SNAPP Questions Unit 4

Question 1

Which of the following signs would be consistent with an actual arthritis diagnosis as opposed to periarticular pain?

Decreased range of motion
Effusion
Deformity
Pain is worse with active motion

Answer 1

Effusion

Periarticular pain refers to pain arising from structures around the joint such as tendons or bursae. It can be distinguished from true arthritis by the lack of effusion, the presence of point tenderness over the area of inflammation, and joint pain made worse with active motion. There could be decreased range of motion and deformity in either case so neither A nor C is a good answer. Finally, pain worse with active motion is not consistent with arthritis and is more associated with periarticular pain so would not be sign used in diagnosing arthritis.

Question 2

Staph Aureus is placed on a slide and incubated with a patient’s serum. After washing, a fluorescently-labeled antibody is incubated with the samples. Some samples are fluorescent after a second wash; what is this assay proving the presence of?

Presence of staph aureus in the patient’s serum
Patient antibody to staph aureus
Patient antibody to the fluorescently labeled antibody
Immune complexes of staph aureus and patient antibody

Answer 2

patient antibody to staph aureus

INDIRECT IMMUNOFLORESCENCE TELLS ABOUT PRESENCE OF ANTIBODY olliPlate known antigen/liliAdd patient serum/liliAdd a florescent antibody to the patient’s antibody/li/ol

Question 3

Which of the following is consistent with a positive synovial fluid analysis for rheumatoid arthritis?

> 2000 WBC/ul
200-2000 WBC/ul, 25% neutrophils and type 1 fluid
Positive crystal exam
Elevated complement and serum glucose

Answer 3

> 2000 WBC/ul

2000 WBC/ul with predominantly neutrophils. Complement and glucose levels are usually LOW in RA. B. would be correct for osteoarthritis: 200-2000 WBC/ul with 25% neutrophils (negative crystals and normal glucose)/p

Question 4

Initial recognition of MSU crystals is done by:

Macrophages
B cells
T cells
Toll-like receptors

Answer 4

TLR

Toll like receptors 2 and 4 recognize MSU crystals. This triggers an innate immune response by activation of mast cells and monocytes via cytokines TNFa, IL6, IL8. The inflammatory response to MSU crystals depends on IgG binding to MSU crystals, subsequent activation of complement and neutrophil chemotaxis. /p Phagocytosis of MSU crystals by neutrophils causes neutrophil lysis and release of proteolytic enzymes into the extracellular space. This is part of why gouty attacks are self-limited in nature.

Question 5

Abnormal pyrophosphate (PPi) metabolism is found in CPDD and osteoarthritis. Which of the following is true of PPi levels in the synovial fluid of CPDD and osteoarthritis?

PPi levels are elevated in both CPDD and osteoarthritis
PPi levels are decreased in both CPDD and osteoarthritis
PPi levels are decreased in CPDD and elevated in osteoarthritis
PPi levels are elevated in CPDD and decreased in osteoarthritis

Answer 5

PPi levels are elevated in both CPDD and osteoarthritis

Synovial PPi levels are elevated in both CPDD and osteoarthritis. The PPi then bind to the calcium forming CPDD crystals in the mid-zonal cartilage layers around the chondrocytes. Where as MSU crystals precipitate spontaneously in the synovial fluid, CPDD crystals are released into synovial fluids via shedding from the cartilage itself

Question 6

What effect would inhibition of COX-1 have on hemostasis?

Increased binding of vWF to damaged endothelium
Decreased amounts of thromboxane A2
Increased release of alpha granules
Decreased binding of glycoprotein Ib to vWF

Answer 6

Decreased amounts of thromboxane A2

COX-1 converts arachidonic acid to prostaglandin H2, which in turn is converted to thromboxane A2 by thomboxane synthetase. Thromboxane A2, along with other agonists, is released, acting to amplify platelet activation.<p>A is not correct because vWF binds to endothelium before COX-1 is activated and regardless, this action would INCREASE platelet aggregation.</p><p>An increase in release of alpha granules would not be caused by COX-1 either and this would also INCREASE platelet aggregation.</p><p> Finally, even though D would lead to decreased platelet activation (like inhibition of COX-1), this happens before COX-1 activation, so inhibiting COX-1 would not affect this.</p>

Question 7

The major cell type found in the Pannus of RA is:

Neutrophils
T cells and macrophages
Fibroblasts
Dendritic cells

Answer 7

T cells and macrophages

Major cells in a Pannus are MEMORY T CELLS and macrophages Minor cells in Pannus: Fibroblasts, plasma cells, endothelium, DCs /p Major cells in Synovial fluid of RA: neutrophils/p

Question 8

What is unique about elite controllers?

They make effective antibodies for HIV
Their T cells are more diverse
Their T cells are more aggressive
Their T cells have more TCRs and thus rendered more effective at recognizing the antigen presented on the MHC

Answer 8

T cells are more diverse

HLA-B57 has been associated with resistance to HIV infection. These individuals produce a wider variety of CTL’s targeted against HIV. Elite controllers have T cells that HAVE been infected with HIV, but greater CTL diversity means more efficacious killing of infected T cells. These people will retain normal immune function for a long time, but probably not forever as infected T cells are being destroyed.

Question 9

People with bombay blood make antibodies against which carbohydrates complexes?
A
B
O

Answer 9

all

A,B, and O all have a gene that allows it to make the H antigen in which they hang their other antigens (an A antigen, a B antigen, or in the case of O, nothing). Bombay type does not even have the gene that makes the H antigen, so therefore all of these types of blood seem foreign to it. Bombay blood individuals can only receive other bombay blood. In their case, the saying that O is the universal donor is a misnomer because they are unable to even receive O blood.

Question 10

Which of the following is a feature of osteoarthritis?

Frequent joint inflammation
Increased joint mobility
Radiographic osteophytes
Systemic involvement

Answer 10

radiographic osteophytes

A common feature of osteoarthritis is hypertrophic bony spurs or (osteophytes). In osteoarthritis there is INFREQUENT joint inflammation because it is usually not considered an inflammatory type of arthritis. There is DECREASED joint mobility from the destruction of the cartilage and the hypertrophy of the contiguous bone. In osteoarthritis, there is no systemic involvement.

Question 11

Which joint is most classically affected during an acute gouty arthritic attack?

The first DIP
The first MCP
The elbow joint
The first MTP

Answer 11

first MTP

The MTP (metatarsophalangeal) of the great toe. This is called podagra and is by far the most common joint for gout to occur. Other commonly affected areas include the insteps, ankles, heels, knees, wrists, fingers and elbows.

Question 12

Early pathologic findings of rheumatoid arthritis include which of the following?

Destruction of articular cartilage, bone and periarticular structures
An increase in synovial lining cells (type a, macrophages, type b, fibroblast-like)
Growth of new blood vessels and focal aggregates of CD4 and B cells
Inflammation of the synovium

Answer 12

Inflammation of the synovium

RA begins with inflammation of synovium (early on mono-nuclear cells predominate). Mild inflammation, microvascular injury, edema. Later: Increased type A and B cells in synovium, fibroblast proliferation New blood vessels Focal aggregates of CD4 and B cells Continued microvascular injury Pannus forms under the influence of cytokines Primarily neutrophil response/p

Question 13

Takayasu’s Artheritis affects which sized vessels?

Large
Medium
Small
Arterioles/capillaries

Answer 13

large

Large vessel vasculitis includes Giant Cell arteritis and Takayasu’s. Giant Cell arteritis is related to HLA-DR4, suggesting its antigen driven. Medium size vessels: Polyarteritis Nodosa and Kawasaki’s disease Small vessles: Wegener’s (cANCAs), Microscopic polyangiitis (pANCAs), and Churg-Straus (p-ANCAs) /p VASCULITIS INVOLVES PLATELET ACTIVATING FACTOR which is permissive for immune complex deposition in vessels.

Question 14

Mucocutaneous bleeding is an indication of:

primary hemostasis defect
secondary hemostasis defect
fibrolytic disorders
a local vascular defect

Answer 14

primary hemostasis defect

deep muscle bleeding=secondary

Question 15

Fifteen years after following off of a roof and shattering his left ankle, a patient presents to you with worsening pain and stiffness in the left ankle. Upon examination, his ankle has lost all range of motion. You suspect that his joint was trying to protect itself from the ongoing disease process and fused. What do you call this fixation?

Spondylitis
Synchrondrosis
Ankylosis
None of the above

Answer 15

ankylosis

Ankylosis is the fixation of a joint as the result of a disease process, with fibrous or bony union across the joint. Spondylitis refers to inflammation of specifically the sacroiliac joint and synchondrosis is also a union like ankylosis, but it is a natural union (not from a disease process). It is the union of two bones formed by cartilage seen in the pubic symphysis or costosternal joints.

Question 16

In Reverse Passive Agglutination:

Latex beads coated with antigen are crosslinked by bacterial antigens
Latex beads coated with antigen are crosslinked by bacterial antibodies
Latex beads coated with antibody are crosslinked by bacterial antigens
Latex beads coated with antibody are crosslinked by bacterial antibodies

Answer 16

Latex beads coated with antibody are crosslinked by bacterial antigens

Reverse passive agglutination: olliCoat latex beads separately with known antigen/liliAdd patient serum/liliIf bacteria in CSF, bacterial ANTIGENS will crosslink the latex beads

Question 17

A 35 year-old Caucasian male comes to the ED complaining of pain in his ankles that seems to have started pretty abruptly, with his right ankle having more pain than the left. On exam, you see that in addition to inflammation in his ankles, he has dactylitis (sausage digits) in his toes, as well as signs of conjunctivitis. He also informs you that he has had diarrhea for about the last 2 weeks, along with some pain and dysuria. Based on this history and exam, which of the following is the most likely diagnosis?

Rhematoid arthritis
Reactive arthritis
Ankylosing spondylitis
Psoriatic arthritis

Answer 17

reactive arthritis

Reactive arthritis is correct–characterized by asymmetric peripheral arthritis affecting lower extremities (knees and ankles most commonly), male: female ratio of 5-10:1, dactylitis in 20-50% of patients, and a history of diarrhea for 2-4 weeks. Patients can also present with inflammatory back pain symptoms, inflammatory eye disease (also present in some AS patients). Step 1 Gem: Reiter’s Disease is a triad of reactive arthritis, uveitis and conjunctivitis that can occur as the result of sexually transmitted diseases as well asl GI pathogens. Patients with Reithers quot;can’t see, can’t pee, can’t climb a tree.quot; Sexually transmitted arthritis… who knew?

Question 18

Which of the following mother/fetus combinations is a likely set up for erythroblastosis fetalis? (Assume that the mother only has two children total)

RhD(-) mother, first baby RhD(+), second baby RhD (+)
RhD(-) mother, first baby RhD(+), second baby RhD(-)
RhD(-) mother, first baby RhD(-), second baby RhD(-)
RhD(-) mother, first baby RhD(-), second baby RhD(+)

Answer 18

RhD(-) mother, first baby RhD(-), second baby RhD(+)

HLA genes are expressed co-dominately. You receive one allele from each parent per HLA type and both of these alleles are expressed equally.p The HLA genes are located on chromosome 6 so A is incorrect. /pp HLA A and B code for MHC class I and HLA-DR codes for MHC class II so B is incorrect. HLA DR is the most important for considering rejection in transplantation cases so C is incorrect

Question 19

Which of the following statements is most correct regarding the major histocompatibility antigen that is strongly associated with spondlyoarthropathies?

90% of patients are positive for HLA-B27
90% of patients who are HLA-B27 will get the disease
HLA-B27 is necessary for the disease
HLA-B27 is sufficient for the disease

Answer 19

90% of patients are positive for HLA-B27

Over 90% of Caucasian AS patients are HLA- B27 positive, but only 1-2% of HLA-B27 positive people ever get AS in their lifetime. Remember that although this is the strongest association between a Class I HLA and disease, it is neither necessary nor sufficient for causing disease. /p Only 7% of the general population have the HLA B27 gene

Question 20

If an individual has a HLA-B27 genotype but does not have any first degree relatives with Anklosing Spondylitis, what are his chances of getting it?

2%
20%
50%
90%

Answer 20

2%

2% if HLA-B27 and no family history. If you are HLA-B27 and also have a first degree relative with AS then 20% chance. 90% of ppl with AS have HLA-B27 but only a small percentage of people with this genotype get the disease. /p Predominant cytokine in AS is TNFa /p Rats with genotype do NOT get disease if kept in sterile environment indicating that disease onset requires BOTH environmental trigger and a suseptible host.

Question 21

You place a bacterial specimen on slide, and add a patient’s serum. You then add a fluoro-labeled anti-human IgG antibody, incubate, and the wash the specimen When you look at your experiment under the microscope, you observe flourescence. From your test, which of the following conclusions is MOST correct?

IgG is present, because the secondary antibody is bound to it
Bacterial antigen is present, because the secondary antibody is bound to it
IgG is absent, because the secondary antibody is bound to the bacteria
Bacterial antigen is absent, because the secondary antibody is bound to the IgG

Answer 21

IgG is present, because the secondary antibody is bound to it

This experiment is describing an INDIRECT immunoflourescence test, which tests for the presence of an antibody in the patient’s serum. A direct immunofluorescence test is used to detect the presence of an ANTIGEN, by adding a flourescent antibody directly to it./

Question 22

Approximately how many people in the world become infected with HIV every year?

3 million, mostly adults
3 million, mostly kids
30 million, mostly adults
30 million, mostly kids

Answer 22

3 million, mostly adults

Worldwide, approximately 2.7 million people became infected with HIV. Of these, 2.3 million were adults, and 430,000 were kids under age 15. The indicence of HIV infection is speculted to be underestimated by as much as 40%. In 2008, there were approximately 33.4 million people worldwide living with HIV. /p AIDS diagnosis means that an HIV positive individual EITHER develops symptoms of opportunistic infections or Kaposi

Question 23

Which of the following best describes a ligament?

Connect muscle to bone
Active drivers of joint motion
Composed of bundles of parallel type 1 collagen
Connect muscle to muscle

Answer 23

Composed of bundles of parallel type 1 collagen

Ligaments are made of type 1 collagen and connect bone to bone. They prevent inappropriate motion at joint (hinge joints are bordered by collateral ligaments to limit flexion/extension). Tendons are the active drivers of motion.

Question 24

65% of people who are ‘elite controllers’ have HLA-B57 and:

Retain high numbers of CD8 cells even after HIV infection
Have greater numbers and diversity of CTLs against HIV peptides
Do not have CCR5 and are immune to HIV infection
Make antibody to HIV-1 and HIV-2

Answer 24

Have greater numbers and diversity of CTLs against HIV peptides

HLA-B57 = more CTL and they are more diverse against HIV peptides. 65% of elite controllers have HLA-B57 (this allele also causes DTH reaction to one of the treatments for AIDS and genotyping is now required before receiving that treatment) /p Elite controllers = harbor HIV but retain normal immune function for many years.

Question 25

A 65 year old female patient presents to your clinic with complaints of a unilateral headache, visual loss and scalp tenderness. Which of the following additional symptoms might you also expect, given this information?

Jaw claudication
Rash on the buttocks and legs
Thrombosis of peripheral veins
Conjunctivitis

Answer 25

Jaw claudication

Giant Cell arteritis = most common arteritis affecting med and large arteries, usually branches off the carotid. Affects elderly females. Temporal arteries originating from aortic arch/p Unilateral headache, jaw claudication, vision loss (occlusion of opthalmic artery may cause irreversible blindness), scalp tenderness Disruption of internal elastic lamina Treat with high dose steroids.

Question 26

Which type of pathophysiologic process is rheumatoid arthritis considered?

Metabolic
Inflammatory
Degenerative
All of the above

Answer 26

inflammatory

RA is a classic example of inflammatory arthritis. It is causes by an autoimmune disease that creates inflammation in the joint.

Question 27

Asymptomatic hyperuricemia is defined as which of the following?

Abrupt pain, with warm/red swelling of joints in the morning or overnight
An MSU serum level above 7 mg/dl but without gouty arthritis, tophi or uric acid nephrolithiasis
Attacks that spontaneously resolve within 3-10 days
No symptoms in between acute gout attacks

Answer 27

An MSU serum level above 7 mg/dl but without gouty arthritis, tophi or uric acid nephrolithiasis

Asymptomatic hyperuricemia is defined as 7 mg/ul MSU in serum but no symptoms or signs of gout, tophi or uric acid nephrolithiasis. Acute gouty arthritis is characterized by attacks that occur in morning/overnight and include painful, red, hot swelling. These attacks are self-limiting and resolve in 3-10 days. /p Intercritical gout describes the asymptomatic period between gouty attacks /p Chronic tophaceous gout results from the development of MSU deposits over time

Question 28

Which of the following is NOT a part of the clinical triad of Reiter’s syndrome?

Arthritis
Urethritis
Conjunctivitis
Cellulitis

Answer 28

cellulitis

Reiter’s Triad includes conjunctivitis, urethritis, arthritis (REACTIVE ARTHRITIS). Can’t see, can’t pee, can’t climb a tree! Caused by either sexually transmitted infections such as chlamydia or enteric bacterial infection. HLA-B27 Theory: misfolding of HLA receptor that occurs while it is still being processed in the endoplasmic reticulum triggers a cellular stress response

Question 29

A 50 year old basket weaver presents to your clinic complaining of pain in her fingers. After receiving his history, which of the following features indicate that osteoarthritis is the most likely diagnosis?

The pain is worse during exercise and improves with rest
The pain is present in the first couple hours of the morning
He experiences frequent afternoon fatigue
He describes the pain in his MCPs and PIPs, but reports no pain the DIPs

Answer 29

The pain is worse during exercise and improves with rest

Osteoarthritis has classically pain with use with improvement during rest. The other answers are commonly seen in rheumatoid arthritis.

Question 30

Which of the following is true regarding gout disease?

It is an auto-immune process
It is usually polyarticular
It lacks systemic symptoms
It causes crystal deposition in joints

Answer 30

It causes crystal deposition in joints

Gout is a disease process in which there is a build up of monosodium urate. This can precipitate out and causes crystals in the joint space. Gout is a metabolic process that creates inflammation; however it is NOT an auto-immune process. Gout can affect more than one joint and be polyarticular, however it is more likely seen as a monoarticular disease (potentially oligoarticular, but usually not polyarticular). Gout can have systemic symptoms associated with it.

Question 31

The most important cells in tumor resistence are:

CTLs
NKs
Th1s
B cells

Answer 31

CTLs

CTLs are the most important in tumor resistence. Recognize TAA’s on MHC class I’s. Clonal expansion = acquire lytic function, migrate to tumor, induce apoptosis in tumor cells (perforin or Fas-mediated pathways), secrete INFy upon TCR binding with MHC class I (increases Th1 response which recognize TAAs, attract M1’s)/p

Question 32

Thrombin (factor IIa) activates platelets by:

cleaving protease activated receptor (PAR1)
releasing ADP and serotonin
releasing thromboxane A2
binding factor VII

Answer 32

cleaving protease activated receptor (PAR1)

thrombin activates platelets by cleaving PAR1. Thrombin is formed when factor Xa converts II (prothrombin) to IIa (thrombin).

Question 33

A 25-year-old female presents to your clinic complaining of increasing pain in her fingers, wrists and knees. She has an erythematous, discoid-shaped rash on her chest and trunk, as well as a rash across her checks. She has also noticed small lesions in the back of her throat and is concerned about a possible “strep” infection. Based on this presentation, you order a few labs. She has positive antibody titers for Smith and dsDNA. Which of the following correctly describes this type of hypersensitivity?

Type I
Type II
Type III
Type IV

Answer 33

type 3

SLE is considered a Type III hypersensitivity in which immune complexes are formed and deposisted throughout the body, provoking further immune reactivity. Type I hypersensitivity is an allergic type reaction and can lead to anaphylaxis. Lupus does not involve an allergy. In Type II, antibodies are produce and bind to the surface of organs (like Goodpastures). This would not create the wide variety of symptoms seen in SLE (circulating immune complexes do). Type IV is often called quot;delayed type sensitivityquot; and involves T Cell activation. While it can be involved in some autoimmune conditions (i.e. Hashimoto’s Thyroiditis), this is not the pathophysiology of SLE.

Question 34

Which of the following vasculitis diagnoses involves small blood vessels?

Takayasu’s arteritis
Polyarteritis Nodosa
Kawasaki’s disease
Churg-Strauss

Answer 34

churg-strauss

Small vessel arteritis includes both Churg-Strauss and Wegener’s granulomatosis with polyangiitis. Churg-Strauss is characterized by: asthma, eosinophilia, multiorgan involvement and neuropathy (foot drop). Serum positive for p-ANCA’s. /p Wegener’s is a NECROTIZING GRANULOMATOUS disease characterized by:olliNECROTIZING GRANULOMAS in the lung and upper airway,/liliFocal NECROTIZING glomerular nephropathy/liliNECROTIZING GRANULOMATOUS vasculitis /li/ol/p Patients often have resulting hemoptysis and hematuria. Serum is positive for c-ANCA’s (You can remember that neCrotizing has a C, or that your NECk hurts from the Chuck WEGon)./p

Question 35

Shortly after birth, an individual naturally makes anti-A antibodies and Rh(D) proteins. Which of the following is a possible genotype for this person?

AO, Rh dd
AA, Rh Dd
AB, Rh Dd
BB, Rh Dd

Answer 35

BB, Rh Dd

If they make A antibodies they are type B, so the answer would be either BO or BB. If they make RhD they are either Dd or DD. The key to this question is shortly after birth and naturally. RhD proteins are not ubiquitous in nature, so if this person is making these, it is not because they have been exposed to the antigen and are making protein antibodies against, but that there body naturally makes these proteins making their bloodtype B positive.

Question 36

Which of the following may decrease the effects of warfarin when administered with it in combination?

Aspirin
Rifampin
Antibiotics
Phenytoin

Answer 36

Rifampin

There are many drug-drug interactions with warfarin. Some drugs increase the effects of warfarin and some decrease the effects.<p>Drugs that INCREASE warfarin action:<br></br>Aspirin (inhibits platelets)<br></br>Antibiotics (inhibit vitamin K synthesis by microbes) <br></br>Clofibrate and Phenytoin (displace warfarin from plasma proteins = increase FREE warfarin)<br></br> Cimetadine, Amiodarone, Phenylbutazone (reduce liver metabolism of warfarin) </p><p>Inducers that DECREASE warfarin effects:<br></br>Bartiburates and rifampin (induce liver enzymes)<br></br>Cholestyramine (decrease GI absorption)</p>

Question 37

Which of the following is true regarding SLE?

More often than not, it is fatal
It is more common in males than females
Affected patients can show signs of psychosis
There is no treatment

Answer 37

Affected patients can show signs of psychosis

Psychosis is one of the eleven criteria that can be used to diagnose SLE. These days SLE is RARELY fatal. It is more common in females. Thare are treatments, but there are no cures (cyclophosamids, NSAIDs and corticosteroids are often used as treatment)

Question 38

A patient with a long history of psoriasis presents to your office complaining of stiffening joints. He says the stiffness is not in every joint; in fact he only complains of his left second and third MCP and his right second MCP. How would you describe this type of arthritis?

Monoarticular
Oligoarticular
Multiarticular
Polyarticular

Answer 38

Oligoarticular

Monoarticular=1 joint
Oligoarticular= 2-4 joints
Multiarticular=fictitious term, not used to describe joint involvement
Polyarticular=greater than 4 joints

Question 39

A child presents with petechiae and chronic nosebleeds that started about a month ago. Just before his symptoms began he had a viral infection that you suspect to be the cause of his current symptoms. Which of the following is most likely?

He has chronic immune thrombocytopenia purpura
He should be treated with IVIG to block Fc receptors in the spleen and steroids to deplete autoantibody-producing B-cells.
He will likely get better in 2-6 weeks without treatment
He likely has an underlying autoimmune disease that is causing his symptoms

Answer 39

He will likely get better in 2-6 weeks without treatment

Common in kids/young adults post viral infection. Antibodies to platelets, will resolve without treatment in 2-6 weeks but may cause Petechiae and nosebleeds. Chronic ITP: Adults, usually with other autoimmunities (HIV, lymphoma). Often idiopathic and spontaneous remission is infrequent. Treat with corticosteroids, IVIG (effects in 1-2 days, blocks Fc receptors on spleen), splenectomy (60-70% effective). Steroids dampen proliferation of B cells clones that are making the antibody. Effects seen in 7-10 days.

Question 40

Which of the following is true regarding cartilage?

It is composed almost solely of proteoglycans
It contains no water so is amazingly rigid
It contains no vessels or nerves
It receives its nutrients directly from the bloodstream

Answer 40

It contains no vessels or nerves

Cartilage does not contain any vessels or nerves and so must get its nutrients from synovial fluid in the synovium. This is why osteoarthritis is not initially painful! Cartilage is composed primarily of collagen, but is also composed of some proteoglycans. Besides the collagen, cartilage is almost all water which in combination with collagen and proteoglycans forms a turbid gel.

Question 41

A protein expressed on normal fetal cells is being expressed abnormally by a tumor cell. This antigen is called a:

A tumor specific antigen (TSA)
A tumor rejection factor (TRF)
Tumor associated antigen (TAA)
Oncofetal antigen

Answer 41

Oncofetal antigen

Oncofetal antigens = normal in fetus, abnormal in tumor. CEA = carcinoembryonic antigen in blood of pts with colon carcinomas. Lots of false positives, smoking increases false positives. Only use if you have a high suspicion of cancer. /p TSA = tumor specific, not the same person-to-person TRF = a TAA that is recognized by host immune response Differentiation antigen = specific to a cell line (EGFR-2 and HER 2 in breast cancer, PSA in colon cancer) Clonal antigens = unique to malignant clone (B and T cell malignancies)/p

Question 42

Uric acid is a product of:

Purine metabolism
Pyrimidine metabolism
Uricase reaction
Precipitation of Calcium to pyrophosphate

Answer 42

Purine metabolism

Uric acid is product of purine metabolism Humans lack uricase enzyme that makes uric acid more soluble (due to 2 non-sense mutations)/p Increased production of uric acid can result from:olliHGPRT deficiency. Complete HGPRT deficiency causes Lesch-Nyhan Syndrome which involves intellectual disability, self-mutilation and chronic gouty attacks./liliPRPP deficiency (this enzyme is part of the metabolic pathway that breaksdown purines) /p Synthase overactivity/li/ol/p

Question 43

Which marker is used to determine TOTAL number of T cells?

CD3
CD4
CD20
CD8

Answer 43

CD3

CD3 is used because it is present on all T cells. CD4 is specific for helper T cells CD8 is specific for CTL’s CD19 and CD 20 are B cell markers/p

Question 44

While inspecting a 45-year-old woman’s hands who complains of AM stiffness, you notice her MCP joints are enlarged with effusion and exhibit slight warmth to the touch. Which of the following is the correct term to describe this inflammation?

Synovitis
Articulitis
Rheumatitis
Dermatitis

Answer 44

Synovitis

The synovium is a thin layer of cells and a capsule that surround joints. There are different cells that make up the synovium (macrophages and fibroblasts). These cells can become angry and make the whole synovium inflamed. This is called SYNOVITIS. Articulitis and rheumatics are fictitious terms and dermatitis is seen when skin becomes inflamed.

Question 45

A person with Hemophilia B will have a deficiency in:

factor IX, part of the intrinsic pathway
factor IX, part of the extrinsic pathway
factor VIII, part of the intrinsic pathway
factor VIII, part of the extrinsic pathway

Answer 45

factor IX, part of the intrinsic pathway

Hemophilia B = factor IX deficiency (part of intrinsic pathway)<p>Both Hemophilia A (factor VIII deficiency) and B (factor IX deficiency) will have an increased PTT. Both are part of the intrinsic pathway.</p><p> Common presentation includes macrohemmorrhage, hemarthrosis (bleeding into joints), easy bruising, and increased PTT.</p><p><strong>NOTE:</strong> PT is not affected in Hemophilia A or B. Recall that PT tests I, II, V, VII, X of the extrinsic pathway whereas PTT tests ALL factors except VII and XIII</p>

Question 46

Natural killer cells:

Have T and B cell receptors
Are activated by MHC class I binding
Have receptors for IgG Fc and for DAMPS
Require previous exposure to antigens in order to become active

Answer 46

Have receptors for IgG Fc and for DAMPS

NK cells = Fc IgG receptors and DAMP recognition receptors. No previous exposure required. Kill cells that have DAMPS, have been bound by IgG, or lack MHC class I 5% of all lymphocytes Large Granulocytic Lymphocytes (LGL’s)br/ Kill tumors and virusesbr/ Do not have B or T receptors./p

Question 47

BCG is injected directly into a tumor. The tumor regresses due to:

Illicit help
Delayed T cell hypersensitivity
Increased RES activity
Invoking ADCC

Answer 47

Delayed T cell hypersensitivity

Innocent bystander destruction of tumor. REACTION IS AGAINST BCG NOT TUMOR CELLS OR TUMOR ANTIGENS. DTH

Question 48

Anklosing spondylitis can be characterized by which of the following?

Inflammatory back pain lasting >3 months
Absence of morning stiffness
Improvement of pain with rest
Neurologic sequelae

Answer 48

Inflammatory back pain lasting >3 months

Inflammatory back pain for 3 mo, prolonged morning stiffness (30-60 mintues), and pain that improves with exercise. No neurologic sequellae HLA-B27 is seen in 90% of patients, 60% concordance in monozygotic twins /p Other features of AS include:ulliSI tenderness, global loss of spinal range of motion, back deformities and reduced chest expansion in late disease./lili25% will experience peripheral arthritis, usually hips and shoulders/liliAffects synchondroses (cartilagenous union with bone)/liliIncreased ESR, negative RF, negative ANA/lili100% have bony fusion/erosion of SI joint by age 45

Question 49

Which of the following steps in primary hemostats happens first?

GPIIb-IIIa increases its affinity fo von Willebrand Factor
Collagen, laminin and fibronectin interact with Beta1 integrins on the platelet surface
von Willebrand Factor binds to exposed subendothelium
Platelets contact exposed subendothelium and bind to von Willebrand Factor

Answer 49

von Willebrand Factor binds to exposed subendothelium

First, vWF binds to exposed, damaged subendothelium. Then circulating platelets bind to vWF via their surface glycoprotein, GP1b. The platelets are then exposed to thrombin, ADP, epinephrine, and thromboxane A2, which makes platelet integrin GPIIB-IIIa increase its affinity for vWF, leading to tighter binding. Numerous ligands in the subendothelium, such as collagen, laminin and fibronectin also interact with the Beta1 integrins on the platelet surface. All of these interactions lead to firm adherence of platelets to the subendothelium surface.

Question 50

As a hematologist, a patient is referred to you from their rheumatologist. In the physician’s note it explains that this patient suffers from osteoarthritis of their right knee and often has the fluid drained from it. The last two aspirations had more blood in the synovial fluid than would be expected from a typical aspiration. The rheumatologist wants you to work this patient up for a coagulopathy. From this information, you suspect a defect in secondary hemostasis. What else would you look for that would help you confirm that this was a secondary hemostasis problem, as opposed to a primary one?

Nose bleeds
Menorrhagia
Muscle Bleeds
petechiae

Answer 50

Muscle Bleeds

Secondary hemostasis coagulopathies are associated with soft tissue, joint and deep bleeding while primary hemostasis is associated with mucocutaneous bleeds (bruising, petechia, epistaxis, menorrhagia, prolonged oozing after tooth extraction, increased bleeding with aspirin use, etc.).

Question 51

Which of the following is the correct mechanism of most NSAIDs?

inhibit cyclooxygenase
inhibit dihydrofolate reductase
inhibit dihydroorate dehydrogenase
inhibit metalloproteinases

Answer 51

Most NSAID’s are cyclooxygenase inhibitors.

Question 52

A 35 year old female presents with fever, night sweats, muscle weakness and ocular disturbances. She denies headache or jaw pain but does point out that there is a newly formed nodule on her right elbow. On physical exam you notice weak pulses in the upper extremities. After ruling out other infectious causes of her symptoms, which of the following diagnoses is most likely?

Giant Cell arteritis
Takayasu’s arteritis
Polyarteritis nodosa
Kawasaki’s disease

Answer 52

Takayasu’s arteritis

Takayasu’s arteritis = quot;pulseless disease,quot; large vessels, most commonly female asian patients under 40. Fever, arthritis, night sweats, myalgia, skin nodules, ocular disturbances, weak pulses (FAN my SKIN on Wednesdays). Affects aortic arch and its branches. Claudication of upper lower extremities

Question 53

Using polarizing microscopy, you‘ re examining a sample of fresh synovial fluid in a patient you suspect has gout. The intracellular crystals in PMN‘s are needle-shaped, negatively birefringent, and yellow when parallel to the axis of the red compensator. What do you conclude?

These are MSU crystals and thus the patient most likely has gout.
These are CPDD crystals and thus the patient most likely has pseudogout
Therse are MSU crystals and thus the patient most likely has pseudogout
These are CPDD crystals and thus the patient most likely has gout

Answer 53

These are MSU crystals and thus the patient most likely has gout.
These are MSU crystals and the patient most likely has gout. MSU crystals are associated with gout, while CPDD crystals are associated with psuedogout. When the polarizer is parallel, the MSU crystals (needle-shaped) appear yellow, and the CPDD crystals (rhomboid-shaped) appear blue. When the polarizer is perpendicular to the crystals, the effect is opposite: the MSU needle-shaped crystals are blue, and the CPDD rhomboid crystals are yellow.

Question 54

In order to confirm your diagnosis, you order an X-ray of the aforementioned basket weaver’s hand. Which of the following are you most likely to see if osteoarthritis is the correct diagnosis?

Marginal erosion
Loss of cartilage space
Pencil and cup deformity
Interosseous tophi

Answer 54

Loss of cartilage space

Loss of cartilage space is a classic sign of OA because the articular cartilage is destroyed in OA. Cartilage is the quot;paddingquot; that under normal circumstances, holds the joint apart. Marginal erosion is seen in RA. Pencil and cup deformity is a sign of psoriatic arthritis. Interosseous tophi are a typical radiographic sign of gout.

Question 55

You suspect vWD in a patient. Based on his lab results, which of the following is the most likely?

Bleeding time: 10 minutes

Factor VIII level: 55%

type 1
type 2
type 3
these lab values are not consistent with vWF

Answer 55

type 1

Type 1: 30% activity, 40% antigen, 50% factor VIII, bleeding time 7-15 min
Type 2: 30% activity, 60% antigen, 70% factor VIII, bleeding time 10-30 min, no large multimers
Type 3: 30min
Factor VIII deficiencies are usually acquired, prolonged PTT that doesn’t correct after 2 hours if mixed with normal plasma.

Question 56

Thromboxane A2 is synthesized during platelet adherence due to which process?

Increase intracellular calcium
externalization of phosphatidyl serine to platelet surface
platelet shape change and granule release
phospholipase A2 liberating arachidonic acid

Answer 56

phospholipase A2 liberating arachidonic acid

phospholipase A2 liberates arachidonic acid (triggered by platelet adherence) leading to thromboxane A2 synthesis.

Question 57

Biological Response Modifiers are a loose class of drugs that primarily target:

cytokines and their receptors
neutrophils
folic acid enzymes
adhesion molecules on endothelial cells

Answer 57

cytokines and their receptors

Question 58

An individual tests seropositive for HIV antibodies and has a CD4 count of 450/ul. This individual:

Lacks CCR5 receptor and is immune to HIV
Meets the criteria for AIDS diagnosis
Would meet the criteria of AIDS diagnosis if they develop Kaposi’s sarcoma
Has an HLA B57 phenotype and more effective CTL defenses against HIV

Answer 58

Would meet the criteria of AIDS diagnosis if they develop Kaposi’s sarcoma

Seropositive for HIV = HIV antibody AIDS diagnosis requires HIV antibody + opportunistic infection/kaposi’s sarcoma OR a CD4 count of less than 200. After HIV infection, it takes an average of 9 years to develop AIDS. /p HIV-1 is the AIDS virus, which is a non-transforming retrovirus (carries no oncogene)./p

Question 59

After a transplant, which of the following will hasten the recovery of the recipient?

G-CSF and GM-CSF
cyclosporine A
tacolimus
methotrexate

Answer 59

G-CSF and GM-CSF

G-CSF and GM-CSF will hasten recovery, while cyclosporine A, tacrolimus, glucocorticoid and methotrexate will maintain immunosupression

Question 60

A 50-year-old man is in the ER after suffering from a pulmonary embolism. You missed the beginning of his care, but your attending orders you to immediately administer protamine sulfate. From this information you know that man was initially given:

Warfarin and he is hemorrhaging
Heparin and he is has developed heparin-induced thrombocytopenia syndrome
A fibrinolytic agent and he is having an allergic reaction
Heparin and he is hemorrhaging

Answer 60

Heparin and he is hemorrhaging

Heparin can have a side effect of excessive bleeding. If life-threatening hemorrhage occurs, the effect of heparin can be reversed with protamine sulfate, a positively charged compound that neutralizes heparin.

Question 61

A patient presents with proximal muscle weakness that impairs her ability to lift her arms over her head. On physical exam you notice a rash over her cheeks, orbital surfaces, and distal interphalangeal joints bilaterally. Labs show elevated creatinine kinase, elevated aldolase and a positive ANA and anti-jo 1. Which of the following is considered first line treatment for this patient?

Monoclonal antibodies
Prednisone
Antibiotics
Methotrexate

Answer 61

Prednisone

Prednisone is first line treatment for dermatomyositis. Dermatomyositis is characterized by:ulliProximal muscle weakness, most commonly affecting the shoulders/liliMalar, heliotropic rashes and Gottron’s papules over the DIP’s (quot;shawl and facequot; or quot;mechanic’s handsquot;)./liliIncreased risk of malignancy/liliMuscle biopsy will show perifascicular atrophy (diagnostic)/li/ul/p Labs: Elevated CK and aldolase, positive ANA and anti-jo 1 Treatment: Steroids/p

Question 62

You have a patient with gout and you‘re trying to decide if the hyperuricemia is the result of increased production or decreased renal excretion of urate. The patient‘s 24hr urinary excretion of uric acid is

Answer 62

This patient is an underexcretor, like the majority of patients with gout. The majority (90%) of patients with primary gout are underexcretors of uric acid.

Question 63

The laboratory production of monoclonal antibodies won a Nobel prize for the scientist who created a method to generate hybridomas using what reagent(s)?

Lipopolysaccharide
IL-4 and an anti-CD40 immunoglobulin
A myeloma clone
BAFF

Answer 63

A myeloma clone

A monoclonal antibody is derived from the progeny of a single B cell, that has been fused with a multiple myeloma tumor cell so that it can grow forever in culture like its tumor parent, but make only the specific antibody of its B cell parent.

Question 64

A person with HIV immunosuppression is likely to have the strongest defense against:

Fungi
Intracellular bacteria
Parasites
Extracellular pathogens

Answer 64

Extracellular pathogens

They still have antibodies that work just fine but anything that is T-cell mediated will be compromised. Opportunistic intracellular bacteria, protozoa, viral infection, TB, mycobacterium avium. /p Kaposi’s Sarcoma = tumor of endothelial cells lining lymphatics (more common in homosexual males with AIDS), caused by KSHV (kaposi’s sarcoma herpes virus) or HHV8 (human herpesvirus 8)/p

Question 65

Platelets support thrombin generation by:

providing a phospholipid surface for the coagulation cascade to take place
secreting a heparin-like molecule
expressing thrombomodulin which binds to thrombin and activates proteins C and S
secreting nitric oxide and prostacyclin

Answer 65

providing a phospholipid surface for the coagulation cascade to take place

Endothelial cells on intact vessels PREVENT coagulation by: 1. secreting heparin-like molecule. 2. expressing thrombomodulin which binds thrombin and activates proteins C and S. 3. secretion of nitric oxide and prostacyclin which inhibit platelet activation

Question 66

Of the following pro-inflammatory cytokines, which is predominantly involved in the systemic symptoms of RA?

IL1
IL6
IL17
TNFa

Answer 66

IL6

IL6 is involved in systemic symptoms (anorexia, fatigue) IL1 and TNFa = local inflammation (release of PGE2, induction of collagenase, neutral proteinase production) /p IL1, IL17 TNFa = cartilage and bone destruction, stimulate osteoclast release/activity/p

Question 67

Platelet glycoprotein VI binds to:

Integrins
protein C
von willebrand factor
collagen

Answer 67

Glycoprotein VI interacts with collagen in the vessel wall

Question 68

Which of the following would you expect to find in a patient with Von Willebrand’s disease?

non-responsiveness to desmopressin
elevated PT
elevated factor VIII
abnormal platelet-to-collagen adhesion

Answer 68

abnormal platelet-to-collagen adhesion

Abnormal platelet-collagen adhesion. Decreased factor VIII carrying and half life. Treat with Desmopressin (DDAVP) which will release more vWF from stored endothelium.

Question 69

Which of the following is measured using a capture enzyme-linked immunosorbency assay?

Patient’s antibodies
Monovalent agents
Anitgens with at least TWO different epitopes
Immune complexes

Answer 69

Anitgens with at least TWO different epitopes

Capture ELISA = sandwich. Tells you about the presence of divalent antigens (must be at least divalent. won’t detect antigens that are too small) olliPlate with antibodies to one of the antigen epitopes/liliAdd patient serum containing antigen of interest/liliAdd antibody to a different epitope (contains enzyme)/liliadd a color indicator/lilicolor intensity will tell you the amount of antigen present in patient’s serum/li/ol

Question 70

You want to make a monoclonal antibody against HPV using a B cell and a multiple myeloma cell. Which of the following is the correct way to do this?

Obtain a mouse antibody against HPV and combine it with a multiple myeloma tumor to create a hybrid
Culture a B-cell that is genetically determined to make HPV antibodies and combine it with a multiple myeloma tumor to create a hybrid
Culture a multiple myeloma tumor cell and inject HPV antibodies into it
Obtain DNA from a multiple myleoma cell, inject the DNA into a B cell and stimulate its proliferation

Answer 70

Culture a B-cell that is genetically determined to make HPV antibodies and combine it with a multiple myeloma tumor to create a hybrid

B-cell provides the specific antibodies, tumor cell provides immortality. Creat a hybrid that does NOT express the tumor cell Ab’s and has immortal properties.

Question 71

Which of the following correctly describes the mechanism of Heparin?

Potentiates the action of antithrombin
Inhibits Platelet Adhesion
Increases Plasmin
Disrupts hemostasis

Answer 71

Potentiates the action of antithrombin

Heparin and oral anticoagulants interfere with the coagulation cascade and prevents formation of thrombin, which converts fibrinogen to fibrin.

Question 72

Diffuse calcification of a ligament or tendon at a site of bone insertion is called a:

Osteophyte
Syndesmophyte
Ankylosis
Spondylitis

Answer 72

Syndesmophyte

Syndesmophyte = diffuse calcification of ligament/tendon at site of bone insertion.
Osteophyte = bone outgrowth
ankylosis = fixation of joint due to disease
Axial arthropathy = involves spine
sacroiliitis = inflammation of SI
joint spondylitis = inflammation of vertebrae/p

Question 73

You are interested in evaluating a patient’s B-cell function after a 2 year history of monthly bacterial infections. Which of the following tests should be used?

Serum protein electropheresis
Tuberculin skin test
Patch test
Precipitin test

Answer 73

Serum protein electropheresis

Serum Protein Electrophoresis is used to test B cell function by looking at relative levels of antibody classes.

Question 74

On an overnight shift in the ER, a 35 year old man comes in 10 days after being released from the hospital for spinal surgery. He is complaining that is right leg is incredibly painful and is turning blue in color. Because of his recent surgical history, which of the following physical signs would be MOST consistent with the suspected diagnosis?

skin on his right leg is cool to the touch
loss of reflexes in the right leg
3+ pitting edema in the right leg
increased hair growth on the right leg

Answer 74

3+ pitting edema in the right leg

Venous obstruction can produce pain, pitting edema of the extremity, and a warm, dusky, reddish-blue discoloration of the skin.

Question 75

Polymyositis is mediated by which type of cells?

CD4
CD8
Macrophages
Neutrophils

Answer 75

CD8

Auto-antigen is presented in association with MHC-I molecules on the surface of myocytes and is recognized by CD8 cytotoxic T cells that subsequently initiate destruction.

Question 76

Patients who are homozygous for HGPRT deficiency typically

Have severe gout with more frequent attacks
Are incompatible with life
Are hypouricemic
Have Lesch-Nyhan syndrome

Answer 76

Homozygous HGRPT deficiency causes Lesch-Nyhan syndrome

Question 77

You are working a shift in the ER, when a patient is brought in who has lost unsafe amounts of blood. After blood typing him, you find out he is O+. Which type of blood do you order for his transfusion?

A-
B-
AB
O-

Answer 77

O-

This patient will have antibodies already to blood groups A and B (these are called ISOHEMAGGLUTININS and are naturally occurring), so A, B, and AB will be rejected by this patient. Out of the options listed here, the only safe type of blood would be his exact match.

Question 78

Why does the CD4/CD8 ratio decline in HIV patients?

CTL’s kill infected CD4’s
Infected CD4’s are induced to apoptose
Activated Th’s replicate viral DNA and when the virus buds cells lyse
Infected CD8’s are triggered to proliferate

Answer 78

Activated Th’s replicate viral DNA and when the virus buds cells lyse

Viral budding causes CD4 cell lysis 200 CD4 per uL blood = AIDS/p

Question 79

A mom brings her 6 year old child to the clinic because of an acute fever, rash and swelling of the hands and feet. The most likely diagnosis involves which size blood vessels?

Small
Medium
Large
This doesn’t sound like vasculitis

Answer 79

Medium

Kawasaki’s disease is a self-limiting necrotizing vasculitis seen in infants/children. Affects medium sized vessels Acute fever, rash, mucocutaneous changes, edema of hands/feet. Common in asians. Probably a post-infectious cytokine mediated response./p

Question 80

Which of the following treatments are indicated for osteoarthritis and have proven efficacy?

Methotrexate
Ginger extract
Intra-articular corticosteroids
Acupuncture

Answer 80

Intra-articular corticosteroids

Intra-articular corticosteroids are often used in the treatment of OA with proven benefit. Although ginger extract and acupuncture are alternative forms of therapy for osteoarthritis, research has not proven benefit. Methrotrexate is a DMARD that is used in RA and other auto-immune illnesses./p

Question 81

Which of the following autoantibodies is LEAST helpful for making a diagnosis of connective tissue disease?

Anti-nuclear antibody (ANA)
Anti-double stranded DNA (anti-ds-DNA)
Anti-nuclear ribonucleoprotein (snRNP)
Anti-Smith (anti-SM)

Answer 81

Anti-nuclear antibody (ANA)

The ANA test is nonspecific and just shows that the patient is making autoantibodies to nuclear components. It is elevated in a portion of normal people. Anti-ds-DNA, snRNP, and anti-SM are more useful because these antigens are more characteristic of some diseases. Anti-ds-DNA ad anti-SM are often elevated in SLE. snRNP antibodies can be seen in mixed connective tissue disease./p

Question 82

Which of the following is true of DMARDs?

They are usually just used for symptom control
They are moderately toxic, and should only be used in late stage disease
Although effective for rheumatoid arthritis, are not effective for other autoimmune diseases
They can prevent the progression of autoimmune diseases

Answer 82

They can prevent the progression of autoimmune diseases

Although once thought to be toxic and only used in late stage disease, DMARDs are now a widely accepted form of treatment for not only RA, but also other autoimmune diseases, like SLE. They are thought to actually prevent the progression of the disease so it is important to use them early on in the disease process.