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494 Cards in this Set

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What are the congenital/ hereditary diseases of bones and joints?
Achondroplasia, Osteogenesis Imperfecta, and Osteropetrosis
What are the metabolic diseases of bone and joints?
Rickets, Osteomalacia, Osteoporosis, and Hyperparathyroidism
What is Achondroplasia?
The most common form of Dwarfism
What is Osteogenesis imperfecta?
AKA brittle bone disease, pt have abnormal dentition (dentinogenesis imperfecta type I), hearing loss due to fractured ossicles, and pt have blue sclera
What is osteopetrosis?
AKA Marble bone disease, caused by defective resorption of bone by osteoclasts
What is the difference between rickets and osteomalacia?
Rickets is in developing bone and osteomalacia is in developed bone. Both are caused by vitamin D deficiency resulting in deficient mineralization of osteoid
What findings are common in osteomalacia?
Low Ca and P, High alkaline phosphatase. Also fractures, bone pain and cysts
Who gets osteoporosis?
Most commonly seen in post menopausal women
What findings are common in osteoporosis?
Decreased estrogen associated with increased IL-1, IL-6 and TNF promoting bone resorption. Normal serum Ca, P, and alkaline phosphatase
What does parathormone do?
Regulates blood calcium levels?
What causes primary hyperparathyroidism?
80% parathyroid adenoma 20% parathyroid hyperplasia. Pt have increased serum Ca and decreased phosphate
What is Paget’s Disease?
Osteitis Deformans. Possible cause is a slow virus (paramyxovirus) infection
What serum findings are common in pagets disease?
Very High serum alkaline phosphatase and serum Ca may be elevated
Know the metabolic comparisons table on slide 16
Where is the problem in Osteomalacia?
Deficient mineralization
Where is the problem in Osteoporosis?
Deficient Matrix
Where is the problem in hyperparathyroidism?
Increased resorption
What is the most common cause of osteomyelitis?
Staphylococcus aureus which causes a pyogenic infection
What is Brodie’s abscess?
Rim of sclerotic bone surrounding a residual abscess (Chronic subacute osteomyelitis
What is Potts disease?
Tuberculous osteomyelitis of the spine
What is osteoarthritis?
A Chronic degenerative joint disorder
What is the most common agent in Acute suppurative arthritis?
S. aureus
What causes gout?
Tissue accumulation of uric acid. Primary 90% hyperuricemia due to inborn error of purine metabolism. Secondary 10% due to things like chronic renal disease and leukemia
What is seen histologicaly in gout?
Tophi (crystalline aggregates of uric acid
What causes most malignant bone tumors?
Secondary metastases from area like breast, bronchus, kidney, prostate, thyroid, and GI
What is characteristic of osteoid osteoma?
1-2 cm nodule with central osteoid nidus. Benign.
What is osteosarcoma?
The most common primary malignant bone tumor. 3rd most common malignancy in adolescents. It is a Mesenchymal tumor producing osteoid. May be associated with Paget’s disease
Where do osteosarcoms oftem metastasize to?
The lung, this is a highly malignant tumor
What is the typical behavior of a chondroma (Enchondroma)?
Usually benign. Transformation to chondrosarcoma in 30% of pts with multiple lesions
What type of cells are seen in a giant cell tumor?
Spindle and osteoclast multinucleated giant cells (Look at the picture on slide 40. These tumors are usually benign but 50% recur
What causes Ewing’s sarcoma?
11
How common is Ewing’s sarcoma?
2nd most common bone malignancy in children. This is a highly aggressive tumor
What are the manifestations of Albright’s syndrome?
1. Polyostotic fibrous dysplasia 2. Café au lait spots 3. Precocious puberty in females
What disease did the elephant man have?
Proteus syndrome
What is the most common cause of myofiber atrophy?
Type II (disuse atrophy)
What causes Myasthenia gravis?
Autoimmunity to ACH receptor
How do pts with myasthenia gravis present?
15-20% have thymoma, 65% have thymic hyperplasia. Prominent ptosis and diplopia
What is Duchenne dystrophy?
Absence of dystrophin (attaches the actin cytoskeleton to the sarcolemma). X linked disease
That is Becker dystrophy caused by?
X linked, Dystrophin present but abnormal less severe disease than Duchenne dystrophy
What is a malignant skeletal muscle tumor called?
Rhabdomyosarcoma
What is the function of the gallbladder?
Digestion, storage of bile (~50ml)
What does the cystic duct do?
Connects neck of gallbladder to the biliary tree, joins with the common hepatic duct to form the common bile duct which empties directly into duodenum
What are the 5 layers of the gallbladder (luminal to serosal)?
1. Epithelium 2. Lamina propria 3. Muscularis 4. Perimuscular layer 5. Serosa
What does the epilthelium of the gallbladder look like?
It has mucin containing columnar cells and finger like projections
How common are lymphocytes in the lamina propria of the gallbladder?
Scant lymphocytes
What is the pathway of the biliary tract within the liver?
Bile canaliculi to canals of hering to intrahepatic bile ducts to R and L hepatic ducts which merge to form the common hepatic duct which exits the liver
What is the pathway of the extrahepatic biliary tract?
Common hepatic duct joins the cystic duct to form the common bile duct which joins the pancreatic duct and empties into the duodenum through the ampulla of vater
What type of emithelium is found in bile canaliculi?
Low cuboidal biliary epithelium
What 2 things make up 95% of biliary tract disease?
Cholelithiasis and cholecystitis
What is cholelithiasis?
Presence of calculi (stones) in the gallbladder
What are the 2 types of gallstones?
Cholesterol stones – 80% of cases in west, crystalline cholesterol monohydrate. Pigment stones – calcium bilirubinate salts
How is excess cholesterol removed from the body?
Only as free cholesterol or as bile salts, cholesterol is water insoluble if the concentration exceeds capacity of bile to solubilize solid cholesterol monohydrate crystals are formed leading to cholesterol stones
The pathogenesis of cholesterol stones involves what 4 simultaneous conditions?
1. Supersaturation of bile with cholesterol 2. Establishment of nucleation sites by precipitation of calcium salts 3. Hypomobility of gallbladder (stasis – promotes nucleation) 4. Mucous hypersecretion that traps crystals
What promoted pathogenesis of pigment stones?
Presence of unconjugated bilirubin in biliary tree
What 3 things are more specific of cholesterol stones?
In the gallbladder only, paleyellow to gray-white, most are radiolucent 80%
What three things are common of pigment stones?
Occur anywhere in biliary tree, black (sterile) and brown (infected), and most are radiopaque 50-75%
What is cholecystitis?
Inflammation of the gallbladder, usually associated with gallstones
What are the 2 types of acute cholecystitis?
Calculous – associated with gallstones and Non-calculous which have an absence of gallstones
What is the most common complication of gallstones?
Acute calculous cholecystitis. Caused by obstruction of gallbladder neck or cystic duct by stones leading to acute inflammation. This is also the most common reason for emergency cholecystectomy
What is the pathogenesis of acute calculous cholecystitis?
Obstruction of bile outflow leading to 1. Production of toxic biliary lysolechithins by mucosal phospholipases which are toxic to gallbladder mucosa leading to disruption of protective glycoprotein mucous layer exposing epithelial cells directly to bile salts 2. Release of prostaglandins
In what 4 clinical settings is acute non-calculous cholecystitis seen?
1 postoperative state after major surgery 2 severe trauma 3 severe burns 4 sepsis
How is the diagnosis of cholecystitis made?
Detection of gallstones and bile duct distention on ultrasonography
What cell type is found in chronic cholecystitis?
Lymphocytes (chronic inflammation)
What are some complications of cholecystitis?
Bacterial superinfection leading to cholangitis and sepsis. Gallbladder perforation leading to abscess formation. Gallbladder rupture leading to diffuse peritonitis. Gallstone induced intestinal obstruction
What is the most common malignancy of the biliary tract?
Carcinoma of the gallbladder (5th most common cancer of digestive tract)
When is the diagnosis of carcinoma of the gallbladder made?
Preoperative diagnosis is very rare 20% 0f pts. More often diagnosis is made at the time of cholecystectomy
What are most carcinoma of the gallbladder?
Adenocarcinomas
What is the prognosis for carcinoma of the gallbladder?
5 yr survival ~1% at diagnosis it has often invaded liver, cystic duct, adjacent bile ducts, portahepatic lymph nodes
What is choledocholithiasis?
Presence of stones in the biliary tree
How is choledocholithiasis treated?
Extraction of stones by endoscopic retrograde cholangiopancreatography, percutaneous extraction, or shock wave lithotripsy
What is Cholangitis?
Acute inflammation of bile ducts
What is ascending cholangitis?
Acute inflammation of bile ducts, “ascends” to involve intrahepatic bile ducts
What are different etiologies of cholangitis?
Obstruction of bile flow, retrograde spread of organisms and inflammation/ infection
What bacteria are seen in cholangitis?
E. coli, Klebsiella, Clostridium, Bacteriodes, Enterobacter, and group D streptococci
What happens when cholestasis is not corrected?
Inflammation and fibrosis of the liver leading to irreversible changes
What is seen in the bile ducts in secondary biliary cirrhosis?
Distended bile ducts with pigmented material and proliferation of bile ductules
What is biliary atresia?
Destruction or absence of extrahepatic bile ducts leading to complete obstruction of bile flow
What are features of biliary atresia?
1. Inflammation and fibrosing stricture of hepatic or common bile ducts 2. Progressive destruction of intrahepatic biliary tree 3. Features of biliary obstruction on liver biopsy 4. Periportal fibrosis and cirrhosis within 3-6 mo of birth
What are the 4 features of bile duct obstruction?
Bile duct distension, bile ductular proliferation, cholestais, and portal tract edema and fibrosis
*What is the leading cause of death from liver disease in early childhood?
Biliary atresia. prognosis – death within 2 yrs if no surgical treatment
What are the risk factors of cholangiocarcinoma?
Anything causing chronic cholestasis and inflammation. 1 primary sclerosing cholangitis 2. Fibrocystic disease of biliary tree 3. Previous exposure to thorotrast 4 chronic infection by parasite (Opisthorchis sinensis AKA Chinese liver fluke)
What are the lab findings for cholangiocarcinoma?
Elevated serum aminotransferases and elevated serum alkaline phosphatase
What are the three main groups of cells in the skin?
Keratinocytes, Langerhan’s cells, Melanocytes.
Which of the skin cells is the most important according to Dr. G?
Langerhans cells because they are part of the immune system….they are antigen-presenting cells.
What are the 5 layers of the epidermis?
Stratum Basale, Stratum spinosum, Stratum granulosum, stratum lucidum, stratum corneum.
What is a macule?
Flat colored lesion
What is a Papule?
Raised solid area <5mm across
What is a Nodule?
Rased solid area >5mm
What is a Plaque?
Raised flat-topped area, usually >5mm
What is a Vesicle?
Fluid filled raised area < 5mm
What is a Bulla?
Fluid-filled raised area > 5mm
Blister:
Common name for vesicle or bulla
What is a pustule?
A pus filled raised area
What is a scale?
Dry, horny, plate like excrescence
What is Lichenification?
Thickend rough skin, usually due to repeated rubbing
What is Excoriation?
Breakage of the epidermis, due to scratching sometimes self inflicted
What is Onycholysis?
Separation of the nail plate from the nail bed.
What is acanthosis?
Diffuse epidermal dysplasia
What is hyperkeratosis?
Hyperplasia of the stratum corneum
What is Parakaratosis?
Retention of nuclei in the stratum corneum
What is Dyskeratosis?
Abnormal keratinization occurring prematurely within cells below the stratum granulosum
What is acantholysis?
Loss of intercellular connections between keratinocytes
What is hypergranulosis?
Hyperplasia of the statum granulosum, often due to rubbing.
What is papillomatosis?
Hyperplasia of the papillary dermis with elongation of the dermal papillae
What is lentiginous?
Linear pattern of melanocyte proliferation
What is Spongiosis?
Intercellular edema of the epidermis
What is Hydropic swelling/ballooning?
Intracellular edema of keratinocytes, often seen in viral infections.
What is Exocytosis?
Infiltration of the epidermis by inflammatory or circulating blood cells
What is Erosion?
Discontinuity of the skin exhibiting incomplete loss of the epidermis
What is Ulceration?
Discontinuity of the skin exhibiting complete loss of the epidermis.
What are the three acute inflammatory dermatoses that we studied?
Uticaria, Eczematous dermatitis and Erythema multiforme
What is Uticaria?
Usually a type I hypersensitivity, IgE mediated (hives), common to find Eosinophils in the dermis….could also be caused by IgE independent methods like antibiotics (vancomysin)
What is acute eczematous dermatitis?
Aka spongiotic dermatitis….a clinical term describing lesions that are red, papulovesicular, oozing/crusting, and raised/scaly plaques.
What are the five clinical categories of dermatitis?
“allergic” contact, Atopic, Drug-related, Photoeczematous (UV light), and Irritant(repeated trauma or chemical).
What are the three features of acute eczematous dermatitis?
Epidermal spongiosis, Epidermal hyperplasia, and hyperkeratosis?
Why is allergic contact dermatitis a misnomer?
Because it is not atopy and is IgE independent. It is a TH1 reaction, delayed type hypersensitivity (type IV)
What will occur with persistant atigen stimulation of the type IV hypersensitivity type?
Acanthosis (epidermis thickens) and Hyperkeratotic (lesions become scaly)
How do type IV hypersensitivity reactions occur?
Low weight molecules enter sweat glands and antigens act as haptens to activate TH1 response.
What is Erythema Multiforme?
Hypersensitivity to certain infections and drugs….but CTL mediated, can also be caused by carcinomas or lymphomas or SLE
What are the common infections that cause eythema multiforme?
Herpes simplex, mycoplasma, histoplasmosis, Coccidiodomycosis, typhoid, and leprosy
What drugs can cause the erythema multiforme?
Sulfonamides and penicillins are the most common.
What are three clinical presentations of Erythema Multiforme?
Target lesions, Steven Johnsons syndrome, and Toxic epidermal necrolysis
What is steven Johnsons syndrome?
A clinical presentation of erythema multiforme where mucosal lesions are common. It seems to be due to a reaction to antibiotics and is more common in kids. Has a 5% mortality rate…can lead to blindness
What are the 3 chronic inflammatory dermatoses?
Psoriasis, Lichen Planus, and Cutaneous lesions of SLE.
How common is psoriasis?
It is one of the most common skin disorders affecting 1-2% in the US.
What are 3 characteristics of psoriasis?
Skin lesions are well-dmarcated pink plaques covered by silver-white scales, Nail changes are common, and psoriatic arthritis occurs in 5% of patients.
What is diagnostic of psoriasis?
Pitted nails.
What is the likely pathogenicity of psoriasis?
T-cell mediated reaction to unknown antigen with a genetic susceptibility componentt (50% have HLA B27)
Which other condition is closely associated to HLA B27?
Ankylosing spondylitis which also has an arthritic component which makes researches suspect involvement in arthritic conditions.
What is the auspitz sign?
Dilated blood vessels that cause multiple minute bleeding points.
What are the 4 P’s of lichen Planus?
Pruritic, Purple, Polygonal papules.
What are wickham striae?
Papules often highlighted with white dots or lines
How do you treat lichen planus?
It resolves spontaneously after a year or two, but can leave zones of hyperpigmentation. May be due to a cytotoxic autoimmune response.
What are 3 histological characteristics of lichen planus?
Saw tooth rete ridges, epidermal hyperplasia, and lymphocytes along the dermo-epidermal junction.
What are primary and secondary blistering/bullous diseases?
Primary are where the blisters are the most distinctive feature and secondary the blisters are a feature of another disease like HSV and dermatitis.
Where do pemphigus vulgaris blisters appear?
Suprabasal
What is the common site for bullous pemphigoid and dermatitis herpetifomis?
Sub epidermal.
What blister/bullous condition occurs in the subcorneal layer?
Impetigo and pemphigus foliaceaus.
What is pemphigus?
A rare autoimmune blistering disorder that can be fatal with four variants
What variant of pemphigus is the most common?
Pemphigus vulgaris accounts for 80%
What is pemphigus vulgaris like?
Involves the mucosa and skin with superficial vesicles and bullae that rupture easily and leave shallow erosions.
What are two histological characteristics of pemphigus?
Suprabasal acantholysis resulting in intraepidermal blisters.
What causes pemphigus?
It is a type II hypersensitivity reaction with pathogenic IgG autoantibodies to desmoglein 3 in the skin and mucous membranes.
What does the immunoflourescent pattern of pemphigus look like?
A fishnet pattern along the membranes of the epidermal keratinocytes.
What is pemphigoid?
A relatively common autoimmune disease with two forms that cause subepithelial blisters in mouth, skin or eyes. It is a type II hypersensitivity response to basement membrane antigens.
What are the two forms of pemphigoid?
Mucous membrane/cicatricial pemphigoid and Bullous pemphigoid.
Which type pemphigoid is the most common in the oral cavity?
Mucous membrane/cicatricial with gingival lesions in 90% and buccal mucosa, palate affected in 30%. Bullous pemphigoid can cause oral lesions in up to 40%.
What is the site of bullous pemphigoid?
Sub-epidermal blisters…the blister roof is the full thickness of the epidermis, this causes subepidermal separation
What does bullous pemphigoid look like under immunofluorescence?
You will see linear deposition of IgG and complement along the dermo-epidermal junction.
What is dermatitis herpetiformis?
A rare bullous disorder with uticaria, plaques, vesicles etc and has a strong association with celiac disease….can be treated by a gluten free diet.
Where does dermatitis herpetiformis occur?
Begins as dermal abscesses with basal cell vacuolization that coalesce to form subepidermal blister.
What are the four types of skin tumors that we discussed?
Bening and pre malignant epithelial tumors, Malignant epidermal tumors, tumors of the melanocytes, and Malignant melanomas.
What is seborrheic keratosis?
Common epidermal tumors in older people with proliferating basal keratinocytes…Morgan Freeman spots.
What is a keratoancanthoma?
Nodules with central keratin plug, common in sun exposed area like the lower lip and may mimic SCC.
What are verrucae?
Warts….some caused by HPV w/out oncogenic potential, verucca vulgaris are the most common type with epidermal hyperplasia that radiates symmetrically.
What is actinic keratosis?
Skin dysplasia associated with chronic sun exposure, may develop a cutaneous horn and some lesions progress to SCC in situ.
What is the second most common tumor arising in sun exposed site in older people?
SCC also many other predisposing factors.
What is the risk of distant metastasis for SCC?
They don’t usually metastasize to distant sites, 5% metastasize to regional lymph nodes.
How common is basal cell carcinoma?
It is the most common skin cancer, they don’t occur on mucosal surfaces, are slow growing and are sometimes called rodent ulcers due to bone or sinus invasion.
What are some characteristics of basal cell carcinomas?
Pearly telangiectatic nodules composed of nests of basaloid cells within the dermis and palisading of peripheral tumor cells is seen.
What is a pigmented nevus?
Congenital or acquired neoplasm of melanocytes…the common acquired pigmented nevi (moles) are benign and consist of nests of melanocytes.
What are the early and older lesions of acquired pigmented nevi?
Nests of melanocytes at the dermal epidermal junction form junctional nevi or early lesions, within the dermis are intradermal nevi/older lesions or you might have a compound nevus that is both.
What are the two types of dysplastic nevi?
Familial form which are autosomal dominance with a high risk of malignant transformation and the sporadic form which have low risk of transformation
Which type of dysplastic nevi are worse familial or sporadic?
The familial form as they have a high risk of malignant transformation.
What are some of the characteristics for dysplastic nevi?
They are common in both sun-exposed and protected skin.
What is malignant melanoma?
Common, most cured surgically, most arise in sun exposed areas. Can occur in sites that are not usually visible…these are the worst as they can spread and grow unchecked.
Are genetics involved in malignant melanomas?
Yes the P16INK4A (Ch 21) is the most commonly mutated gene in familial melanoma
What is the most important clinical sign of malignant melanoma?
The change in size, color, or shape of a piqmented lesion.
What are the clinical warning signs of a melanoma?
Enlargement of a mole, itching or pain in a mole, development of a new pigmented lesion as an adult, irregular boders of a pigmented lesion, and the change in color within a pigmented lesion.
What type of growth occurs in early melanoma?
Radial growth within the epidermis and superficial dermal layers, cells don’t metastasize at this stage
What type of growth occurs in an advanced melanoma?
Vertical growth with malignant spread into the dermis and vessels. This growth is where cells with metastatic potential are seen. This is why we take deep biopsies for melanomas. Advanced lesions are usually nodular.
What are the common sites for malignant melanoma metastasis?
Regional lymph nodes, liver, lungs, and brain.
What do all esophageal disorders have in common?
They all produce dysphagia
What is dysphagia attributed to?
Either Deranged esophageal motor function or Narrowing or obstruction of the lume
What type of epithelium is seen in Barrett’s esophagus?
The epithelium of the esophagus is replaced with gastric epithelium
What is hematemesis?
Vomiting of blood, massive hematemesis may reflect life threatening rupture of esophageal varices
What is melena?
Blood in the stools
What is a hiatal hernia?
Dilated segment of stomach protrudes above the diaphragm
What does a hiatal hernia permit?
Separation of diaphragmatic crura, and widening of space beteen muscular crura and esophageal wall
What are the two forms of hiatal hernia?
Sliding hiatal hernia, and paraesophageal (rolling) hiatal hernia
What do pts with severe reflux esophagitis also have?
A sliding hiatal hernia. However, most pts with sliding hiatal hernia don’t have reflux esophagitis
Which type of hernia is more common?
Axial (sliding) hernia
What do 9% of adults with hiatal hernias report?
Heartburn or regurgitation of gastric juices into the mouth
What does Achalasia mean?
Failure to relax. Denotes incomplete relaxation of lower esophageal sphincter in response to swallowing. Produces functional obstruction of esophagus leading to dilation of the more proximal esophagus
What are 3 major abnormalities that cause achalasia?
Aperistalsis, Partial or incompleterelaxation of lower esophageal sphincter w/ swallowing, and increased resting tone of lower esophageal sphincter
What is seen in primary achalasia?
Loss of intrinsic inhibitory innervations of the lower esophageal sphincter and progressive dilation of esophagus above level of lower esophageal sphincter
What is one possible cause of secondary achalasia?
Chagas disease - inflammation in the location of esophageal mesenteric plexus is pathognomonic of the disease
How is achalasia characterized clinically?
Progressive dysphagia, inability to completely convey food to the stomach, nocturnal regurgitation, and aspiration of undigested food.
What is the most serious complication of achalasia?
Development of esophageal squamous cell carcinoma in ~5% of pts
Where do varices occur?
At communications between intra-abdominal splanchnic circulation and systemic venous circulation. The esophagus is one potential site
What causes esophageal varices?
Increased pressure in esophageal plexus producing dilated tortuous vessels called varices
what type of cirrhosis is associated with varies?
Most often alcoholic cirrhosis. 50% of pt with advanced cirrhosis of the liver die as a result of rupture of a varix
What do 50% of pts with varices also found to have?
Coexistent hepatocellular carcinoma
What is the outcome when varices bleed?
20-30% of pts die during the 1st episode. Re-bleeding occurs in 70% w/in 1 yr, similar rate of mortality for each episode
What may cause inflammation in the esophagus?
Prolonged gastric intubation, Uremia, ingestion of corrosive or irritant substances, radiation or chemotherapy, and other things
What is esophagitis attributed to in Western countries?
Reflux of gastric contents
What is the dominant symptom of reflux esophagitis?
Recurrent heartburn
What are the presumed contributory factors of esophagitis?
Decreased efficacy of esophageal antireflux mechanisms, inadequate or slowed esophageal clearance of refluxed material, presence of a sliding hiatal hernia, increased gastric volume contributing to the volume of refluxed material, and impaired reparative capacity of the esophageal mucosa by prolonged exposure to gastric juices
What are the 3 histologic features characteristic of uncomplicated reflux esophagitis?
Eosinophils – W/ or w/o PMNs in epithelial layer, Basal zone hyperplasia, and Elongation of lamina propria papillae w/ vascular lakes
What do intraepithelial neutrophils indicate?
Severe injury
What is Barrett Esophagus?
The replacement of normal distal stratified squamous mucosa by metaplastic columnar epithelium containing goblet cells
What can develop from Barrett Esophagus?
Adenocarcinoma. Pts have 30-40 fold greater rick than normal populations
In which racial group is Esophageal carcinoma more common worldwide?
Blacks
What makes up 90% of esophageal cancers worldwide?
Squamous cell carcinomas
What is the most common type of esophageal cancer in the US?
Adenocarcinoma. Exponential increase in incidence of adenocarcinomas associated w/ barrett esophagus
In which group is Barrett esophagus more common?
Whites. Which means more whites are getting adenocarcinomas
With which virus is there a strong association with esophageal carcinoma?
HPV
What is the only recognized precursor of esophageal adenocarcinoma?
Barrett esophagus
What is the pattern of squamous cell carcinoma throughout the esophagus?
20% arise in cervical and upper thoracic esophagus, 50% in middle third, and 30% in lower third
In which part of the esophagus is an adenocarcinoma likely to arise?
Usually in the distal one third of the esophagus in relation to Barrett esophagus
What is the most common gastrointestinal infection?
Gastric infection w/ helicobacter pylori
What does vomited blood look like?
Coffee grounds b/c blood in the stomach quickly congeals and turns brown in acid environment of stomach lumen
What is gastritis?
Inflammation of gastric mucosa
What is chronic gastritis?
Presence of chronic mucosal inflammatory changes leading to mucosal atrophy and epithelial metaplasia
What is chronic gastritis associated with?
Chronic infection by the bacillus Helicobacter
What are the characteristics of Helicobacter?
Noninvasive, non-spore forming, S shaped, Gram neg rod
Where does helicobacter have the highest infection rates?
Developing counties
What is the prognosis for chronic gastritis and H. pylori?
Usually improve symptomatically when treated with antimicrobial agents. Improvement in underlying chronic gastritis may take much longer
What are the 4 layers of a peptic ulceration?
Necrotic debris, inflammatory layer, granulation tissue, and fibrous scar
Where is H. pylori found?
Nestled with in mucus layer overlying superficial mucosal epithelium
What is intestinal metaplasia?
An additional feature of chronic gastritis. Replacement of gastric epithelium with columnar and goblet cells of intestinal variety. Gastrointestinal type carcinomas appear to arise from dysplasia of metaplastic epithelium
What has been implicated as a precursor of gastric lymphoma?
H. pylori induced proliferation of lymphoid tissue with in gastric mucosa
What is hypochlorhydia?
A condition of insufficient gastric acid ro release vitamin B12 from the protein in food. May be a symptom of chronic gastritis when parietal cells are partially destroyed
What causes peptic ulcer?
H. pylori infection
What is the risk of a pt with chronic gastritis developing gastric carcinoma?
For pt with H. pylori associated chronic gastritis risk is increased 5 fold. For autoimmune gastritis risk ranges from 2-4% of affected individuals well above that of normal population
What does acute gastritis cause?
Mucosal hemorrhage and sloughing of superficial mucosal epithelium. The severe erosive form of disease is an important cause of acute gastrointestinal bleeding
What is acute gastritis frequently associated with?
Heavy use of NSAIDs particularly aspirin. Injury is extremely localized
What are markers of acute gastritis?
Mucosal edema, inflammatory infiltrate of neutrophils, and possibly chronic inflammatory cells
What is gastric ulceration?
A breach in mucosa of alimentary tract extending through muscularis mucosa into submucosa or deeper. In contrast to erosion which is a breach in mucosal epithelium only
Where do peptic ulcers occur?
In any portion of GI tract exposed to aggressive action of acid-peptic juices. 98% in the first portion of duodenum or in the stomach
What is the life time risk of developing peptic ulcer disease for men and women in the US?
20% genetic or racial influences play little or no role in causation
What are 4 common conditions that are more frequent in pts with duodenal ulcers?
Alcoholic cirrhosis, chronic obstructive pulmonary disease, chronic renal failure, and hyperparathyroidism
What does hypercalcemia cause in relation of ulcers?
What ever the cause of the hypercalcemia it stimulates gastrin production leading to acid secretion
What are two key factors in peptic ulcer formation?
Fundamental requisite for peptic ulceration – mucosal exposure to gastric acid and pepsin, and strong causal association with H. pylori infection
What % of pts with ulcers have H. pylori infections?
79090% of pts with duodenal ulcers, 70% of those with gastric ulcers
What pro-inflammatory cytokines is a possible mechanism of peptic ulcer formation?
Most notably IL-8 produced by mucosal epithelial cells, it recruits and activates PMNs
What % of people with H. pylori infections actually develop a peptic ulcer?
10-20%
What are the gastroduodenal effects of NSAIDs?
Range from acute erosive gastritis to acute gastric ulceration to peptic ulceration in 1-3% of users. It is a magor cause of peptic ulcer disease in pts who do not have H. pylori infection
What is key to NSAID induced peptic ulceration?
Suppression of mucosal prostaglandin synthesis which increases secretion of hydrochloric acid, reduces bicarb and mucin production, and reduces synthesis of glutathione a free radical scavenger
What effect does smoking have on stomach mucosa?
Impairs blood flow and healing
What association does alcohol have to peptic ulcers?
Has not been proved to directly cause peptic ulceration but alcoholic cirrhosis is associated with increased incidence of peptic ulcers
What association do corticosteroids have on peptic ulcers?
In high dose and with repeated use promote ulcer formation
How is Zollinger-Ellison syndrome associated to peptic ulcers?
Associated with multiple peptic ulcerations in stomach, duodenum, and jejunum resulting from excess gastrin secretion by a tumor leading to excess gastric acid production
What do pts with NSAID associated peptic ulcers need to have to have gastritis?
A coexistent H. pylori infection
What is the chief complication in peptic ulcers?
Bleeding which occurs in up to 1/3 of pts and may be life threatening
What account for the majority of deaths from peptic ulcer disease each yr?
perforation
Where are stress ulcers found?
Generally multiple lesions located mainly in stomach and occasionally in duodenum
What causes stress ulcers?
Severe trauma like major surgical procedures, sepsis, and grave illness of any type
What are stress ulcers associated with extensive burns called?
Referred to as curling ulcers
What are stress ulcers associated with traumatic or surgical injury to CNS or intracerebral hemorrhage called?
Cushing ulcers
How are acute stress ulcers unlike chronic peptic ulcers?
Acute stress ulcers are found anywhere in the stomach
What is the most important determinant of clinical outcome in stress ulceration?
Ability to correct underlying condition
What are gastric polyps?
Any nodule or mass that projects above level of surrounding mucosa in the GO tract term is generally restricted to mass lesions arising in mucosa
What are the different kinds of polyps found in the stomach?
Hyperplastic and fundic gland polyps which are essentially innocuous, and Adenomatous polyps which have a definite risk of harboring adenocarcinoma
What are the characteristics of adenomas?
They are less common, contain dysplastic epithelium and gastric adenomas are true neoplasms
What is the order for frequency of malignant tumors in the stomach?
#1 caricnoma 90-95%, Lymphoma 4%, carcinoids 3%, mesenchymal spindle cell tumors 2%
Which type of gastric cancer has not changed in frequency in past 60 yrs?
Diffuse. Which arise de novo from native gastric mucous cells and are not associated with chronic gastritis accounts for 50% of gastric carcinomas in the US?
What is the most important prognostic indicator in gastric carcinoma?
Stage of tumor at time of resection
What is Oliguria and Anuria?
Reduced and no urine flow
What is Azotemia?
Increased blood urea nitrogen and creatinine levels usual related to decreased GFR.
What is prerenal azotemia vs. postrenal azotemia?
Prerenal azotemia occurs with hypoperfusion of the kidneys and post renal azotemia is due to an obstruction below the kidney.
What is uremia?
Clinical signs and issues associated with azotomia like gastroenteritis, peripheral neuropathy, pericarditis, confussion, coma, and death.
What is a syndrome?
A collection of symptoms and signs….a given syndrome can be caused by one or more diseases
What are the 4 diseases commonly associated with nephritic syndrome?
Acute diffuse proliferative GN, Rapidly progressive/crescentic GN, IgA nephopathy, and Hereditary nephritis.
What are the 4 diseases associated with nephritic syndrome?
Lipoid Nephrosis, Membranous GN, Focal glomerulosclerosis, and membranoproliferative GN.
Which 5 types of GN can become chronic?
The post-infectious GN type of acute diffuse proliferative diffuse GN, Rapidly Progressive GN, Membranous GN, Focal glomeruslerosis, and Membranoproliferative GN.
What are the characteristics of acute nephritic syndrome?
Inflamed glomerulus and compromised filtration.
What are 5 symptoms associated with acute nephritic syndrome?
Gross/visible hematuria, Oliguria, Azotemia, Hypertension and some proteinuria/edema.
What are the main two characteristics of nephritic syndrome?
Heavy proteinuria (over 3.5 g/day), hypoalbuminemia, severe edema, hyperlipidemia, and lipiduria.
What are some other major renal syndromes?
Asymptomatic hematuria or proteinuria, acute renal failure, chronic renal failure, UTI, Neprolithiasis and others (cystic diseases of the kidney, Hydronephrosis, and renal tumors)
Which diseases commonly cause secondary glomerular diseases?
SLE, HTN, and DM
What are the two hereditary glomerular diseases?
Alport syndrome, and Fabry disease.
What are the primary glomerular diseases?
Acute diffuse proliferative, Rapidly progressive, Membranous, Lipoid nephrosis, Focal segment glomerulosclerosis, Membranoproliferatitve, IgA nepropathy, Chronic GN.
How common are immunological factors in the pathogenisis of glomerular disease?
IgG and complement deposits found in over 70% of patients with GN.
What are the two causes of injury to the glomerulus by immunological factors?
Ag-Ab complexes that create a granular immunoflourescent pattern or antibodies that bind directly to the GBM to form a linear immunofluorescent pattern.
What are 4 diseases that cause acute neprhitic syndrome?
Acute diffuse proliferative GN (the type that occurs after strept throat), IgA nepropathy (berger disease), rapidly progressive (crescentic), and Hereditary nephritis (Alport syndrome)
What is the most common cause of post-infectious glomerulonephritis?
Strept Pyogenes.
How common is recovery for children with post-infectious glomerulonephritis?
>95% children
What does post infectious glomerulonephritis look like morphologically?
Granular deposits of IgG and complement on the GBM, It is diffuse, immune complexes seen as subepithelial humps.
What is the most common form of GN in those with SLE?
Diffuse proliferative glomerular nephritis with wire loop immune complex deposits and a lumpy-bumpy pattern.
What is the most common glomerular disease in Asia?
Berger disease (10% in US)
How does Berger disease affect?
Children and young adults.
What does Berger disease look like?
Gross hematuria, one of the most common cause of recurrent hematuria, may present with acute nephritic syndrome
What is the pathogenesis for Berger disease?
Circulating IgA immune complexes deposited in the mesangium often with increased serum IgA, it is more common in patients with celiac disease.
What is the onset of rapidly prossive glomerunephitis similar to?
Nephritic syndrome….if untreated it may cause death due to renal failure.
What does rapidly progressive GN look like histologically?
Crescents in most glomeruli.
How do the three types of rapidly progressive GN differ?
Type I is an anti-GBM antibody issue (12%), Type II is an immune complex disease (44%), Type III is due to anti-neutrophil cytoplasm antibodies (44%)
What is Good pastures’s syndrome?
A rare anti-GBM antibody vs. type IV collagen….antibodies can cross react with bm of lung alveoli resulting in simultaneous lung and kidney lesions.
What is hereditary nephritis?
Alport syndrome that give hematuria and proteinuria but also may cause deafness and eye disorders
What are the main features of neprotic syndrome?
Massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia.
What is ansarca?
Massive amounts of edema can be due to nephritic syndrome.
If you see nephritic syndrome in an adult, what is it often associated with?
Systemic disease like diabetes, amyloidosis, and SLC
What are the primary causes of nephritic syndrome in a child?
Usually a primary kidney disorder like lipoid nephrosis, membranous GN, focal glomerulosclerosis.
What are the primary causes of neprotic syndrome?
Lipoid nephrosis and membranous GN.
What is Lipoid nephrosis?
Aka minimal change disease, this is the most common cause of nephritic syndrome in kids
What is unique about lipoid nephrosis?
Selective proteinuria: esp albumin, cells of the convoluted tubules contain lipids, usually respond to corticosteroid therapy.
What are the two types of membranous glomerulonephritis?
Primary 85% and secondary 15% due to SLE, or gold or mercury toxicity.
How is membranous glomerulonepritis different from Lipoid nephrosis?
Lipoid is specific for albumin where Membranous is a non-selective proteinuria.
What does membranous glomerulonephritis look like with fluorescence microscopy?
Granular deposition of Igs and complement.
What is focal segmental glomerulosclerosis?
Primary disease or secondary to HIV nepropathy, IgA nepropathy, or other forms of GN…associated with neprhotic syndrome…will see deposits of Igs and complement.
What are type I and II membranoproliferative GN like?
Type I: subendothelial deposits of immune complexes common with HBV, HCV, and SLE, and Type II: intra GBM deposits
What is the histology of the membranoproliferative GN like?
There will be a thickening and splitting of the GBM.
What is the leading cause of kidney failure?
Diabetic nephropathy…..occcurs in up to 40% of diabetics one cause of nephritic syndrome.
What are kimmelstiel-Wilson lesions?
Nodular glomerulosclerosis common in diabetes
Which type of primary glomerular disease is most likely to progress to chronic disease?
Rapidly Progressive/crescentic at 90%
Which type of primary glomerular disease is least likely to progress to chronic GN?
Post-streptococcal
What is chronic GN like?
Proteinuria, HTN, Azotemia, Edema and microscopic hematuria. Microscopic hyalinization of the glomeruli is common.
What are the two inflammatory tubulointerstitial nephritic lesions we discussed?
Pyelonephritis and drug induced interstitial nephritis
What type of tubulointerstitial nephritic lesions can toxicity and ischemia cause?
Acute tubular necrosis.
What is the most common cause of acute pyelonephritis?
E. Coli……but also notably Proteus
What is acute pyelonephritis?
Usually an ascending infection by E. coli most commonly, much more common in females than in males. If it occurs in males something is wrong with UT (prostatic hyperplasia)
What are 4 risk factors for acute pyelonephritis?
Vesico-ureteral valve reflux (can be congenital or due to spinal cord injury), diabetes, pregnancy, and catheters
What does acute pyelonephritis look like clinically?
Sudden onset, chills/fever, dysuria, sudden onset, Pyuria, bacteria, and proteinuria.
What does acute pyelonephritis look like morphologically and histologically?
Morphologically: abscesses on renal surface and pyonephrosis (renal pelvis is filled with pus), and histologically you will find suppurative necrosis, lots of PMNs and, wbc casts in urine.
What are the two forms of chronic pyelonephritis?
Chronic obstructive and Chronic reflux-associated (most common)
What are the two types of drug induced interstitial nephritis’s?
Acute drug-induced interstitial nephritis and Analgesic nephropathy.
What are the characteristics of acute-drug induced interstitial nephritis?
Usually with synthetic penicillins, IgE mediated, eosinophilia is common, acute renal failure is common.
What are the characteristics of analgesics nephropathy (chronic interstitial nephritis)?
Usually Asprin or acetaminophen in high doses. Chronic renal failure may occur, can improve with drug cessation, and increased risk of renal and bladder cancer.
What is the most common cause of acute renal failure?
Acute tubular necrosis…..even though it can be reversible
What are the two types of acute tubular necrosis?
Ischemic (blood loss, hemolytic crisis etc.) and Nephrotoxic (Poisons, heavy metals, etc.)
What does the histology of acute tubular necrosis look like?
Proteinaceous casts in distal tubule and collecting ducts consisting of Tamm-Horsfall protein a normal tubule secretion or Myoglobin when due to crush injury.
What are the three phases of acute tubular necrosis?
Initiating (first 36 hrs w/oliguria and azotomeia), Maintenance (begins at 2-6days w/oliguria or anuria and uremia may last 3 weeks and patients can die w/out treatment) and Recovery with a steady increase in urine volume….about 25% of deaths from ATN occur during this phase.
What are two kidney diseases associated with HTN?
Benign nephrosclerosis as in Hyaline arteriosclerosis where patient rarely die from kidney failure, or malignant nephrosclerosis with diastolic over 120 mmHg and protein leaks (hematuria and proteinuria).
What is the prognosis like for those with malignant nephrosclerosis due to HTN?
50% have a 5 yr survival and 90% of deaths are due to renal failure.
What are the three hallmarks of malignant nephrosclerosis?
Petichial hemorrhages on cotical surface that looks flea bitten, Fibrinoid necrosis of arterioles and Onion skin areteriolitis.
What are the two thrombotic microangiopathies of the kidney?
Hemolytic-uremic syndrome and thrombotic thrombocytopenia purpura (rare genetic issue with vWF.
What are the characteristics of Hemolytic-uremic syndrome?
Due to E. coli 0157:H7 with a shigella like toxin, hemolytic anemia, endothelial injury and activation DIC
What is urolithiasis?
Kidney stones
What are kidney stones like?
Mostly unilateral, 75% are calcium oxalate with or without calcium phosphate, They can be smooth or jagged (stag horn: casts of renal pelvis/calyx) and can cause hematuria.
What are 4 types of renal stones?
Calcium (75%), Struvite/ magnesium ammonium phosphate (associated with proteus vulgaris UTI), Uric acid (gout, leukemias), and Cystine (rare genetic defect.)
What are simple cysts in the kidney?
Usually innocuous, in the cortex, filled with clear liquid
What are dialysis associated acquired cysts?
Present in cortex and medulla, may bleed, tumors may arise from the walls of the cysts.
What are the differences between adult polycystic kidney issues and those in children?
Adult: autosomal dominant, ischemic atrophy due to pressure from cysts leading to HTN, associated with Berry aneurisms. Childhood: autosomal recessive, associated with hepatic cysts
What is hydronephrosis?
Dilation of renal pelvis and calyces usually due to obstructed urine outflow.
What are the most common congenital causes of hydronephrosis?
Atresia of the urethra and valve formations in either the ureter or urethra
What are the most common acquired causes of hydronephrosis?
Stones, tumors of prostate, bladder, and cervix, inflammation of prostate or urethritis, and Neurogenic spinal cord damage with paralysis of the bladder.
Where is the blockage if hydronephrosis is bilateral?
Below the level of the ureters….above this level if unilateral.
What can occur to kidney with hydronephrosis after 3 weeks?
Irreversible damage can occur.
What is the most common malignant tumor of the kidneys?
Renal cell carcinoma (85%)
What are the 3 kidney tumors we discussed?
Renal cell carcinoma, Nephroblastoma (Wilm’s tumor), and primary tumors of the calyces and pelvis.
Are bladder tumors common?
2X more common than renal tumors.
What are the characteristics of renal cell carcinoma?
From tubular epithelium, more males, smokers, may produce erythropoiten and present with polycythemia (too many RBC’s in the blood).
Where are the common site for renal cell carcinoma metastasis?
Lung and bones.
What are the 3 types of renal cell carcinomas?
Clear cell, papillary, and chromophobe
What is clear cell carcinoma of the kidneys like?
It is the most common type of renal cell carcinoma (70-80%), Most are sporadic but can be familial with genetic defect on ch.3
What is Wilms tumor?
It usually occurs in kids under 10, can be sporadic or familial with susceptibility autosomal dominance inheritance….this is the most common kidney cancer in kids and one of the most common tumors in children.
What are the two major categories for bladder tumors?
Benign papillomas and carcinomas
What are most bladder carcinomas?
Mostly urothelial with 5% SCC.
At what stage does bladder carcinoma become invasive?
Usually in grades II and III where cells are less differentiated, invasive, metastatic, and aggressive.
How does bladder carcinoma present?
Painless hematuria
What are the risk factors for bladder cancer?
Males 3X more likely, exposure to beta-napthylamine (50X the risk), smoking, and chronic cystitis…..50% 5 year survival.
What is meckel diverticulum?
The most common and innocuous anomaly of the small intestine. It is the failure of involution of the omphalomesenteric duct that leaves a persistent blind-ended tubular protrusion up to 5 to 6 cm long
What is a possible consequence of malrotation of developing bowel?
The cecum can be found anywhere in the abdomen including left upper quadrant instead of the normal right lower quadrant
What is the mortality rate with bowel infarction?
90%
What are common predisposing conditions for hemorrhoids?
Straining at stool in setting of chronic constipation. Venous stasis of pregnancy in younger women. More rarely may reflect portal hypertension resulting from cirrhosis of the liver
What is dysentery?
Low-volume, painful, bloody diarrhea
What is secretory diarrhea?
Net intestinal fluid secretion that is isotonic with plasma and persists during fasting
What is osmotic diarrhea?
Excessive osmotic forces exerted by luminal solutes that abate wth fasting
How do exudative diseases manifest as diarrhea?
Output of purulent bloody stools that persist on fasting, stools are frequent but may be small or large volume
How big of a worldwide problem is infectious enterocolitis?
Second only to common cold in frequency. Most common health problem encountered by people who travel internationally
What is Rotavirus?
It is spread fecal orally w/ 130 million cases/year w/900,000 deaths ww/yr.
What is responsible for most cases of nonbacterial foodborne epidemic gastroenteritis?
Caliciviruses formerly called Norwalk.
What are three types of preformed baxterial toxins that cause diarrhea?
Staph aureus, Vibrio Species and C. perfringes.
What is a commonly ingested bacterial preformed neurotoxin?
Clostridium botulinum.
What do enterocolitis bacterial infections rely on?
Ability to adhere to mucosal cells, the ability to eloaborate enterotoxins (vibrio cholera), and the capacity to invade and lyse epithelial cells (salmonella typhi, and yersinia enterocolitica.
What two organisms can E. coli act like?
Vibrio cholera, and shigella.
Where do salmonella species cause problems?
In the mucosa of the ileum and the colon.
What is unique about typhoid fever?
It can colonize the gallbladder to induce a chronic carrier state.
What is clostridium difficile associated with?
Pseudomembranous colitis-produced.
What are some of the forms that bacterial enterocolitis take?
Explosive diahhrea, can be due to ingested neurotoxins like C botulinum usually followed by diarrhea and dehydration and dysentery usually more severe than viral diseases.
What do Giardia lamblia look like?
Smiley faced balloons.
What is the major cause of childhood diahhrea?
Cryptosporidiosis, water born accounts for 20% of all cases in developing countries.
What are the three most common malabsorption disorders in the US?
Pancreatic insufficiency, Celiac disease, and Chron disease.
What is steatorrhea?
Excess undigested fat in poop.
What is celiac disease?
A gluten sensitive enteropathy due to a reduction in small intestinal absorptive surface area. The mucosal vili flatten out and reduce the surface area principally in the proximal portion of the small intestine.
Where is gluten found?
It is a component of wheat and related grains that contain water-insoluble protein gliadin…early exposure to this protein is a prominent cofactor for the clinical manifestation of celiac disease.
Does celiac disease affect the long-tern risk of malignant disease?
It does…almost 2X increase in usual rate.
What are two disorders that exemplify malabsorption syndromes arising from intestinal infections?
Tropical Sprue and Whipple disease.
What is tropical sprue?
It resembles celiac disease but is not hereditary…you get villus flattening but as opposed to celiac disease the injury is seen at all levels of the small intestine.
What is the hallmark of whipple disease?
You will see small intestinal mucosa laden w/distended PAS-positive macrophages in the lamina propria. This is caused by tropheryma whippelii.
What are the two conditions are referred to as IBD?
Crohn’s Disease, and Ulcerative colitis
What supplies the duodenum with blood?
The celiac and superior mesenteric arteries.
What supplies the Ileum and Jejunum with blood?
Superior mesenteric artery.
What supplies the colon with blood?
Superior mesenteric artery from cecum to the spenic flexure and the inferior mesenteric artery from splenic flexure distally.
What are Crohns and ulcderative colitis referred to as collectively?
Idiopathic inflammatory bowl disease or just IBD.
What is a distinctive feature of Crohns disease?
50% exhibit non-caseating granulomas.
Which IBD is associated with non-granulomatous disease limited to the colon?
Ulcerative colitis.
What are the big differences between crohns and ulcerative colitis?
Skip lesions, strictures, serpentine fissures and is transmural in crohns and continuous colonic involvement beginning in the rectum., Broad ulcers, and pseudopolyps are common to UC.
What are the buzz words for UC?
Pancolitis, retrograde pseudopolyps, and mucosal crypt abscesses
What are the buzz words for Crohns?
Skip lesions, Creeping fat, apthous ulcers, and transmural granulomas.
What is the driving force in IBD?
T cells…..ANCAs seen in some patients.
How could you summarize IBD?
Exaggerated and destructive immune response, tissue injury occurs with inflammation
What dose the inflammation in IBD lead to?
Impaired mucosal epilthelial barrier, loss of surface epithelial absorption, and activation of crypt epithelial cell secretiondestroys mucosa to give intermittent bloody diarrhea.
What are three charactistics of Crohns?
Transmural involvement of bowell, non caseating granulomas in 40-60%, Aphthous ulcers.
What is the string sign?
A radiograph taken with barium that shows the narrowing of the bowels in crohns disease.
Where are the granulomas in the GI tract associated with crohns found?
Anywhere in the alimentary canal, but the absence of these doesn’t preclude a diagnosis of CD.
Where are lymphoid aggregates found that are associated with crohns?
Various tissue layers and in the extramural fat.
What are patients with long standing Chron’s disease at risk for?
5-6 X increase risk of carcinoma, particularly the colon.
What are some of the devastating events that can accompany Crohns?
Massive intestinal bleeding, toxic dilation of the colon, or carcinoma of the colon and small intestine.
How does the increased risk for carcinoma associated with crohns compare to that associated with Ulcerative colitis?
Crohns risk is less than that for UC.
What are psuedopolyps?
Regenerating mucosal upward bulge…seen in UC
What is a toxic megacolon?
Exposed muscularis propria and neural plexus to fecal material can cause complete shutdown of neuromuscular function to cause gangrene…toxic megacolon seen in UC.
What is a crypt abscess?
Seen in UC or CD where neutrophilic infiltration of the epithelial layer.
What is the most serious complication of UC?
Colon cancer
What two factors govern the risk of colon cancer associated with UC?
The duration of the disease and the anatomic extent.
What can a residual of healed UC look like?
Mucosal architectural disarray and atrophy with submucosal fibrosis.
Are bloody stools more common with UC or CD?
UC…larger lesion and near the rectum.
Is relapse common with UC?
Yes most patients experience relapse.
Are extraintestinal manifestation common in UC?
Yes, more than with CD.
What is the most common location for acquired diverticula?
The colon
What are the teaniae coli?
Three equidistant bands that account for the outer longitudinal muscles coats.
Where do most colonic diverticulosis occur?
95% in the sigmoid colon.
What is the treatment for colonic diverticulosis?
High fiber diet recommended but unproven for prevention of the disease.
Which type of diverticula is congenital?
A Meckel diverticulum is the prototype.
Is the small or large bowell most often affected with bowell obstruction disorders?
The small..due to smaller lumen.
What are the four entities that account for 80% of the bowell obstructions?
Hernias, Intestinal adhesions, Intussusception, and volvulus.
Where are the usual sites of weakness that may become sites for hernias?
The inguinal and femoral canals, the umbilicus and surgical scars.
How do adhesions occur?
Peritoneal inflammation due to surgical procedures, infection or endometriosis eventually heal and adhesions may develop
What is intussusceptions?
Telescoping of a proximal segment of bowel into the distal segment, in adults this often points to a mass or tumor that becomes trapped by peristalsis and causes obstruction.
What is a volvulus?
Most often affect the small bowl….a twisting of the bowel
What are the three types of Familial Adenomatous polyposis (FAP)?
Gardner, Peutz-Jegher, and Cowden
What is the host to more primary neoplasms than any other organ in the body?
The colon/rectum
What would be the leading killing cancer if smoking did not exist?
Colorectal…it currently ranks second to bronchogenic carcinoma.
What type of cancer are most (70%) of the colorectal cancers and those in the GI tract?
Adenocarcinomas.
What is a polyp?
A timorous mass that projects into the lumen of the gut, maybe pedunculated or sessile.
What is a hyperplastic polyp?
A non-neoplastic polyp formed as a result of abnormal mucosal maturation, inflammation, or architecture. These increase with age…an most have no malignant potential
What % of hyperplastic polyps are found in the rectosigmoidal region?
Over 50%.
What are adenomatouos polyps/ adenomas?
True neoplastic lesionsprecursors of carcinoma.
What might some (very few) so-called hyperplastic polyps on the right side of the colon become?
May be precursors to colorectal carcinomas via mismatch repair pathway.
What are juvenile polyps?
In kids under 5, hamartomatous proliferations that may be a source of rectal bleeding.
How many adenomatouos polyps become neoplastic?
20-30% if younger than 40 and 40-50% if over 60.
What is one of the possible clinical features of adenomas?
Anemia or may obstruct the ampulla of Vater and cause biliary obstruction.
Where do most sporadic colorectal adenocarcinomas arise from?
They arise in preexisting adenomatous lesions.
What are the three subtypes of adenomatous polyps based on architecture?
Tubular, Villous, and Tubulovillous.
Which subtype of adenomas are most common?
Tubular
What is the risk of an adenomatous polyp becoming malignant associated with?
Polyp size, Histologic architecture, and the severity of epithelial dysplasia.
What is the chief determinant of the risk of an adenoma harboring carcinoma?
The maximum diameter.
Where are most tubular adenomas found?
Over 50% in the rectosigmoid area.
How many larger tubular adenomas (up to 2.5 cm) contain invasive carcinomas?
40%
Where are villous adenomas most common?
In rectum and rectosigmoid…more common in older people…look like cauliflower.
How do villous adenomas compare to tubular adenomas?
They are larger and more ominous with more common rectal bleeding
Are all adenomas potentially malignant?
Upon discovery, yes all are considered to be potentially malignant.
What type of inheritance do Familial Polyposis syndrome have?
Autosomal dominant
What makes Gardner syndrome unique to the other FAPS’s?
common growth outside of the colon, especially osteomas (common in the mandible), gliomas, and soft tissue tumors.
For patients with FAP, what is the risk for colon cancer by midlife?
100% as well as duodenal adenomas.
Why might Peutz-Jeghers be detected by a dentist?
Because they commonly have melanotic mucosal and cutaneous pigmentation. (café-au-lait)
Are Peutz-Jeghers and Cowden syndromes associated with intestinal and extraintestinal malignancies?
Yes, but not as much as Gardners
How lethal are adenocarcinomas?
They account for 15% of all cancer related deaths in the US.
When do people get colorectal carcinoma?
Less than 20% before 50, peak incidence is 60-70 y/o if it occurs in younger people a pre-existing ulcerative colitis or polyposis syndrome should be suspected.
Who in the world gets colorectal cancer?
Mostly in the US and other developed countries pry due to diets low in fiber, high in fats and refined carbs, with decreased vitamin intake.
What does decreased fiber intake cause?
Reduced stool bulk, increased fecal retention and altered bacterial flora.
What have recent studies shown about high-fiber diets protective effects vs. colorectal cancers?
Recent studies challenge this notion.
What might patients with FAP take to prevent colorectal cancer?
Cox-2 inhibitors…may be chemoprentative.
What is the adenoma-carcinoma sequence?
The development of carcinoma from adenomatous lesions.
What are the two pathways for the development of colorectal cancer?
APC/Beta Catenin pathway due to chromosomal instability and accounts for 80% of sporadic colorectal cancers. And the genetic lesions in DNA mismatch repair genes resulting in microsatellite instability (MSI) aka MSI pathway
What must occur in order for an adenoma to develop via the APC/Beta Catenin pathway?
Both copies of the APC gene must be lost. First hitborn with one mutant APC gene, second hitloss of normal copy.
What other mutation occur in the adenoma-carcinoma sequence APC/Beta Catenin pathway?
K-RAS oncogene and TP53 tumor suppressor gene
What is the principal deletion found in 60-70% of colorectal cancers?
18q21 deletion.
Are all colorectal cancers detectable by a digital or protosigmoidal scopic exam?
No because 50 % of them are found in the transverse and ascending colons and can’t be detected this way.
What do tumors in the proximal colon look like?
They grow as polypoid exophytic masses usually w/out obstruction
What do tumors in the distal colon look like?
They tend to be annular and produce napkin-ring constrictions
What types of cancers predominate in the anal zone?
SCC
What are the clinical features of colorectal cancers?
Can be asymptomatic for years.
What might Iron deficiency in men mean?
GI cancer until proven otherwise, in women this may be caused by gynecologic issues.
How do colorectal cancers metastasize?
Through blood vessels and lymphatics to liver, lungs, bones, lymph nodes
What are 5 methods for detecting colorectal neoplasms?
Digital rectal exam, fecal testing for occult blood loss, Barium enema, Sigmoidoscopy, colonoscopy.
What is the most important prognostic indicator of colorectal cancer?
Extent (TNM stage) of tumor at the time of diagnosis.
What is the 5 year survival rate like for colorectal cancer?
T1-97%, T-4 63%, N1: 66%, N2: 37%
What % of GI tumors are found in the small intestine?
3-6&
What does GISTs stand for?
GastroIntestinal Stromal Tumors.
How are GISTs activated?
A mutation affecting KIT a tyrosine kinase receptor
How are GISTs treated?
With tyrosine kinase inhibitor STI-571- (Gleevec)
Where are most small bowel tumors?
in the duodenum this includes the ampulla of vater
What can duodenal lesions in periampullary region cause?
Obstructive jaundice early in the course of disease leading to early detection
Why are Carcinoid tumors designated endocrine?
Phenotypically they resemble endocrine cells in parenchymal organs.
What does carcinoid mean?
It is an old reference to carcinoma like
what % of small intestinal malignant tumors are carcinoid tumors?
50% of small intestinal malignancies but < 2% of colorectal malignancies
which carcinoids infrequently metastasize?
Appendiceal and rectal carcinoids
what are Carcinoid tumors often misdiagnosed as?
Irritable bowel syndrome
what is the most common site of gut carcinoid tumors?
The appendix
what is the most common tumor of appendix?
Carcinoid tumor found in 1 in 300 routine appendectomy specimens
what can the secretory products of carcinoid produce?
A variety of syndromes or endocrinopathies (Zollinger-Ellison syndrome, Cushing syndrome)
what is thought to to be the basis of carcinoid syndrome?
Most manifestations are thought to arise from elaboration of serotonin (5-HT)
what is the 5 yr survival rate for carcinoids not counting appendiceal?
90%
what is the most common extranodal location for disseminated non-Hodgkin lymphoma?
The gut
in what setting might MALT lymphomas arise?
With mucosal activation as a result of Helicobacter associated chronic gastritis
what is the risk for T-cell lymphomas in celiac disease?
> normal risk
what is the most common acute abdominal condition requiring a surgeon?
Appendicitis
how many people get appendicitis?
10% of people in the united states and other Western countries
what is the criteria for diagnosis of acute appendicitis?
Neutrophilic infiltration of muscularis propria
what marks the classic case of appendicitis?
Mild periumbilical discomfort leading to anorexia, nausea and deep constant ache or pain in lower right quadrant.
What happens when the appendix is retrocecal?
Pt can fail to reveal localizing right sided lower quadrant signs and diagnosis of appendicitis will be delayed
Why is it acceptable for conventional diagnostic techniques to only be acute for appendicitis 80% of the time?
It is better to occasionally resect a normal appendix than risk morbidity and mortality due ot appendiceal perforation
What is APUD?
Amine Precursor Uptake Decarboxylase