Qbank Stuff

Question 1

Porphyria Cutanea Tarda

- Blistering cutaneous photosensitivity

Answer 1

E: uroporphynogen decarboxylase
S: uroporphyrin (tea-colored urine)

Question 2

Acute Intermittent Porphyria
- Painful abdomen, port wine-colored urine, polyneuropathy, psychological disturbances, precipitated by drugs/alcohol/starvation

Answer 2

E: porphobilinogen deaminase
S: prophobilinogen, S-ala coporphobilinogen
Rx: glucose and heme (inhibit ALA synthase)

Question 3

Lead Poisoning

• Kids: mental deterioration
• Adults: HA, memory loss, demyelination

Answer 3

E: ferrochelatase, ALA dehydratase
S: protoporphyrin, S-ala
- Microcytic anemia (basophilic stippling)
- GI and kidney disease
- Rx: chelation with EDTA, dimercaprol, succimer

Question 4

Polycythemia Vera

• blurry vision, HA, flushed face, increased risk of venous thrombosis
• Intense itching after hot shower
• Erythromelalgia (severe, burning pain and red-blue coloration)

Answer 4

• Primary polycythemia
• V617 JAK2 mutation (cytoplasmic TK) –> more sensitive to GF
• Responds to aspirin
• plasma volume and RBC mass increased
• EPO decreased

Question 5

Thrombotic Thrombocytopenic Purpura

• Normal PT/PTT, decreased PC, increased BT
• Pentad of neuro and renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia

Answer 5

• Inhibition/deficiency of ADAMTS13 (vWF metalloprotease) –> decreased degradation of vWF multimers –> increaed large vWF multimers –> increased platelet adhesion –> increased platelet aggregation and thrombosis
• Labs: shistocytes, increased LDH
• Rx: plasmapharesis, steroids

Question 6

Cytokines that mediate systemic inflammatory response and stimulate liver to release acute phase proteins?

Answer 6

IL-1, IL-6, TNF-alpha

- Elevate ESR

Question 7

Decreased activity of mesocortical pathway?

- DA

Answer 7

“Negative sx” (flat affect, limited speech)

Question 8

Increased activity of mesolimbic pathway?

- DA

Answer 8

“Positive sx” (delusions, hallucinations)

- Primary target of antipsychotics (schizophrenia)

Question 9

Decreased activity of nigrostriatal pathway?

- DA

Answer 9

Extrapyramidal sx (dystonia, akathisia, parkinsonism, tardive dyskinesia)

• Major DA pathway in brain
• Significantly affected by movement disorders and antipsychotics

Question 10

Decreased activity of tuberoinfundibular pathway?

- DA

Answer 10

Increased prolactin –> decreased libido, sexual dysfunction, galactorrhea

Question 11

Hereditary Hemorrhagic Telangiectasia

- Aka Osler-Weber Rendu Syndrome

Answer 11

• AD inherited disorder of blood vessels

- Telangiectasias, recurrent epistaxis, skin discolorations, AVMs, GI bleeding, hematuria

Question 12

C1 Esterase Inhibitor Deficiency

- Facial swelling, laryngeal edema, GI probs

Answer 12

• Unregulated activation of kallikrein –> increased bradykinin
• Excessive C1 –> C2 and C4
• ACEi contraindicated

Question 13

C3 Deficiency

Answer 13

• Increases risk of severe, recurrent pyogenic sinus and respiratory tract infections
• Increased susceptibility to type III HSN rxns (SLE, PSGN)

Question 14

C5-C9 Deficiency

Answer 14

Terminal complement deficiency –> increased susceptibility to recurrent Neisseria bacteremia

Question 15

DAF (GPI-Anchored Enzyme) Deficiency

Answer 15

Causes complement-mediated lysis of RBCs –> paroxysmal nocturnal hemoglobinuria

Question 16

Vitamin A Deficiency

Answer 16

Squamous metaplasia with keratinization

Question 17

Watershed areas of bowel (prone to ischemia)?

Answer 17

Splenic flexure (between SMA and IMA)
Rectosigmoid junction (between sigmoid a and superior rectal a)