5/26 - UWorld

Question 1

What is CREST syndrome

Answer 1

· Calcinosis / anti-centromere antibody

· Raynoud

· Esophageal dysmotility

· Sclerodactyly (tightening of skin with loss of wrinkles)

· Telangiectasias

Question 2

Cause and presentation of Hyper-IgE Syndrome (aka Job)

Answer 2

o Deficiency in IFN-y lead to impaired neutrophil recruitment

o Presentation - FATED

§ F – coarse Facies

§ A – Abscesses

§ T – retained primary Teeth

§ E – increased IgE (all other immunoglobulins are normal)

§ D – dermatologic problems (eczema)

Question 3

Release and function of TNF-a

Answer 3

§ Secreted by activated macrophages

§ Mediates septic shock

§ Causes neutrophil and lymphocyte recruitment

§ Responsible for fever, anorexia, corticotrophin releasing hormone, septic shock, and cachexia

Question 4

Infliximab

Answer 4

• TNF-a inhibitor
  
  • Recall: TNF-a is an acute phase reactant produced by activated macrophages to mediate inflammation by accelerating neutrophil migration, and facilitate lymphocyte proliferation; TNF-a is responsible for fever, anorexia, corticotropin releasing hormone, septic shot, and cachexia
• Mechanism of action:
  
  • Is a monoclonal antibody to TNF-a
• Uses:
  
  • Used for autoimmune conditions due to anti-inflammatory effect
  • Rheumatoid arthritis, psoriasis, ankylosing spondylitis

Question 5

Cyclosporine

Answer 5

• Immunosuppressant
  
  • Blocks lymphocyte activation and proliferation
• MOA:
  
  • Binds cyclophilin (protein within cytosol of T-cells)
  • Inhibits calcineurin (which stimulates IL-2) = prevention of IL-2 transcription
• Uses:
  
  • Transplant rejection
  • Psoriasis
  • Rheumatoid arthritis
• Toxicity
  
  • Nephrotoxicity

Question 6

Tacrolimus

Answer 6

• Immunosuppressant
  
  • Blocks lymphocyte activation and proliferation
• MOA:
  
  • Binds FK506 binding protein
  • Inhibits calcineurin à prevention of IL-2 transcription
• Uses:
  
  • Transplant rejection prophylaxis
• Toxicity:
  
  • Nephrotoxicity

Question 7

Sirolumus (aka Rapamycin)

Answer 7

• Immunosuppressant
  
  • Blocks lymphocyte activation and proliferation
• MOA:
  
  • Binds FKBp12 = inhibition of mTOR
  • Prevents response to IL-2

Question 8

Dacluzimab

Answer 8

• Immunosuppressant
  
  • Blocks lymphocyte activation and proliferation
• MOA:
  
  • Binds CD25 (IL-2 receptor) = preventing response to IL-2

Question 9

Describe Wiskott Aldrich syndrome

Answer 9

• X-linked recessive immunodeficiency
  
  • Mutation in WASp gene
  • T cells unable to reorganize actin skeleton = defective antigen presentation
• WATER - Wiskott Aldrich, Thrombocytopenia, Eczema (especially truncal), Recurrent infections

Question 10

Antibody associated with mixed connective tissue disease

Answer 10

Anti-U1 RNP (ribonucleoprotein)

Question 11

What are the different defects that cause homonymous hemianopia with macular involvment vs homonymous hemianopia with macular sparing

Answer 11

• E: homonymous hemianopia with macular involvement is consistent with involvement of the temporal lobe (e.g. occlusion of the middle cerebral artery)
• H: homonymous hemianopia with macular sparing is consistent with occlusion of the posterior cerebral

Question 12

When after fertilization will b-hCG become positive in maternal serum and urine?

Answer 12

• ~8 days after fertilization in maternal serum
• ~14 days after fertilization in urine

Question 13

What are the muscles of the rotator cuff?

Answer 13

Supraspinatus

Infraspinatus

Teres minor

Subscapularis

Question 14

What is the supraspinatus test?

Answer 14

“empty can” test

Question 15

What murmur might radiate to the neck

Answer 15

Aortic stenosis

Turbulent blood pushed out of the aorta may still be turbulent when it reaches the carotids

Question 16

What murmur is associated with an opening snap vs. an ejection click

Answer 16

Opening snap = mitral stenosis

Ejection click = Aortic stenosis

Question 17

Common side effects of Chloramphenicol

Answer 17

Pancytopenia: anemia, leukopenia, and/or thrombocytopenia

Question 18

What are the parasites that can cause disease in red blood cells

Answer 18

Plasmodium (malaria) and Babesia

Question 19

What is the most common location of patients infected with Babesia

Answer 19

NE United States (NE on maltese cross on floor)

Question 20

Presentation of babesiosis

Answer 20

Fever and hemolytic anemia (can result in jaundice)

Question 22

Blood smear of Babesiosus

Answer 22

Ring form or Maltese cross

Question 23

Clinical manifestation of carcinoid syndrome

Answer 23

Bronchospasm, Flushing, Diarrhea, R-sided heart disease/murmur

Question 24

Describe the mechanism behind glucose-induced insulin release

Answer 24

• Glucose enter beta cells of pancreas through GLUT-2 transporters
• Glucose is metabolize by glucokinase to glucose-6-phosphate
• Glucose-6-phosphate is further metabolized by glycolysis and the Krebs cycle to produce ATP
• High ATP leads to closure of ATP-sensitive potassium channels
• Closure of potassium channels leads to depolarization which results in opening of voltage-gated calcium channels (= inflow of calcium)
• High intracellular calcium causes exocytosis of insulin vesicles

Question 25

Where is the inflammation and with what T-cell (CD4+ or CD8+) is inflammation caused by in Dermatomyositis vs. Polymyositis

Answer 25

* Dermatomyositis * Perimysial (THINK: periphery b/c of muscle + skin involvement) inflammation with CD4+ T cells * Polymyositis * Endomysial inflammation with CD8+ T cells

Question 26

What are Class I vs Class II human leukocyte antigens

Answer 26

Class I: HLA-A, HLA-B, and HLA-C Class II: HLA-DP, HLA-DQ, and HLA-DR

Question 27

What are the seronegative spondyloarthopathies

Answer 27

PAIR: Psoriatic arthritis, ankylosing spondylitis, inflammatory bowel disease, reactive arthritis

Question 28

What human leukocyte antigen is associated with the seronegative spondyloarthropaties

Answer 28

HLA-B27

Question 29

What human leukocyte antigen is associated with Rheumatoid arthritis

Answer 29

HLA-DR4

Question 30

What is the procedure performed in a vasectomy?

Answer 30

Transection of the vas deferens Viable sperm can remain in the portion of the vas deferens distal to ligation for 3 months and at least 20 ejaculations

Question 31

Occlusion of the L circumflex artery will affect what part of the heart, which corresponds to ST elevations in what leads?

Answer 31

L circumflex artery provides to the lateral aspect of L ventricle Will see ST elevations in leads I and aVL

Question 32

Describe the presentation of the 3 phases of Lyme disease

Answer 32

* o Stage I * Erythema Chronic Migrans ("Bulls Eye" rash) = “Stage 1” with bulls eye with spiral arrow (spirochete) * Non-painful and non-pruritic * Sweating and feverish, flu like illness = Sir Wright is sweating and looking feverish - * Stage II * Heart block caused by myocarditis = heart shield * Bilateral facial nerve palsy = 2 bells for Bell’s Palsy * o Stage III * Joint pain arthritis of large joints, symptoms may move from joint to joint (migratory polyarthritis) à * Arrow in knee = arthritis of large joint * Straw man swinging back and forth = migratory polyarthritis * Subtle encephalopathy = Memory difficulty, lymphocytic meningitis. * Arrow in the head of straw man and Sir Giemsa is confused

Question 33

Treatment of Lyme disease

Answer 33

Stage 1 = Doxycycline Later stages = Ceftriaxone

Question 34

Cause of acute primary adrenal insufficiency

Answer 34

Waterhouse-Friederichsen syndrome Acute primary adrenal insufficiency due to adrenal hemorrhage associated with septicemia (usually Neisseria meningitides), DIC, endotoxic shock

Question 35

Which hormones use the cAMP pathway (Gs and Gi)

Answer 35

Most hormones of anterior pituitary FSH, LH, ACTH, TSH, hCG, MSH, GHRH, CRH, PTH, calcitonin, glucagon, V2 vasopressin receptor * THINK: FLAT ChAMP * FSH, LH, ACTH, TSH, CRH and calcitonin, hCG, ADH (V2), MSH, PTH

Question 36

Which hormones use IP3 pathway (Gq)

Answer 36

Hormones of posterior pituitary minus V2 GnRH, TRH, oxytocin, V1 vasopressin receptor, H1 histamine receptor, angiotensin II, gastrin * THINK: GOAT HAG * GnRH, Oxytocin, ADH (V1), TRH, Histamine (H1), Angiotensin II, Gastrin

Question 37

Which hormones use cGMP pathway

Answer 37

Vasodilators: Nitric oxide (NO), Atrial natriuretic peptide (ANP)

Question 38

Which hormones use steroid receptors

Answer 38

Estrogens, progesterone, testosterone, glucocorticoids, aldosterone, thyroid hormone (T3/T4), vitamin D * THINK: VETTT CAP * Vitamin D, Estrogen, Testosterone, T3, T4, Cortisol, Aldosterone, Progesterone

Question 39

Which hormones use tyrosine kinase receptors

Answer 39

Insulin, insulin-like growth factor (IGF-1), platelet derived growth factor (PDGF), fibroblast growth factor (FGF)

Question 40

Which hormones use nonreceptor tyrosine kinase (e.g. JAK/STAT)

Answer 40

Prolactin, cytokines (IL-2, IL-6, IFN), GH, G-CSF, Erythropoietin, Thrombopoeitin * THINK: PIGGLET * Prolactin, Immunomodulators (cytokines, IL-2, IFN), Growth hormone, Granulocyte colony stimulating factor, (No L), EPO, Thrombopoietin

Question 41

MOA of Psyllium

Answer 41

* Bulk-forming laxative * Is an indigestible hydrophilic colloid = will absorb water = distention = peristalsis * Used for treatment of constipation * May lead to increased bloating and flatulence

Question 42

MOA of Docusate

Answer 42

* Stool softener * Facilitates the penetration of stool by water and lipids

Question 43

MOA of Senna

Answer 43

* Stimulant laxative * Directly stimulate the enteric nervous system and colonic secretions * Chronic use causes melanosis coli (brown pigmentation of the colon)

Question 44

MOA of Bismuth

Answer 44

MOA: Binds to ulcer base, providing physical protection and allowing HCO3- secretion to reestablish pH gradient in the mucus layer USES: Increased ulcer healing Traveler's diarrhea

Question 45

What are the rapid-acting, intermediate-acting, and long-acting insulins

Answer 45

* Rapid acting = Girls and Lads * Glulisine, Aspart, Lispro * Intermediate acting = Rest Now * Regular insulin, NPH * Long acting = Don't Go * Detemir, Glargine

Question 47

What type of drug are Tolbutamide and Chlorpropramide?

Answer 47

1st generatino sulfonylureas ("-amide" = goose in maid outfit) Treat T2DM

Question 48

MOA of Exanatide and Liraglutide

Answer 48

"-tide" = GLP-1 agonists * These drugs activate GLP-1 receptor (glucagon-like peptide receptor) – opposite of glucagon * Increases insulin release and satiety * Decreases glucagon release and gastric emptying

Question 49

MOA of Sitagliptin and Linagliptin

Answer 49

"-gliptin" = DDP-4 inhibitors * DPP-4 (dipeptidyl peptidase) is the enzyme that breaks down endogenous GLP-1 * If you inhibit DPP-4, you will have more endogenous GLP * Increased insulin and decreased glucagon * Increased satiety and delayed gastric emptying

Question 50

MOA of Pramlintide

Answer 50

* Amylin analog * Amylin is a peptide normally present in insulin secretory granules and secreted along with insulin * At pharmacologic doses, it reduces glucagon secretion, gastric emptying, and appetite * Used to control postprandial glucose spike in both Type I and Type II DM

Question 51

MOA of alpha-glucosidase inhibitors (Acarbose and Miglitol)

Answer 51

Sketchy: A-carb wigglers MOA: Inhibition of a-glucosidase on the intestinal brush border decreases the conversion of disaccharides to absorbable monosaccharides --\> delayed carbohydrate absorption USES: Type 2 DM ADVERSE EFFECTS: Diarrhea, flatulence (due to fermentation of undigested carbs)

Question 52

MOA of SGLT2 inhibitors ("-flozin")

Answer 52

Sketchy: Flossing teacher and Salty Glucose Co. bag MOA: Inhibition of the sodium-glucose cotransporter in the proximal tubule USES: Type 2 DM ADVERSE EFFECTS: UTI Vaginal candidiasis Dehydration