermis which become damaged by immune complexes in Celiac disease resulting in Dermatitis hermatiformis
What is needed for the treatment of Dermatitis hermatiformis?
Gluten free diet
IgA antibodies can be elevated against what 3 substances in celiac disease?
Endomysium, tTG, Gliadin
What pre-existing illness can increase the risk of developing Celiac disease?
IgA deficiency
In patients with IgA deficiency what auto-antibody could be elevated?
IgG antibodies
In celiac disease on biopsy how will the villi look? how will the crypts look? What cell infiltrate will be present?
Flattening of the villi
Hyperplasia of the crypts
Lymphocytes in the intraepithlium
Which segment of the small bowel is most commonly damaged in celiac disease?
Duodenum
All symtpms of Celiac disease will resolve with what dietary modification?
Gluten free diet
There is an increased risk of what malignancies in patients with Celiac disease?
Small bowel carcinoma and T-Cell carcinoma
A transfer from Ross medical school has chronic diarrhea and bloating, upon question they say that they thought they had Celiac disease but symptoms persisted- what is the diagnosis?
Tropical Sprue (Ross is located in the Caribbean)
What is the pathogenesis of Tropical Sprue?
Damage to the small bowel by an unknown organism resulting in malabsorption
What geographic region has an increased incidence of Tropical sprue?
Caribbean
Tropical sprue most often arises after what illness?
Infectious diarrhea
What is the treatment for Tropical sprue?
Antibiotic - Most commonly Tetracycline
What regions of the bowel are most commonly damaged in Tropical Sprue?
Jejunum and ileum
Whipple disease is due to what organism?
Tropheryma whippelii
What cell type contains patially destroyed Tropheryma whippelii in Whipple disease?
Macrophages
Affected tissue in Whipple disease will stain positive to what stain?
PAS +
What is the most common site of involvement in Whipple disease?
Small bowel lamina propria
Fat in the small intestine is broken down by what enzyme?
Lipase
What is the function of bile acids in fat absorption?
Emulsify fat
What is a micelle?
phospholipid unit surrounding emulsified fat absorbed by enterocytes
Enterocytes package fatty acids in what lipoprotein?
Chylomicrons
How do chylomicrons enter circulation?
They enter through lacteals in the lamina propira travels through the lymphatics and enter into venous circulation through the opening of the thoracic duct
Why does Whipple disease result in fat malabsorption?
Increase in macrophages in the lamina propria obstruct the lacteals blocking chylomicron entry
What are other common locations of Whipple disease involvement?
Joints, Heart Valves, Lymph Nodes, and CNS
3 month old presents with failure to thrive, steatorrhea, and acanthocytosis, ataxia, and night blindness- what is the diagnosis?
Abetelipoproteinemia
What is the genetic inheritance pattern of Abetelipoproteinemia?
Autosomal recessive
What is the defect in Abetelipoproteinemia?
B-48 and B-100 deficiency
Why do patients with Abetelipoproteinemia suffer from malabsorption?
They cannot package and secrete chylomicrons
What lipoproteins will be extremely low or absent from the blood in Abetelipoproteinemia?
Chylomicrons, VLDL, LDL
A Carcinoid tumor is a malignant proliferation of what type of cell?
Neuroendocrine
What will a Cacinoid tumor stain positive for?
Chromogranin
Where is the most common site for a Carcinoid tumor
Small bowel
On gross exam how will a Carcinoid tumor look?
Sub-mucosal polyp like nodule
What is the most commonly secreted substance from a Carcinoid tumor?
Serotonin
What enzyme and in which organ is the excess Serotonin metabolized?
Monoamine Oxidase (MAO) in the liver
What product of metabolism is found in the Urine in Carcinoid tumor?
5-HIAA
Metastasis of a Carinoid tumor to what organ allows serotinin to enter into circulation?
Liver
What vasculature does Serotinin leak into to reach systemic circulation?
Serotonin is released into the hepatic vein and then leaks through hepato-systemic shunts
Patient presents with bronchospasm, diarrhea, and skin flushing following an outburst of crying - what is the diagnosis?
Carcinoid syndrome
Carcinoid heart disease is characterized by fibrosis of which valves most commonly?
Right sided heart valves
- Tricuspid Regurgitation
- Pulmonic stenosis
Why are left valved heart lesions not seen in Carcinoid heart disease?
The lung contains MAO
What is the most common tumor of the appendix?
Carcinoid tumor (This is not the most common location of a Carcinoid tumor, but rather the most common tumor of the appendix)
11 year old boy presents to the ER with fever and nausea, on physical exam Rovsing's sign, obturator sign, and psoas sign were all positive - what is the diagnosis?
Acute appendicitis
Pain in acute appendicitis localizes to what point?
McBurney point in RLQ
What is the cause of acute appendicitis in children?
Lymphoid hyperplasia
What is the cause of acute appendicitis in adults?
Fecalith
In a patient with suspected acute appendicitis that presents with guarding and rebound tenderness likely has experienced what complication?
Appendix rupture
What is the most common complication of acute appendicitis?
Periappendiceal abscess
A 28 year old woman has had a 2 year history of recurrent bouts of bloody diarrhea and abdominal pain - infection, neoplasm, and ischemic disease have been ruled out - what is the diagnosis?
Inflammatory Bowel Disease (IBD)
What is the possible etiology of IBD?
Abnormal immune reaction to enteric flora
What is the most common gender and age group to suffer from IBD?
Women teens- 30s
What ethnic groups are most likely to have IBD?
Caucasians and Eastern European Jews
IBD is a diagnosis of____
Exclusion
IBD can be sub-classified into what two diseases?
Ulcerative colitis and Crohn disease
What is the extent of wall involvement in Ulcerative Colitis (UC)?
Mucosal and submucosal ulcers
What is the location of UC?
Starts at Rectum and extends to the cecum
What are the symptoms of UC?
Left lower quadrant pain and blood diarrhea
What is the type of inflammation in UC?
Crypt abscesses with neutrophil
What is the Gross Appearance of UC? What does UC show on imaging?
Pseudopolyps; loss of haustra and the involvement is continuous - lead pipe
What are two major risks of UC?
Toxic megacolon and carcinoma
There is an increased risk of Carcinoma from UC based on what two variables?
Extent and duration of disease
What gallbladder pathology has a higher association in patients with UC?
Primary sclerosing cholangitis
What auto-antibody is elevated in UC
p-ANCA
Name 4 diseases that have an increased p-ANCA
UC
Primary sclerosing cholangitis
Microscopic Polyangitis
Churg-Straus
What is the extent of wall involvement in Crohn Disease?
Full-thickness inflammation with knife-like fissures
What is the location of Crohns? What is the most common site and what is the least common?
Anywhere from mouth to anus
Most- Terminal ileum
Least - Rectum
What are the symptoms of Crohns?
Right lower quadrant pain and non-bloody diarrhea
What is the type of inflammation in Crohns?
Lymphoid aggregates with granulomas
What is the Gross Appearance of Crohns? What does Crohns show on imaging?
Cobblestone mucosa, creeping fat, and strictures, with skip lesions
What cell type is responsible for the stricture formation?
Myofibroblasts have contractile properties that cause the lumen of the bowel to decrease in diamater
Crohn's patients develop what type of kidney stone more often?
Calcium Oxalate
When do Crohn's patients have an increased chance for the development of colonic carcinoma?
If the disease is present within the Colon
What diseases are associated with Crohn's?
Psoriasis
Ankylosing Spondylitis
Sacrolitis
Migratory polyartheritis
Uveitis
Erythema Nodosa
(These are all HLA-B27 diseases -- this association was not mentioned in Pathoma)
Smoking has a protective effect on which inflmmatory bowel disease
Ulcerative Colitis
If a bowel with Crohn's disease ruptures and attaches to another piece of bowel what is this called?
Fistula
Newborn has failed to pass meconium in the first 72 hours after birth, on digital rectal exam there is an empty rectal vault, imaging shows massive dilation just distal to the Splenic flexure- what is the diagnosis?
Hirschprung disease
What is the pathogenesis of Hirschprung disease?
Defective relaxation and peristalsis of the rectum
Hirschprung disease has an association with what trisomy disorder?
Trisomy 21 - Down syndrome
A rectal suction biopsy of a patient with Hirschprung disease would show an abscence of what cell type?
Ganglion cells
Ganglion cells are derived from what embryonic germ layer
Neural crest
Ganglion cells should normally be found in what two plexuses?
Auerbach and Meisner
Where is the Auerbach plexus located?
Between the inner circular and outer longitudinal muscularis propria
What is the function of the Auerbach plexus?
Control motility
Where is the Meissner plexus located?
Submucosa
What is the function of Meissner's plexus?
Control blood flow, secretions, and absorption
Why is it not acceptable to only do a standard biopsy on a patient with Hirschprung disease?
Because a standard biopsy will only get the mucosa and the submucosa and muscularis levels must be examined
What is the treatment for Hirschprung disease? Why is this treatment effective?
Resection of the involved bowel - bowel proximal to the affected area will have normal levels of ganglion cells and function
An elderly adult states that has struggled with constipation, straining, and eats a low fiber diet- he reports that he has no other additional symptoms- what is the likely diagnosis?
Colonic Diverticula
What is a Colonic Diverticula?
Outpouchings of mucosa and submucosa through the muscularis propria
Is a Colonic Diverticula a true or false diverticula?
False
What finding on histology would suggest a piece of the colon is weak and susceptible to a Colonic diverticula?
They arise where the vasa recta traverse the muscularis propria
What is the most common region in the colon to develop a Colonic diverticula?
Sigmoid colon
If Colonic Diverticula presents with bleeding will the color of the blood be?
Bright Red (unlike bleeding from more proximal regions of the bowel)
An elderly patient with a history of severe constipation comes to the ER and is sure he has appendicitis you ask him to point to where it hurts and he points to the lower left side of the abdomen, psoas, obturator, and Rovsing's signs are negative- what is the diagnosis?
Diverticulitis
What is the underlying pathology of Diverticululitis?
Obstructing fecal matter
If a colonic diverticlua ruptures and forms a fistula with a surrounding structure what is the most common structure it attaches to?
Bladder forming a colovesicular fistula
What will be the presenting signs if a Colovesicular fistula has formed?
Air and stool in the urine
Elederly man presents with new onset isolated rectal bleeding, he has no history of colonic diverticula, no history of recent GI infection, no significant family history, it is determined that the bleed originated in the right side of the colon- what is the diagnosis?
Angiodysplasia
What is the pathogenesis of Angiodysplasia?
Aquired malformation of mucosal and submucosal capillary beds
Where is the most common locations to develop Angiodysplsia?
Cecum and right colon
Why are the Cecum and right colon at higher risk of developing Angiodysplasia?
High wall stress
17 year old man comes in and says he has recently experienced bleeding from his lips and mouth, as well as noticing blood in the toilet- he says his dad, and his paternal grandmother have had similar symptoms for years-what is the diagnosis?
Hereditary Hemorrhagic Telangiectasia
What is the genetic mode of inheritance for Hereditary Hemorrhagic Telangiectasia?
Autosomal Dominant
What would the relative wall thickness of the vessels involved in Hereditary Hemorrhagic Telangiectasia?
These are thin walled blood vessels
Patient presents with abdominal pain shortly following each meal, and she has had a 7 pound weight loss since these symptoms started, within the last 24 pain has worsened and there is bloody diarrhea what is the diagnosis?
Ischemic colitis - (Presence of worsening pain and bloody diarrhea suggests ischemia has progressed to infarct)
Where is the most common location of Ischemic colitis?
Splenic Flexure
What is the most common cause of Ischemic colitis?
Atherosclerosis of Superior Mesenteric Artery (SMA)
Why is the Splenic Flexure the most common site of Ischemic colitis?
This is the watershed area for the SMA
33 year old woman has alternating periods of diarrhea and constipation, she also experiences abdominal pain, bloating, and flatulence- these symptoms seem to pass with defecation- what is the diagnosis?
Irritable Bowel Syndrome
What is the pathogenesis of Irritable Bowel Syndrome?
Disturbed Intestinal Motility- no identifiable pathological changes however
What would be one lifestyle modification to suggest to a patient with Irritable Bowel Syndrome?
Increased dietary Fiber
The mother of a male patient aged 4 years had noticed rectal bleeding for 15 months. But says she did not come in sooned because she thought it would stop on its own. A prolapsed rectal polyp has been observed. - What is the diagnosis?
Juvenile polyp
Is a a polyp in Juvenile polyposis a neoplasm or a hamartoma?
Benign hamartoma
Endoscopy of a child with Juvenile polyposis would most often reveal hamartomas in which locations?
Stomach and Colon
Does an increase number of Juvenile polyps increase the risk for carcinoma?
Yes
What is the average age of onset for Juvenile polyps?
Less than 5
If one child has Juvenile polyps and the mother asks what are the chances her next child will has have the condition what is the best response?
Unlikely- since Juvenile polyps are sporadic
6 year old boy comes into the office for a check up, at first glance the boy seems to have dirt around his mouth -on closer exam it is not dirt but many freckle like spots on the lips and oral mucosa, on physical exam these same spots are seen in the genital region- the father says the boys mother and sister have these same spots- what is the diagnosis?
Peutz-Jeghers Syndrome
What would endoscopy of the GI tract reveal in a patient with Peutz-Jeghers Syndrome?
Hamartomas throughout the GI tract
What is the genetic pattern of inheritance of Peutz-Jeghers Syndrome?
Autosomal dominant
There is an increased risk for develepoing cancer in which 3 regions with Peutz-Jeghers Syndrome?
Colorectal, Breast, and Gynecologic
What are Colonic polyps?
Raised protrusions of colonic mucosa
What is the most common type of Colonic polyp?
Hyperplastic polyps
Hyperplastic polyps are caused by what growth adaptation?
Hyperplasia of glands
What is the classic appearance of a Hyperplastic Colonic Polyps on microscopy?
Serrated appearance on microscopy
Where is the most common location of a Hyperplastic Colonic Polyps?
Rectosigmoid region of left colon
Is a Hyperplastic Colonic Polyps benign or malignant?
Benign
Does a Hyperplastic Colonic Polyps have malignant potential?
No
What is the second most common type of Colonic Polyps?
Adenomatous polypls
What type of tissue comprises Adenomatous Colonic Polyps?
Neoplastic proliferation of glands
Are Adenomatous Colonic Polyps benign or malignant?
Benign
Does Adenomatous Colonic Polyps carry an increased risk for the development of adenocarcinoma?
Yes-they are precancerous
Via what sequence can Adenomatous Colonic Polyps progress to adenocarcinoma?
Adenoma-carcinoma sequence
Give the in order the adenoma-carcinoma mutation sequence
APC mutation
K-ras mutation
p53 mutation
Mutation of APC gene increases risk for what?
Risk for polyp formation
Mutation of K-ras leads to the formation of what?
Increase in Poylp size
p53 mutation must occur in with an increased expression of what enzyme to lead to carcinoma?
COX
What medication can decrease the progression of adenoma to carcinoma?
Aspirin- decreases COX
What are two screening methods for Colonic polyps?
Colonoscopy and testing for fecal occult blood
What is the goal of screening for Colonic polyps?
Remove adenomatous polyps before progression to carcinoma
How can you distinguish hyperplastic and adenomatous polyps on colonoscopy?
You can't, they look the exact same
What is is done to every polyp found during colonoscopy?
They are removed and examined microscopically
What are three factors that increase the risk of progression from colonic adenoma to colon carcinoma?
Polyp size greater than 2 cm, sessile growth, and villous histology
What is sessile growth?
Polyp that is growing flat with no stalk
What is the genetic inheritance pattern of Familial Adenomatous Polyposis (FAP)? What is the gene and chromosome affected?
Autosomal dominant; APC gene on chromosome 5
How many polyps tend to be present in FAP?
100s-1000s
If a patient is diagnosed with FAP- whtat is the next step in treatment?
Remove rectum and colon
Why is it necessary to remove the colon and rectum in a patient with FAP?
Because almost all patients will develop carcinoma by the age of 40
Patient with a history of FAP also develops fibromatosis and osteomas- what is the diagnosis?
Gardner syndrome
What is fibromatosis?
non-neoplastic proliferation of fibroblasts
Where is the most common location for fibromatosis to occur?
Retroperitoneum and locally destroys tissue
What is an osteoma?
Benign tumor of bone
Where is the most common location of an osteoma
Skull
A patient with FAP also develops has CNS tumors- what is the diagnosis?
Turcot syndrome
What are the two most common CNS tumors in Turcot syndome?
Medulloblastoma and Glial tumors
Colorectal carcinoma arises from what tissue?
Mucosa of rectum or colon
In terms of incidence and mortality where does Colorectal carcinoma rank?
Incidence - 3rd
Mortality - 3rd
What is the peak incidence of age in Colorectal Carcinoma?
60-70 years
Colorectal carcinoma most commonly arises from what?
Adenoma-carcinoma sequence
What is a second molecular pathway that can often lead to colorectal carcinoma?
Microsatellite Instability
What are Microsatellites?
Repeating sequences of noncoding DNA
Instability of the Microsatellites indicate what mechanism is defective?
DNA copy mechanism (e.g DNA mismatch repair enzymes)
What is the cause of Hereditary Nonpolyposis Colorectal Carcinoma?
Inherited mutation in DNA mismatch repair enzymes
Patients with Hereditary Nonpolyposis Colorectal Carcinoma are at an increased risk for developing what two other types of cancer?
Ovarian and endometrial
Is there any precursor lesion/ polyp in Hereditary Nonpolyposis Colorectal Carcinoma?
No - it arises de novo
Is Hereditary Nonpolyposis Colorectal Carcinoma usually right sided or left sided in the colon?
Right
At what age screening for colorectal carcinoma begin?
50
Screening for colorectal carcinoma is done with what 2 tests?
Colonoscopy and fecal occult blood testing
Patient presented with decreased stool caliber, blood streaked stool, and a malignant "napkin ring" lesion was seen on biopsy - what side of the colon is this carcinoma on?
Left sided
Elderly adult presents with a iron-deficiency anemia and vague pain, and a malignant lesion is seen on biopsy- what side of the colon is this carcinoma on?
Right sided
An older adult with an iron deficiency anemia has_____ _____until proven otherwise?
Colorectal carcinoma
What is the characteristic lesion of left sided colonic carcinoma?
Napkin-ring lesion
What is the characteristic lesion of right sided colonic carcinoma?
Raised lesion
Colonic carcinoma is associated with an increased risk of endocarditis due to what organism?
Strep. Bovis
Do colorectal tumors that are located to the mucosal layer usually spread to the lymph nodes?
No
Why do colorectal tumors located in the mucosa not often spread to the lymph nodes?
There is no lymph located in in the mucosal layer
What is the most common site of metastasis in colorectal carcinoma?
Liver
What serum marker is elevated in colorectal carcinoma?
CEA
Out of the following three an increase CEA is beneficial in:
Assessing Treatment Response
Screening
Detecting Recurrence
Assessing treatment response
Detecting recurrence
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