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Flashcards in Qbank Stuff Deck (17)
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Porphyria Cutanea Tarda
- Blistering cutaneous photosensitivity

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


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

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


Lead Poisoning
- Kids: mental deterioration
- Adults: HA, memory loss, demyelination

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


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)

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


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

- 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


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

IL-1, IL-6, TNF-alpha
- Elevate ESR


Decreased activity of mesocortical pathway?
- DA

"Negative sx" (flat affect, limited speech)


Increased activity of mesolimbic pathway?
- DA

"Positive sx" (delusions, hallucinations)
- Primary target of antipsychotics (schizophrenia)


Decreased activity of nigrostriatal pathway?
- DA

Extrapyramidal sx (dystonia, akathisia, parkinsonism, tardive dyskinesia)
- Major DA pathway in brain
- Significantly affected by movement disorders and antipsychotics


Decreased activity of tuberoinfundibular pathway?
- DA

Increased prolactin --> decreased libido, sexual dysfunction, galactorrhea


Hereditary Hemorrhagic Telangiectasia
- Aka Osler-Weber Rendu Syndrome

- AD inherited disorder of blood vessels
- Telangiectasias, recurrent epistaxis, skin discolorations, AVMs, GI bleeding, hematuria


C1 Esterase Inhibitor Deficiency
- Facial swelling, laryngeal edema, GI probs

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


C3 Deficiency

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


C5-C9 Deficiency

Terminal complement deficiency --> increased susceptibility to recurrent Neisseria bacteremia


DAF (GPI-Anchored Enzyme) Deficiency

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


Vitamin A Deficiency

Squamous metaplasia with keratinization


Watershed areas of bowel (prone to ischemia)?

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