Systemic Path Final

Front 1

Achondroplasia

Back 1

Congenital bone disease. Results in impaired maturation of cartilage, decreasing the epiphyseal bone.

Front 2

Most common form of dwarfism

Back 2

Achondroplasia

Front 3

Which bones are spared in achondroplasia?

Back 3

Cranium and vertebrae

Front 4

Osteogenesis imperfecta

Back 4

Congenital bone disease caused by abnormal type 1 collagen.

Front 5

"Brittle bone disease"

Back 5

Osteogenesis imperfecta

Front 6

What are the dental manifestations of osteogenesis imperfecta?

Back 6

Dentinogenesis imperfecta type 1

Front 7

Which bones are most affected by osteogenesis imperfecta?

Back 7

The long bones, they become bowed. An interesting side note is that the sclera of the eyes are blue

Front 8

Osteopetrosis

Back 8

Congenital condition also called marble bone disease. Result of defective resorption of bone by osteoclasts

Front 9

This bone disease causes diffuse skeletal sclerosis with loss of medullary cavity (impaired hematopoiesis)

Back 9

Osteopetrosis

Front 10

What are the metabolic bone diseases?

Back 10

Rickets and osteomalacia

Front 11

What causes rickets and osteomalacia?

Back 11

Vitamin D deficiency

Front 12

What is the defect in rickets and osteomalacia?

Back 12

Deficient mineralization of osteoid

Front 13

How do rickets and osteomalacia differ?

Back 13

Rickets is when developing bone has defective mineralization (children), and osteomalacia affects developed bone (adults)

Front 14

Osteoporosis

Back 14

Metabolic bone disease that results in decreased total bone mass and density

Front 15

Who are most affected by osteoporosis?

Back 15

Post-menopausal women, due to decreased estrogen levels

Front 16

How would serum calcium levels in osteoporosis compare to healthy individuals?

Back 16

Serum calcium levels are normal in osteoporosis. The effect is more associated with high production of certain cytokines that promote bone resorption

Front 17

Hyperparathyroidism

Back 17

Metabolic bone disease due to increased parathormone. Leads to demineralization, microfractures, and bone cysts

Front 18

What is the mechanism of effect in hyperparathyroidism?

Back 18

Osteoclast activation leading to bone resorption. Increased calicum sparing in the kidneys plus bone resorption leads to increased serum clacium.

Front 19

Which mineral is found in lesser quantity in hyperparathyroidism?

Back 19

Serum phosphate is decreased

Front 20

Paget's disease

Back 20

Also called osteitis deformans. Intense osteoclastic activity followed by disorganized osteoblastic activity

Front 21

What histologic finding is pathognomonic of Paget's disease?

Back 21

Mosaic pattern of bone

Front 22

What serologic findings may indicate Paget's disease?

Back 22

Very high serum alkaline phosphatase and elevated calcium

Front 23

Metabolic bone disorder review

Back 23

Slide 16 of lecture 21

Front 24

How do osteomalacia and osteoporosis differ?

Back 24

Osteomalacia pts have normal bone matrix and deficient mineralization. Osteoporosis pts have deficient matrix with normal mineralization

Front 25

Causes of osteomyelitis

Back 25

Staph aureus and TB are the main ones

Front 26

What is a Brodie's abscess?

Back 26

A rim of sclerotic bone surrounding a residual abscess

Front 27

Potts disease

Back 27

TB that invades the spine, considered a form of osteomyelitis

Front 28

Osteoarthritis

Back 28

Chronic degenerative joint disorder due to age, wear and tear. Causes pain and loss of function in joints.

Front 29

Acute suppurative arthritis

Back 29

Acute synovitis with purulent joint effusion and destruction of articular cartilage. Caused by Staph aureus commonly

Front 30

Gout

Back 30

Tissue accumulation of uric acid in the joints

Front 31

What causes 90% of gout cases?

Back 31

Hyperuricemia or inborn error of purine metabolism. The other 10% are caused by chronic renal disease or leukemias

Front 32

What are the crystalline aggregates of uric acid in gout called?

Back 32

Tophi

Front 33

Are benign or malignant bone tumors more common?

Back 33

Benign are more common. Any malignant bone tumor is likely a secondary metastasy

Front 34

Where would you develop an osteoid osteoma?

Back 34

In the leg (femur/tibia). It is benign

Front 35

Most common primary malignant bone tumor

Back 35

Osteosarcoma, a mesenchymal tumor producing osteoid

Front 36

Where is an osteosarcoma likely to metastasize

Back 36

It is highly malignant and will often metastasize to the lung

Front 37

What is the biggest risk of chondromas?

Back 37

They are usually benign but can transform to chondrosarcoma in 30% of pts

Front 38

Ewing's sarcoma

Back 38

Highly aggressive bone malignancy of neural origin stemming from a gene translocation.

Front 39

2nd most common bone malignancy in children

Back 39

Ewing's sarcoma (osteosarcoma is first)

Front 40

Fibrous dysplasia

Back 40

Progressive distorting, disfiguring, fibrous replacement of bone. Considered benign none the less

Front 41

Albright's syndrome

Back 41

Polyostotic fibrous dysplasia, cafe au lait spots, and precocious puberty in females

Front 42

Myasthenia gravis

Back 42

Muscular disease caused by autoimmunity to ACH receptor. Antibodies bind to the receptors and ACH can't bind

Front 43

Presentation of myasthenia gravis

Back 43

Muscle weakness, particularly extrinsic muscles of the eyes (ptosis). 65% have thymic hyperplasia

Front 44

Muscular dystrophies

Back 44

Progressive myopathies that result in muscle degeneration and death by 20's

Front 45

Two types of muscular dystrophies

Back 45

Duchenne where dystrophin is absent, and Becker where dystrophin is present but abnormal

Front 46

What is dystrophin?

Back 46

It plays a role in the structural and functional integrity of myocytes. It attaches the actin to the sarcolemma. Deficiencies cause muscular dystrophies

Front 47

Excoriation

Back 47

Breakage of the epidermis, ie a deep scratch

Front 48

Onycholysis

Back 48

Separation of nail plate from the nail bed

Front 49

Acanthosis

Back 49

Diffuse epidermal hyperplasia

Front 50

Acantholysis

Back 50

Loss of intercellular connections between keratinocytes

Front 51

Lentiginous lesion

Back 51

One with a linear pattern of melanocyte proliferation

Front 52

Papillomatosis

Back 52

Hyperplasia of the papillary dermis with elongation of the dermal papillae

Front 53

Spongiosis

Back 53

Intercellular edema of the epidermis

Front 54

Hydropic swelling

Back 54

Intracellular edema of keratinocytes, often seen in viral infections

Front 55

Urticaria

Back 55

Hives, usually due to type 1 hypersensitivity

Front 56

What are some IgE independent causes of urticaria?

Back 56

Opiates and antibiotics (they cause mast cell degranulation)

Front 57

Histologic findings in urticaria

Back 57

Eosinophil infiltration into the dermis with edema fluid

Front 58

Acute eczematous dermatitis

Back 58

aka spongiotic dermatitis, a clinical term describing lesions that are red, papulovesicular, ozzing/crusting, with raised scaly plaques

Front 59

What are the five clinical categories of dermatitis

Back 59

Contact, atopic, drug-related, photoeczematous, and irritant

Front 60

Histologic findings of acute eczematous dermatitis

Back 60

Epidermal spongiosis, epidermal hyperplasia, hyperkeratosis, and dermal edema

Front 61

Contact dermatitis

Back 61

Th1 reaction, delayed type hypersensitivity (type 4). NOT ATOPY

Front 62

Effects of contact dermatitis with persistent antigen exposure

Back 62

Epidermis thickens (acanthosis) and lesions become scaly (hyperkeratosis)

Front 63

Erythema multiforme

Back 63

Uncommon, self-limiting disorder. Appears to be hypersensitivity response of some kind

Front 64

What is thought to provoke erythema multiforme?

Back 64

Infections (herpes, Mycoplasma), drugs (sulfonamides, penicillin), carcinomas/lymphomas, and SLE

Front 65

Characteristic lesion in erythema multiforme

Back 65

Red macule or papule with central red zone (from epidermal necrosis) and pale outer rim.

Front 66

Stevens-Johnson syndrome

Back 66

Acute erythema multiforme associated with sulfonamides, anticonvulsant agents, and NSAIDS. Causes painful mucosal lesions and can be deadly

Front 67

Psoriasis

Back 67

Well demarcated pink plaques covered by white scales. Usually affects skin of elbows, knees, and scalp

Front 68

Nail changes in psoriasis pts

Back 68

About 30% have thickened, discolored, and pitted nails. Pitted nails is very diagnostic for psoriasis

Front 69

Pathogenesis of psoriasis

Back 69

Not a lot known, thought to be T cell mediated but don't know the antigen. Genetic susceptibility factor (HLA B27)

Front 70

Auspitz sign

Back 70

Dilated blood vessels in the dermis cause multiple minute bleeding points. Found in psoriasis pts

Front 71

Lichen planus

Back 71

Pruritic, purple, polygonal papules (PPPP). Lesions often found on extremities or in oral cavity in 70% pts. Associated with areas of hyperpigmentation frequently

Front 72

Wickham striae

Back 72

White lines highliting lichen planus lesions

Front 73

Pemphigus

Back 73

Rare autoimmune blistering disorder. 4 variants, pemphigus vulgaris accoutns for 80%

Front 74

Pathology of pemphigus

Back 74

Loss of epidermal intercellular attachments. Superficial vesicles/bulla that rupture easily

Front 75

What type of blisters result from pemphigus

Back 75

Suprabasal blisters (intraepidermal) that result from acantholysis

Front 76

Pathogenesis of pemphigus

Back 76

Type 2 hypersensitivity reaction due to IgG autoantibodies to desmoglein 3 (intercellular cement substance of skin)

Front 77

Histologic findings in pemphigus pts

Back 77

Immunoflourescence of lesional skin shows a fishnet pattern due to deposition of IgG along plasma membranes of epidermal keratinocytes

Front 78

Pemphigoid

Back 78

Relatively common autoimmune disease due to type 2 hypersensitivity response to basement membrane antigens. Causes blistering of skin/mucous membranes

Front 79

Two forms of pemphigoid

Back 79

Mucous membrane (cicatricial) and bullous (skin)

Front 80

What type of blistering happens in pemphigoid?

Back 80

Subepithelial blisters

Front 81

Do pemphigus or pemphigoid blisters rupture more easily?

Back 81

Pemphigus blisters rupture more easily because they are more superficial

Front 82

Histologic findings in pemphigoid

Back 82

Linear deposition of IgG and complement along the dermo-epidermal junction seen via immunoflourescence

Front 83

Dermatitis herpetiformis

Back 83

Rare disorder of urticaria associated with celiac disease. Pt has gluten antibodies that cross react with reticulin (anchors epidermal BM to dermis)

Front 84

Which skin lesion is often mistaked for squamous cell carcinoma?

Back 84

Keratoancanthoma

Front 85

Most common type of verrucae?

Back 85

Verruca vulgaris, caused by HPV types without oncogenic potential

Front 86

Actinic keratosis

Back 86

Skin dysplasia associated with chronic sun exposure. Build-up of excess keratin that can form a cutaneous horn.

Front 87

Can actinic keratosis lesions progress to cancer?

Back 87

Some progress to carcinoma in situ

Front 88

Prevalence of squamous cell carcinoma

Back 88

Second most common tumor arising on sun exposed sites in older people

Front 89

What percentage of SCC will metastasize to regional nodes?

Back 89

5%

Front 90

Basal cell carcinoma

Back 90

Most common skin cancer, but less aggressive and rarely metastasize

Front 91

Pigmented nevus (mole)

Back 91

Refers to any congenital or acquired benign neoplasm of melanocytes.

Front 92

Histologic finding of nevi

Back 92

Nests of melanocytes at the dermal-epidermal junction (junctional), within the dermis (intradermal), or both (compound nevus)

Front 93

Dysplastic nevi

Back 93

Precursors of malignant melanoma. Familial and sporadic forms exist. Lesions are larger and more numerous than normal nevi

Front 94

Which form of dysplastic nevi are more likely to be malignant?

Back 94

Familial form, includes heritable melanoma syndrome and dysplastic nevus syndrome.

Front 95

Malignant melanoma

Back 95

Most arise in sun exposed skin and are cured surgically

Front 96

Clinical warning signs of melanoma

Back 96

Change in color, size, or shape of pre-existing pigmented mole or new lesion. Also irregular borders and varation in color are signs

Front 97

Growth patterns of malignant melanoma

Back 97

Radial (horizontal growth) initially. During this phase cells can't metastasize. Then vertical growth ensues into dermis and cells can metastasize

Front 98

Macular and nodular melanomas

Back 98

If it macular, it is likely a young lesion. If it is nodular it has likely invaded dermis and metastasized

Front 99

Oliguria/anuria

Back 99

Reduced/no urine flow

Front 100

Azotemia

Back 100

Increased BUN and creatinine due to decreased GFR. Prerenal means the problem is before the kidneys(hypoperfusion), postrenal means its after

Front 101

Uremia

Back 101

Clinical signs and symptoms of azotemia, ie gastroenteritis, peripheral neuropathy, pericarditis, and confusion

Front 102

What is the difference between syndromes and diseases?

Back 102

Syndromes are the signs and symptoms of diseases. A syndrome may be caused by one or more diseases

Front 103

Nephritic disorders

Back 103

Acute diffuse proliferative GN, rapidly progressive (crescentic) GN, IgA nephropathy, hereditary nephritis

Front 104

Nephrotic syndromes

Back 104

Lipoid nephrosis, membranous GN, focal glomerulosclerosis, membranoproliferative GN

Front 105

Which of the nephrotic and nephritic syndromes can become chronic?

Back 105

Post-infectious GN, rapidly progressive GN, membranous GN, focal glomerulosclerosis, and membranoproliferative GN

Front 106

Acute nephritic syndrome

Back 106

Inflamed glomerulus, compromised filtration. This leads to visible hematuria, oliguria, azotemia, and hypertension (little proteinuria)

Front 107

Nephrotic syndrome

Back 107

Leaky glomerulus, massive protein loss. This leads to heavy proteinuria, hypoalbuminemia, severe edema, hyperlipidemia, and lipiduria.

Front 108

Glomerular diseases

Back 108

All the diseases listed under nephrotic/nephritic syndromes are primary diseases. Secondary glomerular disease can result from SLE, hypertension, or diabetes

Front 109

Pathogenesis of glomerular diseases

Back 109

Ag-Ab complexes and complement deposit in the glomeruli or in BM. This happens in >70% of pts with GN. Some damage also caused by activation of PMN and monocytes

Front 110

Goodpasture's syndrome

Back 110

Antibodies bind directly to the glomerular BM and create a linear pattern of Ig deposition seen by immunoflourescence. The antigen is the a-3 chain of type 4 collagen. Causes rapidly progressive GN

Front 111

When would you see a granular pattern in immunoflourescence of glomerulus?

Back 111

When immune complexes deposit in the glomeruli. When antibodies bind to BM only, it creates a linear pattern

Front 112

Post-infectious GN

Back 112

Develops 1-4 weeks after skin or pharyngeal infections with Strep pyogenes. Leads to nephritic syndrome. <95% will recover

Front 113

Histologic finding of post-infectious GN

Back 113

Deposits of IgG and complement on glomerular BM. Affects nearly all glomeruli (diffuse) and appears as subepithelial "humps"

Front 114

What is the most common form of GN in SLE?

Back 114

Diffuse proliferative glomerulonephritis. Subendothelial deposits of immune complexes make a "wire loop" appearance

Front 115

IgA nephropathy (Berger disease)

Back 115

Causes acute nephritic syndrome, one of the most common causes of refurrent hematuria. Gross hematuria recurs every few months. Slow progressin to renal failure in up to 50%

Front 116

Epidemiology of IgA nephropathy

Back 116

Most common glomerular disease in Asia, commonly affects children and young adults

Front 117

Pathogenesis of IgA nephropathy

Back 117

Circulating IgA immune complexes deposit in mesangium, more common in pts with celiac disease

Front 118

Rapidly progressive (crescentic) GN

Back 118

Causes nephritic syndrome, rapid loss of renal function that may require dialysis or transplantation. If untreated, death from renal failure.

Front 119

Histology of rapidly progressive GN

Back 119

Epithelial cells and leukocytes form crescent shapes in the glomeruli. More crescents means worse prognosis

Front 120

Which of the three types of rapidly progressive GN has the best prognosis?

Back 120

Type 1 (anti-GBM antibodies). It has the best prognosis and can benefit from plasmapheresis but is the least common

Front 121

What antibodies are present in the three types of rapidly progressive GN?

Back 121

Type 1 is anti-GBM antibodies, type 2 is immune complex disease (like SLE), and type 3 is anti-neutrophil cytoplasmic antibodies (ANCAs)

Front 122

What other organ is affected in Goodpasture's syndrome?

Back 122

The lungs because the antibodies can cross-react with BM of lung alveoli and glomerular BM

Front 123

Hereditary nephritis (Alport syndrome)

Back 123

Abnormality in collagen type 4 synthesis, presents at early age. Causes hematuria, proteinuria, deafness and eye disorders. Other than this it has a benign course

Front 124

Which syndrome would lead to anasarca?

Back 124

Nephrotic syndrome

Front 125

How do causes of nephrotic syndrome vary between children and adults?

Back 125

In children it is almost always caused by a primary kidney disease (lipoid nephrosis), and in adults it is often associated with systemic disease (SLE, diabetes)

Front 126

Which primary glomerular disease is more importnat in adults?

Back 126

Membranous GN

Front 127

Which primary glomerular disease is more importnat in children?

Back 127

Lipoid nephrosis (minimal change disease)

Front 128

Most common cause of nephrotic syndrome in children

Back 128

Lipoid nephrosis (minimal change disease)

Front 129

What type of proteinuria happens in lipoid nephrosis?

Back 129

Selective proteinuria where only albumin escapes the glomerulus

Front 130

How would you treat lipoid nephrosis?

Back 130

Corticosteroid therapy

Front 131

Histology of lipoid nephrosis

Back 131

Tubular cells contain lipid

Front 132

Membranous GN

Back 132

Subepithelial deposits of immune complexes, granular pattern seen. Causes nephrotic syndrome and induces non-selective proteinuria

Front 133

Is primary or secondary membranous GN more common?

Back 133

Primary is 85% of cases, the secondary causes include chronic HBV, cancer, SLE, and toxic gold/mercury salts

Front 134

Focal segmental glomerulosclerosis

Back 134

Deposits of Ig and complement, causes hyalinization and sclerosis. Often associated with nephrotic syndrome

Front 135

Membranoproliferative GN

Back 135

Type 1 is sub-endothelial deposits of immune complexes and type 2 is intra-GBM deposits. Causes combined nephrotic/nephritic syndromes

Front 136

Which infectious organism is involved in membranoproliferative GN?

Back 136

HBV implicated in type 1, type 2 idiopathic

Front 137

Histology of membranoproliferative GN

Back 137

Thickening and splitting of glomerular BM in both type 1 and 2

Front 138

What systemic condition is the leading cause of kidney failure?

Back 138

Diabetes

Front 139

Kimmelstiel-Wilson lesion

Back 139

Nodular glomerulosclerosis lesion that is very specific to diabetes

Front 140

What type of renal syndrome will diabetics have?

Back 140

Nephrotic syndrome

Front 141

Which primary glomerular disease most often progresses to chronic GN?

Back 141

Rapidly progressive (crescentic) GN progresses in 90% of pts

Front 142

Chronic GN

Back 142

Insidious onset, leading to proteinuria, hypertension, azotemia, microscopic hematuria, and edema.

Front 143

Histology of chronic GN

Back 143

Hyalinazation of the glomeruli, interstitial fibrosis, tubular atrophy

Front 144

Acute pyelonephritis

Back 144

Kidney infection that ascends from bladder/ureters or stems from bacteremia. Most commonly caused by E. coli

Front 145

Risk factors for acute pyelonephritis

Back 145

Vesico-uretral valve reflux, diabetes, pregnancy, and catheterization/instrumentation

Front 146

Symptoms of acute pyelonephritis

Back 146

Pain, chills, fever, dysuria, pyuria, bacturia, proteinuria. Abscesses on renal surface and renal pelvis filled with pus.

Front 147

Histology of acute pyelonephritis

Back 147

Suppurative necrosis and white cell casts in urine

Front 148

Two forms of chronic pyelonephritis

Back 148

Chronic obstructive pyelonephritis, chronic reflux-associated pyelonephritis (most common)

Front 149

Acute drug-induced interstitial nephritis

Back 149

Caused by synthetic penicillins and is mediated by IgE (allergy). Leads to acute renal failure commonly

Front 150

Analgesic nephropathy

Back 150

Caused by aspirin and acetaminophen in large quantities. Condition may improve with cessation of drug use

Front 151

Analgesic nephropathy leads to higher risk of what cancers?

Back 151

Renal and bladder

Front 152

Most common cause of acute renal failure

Back 152

Acute tubular necrosis. Can be due to ischemic damage from blood loss, or nephrotoxic poisons, heavy metals, drugs, etc

Front 153

Histology of acute tubular necrosis

Back 153

Proteinaceous casts in distal tubules, most commonly Tamm-Horsfall protein, but can be myoglobin as well

Front 154

3 stages of acute tubular necrosis

Back 154

Initiating (36 hours) maintenance (days 2-6) during which time pts may die without tx, and recovery

Front 155

Benign nephrosclerosis

Back 155

Associated with benign hypertension, causes hyaline arteriolosclerosis. Pts rarely die from renal failure, more likely to die from heart disease

Front 156

Malignant nephrosclerosis

Back 156

Associated with malignant hypertension (diastolic >120). Leads to arteriosclerosis of kidneys and has rapid course.

Front 157

Survial rate of malignant nephrosclerosis

Back 157

50% 5 year survival rate, 90% of deaths related to renal failure as opposed to heart disease like in benign NS

Front 158

Histology of malignant nephrosclerosis

Back 158

Pinpoint petechial hemorrhages that are described as "flea-bitten"

Front 159

Hemolytic-uremic syndrome

Back 159

Formation of intravascular thrombi due to endothelial damage from shiga-like toxin produced by E. coli. Leads to DIC

Front 160

Thrombotic thrombocytopenic purpura

Back 160

Acquired or genetic defect in proteolytic cleavage of vWF. This promotes platelet aggregation and micro-thrombus formation

Front 161

Renal stones (urolithiasis)

Back 161

Familial tendency, form unilaterally mostly. Made of calcium oxalate and calcium phosphate.

Front 162

Clinical symptoms or manifestations of renal stones

Back 162

Main one is hematuria, but may be asymptomatic

Front 163

Conditions associated with renal stones

Back 163

Hypercalcemia or hypercalcuria (75%), struvite (magnesium ammonium phosphate) associated with Proteus vulgaris, uric acid, and cystine

Front 164

Polycystic kidney disease

Back 164

Genetic defect causing multiple bilateral fluid-filled cysts. Pressure from these cysts leads to ischemic atrophy. In children, hepatic cysts develop as well and can lead to cirrhosis

Front 165

Which kidney disease is associated with Berry aneurisms?

Back 165

Polycystic kidney disease

Front 166

Hydronephrosis

Back 166

Dilation of renal pelvis and calyces due to obstruction to urine outflow. Can be congenital or acquired

Front 167

Most common kidney tumor

Back 167

Renal cell carcinoma (80-85%)

Front 168

Are bladder or kidney tumors more common?

Back 168

Bladder tumors are twice as common

Front 169

Renal cell carcinoma

Back 169

Biggest risk factor is smoking. Hematuria, polycythemia, and pain are symptoms. Metastases in lung and bones

Front 170

Most common type of renal cell carcinoma

Back 170

Clear cell carcinoma accounts for 70-80%.

Front 171

Wilms tumor

Back 171

Typically occurs in children <10 yrs old, susceptibility can be inherited, but sporadic cases occur as well.

Front 172

Bladder tumors

Back 172

Most are carcinomas, mainly urothelial. Grade 1 are rarely invasive, grades 2 and 3 are invasive and metastatic

Front 173

Risk factors and symptoms of bladder cancer

Back 173

Smoking and exposure to beta-napthaline (50X risk) are risk factors, and main symptom is painless hematuria

Front 174

What is the cystic duct?

Back 174

It connects the neck of the gallbladder to the biliary tree. It then joins the common hepatic duct to form the common bile duct that empties into the duodenum

Front 175

Histology of gall bladder

Back 175

Columnar epithelium containing mucin, arranged into finger-like projections

Front 176

Biliary tract route

Back 176

Bile canaliculi --> canals of Hering --> intrahepatic bile ducts --> R and L hepatic ducts --> common hepatic duct

Front 177

Histology of biliary tract

Back 177

Low cuboidal epithelium with underlying actin and myosin that propel bile through lumen

Front 178

These two conditions account for 95% of biliary tract disease

Back 178

Cholelithiasis and cholecystitis

Front 179

2 types of gallstones

Back 179

Cholesterol stones (80% of cases) and pigment stones (bilirubin)

Front 180

Formation of bile stones

Back 180

If cholesterol concentration exceeds capacity of bile to solubilize, solid cholesterol crystals form

Front 181

Pathogenesis of bile stones

Back 181

Supersaturation of bile with cholesterol, nucleation sites from calcium precip, hypomobility of gall bladder, mucous hypersecretion that traps crystals

Front 182

Pathogenesis of pigment stones

Back 182

Promoted by presence of unconjugated bilirubin in biliary tree

Front 183

Risk factors for cholelithiasis

Back 183

Increased age, female gender, Native American, family history, increased biliary cholesterol secretion, reduction in gallbladder motility

Front 184

Gross morphology of cholesterol gall stones

Back 184

Found in gallbladder only, are yellow to gray-white. Most are radiolucent

Front 185

Gross morphology of pigment gall stones

Back 185

Found anywhere in biliary tree, are black (sterile) or brown (infected). Most are radiopaque

Front 186

Complications of cholelithiasis

Back 186

Empyema, fistula formation , perforation, or inflammation of bile ducts. Small stones can enter the cystic or common ducts and obstruct them

Front 187

Cholecystitis

Back 187

Inflammation of the gallbladder, usually associated with gallstones

Front 188

2 types of cholecystitis

Back 188

Acute (calculous/non-calculous) and chronic. Calculous means assoc. with gall stones

Front 189

Most common reason for emergency cholecystectomy

Back 189

Acute calculous cholecystitis that leads to obstruction of gallbladder neck or cystic duct. (most common complication of gallstones)

Front 190

Pathogenesis of acute calculous cholecystitis

Back 190

Obstruction of bile outflow, production of toxic lysolechithins that disrupt the protective glycoprotein mucous layer. Epithelium of gallbladder exposed to bile salts leading to prostaglandin release and inflammation

Front 191

Is cholecystitis more commonly caused by gallstones or non-gallstone related causes?

Back 191

Calculous (gallstone) cholecystitis is more common

Front 192

What factors can contribute to non-calculous cholecystitis?

Back 192

Post-op state, severe trauma, severe burns, sepsis, dehydration, bacterial infection

Front 193

Why is non-calculous cholecystitis more difficult to diagnose?

Back 193

Because the symptoms are usually masked by the primary medical problems causing it

Front 194

Main symptom of chronic cholecystitis?

Back 194

Recurrent R upper quadrant pain that can radiate to the shoulder. Also, intolerance to fatty foods (vomiting)

Front 195

Major complications of cholecystitis?

Back 195

Bacterial superinfection leading to cholangitis,

Front 196

Most common malignancy of the biliary tract

Back 196

Carcinoma of the galbladder (usually adenocarcinomas). Presumably due to chronic inflammation or recurrent trauma

Front 197

When is carcinoma of the gallbladder normally diagnosed?

Back 197

During surgery for cholecystectomy the tumor is usually discovered. If pts develop early symptoms of blockage and get surgery, they may get it removed before the tumor metastasizes

Front 198

At time of dx of gallbladder carcinoma, what metastases have developed?

Back 198

Usually liver, cystic duct, adjacent bile ducts, portohepatic lymph nodes

Front 199

Choledocholithiasis

Back 199

Presence of stones in biliary tree, may derive from gallbladder or may develop in ducts

Front 200

Does choledocholithiasis usually require surgery to remove structures?

Back 200

No, stones can be extracted via endoscopy or shock wave lithotripsy

Front 201

Cholangitis

Back 201

Acute inflammation of bile ducts. Can ascend into the intrahepatic bile ducts

Front 202

Most common cause of cholangitis

Back 202

Choledocholithiasis

Front 203

What organsims are commonly associated with cholangitis?

Back 203

E. coli, Klebsiella, Clostridium (mostly bacterial, usually not parasites)

Front 204

Secondary biliary cirrhosis

Back 204

Prolonged obstruction of extrahepatic biliary tree leading to secondary inflammation and fibrosis of liver

Front 205

Is secondary biliary cirrhosis reversible?

Back 205

If obstruction is corrected early the damage is reversible. If not, the damage is irreversible = SBC

Front 206

Biliary atresia

Back 206

Destruction or absence of extrahepatic bile ducts leading to complete obstruction of bile flow

Front 207

What serious condition can biliary atresia lead to?

Back 207

Secondary biliary cirrhosis

Front 208

Leading cause of death from liver disease in early childhood

Back 208

Biliary atresia. Can be corrected with surgery, but without the pt will die by age 2

Front 209

Cholangiocarcinoma

Back 209

Carcinoma of bile duct epithelial cells (can be intra or extrahepatic)

Front 210

What symptom is universally common to most esophageal diseases?

Back 210

Dysphagia (difficulty swallowing), usually due to either deranged esophageal motor function or narrowing/obstruction of the lumen

Front 211

What would indicate severe inflammation, ulceration, or laceration of the esophagus?

Back 211

Hematemesis (vomiting blood) or melena (blood in the stools)

Front 212

Hiatal hernia

Back 212

Dilated segment of stomach protrudes above the diaphragm, separating the diaphragmatic crura

Front 213

Axial (sliding) hiatal hernia

Back 213

More common, 95% of cases, bell-shaped dilation bounded below by the diaphragmatic narrowing. Those with severe reflux esophagitis likely to have sliding hiatal hernia

Front 214

What condition do most pts with severe reflux esophagitis likely have?

Back 214

Sliding hiatal hernia

Front 215

Para-esophageal (rolling) hiatal hernia

Back 215

Separate portion of stomach, usually along the greater curvature. Enters thorax through the widened foramen. Only 5% of hiatal hernia

Front 216

What would indicate severe inflammation, ulceration, or laceration of the esophagus?

Back 216

Hematemesis (vomiting blood) or melena (blood in the stools)

Front 217

Achalasia

Back 217

Esophageal dysmotility and failure of the lower esophageal sphincter to fully relax. Results in functional obstruction and dilation of the proximal esophagus.

Front 218

Three major abnormalities in achalasia

Back 218

Aperistalsis, incomplete relaxation of lower esophageal sphincter, increased resting tone of sphincter

Front 219

Example of disease causing secondary achalasia

Back 219

Chagas disease, leads to destruction of myenteric plexus of esophagus and inflammation of esophageal mesenteric plexus (pathognomonic sign)

Front 220

Symptoms of achalasia

Back 220

Progressive dysphagia, inability to convey food to stomach, nocturnal regurgitation, and aspiration of undigested food. Usually manifests in young adults

Front 221

What is the most serious complication that can arise from achalasia?

Back 221

Development of squamous cell carcinoma

Front 222

Cause of esophageal varices

Back 222

Portal hypertension secondary to liver damage causes portal blood to spill into venous circulation through shunts. This increases the BP in esophageal vessels and causes them to dilate

Front 223

What condition are esophageal varices most commonly associated with?

Back 223

Alcoholic cirrhosis

Front 224

What is the major cause of death in pts with advanced cirrhosis of the liver?

Back 224

Rupture of esophageal varix that leads to massive hemmorhage

Front 225

What tumor is found in 50% of pts with rupture of esophageal varices?

Back 225

Hepatocellular carcinoma

Front 226

Dominant symptom of GERD

Back 226

Recurrent heartburn

Front 227

Factors contributing to esophagitis

Back 227

Sliding hiatal hernia, increased gastric volume, impaired reparative capacity of mucosa damaged by gastric juices, decreased antireflux mechanisms

Front 228

Histologic features of esophagitis

Back 228

Main one is presence of eosinophils +/- neutrophils in the epithelial layer. Others are basal zone hyperplasia and elongation of papillae

Front 229

Presence of intraepithelial neutrophils in esophagitis pt indicates what?

Back 229

It is a marker of a more severe injury, not just irritation from gastric juices

Front 230

Barretts esophagus

Back 230

A complication of long standing GERD. Normal stratified squamous mucosa replaced by columnar epithelium containing goblet cells

Front 231

What is the chief concern with Barretts esophagus?

Back 231

Development of adenocarcinoma is 30-40x more likely in these pts

Front 232

What is the most common esophageal cancer worldwide?

Back 232

90% are squamous cell carcinomas, the rest are mostly adenocarcinomas.

Front 233

What race has higher prevelance of esophageal SCC?

Back 233

Blacks

Front 234

What is the most common esophageal cancer in the US?

Back 234

Adenocarcinoma associated with Barretts esophagus. Much more common in whites

Front 235

What virus is strongly associated with esophageal squamous cell carcinoma?

Back 235

HPV

Front 236

In what part of the esophagus do most SCCs arise?

Back 236

50% arise in the middle third, 30% in lower third, and 20% in cervical and upper thoracic esophagus

Front 237

In what part of the esophagus do most adenocarcinomas arise?

Back 237

The lower third

Front 238

Most common gastrointestinal infection

Back 238

Helicobacter pylori

Front 239

Vomited blood that has the appearance of coffee grounds likely came from what?

Back 239

Bleeding from the stomach due to breach of gastric mucosa

Front 240

Gastritis

Back 240

Inflammation of gastric mucosa, usually chronic but can be acute

Front 241

Histologic findings in chronic gastritis

Back 241

Mucosal atrophy and epithelial metaplasia (usually changes to columnar with goblet cells)

Front 242

Most important etiologic association with chronic gastritis

Back 242

Helicobacter pylori

Front 243

Where does H. pylori colonize on the mucosa of the stomach?

Back 243

It is in the mucus layer overlying the superficial mucosal epithelium

Front 244

How could H. pylori contribute to gastric lymphoma?

Back 244

It induces proliferation of lymphoid tissue within gastric mucosa

Front 245

What two conditions can develop due to chronic gastritis?

Back 245

Peptic ulcer and gastric carcinoma

Front 246

Important cause of acute GI bleeding

Back 246

Severe erosive form of acute gastritis

Front 247

Associations with acute gastritis

Back 247

Heavy use of NSAIDs, especially aspirin. Others include excessive alcohol and smoking and uremia

Front 248

What type of acute gastritis does NSAID overuse lead to?

Back 248

Extremely localized lesion, not diffuse.

Front 249

Common histologic findings in acute gastritis

Back 249

Mucosal edema, inflammatory infiltrate of neutrophils, possibly chronic inflammatory cells as well.

Front 250

What is the difference between gastric ulcers and erosions?

Back 250

Ulcers extend through the muscularis mucosa into submucosa. Erosions are a breach in epithelium only and heal quickly

Front 251

Where do most peptic ulcers occur?

Back 251

98% occur in the stomach and first portion of the duodenum

Front 252

What two conditions can lead to duodenal ulcers?

Back 252

Chronic renal failure and hyperparathyroidism because they cause hypercalcemia which stimulates gastrin production and acid secretion

Front 253

What has to happen in order for a peptic ulcer to form?

Back 253

Mucosal exposure to gastric acid and pepsin. Also, H. pylori is commonly involved

Front 254

Mechansims that lead to ulcers from H. pylori infection

Back 254

Increased production of pro-inflammatory cytokines, especially IL-8 (PMN recruitment/activation). Also, bacterial gene products can cause epithelial damage

Front 255

What percentage of people infected with H. pylori will actually develop a peptic ulcer?

Back 255

Only about 10-20%, probably due to some strains being more virulent

Front 256

Gastroduodenal effects of NSAIDs

Back 256

Acute erosive gastritis, acute gastric ulceration, and peptic ulceration

Front 257

How do NSAIDs contribute to peptic ulcers?

Back 257

They supress prostaglandin synthesis by mucosa. This leads to increased acid and decreased bicarbonate

Front 258

How does smoking contribute to peptic ulcers?

Back 258

It impairs mucosal blood flow and healing

Front 259

Etiologic factors associated with peptic ulcers

Back 259

Corticosteroid use and alcohol consumption leading to alcoholic cirrhosis. Also, chronic gastritis

Front 260

Zollinger-Ellis syndrome

Back 260

A pancreatic tumor produces excess gastrin which then leads to excess gastric acid production and multiple ulcerations

Front 261

Will pts with NSAID-associated ulcers have gastritis?

Back 261

No, unless there is coexistent infection with H. pylori

Front 262

What would lead to stress ulcers?

Back 262

Severe trauma, sepsis, surgical procedures, burns, chronic exposure to gastric irritant drugs

Front 263

What is a 'Curling ulcer"?

Back 263

A stress ulcer due to extensive burns

Front 264

What is a "Cushing ulcer"?

Back 264

Ulcer due to traumatic or surgical injury to the CNS or intracerebral hemorrhage

Front 265

How do acute stress ulcers and chronic peptic ulcers differ?

Back 265

Stress ulcers are found anywhere in the stomach, not localized to a particular section. Also, multiple stress ulcers generally form

Front 266

What is the most important care for a pt with stress ulcers?

Back 266

You have to treat the primary disease or trauma that is causing them (ie sepsis, burns, trauma)

Front 267

What part of the stomach is most likely to develop gastric carcinoma?

Back 267

The lower third (antrum)

Front 268

Gastric polyps

Back 268

Nodule or mass that projects above the level of surrounding mucosa. This term refers to mass lesions arising in mucosa

Front 269

What do most gastric polyps end up being?

Back 269

Just hyperplastic polyps, only 5% are adenomas

Front 270

What type of cancer is most common in the stomach?

Back 270

Carcinomas represent 90-95%

Front 271

What are the two morphologic types of gastric cancer?

Back 271

Intestinal and diffuse

Front 272

Intestinal gastric cancer

Back 272

Arises from gastric mucosa that have undergone intestinal metaplasia in setting of chronic gastritis.

Front 273

Diffuse gastric cancers

Back 273

Arise de novo from gastric mucosa, not associated with chronic gastritis. This form hasn't decreased in frequency like intestinal GCs have

Front 274

Meckel diverticulum

Back 274

It is a developmental anomalie of the small intestine. The ophalomesenteric duct fails to involute properly and leaves a tubular protrusion

Front 275

What is a volvulus?

Back 275

It is a bowel blockage due to a loop of bowel that has abnormally twisted on itself. Usually due to malrotation of developing bowel

Front 276

What condition does a transmural infarction of the gut imply?

Back 276

Acute occlusion of a major mesenteric artery. Smaller arteries may only lead to a mural infarct

Front 277

Major complication of bowel infarction

Back 277

There is a small windown of time between onset of symptoms and perforation caused by gangrene. 90% mortality rate if perf

Front 278

Angiodysplasia (tortuous dilations of mucosal blood vessels in gut) is associated with what other disorder?

Back 278

CREST (limited scleroderma)

Front 279

Conditions predisposing to hemorrhoids

Back 279

Straining at stool with chronic constipation, pregnancy, or portal hypertension secondary to cirrhosis of the liver

Front 280

Second most frequent affliction?

Back 280

Infectious enterocolitis, second only to the common cold in frequency

Front 281

What is the most common health problem encountered by people who travel internationally?

Back 281

Infectious enterocolitis

Front 282

Most common pathogenic causes of infectious enterocolitis

Back 282

Rotavirus, calciviruses, and E. coli

Front 283

What microbe is responsible for most cases of non-bacterial foodborne diarrhea?

Back 283

Caliciviruses (previously called Norwalk viruses)

Front 284

Which bacteria produce preformed toxins that cause diarrhea if ingested?

Back 284

S. aureus, Vibrio, Clostridium perfringens and botulinum

Front 285

What areas of the intestines are affected by Salmonella infections?

Back 285

The ileum and colon

Front 286

At what point in typhoid fever does a person usually become a chrnoic carrier?

Back 286

Usually after the thrid week once the gallbladder becomes colonized

Front 287

Which diarrhea-causing bacteria can also cause pseudomembraneous colitis?

Back 287

Clostridium difficile

Front 288

Major cause of childhood diarrhea

Back 288

Cryptosporidiosis caused by ingestion of contaminated water

Front 289

What is steatorrhea and what causes it?

Back 289

It is excess output of undigested fat in stool and is caused by defective intraluminal digestion

Front 290

What is the basic disorder in celiac disease?

Back 290

Sensitivity to gluten due to a CD4+ T cell immune response. This response causes damage to the villi and reduce the absortpive surface area of the small intestine

Front 291

What is the protein in gluten that is responsible for the immune response in celiac disease?

Back 291

Gliadin

Front 292

How do people develop celiac disease?

Back 292

They have a genetic susceptibility, usually conferred by the HLA-DQ2 or DQ8 haplotype

Front 293

What structures in the small intestine are affected by celiac disease?

Back 293

The mucosal villi. Usually the proximal intestine is more affected than distal intestine

Front 294

Are people with celiac disease at significantly greater risk of developing GI malignancies?

Back 294

Not really, only two-fold increase in risk. If anything, it would be an intestinal T cell lymphoma or GI carcinoma

Front 295

Two examples of malabsorption disorders arising from intestinal infection

Back 295

Tropical sprue and Whipple disease

Front 296

Tropical sprue

Back 296

A malabsorption ayndrome with symptoms like celiac disease, but thought to be caused by some type of infection. Usually leads to loss of villi similar to celiac disease

Front 297

How do celiac disease and tropical sprue differ?

Back 297

Celiac is caused by immune rxn and is mostly in the proximal intestine. Tropical sprue is from an infection and affects all parts of small intestine

Front 298

Whipple disease

Back 298

A malabsorption disorder caused by systemic infection of Tropheryma whippelii. Hallmark finding is PAS+ macrophages in the lamina propria of the small intestinal mucosa

Front 299

What are the two idiopathic inflammatory bowel diseases (IIBD)?

Back 299

Crohn disease and ulcerative colitis

Front 300

What causes Crohn and ulcerative colitis?

Back 300

Cause is unknown, for this they are called the idiopathic inflammatory bowel diseases

Front 301

Characteristics of Crohn disease

Back 301

50% of cases exhibit non-caseating granulomatous inflammation that can happen anywhere from the esophagus to the anus

Front 302

Characteristics of ulcerative colitis

Back 302

Non-granulomatous disease limited to the colon (unlike Crohn that can be anywhere in the GI tract)

Front 303

Is ulcerative colitis or Crohn disease more amenable to surgery?

Back 303

Surgery for UC can be curative, but its a bad idea for Crohn

Front 304

Even though ulcerative colitis and Crohn disease are idiopathic, what is thought to underly the disease?

Back 304

Genetic predisposion, there have been associations discovered with certain MHC type II alleles and it is thought to cause dysregulation of mucosal immunity

Front 305

What immune factor is the driving force in IBD?

Back 305

Activated T cells found in the gut mucosa

Front 306

Common characteristic sign of IBD?

Back 306

Intermittent bloody diarrhea caused by mucosal destruction

Front 307

Radiographic findings in Crohn disease

Back 307

In the small intestine there will be a thin stream of barium passing through the diseased segment and this is called the "string sign"

Front 308

Early lesions in Crohn diseases resemble what other type of lesion?

Back 308

Apthous ulcers

Front 309

Does absence of granulomas preclude the diagnosis of Crohn disease?

Back 309

No, only about 40-60% of Crohn pts will have granulomas

Front 310

What is the relative risk for people with longstanding Crohn disease of developing GI malignancy?

Back 310

5-6 fold increase in risk of carcinoma, particularly of the colon

Front 311

Does ulcerative colitis or Crohn carry a greater risk of developing malignancies?

Back 311

Ulcerative colitis

Front 312

Debilitating consequences of Crohn disease

Back 312

Fistula formation between gut and bladder, vagina, perianal skin, etc. Intestinal stricture or obstruction, and peritonitis

Front 313

What structure is affected in ulcerative colitis?

Back 313

The lesions begin in the rectum and extend proximally to the colon

Front 314

Which IIBD doesn't exhibit granulomas or skip lesions and has lesions that don't extend below the submucosa?

Back 314

Ulcerative colitis

Front 315

What are pseudopolyps?

Back 315

They are isolated islands of regenerating mucosa that bulge upward from the colon. They are found in ulcerative colitis pts

Front 316

How does toxic megacolon come about?

Back 316

In ulcerative colitis pts the muscularis propria and neural plexus can get exposed to feces and neuromuscular function can shut down. Colon then swells and becomes gangrenous

Front 317

What are crypt abscesses?

Back 317

Neutrophil infiltration of the epithelial layer of the gut produces collections of PMNs in crypt lumina

Front 318

What characterisitcs remain in the colon following healed ulcerative colitis lesions?

Back 318

Submucosal fibrosis, mucosal architectural disarray and atrophy

Front 319

Is ulcerative colitis or Crohn disease more likely to exhibit bloody stools?

Back 319

Ulcerative colitis

Front 320

What disease course do most people with IIBD experience?

Back 320

A relapsing course

Front 321

Both ulcerative colitis and Crohn disease can have extraintestinal manifestations. Which is more common?

Back 321

They are more common in ulcerative colitis than Crohn disease, especially migratory polyarthritis

Front 322

What is a Meckel diverticulum?

Back 322

A true congenital diverticulum, not acquired like most others

Front 323

Most common location for acquired diverticulum

Back 323

The colon (diverticulosis)

Front 324

What is unique about the colons musculature?

Back 324

Its outer longitudinal muscle is split into three equidistant bands called taeniae coli (so the muscle doesn't totally wrap around the colon)

Front 325

What are the two factors that lead to development of diverticulum?

Back 325

Exaggerated peristaltic contractions with abnormal intraluminal pressure and focal defects in the muscular colonic wall

Front 326

What part of the colon are acquired diverticula most likely to develop?

Back 326

95% are in the sigmoid colon

Front 327

Treatment of diverticulosis

Back 327

High fiber diet to help reduce the exaggerated peristalsis of the colon

Front 328

Most dangerous complications of diverticulosis

Back 328

Perforation with pericolic abscess or fistula formation

Front 329

Most common part of gut to become obstructed

Back 329

The small intestine

Front 330

What four entities account for 80% of bowel obstructions?

Back 330

Hernias, intussusception, volvulus, and intestinal adhesions

Front 331

What is an intestinal adhesion?

Back 331

Following surgery, the healing operative site may adhere to other segments of gut or the abdominal wall. THis creates closed loops through which intestines can slide and get trapped (internal hernia)

Front 332

Intussusception of the gut

Back 332

Telescoping of a segment of bowel into an adjacent section. Can be caused by a tumor that gets trapped during peristaltic wave

Front 333

Volvulus

Back 333

Twisting of a loop of bowel about its base of attachemnt. This affects small intestine mostly and can lead to constriction of blood flow and obstruction of lumen

Front 334

Which organ is host to more primary neoplasms than any other organ?

Back 334

The colon

Front 335

Colorectal cancer stats

Back 335

Second only to bronchogenic carcinoma among cancer killers. 40% death rate

Front 336

What type of cancer predominates in the GI tract (especially the colon)?

Back 336

Adenocarcinomas make up about 70% of cancers in the GI tract

Front 337

What is a polyp?

Back 337

A hyperplastic, non-neoplastic tumorous mass that protrudes into the gut lumen

Front 338

What is the difference between an adenoma and a polyp?

Back 338

An adenoma has neoplastic potential and arises due to epithelial dysplasia

Front 339

Where do the majority of intestinal polyps form?

Back 339

90% are in the large intestine, mostly in the rectosigmoid region. They are found mostly in persons older than 60 y/o

Front 340

Can hyperplastic polyps become neoplastic?

Back 340

No, not true hyperplastic polyps. Sometimes they are misdiagnosed on the right side of the colon and can give rise to cancer by mismatch repair pathway

Front 341

Juvenile polyps

Back 341

Esentially hamartomatous proliferations with no malignant potential. They can be a cource of rectal bleeding though

Front 342

How do most adenocarcinomas arise?

Back 342

From preexisting adenomatous lesions (often happens in familial patterns)

Front 343

What are the two subtypes of adenomas in the colon?

Back 343

Tubular adenomas, villous adenomas, and tubulovillous adenomas

Front 344

What are the most common type of GI adenomas?

Back 344

Tubular adenomas make up about 90%.

Front 345

What determines malignant risk of GI adenomas?

Back 345

The size, histologic architecture, and severity of epithelial dysplasia. (diameter is the chief determinant of risk though)

Front 346

Difference between tubular and villous adenomas

Back 346

Tubular are usually pedunculated with a slender stalk. Villous are mostly sessile. Both occur more commonly in older persons and in the rectosigmoid area

Front 347

Which type of GI adenoma is more symptomatic and more ominous?

Back 347

Villous adenomas usually cause rectal bleeding and 40% will be neoplastic.

Front 348

Where is the genetic defect in familial adenomatous polyposis (FAP)?

Back 348

The APC gene on chromosome 5q2.1

Front 349

Gardner syndrome

Back 349

Similar to FAP but also tends to have extra-intestinal tumors such as osteomas, gliomas, and soft tissue tumors

Front 350

How many adenomatous polyps must a pt have in order to be diagnosed with FAP?

Back 350

Minimum of 100

Front 351

What is the incidence of colon cancer in FAP pts?

Back 351

Nearly 100%

Front 352

Peutz-Jeghers

Back 352

Uncommon autosomal dominant syndrome causing hamartomatous polyps and melanotic mucosal and cutaneous pigmentation (cafe-au-lait)

Front 353

Cowden syndrome

Back 353

Similar to Peutz-Jeghers, hamartomatous polyps of GI tract. Both also carry increased risk of malignancies in and outside the GI tract

Front 354

When a young person is found to have colorectal carcinoma, what must be suspected?

Back 354

Preexisting ulcerative colitis or one of the FAP syndromes. Otherwise it mainly happens in those 60-70 y/o

Front 355

What factor is generally considered to explain the geographic distribution of colorectal cancer?

Back 355

Dietary factors, such as low fiber, high refined carbs, high fat, and decreased vitamin intake (this has not been proven though)

Front 356

What drug class has been approved by the FDA for chemoprevention in FAP pts?

Back 356

COX-2 inhibitors that stop prostaglandin synthesis (which stops production of VEGF) and thus angiogenesis

Front 357

What are the two pathways for development of colorectal carcinoma?

Back 357

The APC pathway and the DNA mismatch repair pathway (or MSI pathway)

Front 358

What is the molecular signature of a defective DNA mismatch repair gene?

Back 358

Microsatellite instability (MSI)

Front 359

Do more colorectal carcinomas result from the APC or the MSI pathway?

Back 359

80% from APC pathway, even though you need several mutations in order for the carcinoma to develop (you only need one in MSI)

Front 360

Where are most adenocarcinomas of the colon found?

Back 360

They're pretty well spread out, not just in the sigmoidorectal area. Many go undetected by endoscopic procedures

Front 361

How do colorectal tumors of the distal and proximal colon differ in morphology?

Back 361

Usually those in the proximal are polypoid and don't cause obstruction. The distal tumors are annular, encircling lesions that cause "napkin ring constrictions"

Front 362

What type of cancers tend to predominate in the anal zone?

Back 362

Mostly SCC instead of adenocarcinomas like in the rest of the colon

Front 363

What does iron deficiency anemia in an older man almost always mean?

Back 363

That he has a GI cancer, until proven otherwise

Front 364

What procuct do colorectal carcinomas produce that makes them more invasive?

Back 364

Mucin, which is secreted into the interstitium oftn and ends up dissecting through the gut wall and facilitating extension of the tumor = worse prognosis

Front 365

How do colorectal tumors spread?

Back 365

Through lymphatics and blood vessels to the regional lymph nodes, liver and lungs most commonly

Front 366

Detection methods for colorectal cancer

Back 366

Rectal exam (finger up butt), fecal testing for blood, barium enema, colonoscopy/sigmoidoscopy

Front 367

What is the most important prognostic indicator for colorectal carcinoma?

Back 367

The TNM stage at time of diagnosis

Front 368

What type of tumors would you find in the small intestine?

Back 368

Mostly benign stromal tumors from smooth muscle, adenoma, lipoma, hamartoma, etc

Front 369

What is Gleevec used for?

Back 369

It was originally developed for chronic myeloid leukemia, but has been used recently for GI stromal tumors.

Front 370

Method of action of Gleevec

Back 370

It is a tyrosine kinase inhibitor that prevents mutant KIT from causing cancer

Front 371

Where do most small intestine carcinomas develop?

Back 371

In the duodenum (including the ampulla of Vater)

Front 372

What is a carcinoid tumor?

Back 372

It resembles a carcinoma but originates from endocrine cells of GI tract. Most of this type of tumor are found in the GI, but some in pancreas, liver, lungs, etc.

Front 373

What type of malignant tumors are most common in the small intestine?

Back 373

Carcinoid tumors arising from endocrine cells of the GI tract (insulinoma, gastrinoma, somatostatinoma, etc)

Front 374

Most common site of GI carcinoid tumors

Back 374

In the appendix, which is lucky because carcinoid tumors of the appendix and rectum almost never metastasize

Front 375

Most common tumor of the appendix

Back 375

Carcinoid tumors

Front 376

Endocrinopathies resulting from carcinoid tumors

Back 376

Zolliner-Ellison syndrome due to excess gastrin production, and Cushing syndrome due to ACTH or insulin over production

Front 377

Carcinoid syndrome

Back 377

Thought that most manifestations due to elaboration of serotonin and other secretory products of a carcinoid tumor

Front 378

What is the prognosis for carcinoid tumors?

Back 378

It good, ~90% 5-year survival rate

Front 379

What is the most common location of extranodal lymphomas?

Back 379

The GI tract

Front 380

Where do most GI lymphomas originate?

Back 380

From B cells of MALT, usually in stomach or small intestine

Front 381

What pathologic agent is thought to contribute to GI lymohomas?

Back 381

Helicobacter pylori due to its intense activation of T and B cells in the gastric mucosa. Can lead to monoclonal B-cell neoplasm

Front 382

What GI disorder predisposes to T-cell lymphomas?

Back 382

Celiac disease

Front 383

Prevalence of appendicitis

Back 383

10% of people in Western countries will develop it at some time

Front 384

Most common cause of acute appendicitis

Back 384

Obstruction of appendix, usually by a fecalith, gallstone, tumor, or ball of worms

Front 385

Criteria for dx of acute appendicitis

Back 385

Neutrophilic infiltration of muscularis propria

Front 386

In what circumstance is appendicitis generally asymptomatic?

Back 386

Right-sided lower quadrant pain absent when appendix is retrocecal or colon malrotated

Front 387

Is it better to take no action, or resect a normal appendix to be on the safe side ?

Back 387

Yes, you should err on the side of caution due to the significant mortality and morbidity that is associated with perforated appendix

Front 388

What serologic marker is used to diagnose infectious mononucleosis?

Back 388

Heterophil anti-sheep RBC antibodies. The test is called the monospot test

Front 389

What cytologic finding is characteristic of infectious mono?

Back 389

Virus-specific cytotoxic T cells that appear as atypical lymphocytes in circulation

Front 390

What marker of infectious mono infection persists for life?

Back 390

IgG antibodies to the viral capsid

Front 391

If you suspect infectious mono but the pt doesn't have fever (only malaise, fatigue, and lymphadenopathy), what should your differential dx include?

Back 391

Leukemia and lymphoma

Front 392

What is the most common organ to be affected in infectious mono?

Back 392

The liver, can present with elevated enzymes and jaundice or even liver failure (rarely)

Front 393

Follicular hyperplasia

Back 393

A form of chronic nonspecific lymphadenitis associated with infections that activate B cells (early stage of HIV is big one)

Front 394

Paracortical lymphoid hyperplasia

Back 394

A form of chronic nonspecific lymphadenitis characterized by changes within the T cell regions of lymph nodes. Usually found in viral infections

Front 395

Sinus histiocytosis

Back 395

A form of chronic nonspecific lymphadenitis characterized by distention and prominence of lymphatic sinusoids. Usually found in lymph nodes draining cancers

Front 396

Where would you find sinus histiocytosis?

Back 396

In lymph nodes that are draining cancers. May represent an immune response to the tumor or its products

Front 397

Where would lymphadenopathy secondary to cat scratch fever be localized?

Back 397

Most frequently in the axilla and neck

Front 398

Types of lymphoid neoplasms

Back 398

Non-Hodgkin lymphoma, Hodgkin lymphoma, lymphocytic leukemia, plasma cell dyscrasias

Front 399

How are lymphoid neoplasms classified?

Back 399

Based on the stage of lymphocyte differentiation that the cells are in

Front 400

What are myeloid neoplasms?

Back 400

They arise from stem cells that normally give rise to the formed elements of the blood, such as granulocytes, RBCs and platelets

Front 401

Where are plasma cell tumors usually found?

Back 401

They usually present as masses within bone

Front 402

What causes the symptoms of plasma cell dyscrasias?

Back 402

The inappropriate production of a complete or partial monoclonal Ig polypeptide

Front 403

Why are lymphomas and leukemias hard to differentiate?

Back 403

Because lymphomas can spill over into the peripheral blood and leukemias can infiltrate lymph nodes so its hard to tell where it started sometimes

Front 404

What histologic finding distinguishes a Hodgkin from a non-Hodgkin lymphoma?

Back 404

The presence of Reed-Sternberg giant cells

Front 405

Reed-Sternberg giant cells

Back 405

Giant cells found in Hodgkin's lymphoma that are usually dervied from B cells.

Front 406

What is the cell type involved in most lymphoid neoplasms?

Back 406

80-85% are of B cell origin and the rest are mostly T-cell tumors (a few NK cell neoplasms here and there)

Front 407

Cell lineage of all lymphoid neoplasms

Back 407

Dervied from a single transformed cell, therefore they are monoclonal

Front 408

Treatment of non-Hodgkin's lymphomas

Back 408

At time of dx tumor is usually widely disseminated, so only systemic therapies can be curative

Front 409

Treatment of Hodgkin's lymphoma

Back 409

Often presents at a single site so it can be cured with local therapy (excision and local radiation)

Front 410

The REAL classification of lymphoid neoplasms divides them into what 3 groups?

Back 410

Tumors of B cells, T cells and NK cells, and Hodgkin's lymphoma

Front 411

What lymphoid neoplasms predominate in the USA?

Back 411

Precursor B/T cell lymphoblastic leukemia/lymphoma, small lymphocytic lymphoma/chronic lymphocytic leukemia, Burkitt lymphoma, multiple myeloma, and Hodgkin lymphoma

Front 412

What causes the symptoms of acute leukemias?

Back 412

Symptoms arise due to lack of normal RBCs, WBCs, and platelets (all the neoplastic blast cells in the marrow suppress normal stem cells)

Front 413

Therapeutic aim of leukemia tx

Back 413

Reduce the population of leukemic clone enough to allow reconstitution with the progeny of remaining normal stem cells in bone marrow

Front 414

Manifestations of acute leukemia

Back 414

Abrupt onset, fatigue, fever, bleeding, bone pain, generalized lymphadenopathy,

Front 415

Serologic identifiers of acute leukemia

Back 415

Blast cell forms circulating in the blood comprise 60-100% of all the cells

Front 416

How can you differentiate between aplastic anemia and acute leukemia?

Back 416

You have to look at the bone marrow. If its flooded with blast cells then you can rule out aplastic anemia

Front 417

What do both pre-B and pre-T lymphoblastic tumors have in common?

Back 417

They both have clinical appearance of acute lymphoblastic leukemia (ALL) at some point

Front 418

Most common type of leukemia in childhood

Back 418

Acute lymphoblastic leukemia

Front 419

Prognosis of lymphoblastic tumors

Back 419

They have a good prognosis, especially pre-B cell tumors. Most can actually be cured

Front 420

Difference between chronic lymphocytic leukemia and small lymphocytic lymphoma

Back 420

They are basically the same thing, just that CLL means you have large numbers of circulating neoplastic cells

Front 421

Most important type of lymphoma in adults

Back 421

Diffuse large B-cell lymphoma represents about 50% of all adult NHLs

Front 422

What lymphoid tumor has a characteristic "starry sky pattern"?

Back 422

Burkitt lymphoma. This feature is the result of lighter staining normal macrophages interspresed with the neoplastic cells

Front 423

Characteristics of plasma cell dyscrasias

Back 423

B cell neoplasms that result in a single clone of Ig secreting cells producing a single homogenous Ig or fragments

Front 424

2 major plasma cell dyscrasias

Back 424

Multiple myeloma (most common) and localized plasmacytoma

Front 425

Multiple myeloma

Back 425

Clonal proliferation of neoplastic plasma cells in the bone marrow. Usually has associated skeletal lesions

Front 426

Bence Jones proteins

Back 426

Proteins found in the urine of pts with light chain disease (multiple myeloma)

Front 427

Where are the most common areas for development of bone lesions in multiple myeloma pts?

Back 427

The vertebral column and the skull

Front 428

What causes the bone lesions in multiple myeloma?

Back 428

The secretion of certain cytokines by myeloma cells (ie IL1, TNF, and IL6). The cytokines enhance osteoclast activation

Front 429

Characteristic appearance of bone lesions in multiple myeloma

Back 429

Punched-out lesions usually 1-4 cm in diameter

Front 430

What is myeloma nephrosis?

Back 430

Renal involvement from multiple myeloma. Stems from the presence of Bence Jones proteins in the urine that form casts with properties of amyloid

Front 431

Clinical manifestations of multiple myeloma

Back 431

Bone pain, renal insufficiency, and amyloidosis in some

Front 432

Diagnostic tests for multiple myeloma

Back 432

Radiographs to identify punched out lesions of the vertebrae or calvarium, and serum/urine analysis to detect spike of monoclonal Ig

Front 433

Waldenstrom macroblobulinemia

Back 433

This is when you get large macroglobulins secreted in multiple myeloma pts and this creates hyperviscous blood

Front 434

Which cells are most commonly neoplastic in Hodgkin lymphoma?

Back 434

B lymphocytes

Front 435

What do all Hodgkin lymphomas have in common?

Back 435

Reed-Sternburg cells

Front 436

Three general categories of myeloid neoplasia

Back 436

Acute myeloblastic leukemias, chronic myeloproliferative disorders, and myelodysplastic syndromes

Front 437

Acute myeloblastic leukemias

Back 437

Tumors marked by blockage in differentiation of early myeloid cells. Immature myeloid cells accumulate in the marrow and replace normal cells

Front 438

Chronic myeloproliferative disorders

Back 438

Neoplastic cells can differentiate in this type, so you get increased number of a particular formed element (RBCs, or platelets, etc)

Front 439

Myelodysplastic syndromes

Back 439

Neoplastic disorders in which myeloid differentiation occurs but is disordered and ineffective. So you have displastic precursors and differentiated cells

Front 440

Auer rods

Back 440

Distinctive red-staining rodlike structures present in cytoplasm of neoplastic myeloblasts. They are a diagnostic clue for AML

Front 441

Best way to diagnose and differentiate myeloid neoplasms

Back 441

By the karyotype mostly, but also the antigens that they produce (ie CD 13)

Front 442

FAB classification

Back 442

This is the French-American-British classification of AML that divides it into 8 groups (it isn't very prognostic)

Front 443

What translocations seen in a karyotype would be indicative of AML?

Back 443

t(8;21) is the big one. This is usually associated with Auer rods

Front 444

What is the only tx that can lead to cure of AML?

Back 444

Bone marrow transplant is the only way, although chemo is helpful too

Front 445

What is unique about the neoplastic cells in chronic myeloproliferative disorders?

Back 445

There is an increase in neoplastic bone marrow progenitors, but they still have the capacity for terminal differentiation. They also commonly seed the spleen, lymph nodes, and liver

Front 446

Most common chronic myeloproliferative disorder

Back 446

Chronic myelogenous leukemia (CML). This is associated with Philadelphia (Ph) chromosome

Front 447

What is Philadelphia (Ph) chromosome?

Back 447

It is a translocation between the long arms of chromosomes 9 and 22 and is associated with chronic myelogenous leukemia (CML)

Front 448

What is the result of the Ph chromosome translocation?

Back 448

It gives rise to the BCR-ABL fusion gene that leads to neoplastic transformation (CML)

Front 449

Difference between acute and chronic myelogenous leukemias

Back 449

In acute there is a block of stem cell maturation so the cells are stuck in the precursor form. This doesn't happen in chronic, so the cells can differentiate

Front 450

What is one of the first symptoms of myelogenous leukemias?

Back 450

Dragging sensation in the abdomen caused by splenomegaly

Front 451

Oral manifestations following bone marrow transplant for CML

Back 451

Massive graft vs host rxn leads to ulcerations/blisters in the oral cavity

Front 452

New drug used to treat CML that can be quite effective

Back 452

STI-571. It is an ATP analogue that blocks a specific tyrosine kinase BCR-ABL fusion protein that causes CML

Front 453

Polycethemia vera

Back 453

This is the massive increase in myeloid-dervided blood cells, mostly RBCs.

Front 454

Main signs and symtoms of polycythemia vera

Back 454

Pts are plethoric (red-faced) and have intense pruritis (itching) likely due to histamine released from increased number of basophils

Front 455

How do you diagnose polycethemia vera?

Back 455

By doing blood work. All blood cell counts will be increased, especially RBCs

Front 456

Histiocytosis

Back 456

A variety of proliferative disorders involving dendritic cells and macrophages

Front 457

Langerhans cell histiocytosis

Back 457

Clonal proliferation of Langerhans cells, dendritic antigen-presenting cells found largely in the skin.

Front 458

Letterer-Siwe, Hand-Schuller-Christian, and eosinophilic granuloma are all expression of what disorder?

Back 458

Langerhans cell histiocytosis

Front 459

Letterer-Siwe disease (acute disseminated Langerhans cell histiocytosis)

Back 459

This affects kids under 2 y/o usually. They develop cutaneous lesions, hepatosplenomegaly, and destructive bone lesions

Front 460

Multifocal Langerhans cell histiocytosis (H-S-C)

Back 460

Usually affects children. Most common presentations are abnormally loose teeth and large lesions of the scalp and ear canals. Also, it affect the pituitary and causes diabetes insipidus

Front 461

What neoplastic disorder causes diabetes insipidus?

Back 461

Langerhans cell histiocytoses

Front 462

What is the Hand-Schuler-Christian triad?

Back 462

This is a form of Langerhans cell histiocytosis and the triad is calvarial bone defects, diabetes insipidus, and exopthalmos

Front 463

Unifocal and multifocal eosinophilic granuloma

Back 463

A type of Langerhans cell histiocytosis that occurs in medullary cavities of bone and has an eosinophilic component. Usually causes skeletal lesions

Front 464

What syndromes are associated with increased incidence of childhood leukemias?

Back 464

Down syndrome and neurofibromatosis type 1

Front 465

What viruses are implicated as causative agents of lymphohematopoietic neoplasms?

Back 465

TLV-1, EBV, and HHV-8

Front 466

Symptoms of anemia

Back 466

Most are non-specific and include weakness, malaise, fatigue, headache, faintness, and vision problems

Front 467

What type of anemia results from iron deficiency?

Back 467

Hypochromic microcytic anemia

Front 468

What is the most common nutritional deficiency?

Back 468

Iron deficiency

Front 469

Most common cause of iron deficiency

Back 469

Blood loss from female genital tract or GI tract

Front 470

Plummer-Vinson syndrome

Back 470

Hypochromic microcytic anemia, atrophic glossitis, and esophagel webs (dysphagia)

Front 471

What is koilonychia?

Back 471

It is a finger nail deformity caused by iron deficiency anemia

Front 472

Pernicious anemia

Back 472

Autoimmune disorder that causes loss of parietal cells and intrinsic factor needed to absorb Vit B12. No Vit B12 leads to defective megaloblastic RBCs

Front 473

Intraoral signs of pernicious anemia

Back 473

Atrophic glossitis

Front 474

How can Vit B12 deficiency be treated?

Back 474

By parenteral administration of B12 or by folate supplementation. The resulting anemia should improve as a result

Front 475

How do chronic diseases lead to anemia?

Back 475

They decrease the amount of storage iron over time and this leads to insufficient erythropoietin

Front 476

Aplastic anemia

Back 476

Hypocellular bone marrow due to failure of stem cells from irradiation, viral infections, neoplasms, etc

Front 477

Hereditary spherocytosis

Back 477

A form of chronic hemolytic anemia due to mutation in ankyrin gene that normally stabilizes RBC membrane. Cell loses shape and becomes phagocytized by splenic macrophages

Front 478

What are Heinz bodies?

Back 478

They are hemoglobin precipitate in RBCs usually caused by G6PD deficiency. This leads to anemia when RBCs are lysed in the spleen

Front 479

Most common hemoglobin disorder?

Back 479

Sickle cell disease. It is caused by a point mutation of the B globin gene.

Front 480

What is thalassemia?

Back 480

Reduced or no synthesis of globin chains (can be either a or b chains)

Front 481

Result of thalassemia

Back 481

Severe hypochromic microcytic anemia with erythroid hyperplasia and skeletal deformities

Front 482

2 types of autoimmune hemolytic anemias

Back 482

Warm and cold types

Front 483

Warm antibody type immune hemolytic anemia

Back 483

IgG, 37 deg C. Primary is idiopathic, seconday can be due to leukemia, lymphoma, or SLE. Give a positive direct Coombs test

Front 484

Direct Coombs test

Back 484

Detects presence of antibodies against RBC surface antigens that are bound to RBCs. Used to diagnose immune hemolytic anemia

Front 485

Indirect Coombs test

Back 485

Detects anti RBC antibodies unbound in the serum

Front 486

Cold type immune hemolytic anemia

Back 486

IgM, 30 deg C. Can be caused by Mycoplasma pneumoniae and infectious mono

Front 487

What is the most common acquired hemolytic anemia worldwide?

Back 487

Malaria. In this case, parasites destroy the RBCs.

Front 488

How are the parathyroids unique in their function?

Back 488

They are controlled by levels of circulating calcium as opposed to a trophic hormone

Front 489

What is the effect of PTH in the body?

Back 489

It activates osteoclasts, increases renal tubule reabsorption of Ca, and increases GI absorption

Front 490

Causes of hypoparathyroidism

Back 490

Accidental surgical resection, DiGeorge syndrome, and endocrine candidiasis syndrome

Front 491

What would presence of persistent oral candidiasis in a young person signal?

Back 491

Endocrine candidiasis syndrome. Results in hypoparathyroidism.

Front 492

Effects of hypoparathyroidism during odontogenesis?

Back 492

Pitting enamel hypoplasia and failure of tooth eruption

Front 493

Pseudohypoparathyroidism

Back 493

Pts have normal levels of PTH but there is a defective G protein receptor for PTH. Pt appears to have hypoparathyroidism

Front 494

Most common type of pseudohypoparathyroidism

Back 494

Type 1a, in which there is a defective Gsa protein that prevents cAMP formation. They usually have problems with other endocrine glands as well

Front 495

Clinical features of pseudohypoparathyroidism

Back 495

Mild mental retardation, obesity, short stature, and subcutaneous calcifications. Also other endocrine abnormalities (hypogonadism, hypothyroidism, etc)

Front 496

Type 2 pseudohypoparathyroidism

Back 496

Normal production of PTH and G protein, but cell still doesn't respond. Pts are relatively normal except for hypocalcemia

Front 497

Dental manifestations of pseudohypoparathyroidism

Back 497

Enamel hypoplasia, widened pulp chambers, delayed eruption, and pulpal clacifications

Front 498

Dx of pseudohypoparathyroidism

Back 498

Serologic studies that reveal elevated PTH with concurrent hypocalcemia and normal renal function

Front 499

Causes of hyperparathyroidism

Back 499

Usually adenoma/carcinoma of the parathyroids, but can also be hyperplasia of the parathyroids less commonly

Front 500

Most common cause of hypercalcemia

Back 500

Primary hyperparathyroidism

Front 501

What usually causes symptomatic hypercalcemia?

Back 501

Malignancy causes symptomatic hypercalcemia and hyperparathyroidism causes silent hypercalcemia

Front 502

Most common manifestations of hyperparathyroidism

Back 502

Renal calculi, nephrocalcinosis, and peptic ulceration

Front 503

Radiographic features of hyperparathyroidism

Back 503

Brown tumors in the bone that appear as unilocular defects similar to giant cell tumors. Also loss of trabecular bone that give a ground glass appearance

Front 504

Brown tumors

Back 504

These are bone lesions that result from excess osteoclast activity in hyperparathyroidism. Also called central giant cell granuloma

Front 505

Most common cause of secondary hyperparathyroidism

Back 505

End stage renal disease. Causes bone lesions similar to primary disease

Front 506

Triad of symptoms in hyperparathyroidism

Back 506

Stones, bones, and abdominal groans (ulcers)

Front 507

Dental manifestations of hyperparathyroidism

Back 507

Loss of the lamina dura noted from x-rays as well as loss of trabecular bone (replaced by ground-glass appearance)

Front 508

What condition can result from long standing brown tumors from hyperparathyroidism?

Back 508

Osteitis fibrosa cystica

Front 509

GI manifestations of hyperparathyroidism

Back 509

Tendency to develop duodenal ulcers

Front 510

Tx of primary hyperparathyroidism

Back 510

Surgical removal of involved gland

Front 511

Tx of secondary hyperparathyroidism

Back 511

Treat underlying cause, then use Vit D supplement, eliminate exposure to aluminum salts that inhibit bone mineralization

Front 512

What type of salts should be avoided in pts with secondary hyperparathyroidism?

Back 512

Aluminum salts (commonly found in antacids) because they inhibit bone mineralization.

Front 513

Diabetes insipidus

Back 513

This is when there is insufficient ADH secreted by the posterior pituitary or the kidneys don't respond to ADH like they should. Results in lots of dilute urine. Happens in histiocytoses

Front 514

Rathkes pouch tumor

Back 514

Tumor derived from pituitary gland embryonic tissue that arises from nests of odontogenice epithelium. Contains calcium deposits and resembles ameloblastoma histologically

Front 515

What is a common symptom of Rathke puch tumor

Back 515

Bi-temporal hemianopia as the tumor compresses the optic chiasm

Front 516

Sheehan syndrome

Back 516

Results from pituitary hyposecretion and is caused by blood loss during and after child birth. The lack of blood results in pituitary necrosis

Front 517

Pituitary dwarfism

Back 517

Result of underproduction of HGH or reduced capacity to respond to it. Affected patients are short but proportioned right

Front 518

Primary causes of lack of HGH

Back 518

Pituitary aplasia or tumors, radiation, infection. Also can be the hypothalamus effected and GHRH isn't being produced

Front 519

Dental manifestations in pituitary dwarfism

Back 519

Maxilla and mandible smaller than normal, delayed tooth eruption, delayed development of 3rd molars, and reduced tooth size.

Front 520

Treating pituitary dwarfism with HGH normally works, with what exception?

Back 520

If the pt is lacking receptors for HGH. Also, if growth plates have already fused then its too late

Front 521

Most common cause of pituitary hypersecretion

Back 521

Tumor, usually either adenoma or carcinoma

Front 522

Most common types of pituitary adenomas

Back 522

20-30% are prolactinomas, 20% null cell, 15% adrenocorticotrophic and 15% gonadotrophic

Front 523

Pituitary gigantism

Back 523

Increased production of HGH before closure of growth plates so they get very tall. Usually due to a pituitary adenoma

Front 524

What is the height differrence that would make you suspect endocrine problems in a person?

Back 524

Greater than 3 standard deviations taller/shorter than mean height

Front 525

Diagnosing pituitary hypersecretion

Back 525

Radiographic evidence of enlarged sella, likely from an adenoma. Also, levels of HGH in the serum with be elevated

Front 526

Acromegaly

Back 526

Excess production of HGH after closure of the growth plates. Usually the excess is due to functional pituitary adenoma

Front 527

Direct effects of excess HGH production

Back 527

Hypertension, heart disease, arthritis, and peripheral neuropathy

Front 528

Dental manifestations of acromegaly

Back 528

Mandibular prognathism, diastema formation, and macroglossia

Front 529

Treatment of pituitary tumors

Back 529

Most are removed by transhenoidal surgery. If that fails then radiation as a last resort

Front 530

Most common cause of hypothyroidism in the USA?

Back 530

Chronic lymphocytic thyroiditis (Hashimoto)

Front 531

Chronic lymphocytic thyroiditis (Hashimoto)

Back 531

Autoimmune inflammatory disease primarily cause by T cell defect. Cytotoxic T cells destroy thyroid parenchyma and anti-TSH receptor Igs block TSH activity

Front 532

How do Graves and Hashimoto differ?

Back 532

In Graves, the anti-TSH antibodies are agonistic and in Hashimoto they are antagonistic. So ones causes hypo and the other hyper thyroidism

Front 533

What is a Hurthle (oxyphil) cell?

Back 533

Epithelial cells lining the thyroid follicles that are distinguished by the presence of eosinophilic, granular cytoplasm. Found in Hasimoto thyroiditis

Front 534

Predilections of Hashimoto thyroiditis?

Back 534

More common in females 10:1 to 20:1!!

Front 535

Cancer risks in Hashimoto thyroiditis

Back 535

Increased risk for B-cell non-Hodgkin lymphomas within thyroid

Front 536

Common causes of hypothyroidism in adults

Back 536

Hashimoto (autoimmune), iatrogenic factors, radioactive iodine therapy, and surgery for tx of hyperthyroid

Front 537

Manifestations of hypothyroidism

Back 537

Lethargy, dry, coarse, cool skin, swelling of face and extremities, slowed HR and reduced body temp

Front 538

Oral manifestations of hypothyroidism

Back 538

Lips thickened and tongue enlarged as a result of the GAG deposits. Failure of teeth to erupt also

Front 539

Diagnosing hypothyroidism

Back 539

Serum levels of T4 checked. If low, TSH levels are measured. In this way it can be determined if the thyroid or pituitary is disfunctioning

Front 540

Other names for hypothyroidism

Back 540

Cretinism and myxedema

Front 541

Grave's disease

Back 541

Most common cause of hyperthyroidism. Due to agonist autoantibodies to TSH receptors that stimulate TSH release

Front 542

Manifestations of hyperthyroidism

Back 542

Nervousness, heart palpitations, heat intolerance, and weight loss. Much more common in women

Front 543

Serologic findings in hyperthyroidism

Back 543

Elevated leves of T4 and depressed levels of TSH

Front 544

Risk of radioactive iodine therapy and surgery for hyperthyroidism

Back 544

Hypothyroidism can develop...then you have the opposite problem

Front 545

Thyroid storm

Back 545

In hyperthyroidism, certain situations can stimulate large release of thyroid hormone at one time. This can cause delirium , elevated temp, and tachycardia

Front 546

Dental considerations for hyperthyroid pts

Back 546

If they get stressed during or before dental tx, thyroid storm can develop and this is life threatening. Thyroid should be controlled before visit

Front 547

Main concern in pts with thyroid nodules

Back 547

Development of neoplastic diseases such as adenomas or aggressive carcinomas

Front 548

Most relaible criteria for distinguishing carcinomas from adenomas in the thyroid

Back 548

Presence of capsular and/or blood vessel invasion

Front 549

In radioactive iodine imaging, do malignancies take up more or less iodine?

Back 549

Malignancies take up less so they appear "cold". Cold nodules can be malignant, but hot nodules almost never malignant

Front 550

Medullary carcinomas of the thyroid are derived from which cells?

Back 550

Parafollicular cells (C-cells)

Front 551

Most common cause of RET gene mutation in papillary thyroid carcinomas

Back 551

Ionizing radiation

Front 552

Most common form of thyroid cancer

Back 552

Papillary carcinoma resulting from previous exposure to ionizing radiation

Front 553

Psammoma bodies

Back 553

Concentrically calcified structures often present within the papillae in papillary carcinoma of the thyroid

Front 554

Medullary carcinomas of the thyroid can lead to increased secretion of what hormone?

Back 554

Calcitonin (because its produced by C-cells in the medulla)

Front 555

What neoplastic syndrome accounts for many of the familial case of medullary thyroid carcinomas?

Back 555

Multiple endocrine neoplasia syndromes 2A or 2B

Front 556

Distinctive morphologic features of thyroid medullary carcinoma

Back 556

Amyloid deposits and presence of calcitonin within cytoplasm of tumor cells

Front 557

Effects of calcitonin

Back 557

Directly antagonistic to PTH. It lowers serum Ca by reducing GI absorption, decreasing osteoclast activity, and inhibiting renal reabsorption of Ca

Front 558

Does the overproduction of calcitonin in thyroid medullary tumors produce hypocalcemia?

Back 558

Negatory

Front 559

Where in the adrenals is mineralocicoids (aldosterone) produced?

Back 559

In the superficial cortical layer (zona glomerulosa)

Front 560

Where are glucocorticoids like cortisol produced?

Back 560

In the middle cortical layer of the adrenals (the zona fasciculata)

Front 561

Cushings syndrome

Back 561

aka hypercortisolism. Usually due to administration of exogenous corticosteroids, but can be due to endogenous sources from pituitary or adrenal dysfunction

Front 562

Cushing's disease

Back 562

Increase in glucocorticoid (cortisol) levels caused by adrenal or pituitary tumor

Front 563

Clinical features of Cushing's syndrome

Back 563

Weight gain is the big one. Usually accompanied by buffalo hump and moon faces due to fatty tissue deposits

Front 564

Diagnosis of Cushing's syndrome

Back 564

Administer exogenous corticosteroid and see how it effects serum cortisol and ACTH levels. In Cushing's both will decrease unless its due to a tumor

Front 565

Addisonian crisis

Back 565

Acute hypoadrenocorticism. It is the result of corticosteroid use that suppresses normal ACTH production in response to stress

Front 566

Dental considerations for Cushing's syndrome

Back 566

Due to stress of dental visits, it is often necessary to increase corticosteroid dose due to greater need of the body for cortisol

Front 567

Addison's disease

Back 567

Primary hypoadrenocorticism. Characterized by inadequate production of cortisol and other adrenal steroids.

Front 568

Causes of Addison's disease

Back 568

Used to be tuberculosis, but now its idiopathic adrenocortical atrophy (probably of autoimmune origin)

Front 569

Most common characteristic finding in Addison's disease

Back 569

Skin hyperpigmentation along with brown to black macular pigmented lesions affecting the oral mucosa

Front 570

Diagnosing Addison's disease

Back 570

Serology to check plasma ACTH. In primary disease ACTH levels will be high (problem is adrenals). In secondary they will be low due to pituitary gland problem

Front 571

Most importnat disease of the adrenal medulla

Back 571

Neoplasms such as neuroblastomas or pheochromocytomas

Front 572

Pheochromocytoma

Back 572

Neoplasms composed of chromaffin cells in the adrenal medulla that causes over-production of catecholamines

Front 573

Rule of 10's for pheochromocytomas

Back 573

10% associated with MEN 2A/2B, type 1 NF, and Sturge-Weber. 10% extra-adrenal, 10% bilateral, 10% malignant

Front 574

Serious and potentially lethal effects of even benign pheochromocytomas

Back 574

Resulting hypertension from the excess of catecholamines

Front 575

T/F Both capsular and vascular invasion can happen in benign pheochromocytomas

Back 575

True, what classifies these as malignant is the presence of metastases

Front 576

Biggest concern with pheochromocytoma

Back 576

Overproduction of catecholamines leads to hypertension that can cause MI, heart failure, renal injury, or even sudden cardiac death

Front 577

Diagnosis of pheochromocytoma

Back 577

Urinary analysis to identify presence of catecholamines or their metabolites (vanillylmandelic acid VMA)

Front 578

Multiple endocrine neoplasia (MEN) syndromes

Back 578

A group of rare conditions characterized by tumors or hyperplasias of neuroendocrine tissues

Front 579

MEN 2B

Back 579

Causes adrenal pheochromocytomas, medullary thyroid carcinomas, and mucosal neuromas

Front 580

Cause of MEN 2B

Back 580

Autosomal dominant trait caused by mutation of the RET protooncogene on chromosome 10

Front 581

First signs of MEN 2B

Back 581

Oral mucosal neuromas that present as soft, painless, papules or nodules on buccal mucosa, gingiva, and palate

Front 582

Most characteristic oral manifestation of MEN 2B

Back 582

Bilateral neuromas of commisural mucosa

Front 583

Most common significant tumor in MEN 2B

Back 583

Medullary thyroid carcinoma occurs in >90% of cases

Front 584

Tx for MEN 2B

Back 584

Prophylactic removal of thyroid due to risk of thyroid medullary carcinoma and monitoring for pheochromocytoma

Front 585

What type of necrosis do you get in the CNS following ischemic episodes?

Back 585

Liquefactive necrosis. Other tissues will exhibit coagulative necrosis with ischemic injury

Front 586

Major supporting cells of the brain

Back 586

Astrocytes

Front 587

Cells that wrap around axons in the CNS to form myelin sheith

Back 587

Oligodendrocytes, analogous to Schwann cells of the peripheral nervous system

Front 588

The phagocytic/antigen presenting cells of the CNS

Back 588

Microglia, when activated they behave as active macrophages

Front 589

Gitter cells

Back 589

Activated macrophages in the CNS that appear in setting of tissue necrosis and demyelinating disease. They accumulate abundant lipid to form foamy cytoplasm appearance.

Front 590

Two types of brain parenchyma edema

Back 590

Vasogenic and cytotoxic edema

Front 591

Vasogenic cerebral edema

Back 591

Occurs when blood-brain barrier is disrupted and fluid escapes from vasculature into interstitial space of brain

Front 592

Cytotoxic cerebral edema

Back 592

Increase in intracellular fluid secondary to hypoxic-ischemic cellular injury

Front 593

Hydrocephalus

Back 593

Accumulation of excessive CSF within ventricular system of brain. Usually caused by decreased reabsorption of CSF, but can be a result of overproduction

Front 594

Effects of hydrocephalus

Back 594

Before sutures are closed, enlargement of head. After, the ventricles expand and intracranial pressure increases

Front 595

Third most common cause of death in USA

Back 595

CVA (stroke). Only exceeded by heart disease and cancer

Front 596

Most common type of cerebrovascular disease

Back 596

Infarcts caused by local vascular obstruction account for 80% (and most of these are due to emboli not atherosclerotic plaques). The rest are hemorrhages and generalized ischemic injury

Front 597

At what blood pressure point does the brain lose its potential to regulate blood flow?

Back 597

Below 50 mmHg the brain can't compensate and ischemic injury may result

Front 598

Which cells in the CNS are the most vulnerable to ischemic injury?

Back 598

Neurons, far more than glial cells

Front 599

Conditions leading to global hypoxic-ischemic encephalopathy

Back 599

Cardiac dysrhythmias, shock, increase in intracranial pressure (anything that results in generalized decrease in oxygenated blood to brain)

Front 600

Where do the most severe atherosclerotic lesions in the brain form?

Back 600

Internal carotid arteries, proximal middle cerebral arteries, and basilar arteries

Front 601

Most common cause of CVA

Back 601

Mostly caused by emboli from heart. Also, occlusion of vessels by atherosclerotic plaques

Front 602

Which vessels in the brain most commonly become occluded by emboli?

Back 602

Middle cerebral arteries

Front 603

How long does it take necrotic tissue in the CNS to completely liquefy?

Back 603

6 months

Front 604

What is the most important predictor of CNS infarcts?

Back 604

The presence of transient ischemic attacks (TIAs). These are episodes of neurologic dysfunction preceding stroke

Front 605

Most common cause of primary brain parenchymal hemorrhage

Back 605

Hypertension and coagulation disorders

Front 606

Signs of brain hemorrhage

Back 606

Cheyne-Stokes respiration, dilated and nonresponsive pupils, and coma

Front 607

Most common cause of subarachnoid hemorrhage

Back 607

Rupture of saccular (berry) aneurysm

Front 608

4 major types of vascular malformations

Back 608

Arteriovenous malformations, capillary telangiectasias, venous angiomas, and cavernous angiomas

Front 609

Most common congenital vascular abnormality in the brain

Back 609

Arteriovenous malformations

Front 610

Clinical manifestation of arteriovenous malformations in the brain

Back 610

Spontaneous hemorrhage after the first decade of life

Front 611

What type of hemorrhage happens in shaken baby syndrome?

Back 611

Subdural hemorrhage due to tearing of delicate bridging veins between brain and sinuses

Front 612

Diffuse axonal injury

Back 612

Result of sudden accelaration/deceleration that stretches or tears axons in cerebral white matter.

Front 613

Cause of most cases of post-traumatic dementia

Back 613

Diffuse axonal injury in conjunction with hypoxic-ischemic injury (responsible for most cases of persistent vegetative state)

Front 614

Contusion

Back 614

Hemorrhages in superficial brain parenchyma caused by blunt trauma

Front 615

Coup contusion

Back 615

The hemorrhage is most pronounced in the area of the impact (trauma)

Front 616

Contrecoup contusion

Back 616

The hemorrhage is most pronounced on the opposite side of the brain from the impact (trauma)

Front 617

Most common congenital malformations of nervous system

Back 617

Neural tube defects, malformations associated w/hydrocephalus, primary forebrain abnormalities

Front 618

Most common and severe cranial neural tube defect

Back 618

Anencephaly (absence of brain)

Front 619

Meningomyeloceles

Back 619

Herniation of spinal meninges and spinal cord through a posterior vertebral defect

Front 620

Most common group of primary CNS tumors

Back 620

Astrocytomas

Front 621

Most common mutation in astrocytomas

Back 621

TP53

Front 622

Characteristic radiographic finding in oligodendrogliomas

Back 622

Calcifications in the tumor

Front 623

Most common form of neuronal tumor in CNS

Back 623

Ganglion cell tumors

Front 624

Common manifestation of ganglion cell tumors

Back 624

Seizures

Front 625

What viral disease has been associated with increase in CNS lymphomas?

Back 625

AIDS

Front 626

Characteristic histologic features of CNS lymphomas

Back 626

Tendency to grow around and within walls of blood vessels (angiocentric growth)

Front 627

Gender predilection of meningiomas

Back 627

More predominant among females due to progesterone receptors on meningothelial cells

Front 628

What types of neoplasms can commonly spread to the CNS?

Back 628

Hematopoietic neoplasms

Front 629

Lesions of multiple sclerosis

Back 629

Called plaques, well-demarcated lesions of demyelinated axons in the brain

Front 630

Nutritional deficiencies related to CNS disorders

Back 630

Thiamine and Vit B12 deficiencies. Can lead to peripheral neuropathy

Front 631

Dementia

Back 631

Development of memory impairment and other cognitive deficits, but normal level of consciousness

Front 632

Most common cause of dementia in the elderly

Back 632

Alzheimers disease

Front 633

What chromosomal abnormality is associated with greater risk of Alzheimers?

Back 633

Trisomy 21 (Down syndrome), if they survive beyond 40

Front 634

Cause of Alzheimers

Back 634

Unknown, but thought to be associated with amyloid precursor protein and protein tau

Front 635

Buzzwords for Alzheimers

Back 635

Tau protein, amyloid plaques, and Lewy bodies

Front 636

Histologic features of Alzheimers

Back 636

Neurofibrillary tangles, amyloid plaques, and Lewy bodies

Front 637

Lewy bodies

Back 637

Found in Alzheimers and Parkinsons

Front 638

Parkinsonism

Back 638

Disturbance in motor functions and dementia. Causes pill-rolling tremor, and involuntary movements

Front 639

Cause of Parkinsons

Back 639

Disturbance in dopaminergic pathways connecting substantia nigra to the basal ganglia

Front 640

Genetic factors in Parkinsonism

Back 640

Most are sporadic, but some are familial and result from mutation in a-synuclein gene

Front 641

Huntington disease

Back 641

Hereditary, progressive, fatal disorder of extrapyramidal motor system

Front 642

Two clinical features of Huntington

Back 642

Chorea (involuntary movements) and dementia

Front 643

Genetic basis for Huntington

Back 643

Trinucleotide repeat mutations in huntington gene on chromosome 4p. 45-55 CAG repeats

Front 644

Clinical onset of Huntington

Back 644

Usually in the 4th or 5th decade

Front 645

Amyotrophic lateral sclerosis (ALS)

Back 645

aka Lou Gehrig disease. Most common primary neurodegenerative disorder affecting motor neurons