random

Question 1

hydroxyurea

Answer 1

ribonucleotide reductase inihbitor

increases HbF content, break down cells prone to sickling

44% fewer pain crises in RCT
increased HbF, decreased admissions by 56-87% in children

Question 2

hydroxyurea (route, expensive?)

Answer 2

oral, inexpensive

Question 3

hydroxyurea side effects

Answer 3

may impair spermatogenesis, carcinogenic risk

Question 4

5-AZA (azacytozine), decitabine, others

Answer 4

HbF inducer
cytosine- analog

DNA methyltransferase inhibitor
causes direct cytotoxicity
induces hbF in baboons, and SCD

BUT it was not safe, efficacious, It was IV only. cost prohibitive, potential cancer risk

Question 5

Bohr effect

Answer 5

hgb-oxygen binding affinity is inversely related both to acidity and to the concentration of carbon dioxide

Question 6

Haldane effect

Answer 6

deoxygenation of blood increases its ability to carry carbon dioxide. Conversely, oxygenated blood has a reduced capacity for carbon dioxide

Question 7

ribonucleotide reductase

Answer 7

enzyme that catalyzes the formation of deoxyribonucleotides from ribonucleotides. Deoxyribonucleotides in turn are used in the synthesis of DNA