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Flashcards in Qbank Stuff Deck (17)
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1
Q

Porphyria Cutanea Tarda

- Blistering cutaneous photosensitivity

A

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

2
Q

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

A

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

3
Q

Lead Poisoning

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

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

4
Q

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)
A
  • Primary polycythemia
  • V617 JAK2 mutation (cytoplasmic TK) –> more sensitive to GF
  • Responds to aspirin
  • plasma volume and RBC mass increased
  • EPO decreased
5
Q

Thrombotic Thrombocytopenic Purpura

  • Normal PT/PTT, decreased PC, increased BT
  • Pentad of neuro and renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia
A
  • 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
6
Q

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

A

IL-1, IL-6, TNF-alpha

- Elevate ESR

7
Q

Decreased activity of mesocortical pathway?

- DA

A

“Negative sx” (flat affect, limited speech)

8
Q

Increased activity of mesolimbic pathway?

- DA

A

“Positive sx” (delusions, hallucinations)

- Primary target of antipsychotics (schizophrenia)

9
Q

Decreased activity of nigrostriatal pathway?

- DA

A

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

  • Major DA pathway in brain
  • Significantly affected by movement disorders and antipsychotics
10
Q

Decreased activity of tuberoinfundibular pathway?

- DA

A

Increased prolactin –> decreased libido, sexual dysfunction, galactorrhea

11
Q

Hereditary Hemorrhagic Telangiectasia

- Aka Osler-Weber Rendu Syndrome

A
  • AD inherited disorder of blood vessels

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

12
Q

C1 Esterase Inhibitor Deficiency

- Facial swelling, laryngeal edema, GI probs

A
  • Unregulated activation of kallikrein –> increased bradykinin
  • Excessive C1 –> C2 and C4
  • ACEi contraindicated
13
Q

C3 Deficiency

A
  • Increases risk of severe, recurrent pyogenic sinus and respiratory tract infections
  • Increased susceptibility to type III HSN rxns (SLE, PSGN)
14
Q

C5-C9 Deficiency

A

Terminal complement deficiency –> increased susceptibility to recurrent Neisseria bacteremia

15
Q

DAF (GPI-Anchored Enzyme) Deficiency

A

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

16
Q

Vitamin A Deficiency

A

Squamous metaplasia with keratinization

17
Q

Watershed areas of bowel (prone to ischemia)?

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