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211 Cards in this Set

  • Front
  • Back
70-80% of clear cell renal cell carcinomas are associated with mutations of which gene?
VHL gene on chromosome 3p; mutation leads to VEGF proliferation and angiogenesis
Type 1 papillary renal cell carcinoma is associated with mutations of which gene?
Met oncogene on chromosome 7
What is an oncocytoma? What will you see histologically?
Benign lesion that may seem like renal cell carcinoma on a radiograph

Will see dense eosinophilia and a "spoke wheel" vascular pattern
Which kidney malignancy is associated with the Sickle Cell Trait?
Medullary Renal Cancer
What symptoms comprise the Grawitz Triad?
1. Hematuria
2. Flank pain
3. Palpable mass
What is Stauffer syndrome? With what is it associated with?
Hepatic dysfunction NOT due to metastasis.

Is associated with renal cell carcinoma
What paraneoplastic syndromes are associated with renal cell carcinoma?
Hypercalcemia, erythrocytosis, Stauffer syndrome
Immunotherapy will only work for which type of renal cell carcinoma?
Clear cell renal cell carcinoma
Is Sorafenib a multikinase inhibitor, or a tyrosine kinase inhibitor?
Multikinase inhibitor
Is Sunitinib a multikinase inhibitor, or a tyrosine kinase inhibitor?
Tyrosine kinase inhibitor
Where is the most common site for a transitional cell carcinoma?
Bladder
What are five (5) risk factors for bladder cancer?
1. Cigarette smoking
2. Aniline dyes
3. Arylalkamines (rubber/plastic)
4. Cyclophosphamide
5. Schistosomiasis
With what stage does bladder cancer become detrusor muscle invasive?
Stage 2 and beyond
For a stage 1 bladder cancer, what kind of vaccine might you recommened?
BCG vaccine (a Tb vaccine); it causes an inflammatory response that may kill tumor cells
Which urothelioma has a greater chance of cure: bladder or renal pelvis/ureter?
Bladder
What type of carcinoma would you use Finasteride with, and how does it help?
Use with prostate cancer; it inhibits type 2 5-alpha reductase
What is Brachytherapy?
Inserting radioactive seeds into the prostate
When would you NOT use GNRH agonists to treat prostate cancer?
If the cancer has metastasized
What is the most curable solid malignancy in males?
Testicular cancer
What is the most common malignancy in young males (15-35 yrs old)?
Testicular cancer
Type 2 papillary renal cell carcinoma is associated with what condition, and in which demographic?
Leiomyomatosis in young women
What four (4) symptoms might you see with bladder cancer?
1. Urinary frequency
2. Abdominal pain
3. Dysuria
4. PAINLESS hematuria
What is the gold standard treatment for urothelioma of the renal pelvis/ureter?
Nephroureterectomy with bladder cuff excision
Which nodes MUST you examine in the case of testicular cancer?
Supraclavicular nodes (may indicate testicular cancer metastasis)
What is the description of the metastasis you might see with testicular cancer? Where are the common places of metastasis?
"Cannon ball metastasis"

Commonly metastasizes to the LUNGS and RETROPERITONEAL lymph nodes
What is the most common presentation for testicular cancer?
PAINLESS enlargement!

If you have pain, that might indicate a hemorrhage into the tumor
What is the lifetime risk of kidney stones in males? In females?
Males = 12%

Females = 6%
Which US region has the highest prevalence of kidney stones?
Southeastern US
How does vitamin C lead to an occurrence of kidney stones?
Metabolism of vitamin C can yield oxalate
Which protease inhibitor for HIV treatment causes kidney stone formation?
Indinavir!
What is the clinical presentation of kidney stones?
1. Hematuria
2. Flank pain
3. Lower UT symptoms (dysuria, frequency, urgency)
What are four (4) common compositions of kidney stones?
1. Calcium oxalate/phosphate
2. Uric acid
3. Struvite
4. Cystine
How does ALKALINIZATION help treat kidney stones?
The pKa's of urate and cystine dictate their solubility
How does alpha-blockade help treat kidney stones?
Reduces the contraction of the distal ureter to help expulsion
What is the gold standard minimally invasive surgical procedure for BPH?
TURP: Transurethral resection
What is Dietl's Crisis, and with what condition might you see it in?
Acute hydronephrosis with flank pain

May see it in UPJ obstruction
What is a retrocaval ureter? Its presentation is similar to what other condition?
A congenital abnormality in which the RIGHT ureter passes POSTERIOR to the vena cava

Similar presentation to UPJ obstruction
What is the clinical presentation (4 signs) of a UPJ obstruction?
1. Dietl's Crisis
2. Hematuria
3. UTI w/ fever
4. Abdominal mass
Melanocytes originate from where?
Neural crest
Where are Langerhan's Cells derived from?
Bone marrow; are a type of macrophage/dendritic cell
What is the mutation in Epidermolytic Hyperkeratosis? What layer of epidermis is affected?
Mutation in keratins 1 and 10

The stratum SPINULOSUM is affected, not the basal layer
Which layer is involucrin located in?
Stratum Spinulosum
Which layer are loricrin and profilaggrin located in?
Mainly in stratum granulosum and corneum
Which disorder of cornification is associated with an extreme odor? What is the cause of this odor?
Epidermolytic hyperkeratosis. The odor is caused by bacterial colonization.
Which disorder of cornification is associated with a cardboard-like appearance, erosions, and blisters?
Epidermolytic hyperkeratosis.
What is the defect in Harlequin Ichthyosis?
Caused by a block in profilaggrin processing

Also associated with a defect in lipid synthesis
What is the general clinical presentation in Harlequin Ichthyosis?
A general SCALING of the skin; the skin moves as one giant plate and cracks, is almost universally fatal
What keratins are affected in Pachyonychia Congenita?
Keratins 16 and 17, which are preferentially expressed in nails, mouth, palms, soles of feet
Which disorder of cornification presents with wedge-shaped thickening of nails?
Pachyonychia Congenita
Mutilating Palmar-Plantar Hyperkeratosis is associated with a defect in what?
Loricrin
Mutilating Palmar-Planter Hyperkeratosis is associated with what sensory deficit?
Deafness
Which disorder of cornification presents with constricting bands and auto-amputation of fingers?
Mutilating Palmar-Plantar Hyperkeratosis
What mutation causes KID syndrome? What type of infections does it make you susceptible to?
A mutation in the connexin gene

Increase in C. albicans infections
What are the four (4) signs of KID syndrome?
1. Keratitis
2. Icthyosis
3. Deafness
4. Ocular issues
What type of keratinocytes does HPV target?
Basal keratinocytes; this makes it difficult to remove
Which organism, that infects skin, has evolved mechanisms to evade immune surveillance?
HPV!
Verruca usually indicates a failure in what?
Failure of the cytotoxic cellular response
Which virus exhibits retrograde transport to the DRG after infecting mucosal surfaces?
Herpes Simplex Virus
Is psoriasis more common in the extensor or flexor regions?
EXTENSOR - elbows, knees, trunk
What is Koebner's Phenomenon?
Development of psoriasis in areas of TRAUMA
What is Auspitz's Sign?
Mechanical scraping of the silvery scale causes small blood droplets to appear
What is the most common co-morbidity with psoriasis?
Psoriatic ARTHRITIS

It is a spondyloarthropathy!! HLA-B27 what
What is dactylitis? With what condition is it associated with?
Inflammation of an ENTIRE finger or toe.

Associated with psoriatic arthritis
What are the three (3) characteristics of psoriatic arthritis?
1. Arthritis
2. Dactylitis
3. Enthesitis

Look for BAD NAIL INVOLVEMENT to clue you in for PA
What cells play a role in the initiation and maintenance of psoriasis? How do they do so?
TH1 cells.

They secrete cytokines that stimulates keratinocyte hyperproliferation (TNF-alpha, IL-2)
What cells play a role in eczema (atopic dermatitis type)?
TH2 cells

Secrete cytokines such as IL-4, IL-5, IL-10
The TH1 response to leprosy causes what type of leprosy?
Tuberculoid leprosy - cell mediated immunity
The TH2 response to leprosy causes what type of leprosy?
Lepromatous leprosy - antibody response
What are three types of biologic agents (proteins w/ pharmacologic activity)?
1. Recombinant human cytokines
2. Monoclonal antibodies
3. Molecular fusion receptor proteins
What are three (3) treatments for Psoriasis? How do they work?
1. Raptiva - blocks LFA1/ICAM1 co-stimulation
2. Amevive - binds CD2 to block co-stimulation
3. TNF antagonists
Vitamin D synthesis is a result of UVA or UVB?
UVB
Which penetrates skin further - UVA or UVB?
UVA
What are characteristics of photo-aged skin?
Wrinkles, laxity, uneven pigmentation, brown spots, leathery appearance
What determines skin color?
The efficiency of transfer of melanosomes
What is a key enzyme in melanin production, and what is its key cofactor?
Tyrosinase; Copper
What is albinism? What are concurrent symptoms with albinism?
An ABSENCE of melanocytes

Vision, hearing, mental problems (think neural crest)
Do ppl with darker skin have higher or lower levels of vitamin D production?
LOWER per dose of UVB
What is the phenotype of Piebaldism? Is it AD or AR? What is the causative mutation?
Non-progressive hypopigmentation (disorder of melanocyte development)

Autosomal-dominant mutation of KIT
What is the causative mutation of Waardenburg's Syndrome?
Mutations in genes for melanocyte MIGRATION - PAX3, MITF, SOX10
Besides achromia of the skin/hair, what are other symptoms of Waardenburg's Syndrome?
Deafness, heterochromia irides
What is the cause of Menkes Syndrome? What is the phenotype?
Copper deficiency

Pigmentary dilution
What is the cause of vitiligo?
AUTOIMMUNE disease that causes loss of melanocytes
What other conditions is vitiligo associated with?
Other autoimmune conditions: Hashimoto's thyroiditis, Addison's disease, pernicious anemia, diabetes mellitus
In reference to carcinogenesis, what does UVB induce?
Thymine dimers
What is a UVA-induced photoproduct?
8-Hydroxyguanosine
Where are common places for melanoma in Caucasian males?
Trunk, especially UPPER BACK
Where are common places for melanoma in females?
LOWER LEGS, back
Where are common places for melanoma in Blacks and Asians?
Plantar foot, subungal, mucosal sites
Which keratins are located in the basal layer?
Keratins 5 and 14
Which keratins are located in the spinulosum layer?
Keratins 1 and 10
What is the function of Bullous Pemphigoid Antigen 1 and 2?
Anchors the hemidesmosome; they are HD anchoring filaments
Where is laminin located? With what number collagen does it interact with?
Lamina densa of the basal lamina

Interacts with type 4 collagen in the basal lamina
Where is type 7 collagen located? What function does it serve there?
Located in the sublamina densa

Serves as anchoring fibrils
What is the molecular cause of pemphigus? What molecular structure is affected in pemphigus?
Inhibition of desmogleins via IgG antibodies

Desmoglein is part of the DESMOSOME
What is the most common symptom in pemphigus?
Oral lesions; only 10-15% will present with cutaneous lesions
What type of blisters do you see with pemphigus? Where is the blistering occuring?
Will see FLACCID blisters

Blisters occur just ABOVE the basal layer
What will you see in a direct immunofluorescence of pemphigus?
A "chicken-wire" pattern - will stain the keratinocytes in stratum spinulosum/granulosum
How do you treat pemphigus?
Steroids, cyclosporin
What is the most common autoimmune subepidermal blistering disease?
Bullous Pemphigoid
What type of blisters do you see with bullous pemphigoid? Where is the blistering occuring?
Will see TENSE blisters

Blisters occur in the lamina lucida
Does bullous pemphigoid usually occur in the young or the elderly?
Elderly
What will you see histologically w/ bullous pemphigoid?
A clean break b/w the epidermis and the dermis

This space will accumulate fluid and eosinophils
Where on the body does bullous pemphigoid spare?
The face
What is the autoantibody target in bullous pemphigoid?
The hemidesmosome

Specifically BP Antigen 2/Collagen 12
An immunofluorescence of bullous pemphigoid will show what?
A linear staining band of IgG and C3 deposits on the basement membrane zone
What is the autoantibody target in epidermolysis bullosa acquisita?
Auto IgG is directed against anchoring fibril collagen type 7

IgG deposits in the upper dermis, beneath the lamina densa
Can you use immunofluorescence to diagnose EB aquisita? What might be useful in diagnosis?
May see a linear band with IF, but that is not enough.

Need to use EM to see deposits of Ab for a diagnosis
What are the clinical features of EB aquisita?
Marked skin fragility, progressive hair loss, loss of nails, autoamputation of digits
What are the three (3) types of epidermolysis bullosa?
1. EB Simplex
2. Junctional EB
3. Dystrophic EB
What is the abnormality in EB simplex? Where are the blisters located?
Abnormal keratin 5 and 14 in the basal cells causes fractures; an autosomal dominant disorder

Blisters localized to the hands/feet, where there is mechanical shearing
Where are the blisters in Junctional EB? Is Junctional EB autosomal dominant or recessive?
Blisters through the lamina lucida

An autosomal RECESSIVE disorder
What is the abnormality in Dystrophic EB?
Decreased/absent collagen 7 (dermal anchoring fibrils) causes fracturing below BM zone
Is Dystrophic EB autosomal dominant or recessive? Its presentation is similar to what other bullous disease?
An autosomal DOMINANT disorder

Similar in presentation to EB aquisita - skin fragility, nail loss, autoamputation of digits
How do drugs cause toxic epidermal necrolysis (TEN) ?
Drugs may induce keratinocyte FAS ligand to become lytic
What are three (3) types of drugs that are associated with TEN?
1. Anticonvulsants
2. Anti-seizure meds
3. Sulfa drugs
What is a requirement for a condition to be considered TEN?
MUST have oral/mucus membrane involvement
What type of bullae do you see in TEN?
FLACCID bullae

Will see detachment of sheets of skin
What percentage of skin is involved before you diagnose the condition as TEN?
30%
How do you calculate Sensitivity?
TP / (TP+FN)
How do you calculate Specificity?
TN / (TN+FP)
How do you calculate Positive Predictive Value?
TP / (TP+FP)
What are four (4) clinical insults that are involved in the Systemic Inflammatory Response Syndrome?
1. Temp > 38C or < 36C
2. Heart rate > 90bpm
3. Resp. Rate > 20 bpm, or PaCO2 < 32 mmHg
4. WBC > 12000/mm3, < 4000/mm3, or a left shift
How do you differentiate b/c septic shock and sepsis-induced hypotension?
Administer fluids; if blood pressure increases, it is NOT septic shock and it is rather sepsis-induced hypotension
What would you use to treat septic shock, but not sepsis-induced hypotension?
Pressors
What causes the "warm" in warm shock?
Decreased vascular resistance causes vasodilation; skin becomes warm and flushed
What causes the "cold" in cold shock?
Injury to the tight junctions leads to fluid loss and hypovolemic shock
How do you treat compensated septic shock?
Use vasoconstrictors (NE, dopamine, vasopressin) only, since blood pressure is preserved
How do you treat uncompensated shock?
Use an inotropic agent + alpha-agonist to control vasodilation and increase cardiac output
What are three (3) characteristics of the febrile response?
1. Cytokine-mediated rise in core temperature
2. Generation of acute-phase reactants
3. Activation of numerous systems (physiologic, endocrinologic, immunologic)
Where are three (3) places you can measure the core body temperature?
1. Aortic blood
2. Esophageal
3. Tympanic membrane
What are the five (5) temperature sensors of the body?
1. Skin
2. Deep chest and abdomen
3. Spinal cord
4. CNS
5. Hypothalamus
What are the four (4) endogenous pyrogens that I keep on forgetting?
1. Ciliary neurotropic factor
2. Oncostatin M
3. Cardiotropin-1
4. Leukemic inhibitory factor
How do pyrogens increase body temperature? What actually crosses the BBB?
Pyrogens bind to preoptic anterior hypothalamus and activate phospholipase A2

PGE2 crosses the BBB to activate thermosensitive neurons
What is DECREASED in the febrile state (3 things)
1. TSH
2. ADH
3. Sweating
What infections (4) do NOT cause the febrile response in the elderly?
1. Bacteremia
2. Meningitis
3. Endocarditis
4. Pneumonia
How do COX inhibitors work?
Blocks the synthesis of PGE2
How do corticosteroids work as antipyretics?
Inhibits phospholipase A2; blocks mRNA transcription of pyrogenic cytokines
What temperature is considered the critical thermal max?
41.6 - 42.0 C

i.e. 106.9 - 107.6 F
How much should the pulse rise per 1C increase in body temp?
15bpm per 1C increase
What does fever do to calcium levels?
Hypercalcemia due to an increase in bone resorption
What causes neuroleptic malignant syndrome? What would this syndrome cause?
Too much dopamine antagonism due to neuroleptic/antipsychotic drugs

Syndrome will cause hyperthermia
What are some drug causes of hyperthermia?
1. Beta-blockers (leads to unopposed alpha stimulation)
2. Anti-cholinergics
3. Sympathomimetics
4. Salicylate toxicity
Which will cause a high grade fever, atelectasis (collapsed lung) or pneumonia?
Pneumonia
Which will cause a high grade fever, acute cholecystitis or cholangitis?
Cholangitis
Which will cause a high grade fever, acute pancreatitis or an infected pseudocyst?
Infected pseudocyst
What is a common cause of the double quotidian fever?
Miliary tuberculosis
What are the only two infections that can cause a temperature greater than 106F?
1. Malaria
2. Small pox
What kind of heart rate will you see in central fever?
Relative bradycardia; the febrile response is not activated, so you don't have beta agonism via catecholamines
What kind of drugs commonly cause malignant hyperthermia?
General anesthetics
What type of tumors cause fever?
Tumor cells involved in the reticular activating system

Tumor cells that secrete interleukins

Tumors involving macrophage-like cells
Which solid tumors (4) cause fever?
Renal cell carcinoma, hepatoma, ovarian carcinoma, osteosarcoma
What are some events that would trigger DKA?
1. Failure to take meds
2. Infection (mucormycosis)
3. Emesis
4. Other forms of stress
Which two drugs cause fever as a side effect?
1. Amphotericin B (shake and bake)
2. Bleomycin
What is the cause of death in DKA?
OVER-correction of fluids/glucose/electrolytes

Hypoglycemia from treatment may starve brain cells
What is the physiologic explanation for the pseudohyperkalemia in DKA?
Acidotic conditions will drive K+ out of cells

(Insulin drives K+ back into cells)
How would you calculate the amount of pseudo-hyponatremia in a DKA patient?
Na+ is reduced by 1.6 mEq/L for every 100 mg/dL the glucose level is above 100 mg/dL
How would you calculate the pseudohyperkalemia in DKA?
Serum K+ will be increased by 0.6 mEq/L for every 0.1 decrease in pH
What are 4 counter-regulatory hormones released in DKA, and what is the result of each?
1. Catecholamines --> ketone formation, lipolysis, glycogenolysis
2. Glucagon --> gluconeogenesis from lipolysis, ketone formation
3. Cortisol --> gluconeogenesis from muscle
4. Growth Hormone --> lipolysis, antagonism of insulin
What is the result of released cortisol in DKA?
Gluconeogenesis from muscle
What is the result of released catecholamines in DKA?
Ketone formation, lipolysis, glycogenolysis
What is the result of released glucagon in DKA?
Ketone formation, gluconeogenesis from lipolysis, glycogenolysis
What is the result of released growth hormone in DKA?
Lipolysis, antagonism of insulin
What causes the early symptoms of DKA?
Hyperglycemia, muscle breakdown and dehydration
What are the early symptoms of DKA?
1. Polydipsia, polyphagia, polyuria
2. Visual changes
3. Weight loss/weakness
What causes the later symptoms of DKA?
Acidosis, ketonemia, hyperosmolarity
What are the later symptoms of DKA?
1. Acetone breath
2. Abdominal pain
3. Kussmaul hyperpnea
4. Altered mental status
What causes the terminal symptoms of DKA?
Hypokalemia
What are the terminal symptoms of DKA?
1. Cardiac arrhythmias
2. Muscle cramps
3. Gastric stasis/ileus
What are the three vitals you want to measure STAT in DKA?
1. Glucose level in blood
2. Ketones in blood
3. Arterial blood gas to measure acidosis
What causes the abdominal pain in DKA?
Acidosis causes vasoconstriction of the blood supply to the gut viscera
How do you treat DKA (in order of importance)
1. Fluids (BOLUS for fast result)
2. Electrolytes (K+, Mg, Phos)
3. Continuous monitoring
4. CONTINUOUS insulin (to decrease ketones, not to fix hyperglycemia)
What correlates to mental status in DKA?
Serum osmolality

Normal is 285-295 mOSM/L
How do you calculate serum osmolality?
2*Na + Glu/18 + BUN/3
Can DKA be the initial presentation in diabetes mellitus?
Yes (10% of the time)
At which dilution should you look at serum acetone to measure ketones?
1 : 2 dilution
What causes the tachypnea in DKA?
Tachycardia + acidosis...think about it
What causes shock in DKA?
Uncorrected dehydration
What two (2) things will shock cause in DKA?
1. Exacerbation of acidosis via lactic acidosis
2. Decreased GFR, which worsens hyperglycemia and causes acute tubular necrosis
What EKG reading would alert you to stop administering K+ to treat DKA?
Peaked T-waves, which are indicative of hyperkalemia (more K+ rushing into the cell)
When would you administer bicarbonate therapy in DKA?
ONLY if the pH drops < 7.0
How do you treat anaphylactic shock? Can you treat other forms of shock this way?
Treat w/ epinephrine

Do not use it to treat other forms of shock
What is the etiology of hemorrhagic shock?
Rapid blood loss
What is the etiology of septic shock?
Increased capillary permeability
What is the etiology of neurogenic shock?
Loss of sympathetic tone, probably due to spinal cord trauma
What is the etoilogy of cardiogenic shock?
Decreased pumping ability of the heart, which causes inadequate perfusion to tissues
What is the etiology of anaphylactic shock?
Type 1 hypersensitivity rxn causing systemic vasodilation and increased capillary permeability
Which skin infection is more superficial, that of Staph or Strep?
Staph
Which organism infects the skin and loves to go into the lymphatics
Strep
Do subcutaneous arteries/veins/nerves travel above or below the fascia?
Above
How are the gradings for musculotendinous strains determined?
Grade 1: partial tear, NO weakness
Grade 2: partial tear, SOME weakness
Grade 3: complete tear, loss of function, palpable defect
What is tendinosus?
Tendon degeneration from OVERuse

NOT an inflammatory condition!
What word describes a fracture in several pieces?
"Comminuted"
What is the normal time progression for fracture healing?
Day 1-3: Bleeding and clot formation
Week 1: Macrophage infiltration
Weeks 1-6: Clot reorganizes into a CALLOUS
Months 2 - 12: Reorganization into mature bone
What is the time progression for ligament healing?
Immediately - Hemorrhagic phase
1-2 weeks - Inflammatory phase
1-8 weeks - Reparative phase
4 weeks - 1 year - Remodeling phase
What is the healing time for the different grades of sprains?
Grade 1: 2-4 weeks
Grade 2: 4-6 weeks
Grade 3: 2-3 months
What 3 risk factors give the highest risk for melanoma?
1. Changing nevus
2. Familial melanoma w/ dysplastic nevi
3. > 50 nevi that are > 2mm
What happens to the ratio of collagen 3 and 1 in photoaging?
Increase in the 3/1 ratio
If pemphigus is a paraneoplastic lesion, what is the underlying malignancy?
Signifies a lymphoreticular malignancy
What is Nikolsky's sign?
The movement of the entire epidermis with little lateral pressure

Seen in TEN
What is the most common patient profile of bladder cancer?
Caucasian, older, male
What can give a false positive in a screen for beta-HCG to test for choriocarcinoma?
Hypogonadism, marijuana
What can give a false positive in a screen for alpha-fetoprotein to test for yolk sac tumor?
Hepatoma, hepatitis, cirrhosis
What symptom is common in many genitourinary lesions, but does not occur in BPH?
Dysuria!
Which calculi are radio-opaque?
Stones made up of calcium
Which calculi are radio-transparent?
Stones made up of urate...b/c they are complexed w/ SODIUM
Which calculi indicates an infection?
Struvite stones, which are made up of phosphate+Mg+Ca

Due to the presence of ammonia
What would a calculi composed of cystine indicate?
The patient has difficulties reabsorbing amino acids at the proximal tubule
How big does a calculi have to be before you consider surgical removal?
> 5mm
Which drugs are sympathomimetic?
1. Cocaine
2. Amphetamines
3. PCP
4. LSD
Which drugs are cholinergic?
1. Organophosphates
2. Pilocarpine
Which drugs are CNS depressants?
1. Inhalents
2. GHB
3. Opiates/narcotics
4. Ketamine