FA Notes Flashcards

(45 cards)

0
Q

Hereditary cause of sideroblastic anemia? Treatment?

A

X linked delta-ALA synthase

B6, pyridoxine

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1
Q

How does lead poisoning leads to anemia?

A

Lead inhibits ferrochelatase and ALA dehydratase decreasing heme synthesis and inc RBC protoporphyrin

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2
Q

What are the effects of LEAD poisoning? Treatment?

A

Lead lines on gingivae and long bone
Encephalopathy and Erythrocyte basophilic stippling (blocked rRNA degradation)
Abdominal colic and sideroblastic Anemia
Drop of wrist or foot, Dimercaprol and EDTA

Kids: Succimer

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4
Q

What causes orotic aciduria? What’s the clinical manifestation? How is it differentiated from ornithine transcarbamylase def?
Treatment?

A

Inability to convert orotic acid to UMP from UMP synthase, causes megaloblastic anemia, no hyperammonemia
Treatment: uridine monophosphate

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5
Q

What does heparin do? How is it reversed?

A

It activates antithrombin, decreasing both thrombin and factor Xa. Reversal by protamine sulfate

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6
Q

What is HIT?

A

Heparin-induced thrombocytopenia, IgG antibodies against heparin bound PF4. The complex activates platelets and causes thrombosis and thrombocytopenia

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7
Q

What do Argatroban, bivalrudin, and dabigatran do? When are they used?

A

Inhibit thrombin directly, used for patients with HIT

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8
Q

What apixaban, rivaroxaban do?

A

Bind directly to Xa

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9
Q

Which anticoagulant is safe in pregnancy?

A

heparin (not warfarin). It does not cross the placenta

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10
Q

What is warfarin’s toxicity? How is it reversed?

A

Skin/tissue necrosis. Reversed by vit K. Rapidly by FFP

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11
Q

What do alteplase, streptokinase do? How is toxicity treated?

A

They are thrombolytics. They aid activation of plasminogen to plasmin causing cleavage of thrombin and fibrin clots. . Treat toxicity with aminocaproic acid (inhibits fibrinolysis)

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12
Q

What does aspirin do? Toxicity?

A

Irreversible inhibition of COX1 and 2, decreases TXA2 and prostaglandins.
-Causes ulcers, tinnitus, acute renal failure, interstitial nephritis, and Reye syndrome in kids with viral illness

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13
Q

What does overdose look like (acid-base)?

A

Hyperventilation and respiratory alkalosis -> mixed metabolic acidosis and respiratory alkalosis

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14
Q

What does clopidogrel do?

A

It inhibits platelet aggregation by blocking ADP receptors, prevents IIb/IIIa expression on platelet

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15
Q

What cilostazol and dipyridamole do?

A

They are phosphodiesterase III inhibitors. By dec cAMP in platelets, they inhibit aggregation. Vasodilators.

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16
Q

What do abciximab, eptifibatide, and tirofiban do?

A

Bind to IIb/IIIa receptor on activated platelets to prevent aggregation

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17
Q

What does vemurafenib do?

A

It inhibits BRAF positive melanoma

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18
Q

What is trastuzumab? Toxicity?

A

It is a monoclonal antibody against HER-2, tyrosine kinase receptor. Cardiotoxicity

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19
Q

What is tamoxifen, raloxifine? Tamoxifen inc risk of which cancer?

A

Selective estrogen receptor modulator (antagonist in breast, agonist in bone). Tamoxifen inc risk of endometrial cancer

20
Q

What does rituximab do? Toxicity?

A

monoclonal antibody against CD20, inc risk of progressive multifocal leukoencephalopathy

21
Q

What is imatinib?

A

Tyrosine kinase inhibitor of BCR-ABL (Philadelphia in CML)

22
Q

What is erlotinib?

A

EGFR tyrosine kinas inhibitor

23
Q

Bevacizumab

A

Monoclonal antibody against VEGF, inhibits angiogenesis

24
Q

What does Cisplastin/carbolatin do?

A

cross-links DNA, can cause nephrotoxicity (prevent with amifostine and chloride diuresis) or ototoxicity

25
What do etoposide and teniposide do?
It inhibits topoisomerase II, causing DNA degredation
26
What does irrnotecan/toptecan do?
inhibits topoisomerase I, preventing DNA winding and replication
27
What does hydroxyurea do?
Inhibits ribonucleotide reductase, decreases DNA synthesis, increases HbF
28
What cancer drugs are microtubule inhibitors?
Paclitaxel, vincristine, vinblastine
29
What does paclitaxel do?
It hyperstabilizes polymerized microtubules in M phase, prevents breakdown of mitotic spindle
30
What does vincristine/vinblastine do? Toxicity?
Binds to Beta-tubulin and inhibits microtuble polymerization (M phase arrest). Neurotoxic (vincristine) and marrow suppression (vinblastine)
31
What drugs are alkylating agents?
Busulfan, cyclophosphamide, nitrosoureas
32
What does busulfan do?
Cross-links DNA
33
What does cyclophosphamide/ifosfamide do? Important toxicity?
Cross-links DNA at guanine N-7, hemorrhagic cystitis
34
What do nitrosoureas do? Toxicity?
Cross blood brain barrier to cross link DNA. Causes CNS toxicity (convulsions, dizziness, ataxia)
35
What does bleomycin do?
Induces free radical formation
36
What does dactinomycin do?
Intercalates in DNA, used for childhood tumors
37
What does Doxorubicin do? Important toxicity?
Generates free radicals, dilated cardiomyopathy
38
What is methotrexate? Important toxicity?
Folic acid analog, competitive inhibition of dihydrofolate reductase, dec DNA synthesis. Myelosuppresion, reversible with leucovorin
39
What is 5-fluorouracil? Toxicity?
Pyrimidine analog activated to dUMP, inhibits thymidylate synthase, myelosuppression, not revesible
40
What is cytarabine? Toxicity?
Pyrimidine analog, inhibits DNA polymerase, causes pancytopenia
41
What is cladribine?
Purine analog, used for hairy cell leukemia
42
What is azathioprine/6-MP? Toxicity?
Purine analog, activated by HGPRT, myelosuppression. Metabolized by xanthine oxidase
43
First step of Heme synthesis? Co-factor?
Glycine + succinyl CoA -> aminovulinic acid. Pyroxidine
44
Defective enzyme in acute intermittent porphyria?
Prophobilinogen deaminase
45
Defective enzyme in porphyria cutanea tarda?
Uroporphyrinogen decarboxylase