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DHM midterm
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Pre-School
03/22/2012

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Term
Involucrin
Definition
marker for maturity in keratinocytes
Term
Nikolsky sign positive in ____, negative in ____
Definition
pemphigus; pemphigoid

(is a physical diagnostic sign for acantholysis)
Term
desmogleins, desmoplakins, tonofilaments
Definition
remember that tonofilaments (i.e. cytokeratin) are attached transversely in desmosomes & on-end in hemidesmosomes
Term
things melanocytes are stimulated by
Definition
ACTH (MSH), T3, E2, progesterone
Term
apocrine sweat glands are actually...
Definition
merocrine excretion (eccrine sweat glands use ion channels)
Term
what binds hemidesmosome in BM?
Definition
collagen VII
Term
nail growth rate
Definition
1mm/week
Term
dDx when you see clubbing
Definition
lung disease/lung cancer, CHF, A-V malformation, celiac, IBD
Term
Nail pitting
Definition
psoriasis
Term
Beau's lines
Definition
(indentations in nail bed, move with growth)
marker for acute illness (can estimate when by growth rate (~1mm/week)
Term
Mees lines
Definition
White lines that move with growth
(arsenic or thallium poisoning)
Term
Muehrcke lines
Definition
(white lines that don't move w/ growth)
- marker for hypoalbuminemia
Term
bad stuff in black henna
Definition
paraphenylenediamine --> allergic dermatitis
Term
Most common defect in ED
Definition
lysyl hydroxylase (smooth ER)
Term
What inhibits Th1 differentiation? Th2?
Definition
IL-10; IFN-gamma
Term
antibodies induced to be secreated by IL-4 (& 13)
Definition
short term: IgG1; long term: IgE
Term
need this for class switching
Definition
CD40L (CD40 is on T-cells)
Term
This is needed for T-cell activation
Definition
CD28 (T-cells) & B7 (APCs)

(aside: CTLA-4, i.e. CD152, binds B7 and is responsible for anergy)
Term
how do immune complexes get removed?
Definition
complement disrupte antigen-antibody bonds --> solubilized the product --> C3b binds RBCs --> transported to spleen for destruction
Term
what infectious diseases can cause T3 hypersensativity?
Definition
strep (post-strep glomerulonephritis), meningitis, hep, mono, malaria, trypanosomes

(aside: serum sickness is a type 3)
Term
what causes kidney damage in SLE?
Definition
immune complexes
Term
what form of trypanosome lives in sandfly? human?
Definition
promastigote (flagellated), amastigote (non-flagellated, intracellular)
Term
Tx for leishmania
Definition
stibogluconate (antimony compound)
Term
important thing that protects leishmania?
Where does it develop?
Definition
- lipophosphoglycan (LPG)

- it develops in the sandfly gut; it protects the amastigote in a number of ways:
1) binds CR3 via C3bi --> enhances phagocytosis w/out immune system activation
2) resist complement mediated lysis
3) resists acid in lysosome (later infection)
Term
What is messed up when people get chronic cutaneous leishmaniasis (non-healing ulcer for at ~3 months)
Definition
Too much Th2 response
Term
what is a possible late complication of localized, cutaneous leishmaniasis?
Definition
mucocutaneous leishmaniasis
Term
what type of cells do you see in mucocutaneous leismaniasis?
Definition
mononuclear infiltrate w/ lots of granulomas & few, if any, organisms
Term
what mediates tissue damage in mucocutaneous leishmaniasis (& most things)
Definition
Th17
Term
how does Th17 produce tissue damage?
Definition
it's a pro-inflammatory T-cell:
- increases TNF & IFN-g production by other T-cells
- increases neutrophils --> increase necrosis, MMPs
- IL-1,6,17,23
Term
How does IL-10 inhibit Mø?
Definition
IL-10 induces macrophage arginase --> decreases NO production
Term
name precursors for:
BCC
SCC
melanoma
Definition
- none
- actinic keartosis/cheilitis, SCCIS (Bowen's)
- dysplastic nevus
Term
wavelengths for:
Blue light
UVA
UVB
UVC
Definition
-460 nm (DNA damage via ROS)
- 320-400 nm (Black light; same as blue light)
- 290-320 nm (sunburns; pyrimidine dimers; pilots, skiers)
- 100-290 nm (welder's arc)
Term
possible, bad complications of sunburn?
Definition
- high output cardiac failure
- infection
Term
2 types of sunblock
Definition
- chemical (has aromatic ring) --> absorbs UV light (resonance delocalization) (e.g. benzophenones, cinnamates, salicylates)

- Inorganic (e.g. ZnO4): absorb & reflect UVA & UVB
Term
2 types of pyrimidine dimers, which is most common?
Definition
1) cyclobutane (most common)
2) 6-4 dimers (more mutagenic)
Term
what does Pi do to 1-alpha hydroxylase activity?
Definition
high Pi inhibits 1 alpha hydroxylase (AKA CYP27B1)
Term
Ca, 25-OH vitD, & 1,25-OH vitD in Vitamine D toxicity?
Definition
high, high, normal
Term
why is azathioprine a riskfactor for SCC?
Definition
metabolite (6-thioguinine) is inserted into DNA & converted to ROS produce by UVA light
Term
how does inflammation cause SCC?

examples?
Definition
D6 saturation (sequesters chemokines) --> increased inflammation --> Ras-activated proliferation of keratinocytes (SCC)

- epidermolysis bullosa, marjolin's ulcer, scar, burn, lichen sclerosis, lichen planus, discoid lupus
Term
marjolin's ulcer?
Definition
non-healing ulcer; may have underlying osteomyelitis

risk of SCC (chronic inflammation)
Term
HPV types that predispose to SCC
Definition
3,5,8

("epidermodysplasia verruciformis" is an AR condition where people get lots of HPV infections & have an increased risk fo SCC b.c. of that)
Term
radiation & heat predispose to what type of skin cancer
Definition
SCC (ab-igne)
Term
aresenic skin cancer?
Definition
SCCIS (well water, meds, insecticides)
Term
where are the atypical cells in actinic keratosis?
Definition
stratum spinosum (SCCIS is full thickness)
Term
sign of very poor prognosis in SCC?
Definition
perineural invasion
Term
describe features of each type of BCC:
- Nodular
- Morpheaform
- Superficial
Definition
- big nests of cells w/ retractions (most common type)
- looks like a scar; smaller clumps of cells
- scaly & on trunk; cells attached to hair follicles
Term
gene in Gorlin syndrome
Definition
("basal cell nevus syndrome")
PTCH (patched) --> overactivation of smoothened
Term
BCC, palmar pits, jaw cysts, frontal bossing, short 4th metacarpal
Definition
Gorlin syndrome
Term
markers for aggressiveness in BCC
Definition
- bcl-2 (antiapoptotic)
- anti-SMA (local migration)
- Strombolysin 3 (invasion)
Term
most common type of melanoma
Definition
superficial spreading (70%) --> located on upper back
Term
melanoma on feet & hands (non-sun exposed areas)
Definition
acrolentiginous
Term
melanoma on face that isn't raise and stays in-situ for a long time
Definition
lentigo maligna
Term
Melanoma that has no "in situ" form
Definition
nodular (this form has the most mortality)
Term
abridged clark levels (melanoma)
Definition
I:in situe
II: shallow dermal spread
III: fills papilla
IV: fills whole dermis
V: in subcutis
Term
stain for Merkel cell carcinoma
Definition
CK20
Term
virus associated w/ merkel cell
Definition
polyomavirus
Term
what embryonic layer does synovium derive from?
Definition
mesoderm (explains why there is no basement membrane
Term
layers of synovium?
Definition
intima (2-3 cells thick: Mø & fibroblasts that secrete HA)

subintima (has lymphatics, capillaries, nerves)
Term
Type B synoviocytes
Definition
fibroblasts
Term
which form of collagen is secreted but not terminally cleaved?
Definition
procollagen (tropocollagen is cleaved product)
Term
second messanger in RANK-L signaling?
Definition
NF-kB
Term
howships lacunae
Definition
osteoclast resorption pit
Term
Haversian canal
Definition
where vessels live
Term
canaliculi
Definition
where osteocyte processes live (lamellae is compact bond where the osteocytes live)
Term
many ____ are contained in one muscle cell/fiber
Definition
myofibrils
Term
describe SR & T-tubule triad
Definition
- T-tuble in center, 2 SR membranes on each side
- located at A-I junction
Term
Name stages of bone repair:
1 week
2 weeks
1 month
3 months
Definition
- inflammation
- granulation tissue, early mineralization
- woven bone ("bony callus")
- lamellar bone ("remodeling stage")
Term
things that can cause pathologic muscle hypertrophy
Definition
hypothyroid
acromegaly
dystrophies
chronic denervation
Term
wernig hoffmann disease
Definition
infant motor neuron disease (most severe spinal musclar atrophy): SMA1 mutation
Term
SMA1 mutation
Definition
wernig hoffman disease
Term
Duchenne's MD gene
Definition
Xp21 (dystrophin)
Term
other X-linked musclar dystrophy?
Definition
emery-dreifuss (gene is "emerin" on Xq28)
Term
triplet repeat muscular dystrophy
Definition
myotonic dystrophy (CTG repeat of DMPK)
Term
ACTA1 gene
Definition
Nemaline myopathy (Z-band (alpha-actinin) inclusions, i.e. "nemaline rods")
Term
Central core disease (myopathy) gene mutation
Definition
RYR1
Term
X-linked myopathy
Definition
centronuclear myopathy (see lots of central nuclei like the muscle is trying to repair itself)
Term
Parking lot inclusion
Definition
mitochondrial myopathies ("ragged red fibers"); opthalmoplegia & encephalopathy are common sequela
Term
inflammatory myositis w/ inflammation not around atrophic muscle
Definition
dermatomyositis (inflammation is around capillaries)
Term
HLA-DR2 associated with?
DR3?
Definition
- narcolepsy & MS
- SLE & Myasthenia
Term
Which HLA has a small beta subunit?
Definition
HLA-1
Term
job of HLA-DM?
Definition
remove CLiP from MHCII
Term
how many AA on MHCI & MHCII
Definition
8-10 & 13-18
Term
name the 2 stimuli for T-cell activation
Definition
MHC I (CD8) or II (CD4) and B7 (attaches to CD28 on T-cells)
Term
acute rejection is mediated primarily by CD4 or CD8? chronic?
Definition
CD8 mediates acute (activated against graft antigens on graft MHC-I

over time, graft dendritic cells decline and no longer travel to lymph nodes --> host APCs present graft antigens to immune system --> CD4 response --> chronic rejection
Term
HLAI testing?
Definition
mix leukocytes w/ antibodies to different types of HLA and complement --> look for complement activation by dye uptake
Term
concentration that urate crystals precipitate @
Definition
>6.8 mg/dL
Term
high purine foods
Definition
red meat, shellfish, beer
Term
drugs that can contribute to gout

Drugs that reduce the risk?
Definition
Diuretics (HCTZ & furosemide)
Niacin (metabolized to UA)
Cyclosporin (decrease renal function)


Reduce risk: fibrates, Losartan
Term
skin condition that can cause gout
Definition
psoriasis (also remember that myeloproliferative disorders are classically associated with gout)
Term
2 transporters involved in UA transport in kidney and their positions?
Definition
URAT1 (apical)
Glut9 (basolateral)
Term
inflammasome does what?
Definition
important in gout: activates caspase1 to cleave IL-1 to it's active product --> inflammation
Term
UA level in trophaceous gout
Definition
>10 mg/dL
Term
treatment of gout-induced nephrolithiasis
Definition
allopurinol
Term
long standing gout effect in kidney
Definition
urate nephropathy (uric acid crystals in interstitium)
Term
SE of colchicine
Definition
agranulocytosis; neuropathy
Term
Alizarin red stain
Definition
basic pyrophosphate crystalline arthritis (periarthritis, e.g. "frozen shoulder")
Term
other things RF is seen in?
Definition
HepC, SLE, Sjogrens, Cryoglobulinemia, chronic infections, 10% of elderly
Term
thing on APCs/B-cells that activate T-cells

thing on T-cells that activate APCs/B-cells
Definition
B7

CD40L (esp needed for class switching, def = hyper IgM)
Term
HLA associated w/ RA?
Definition
HLA-DR4 (explains why it is a CD4+ mediated pathogenesis)
Term
Types of T-cells in RA
Definition
Th1, Th17 (IL-1,6,17 & IFN-g & TNF)
Term
can psoriatic arthritis be on DIPS?
Definition
yes! usually the first joint affected (usually assymetric: dactylitis, i.e. sausage-finger)
Term
Gene involved in citruilination
Definition
PADI4 (gene that is associated w/ RA), another gene is PTPN22 (regulates B & T cell function)
Term
why do you get boney erosions w/ RA?
Definition
increased RANK-L expression on synovium

get subcondral trabecular thinnign b.c. of influx of inflammatory cells
Term
felty syndrome
Definition
neutropenia, splenomegally, rheumatoid arthritis
Term
increased to get this cancer with RA
Definition
lymphomas
Term
Heart problem with RA
Definition
increased risk of coronary artery disease
Term
markers of systemic inflammation?
Definition
CRP & ESR (fibrinogen) --> both are acute phase reactants

(Check for in RA)
Term
markers for RA
Definition
acute phase proteins

RF, anti-ccp, ANA
Term
sulfasalazine MOA
Definition
immunosupressive agent: sulfapyradine componenet inhibits B-cell function (RA)
Term
Hydroxychloraquine use
Definition
RA: nebulous MOA; hemolysis in G6PD def (so does sulfasalazine!)
Term
TNF inhibitors?
Definition
Etanercept, Infliximab (RA)
Term
CD20 antibody
Definition
Rituximab (RA)
Term
CD20 antibody
Definition
Rituximab (RA)
Term
IL-1 inhibitor
Definition
Anakinra (RA)
Term
what disease do you get subchondral cysts in?
Definition
osteoarthritis
Term
2 joint diseases associated w/ hemochromatosis
Definition
Pseudogout & osteoarthritis

Other things for PG: hyperparathyroidism, osteoarthritis

Other thigns w/ osteoarthritis: wilsons, ochronosis, gout, pseudogout, other inflammatory joint diseases
Term
stickler syndrome & blount disease?
Definition
both associated w/ OA:
- defect in collage (many different types)
- congenital tibia varus
Term
dietary deficiencies associated w/ OA?
Definition
selenium & iodine
Term
what is an absolute indication for CCS injections in OA?
Definition
bakers cyst
Term
normal synovial pressure
Definition
-5.7 mmHg
Term
symptom progression w/ gonococcal septic arthritis
Definition
prodrome (fever, chills) --> migratory arthritis --> settles into a large joint (usually knee)
Term
how can you differentiate gonococcal setptic from non-gonococcal bacterial septic arthritis from physical exam alone?
Definition
gonococcal is frequently accompanied w/ tenosynovitis (rare in non-gonococcal)
Term
Tx for gonococcal septic arthritis
Definition
- 1 week of IV ceftriaxone (may substitute spectinomycin)
- 1+ weeks of oral amoxicillin/clavulanic acid

always add doxycycline for chlamydia
Term
what is first step in management of septic joint. What do you do with this data?
Definition
aspirate & gram stain

if G+cocci, assume staph or strep --> start on vanco (if MRSA), nafcillin, or cefazolin & do sensitivity testing

if G(-) start on ceftriaxone/ofloxacin

if pseudomonas suspected (IVD), start on antipseudomonals: ceftazidime/piperacillin/gentamicin
Term
most common cause of G(-) septic arthritis
Definition
enterbacteraceae
Term
which viral arthritis looks like RA
Definition
parvo
Term
Tx for osteomyelitis?
Definition
B-lactams, fluoroquinolones, rifampin
Term
give answers to each situation for septic arthritis:
- young, healthy adult:
- RA:
- EtOH:
- IC:
- Myeloproliferative disease:
- IVD:
- Neonates:
- Children:
- Patient w/ UTI:
- Cat bite or scratch:
- Rat Bite:
Definition
- young, healthy adult: gonococcal
- RA: S.aureus
- EtOH: S.pneumo
- IC: G(-)
- Myeloproliferative disease: G(-)
- IVD: Pseudomonas & serratia
- Neonates: G(-), S.aureus, Candida
- Children: Salmonella, S.aureus
- Patient w/ UTI: Proteus, E.coli
- Cat bite or scratch: Pastuerella multocida
- Rat Bite: Streptobacillus moniliformis
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