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IOS 11 Exam 1

Prion Disease

QuestionAnswer
Animal Prion Disease are Mad Cow, Scrapies, Chronic wasting disease
HUman Prion diseases are Kruru, Creutzfeldts Jacob, New varient of CCJD, Gertmann Straussler Schinker syndrom, Fatal Familial Insomnia
Kuru characterisitcs Fore lingusistic tribal group in Papua New Guinea, Canabalism. Incubation was 10-13 yeas followed by death within 3months to 2 years
Creutzfeld-Jackob characteristics Onset is 57-62 contagiuos via transplant, surgery. Incubation is 16months -19 years. Onset 7-9months
New varient CJD Cases in UK 1995 time from bovine to human transmission. Age 26 more cerebellum involvement and longer duration of illiness 14 months with psychiatric manidestations
Gerstmann-Straussler Scheinker Syndromw Rare autosomal dominant , onset 43-48yo, average duration is 5 years.
Fatal Familial Insomnia First reported in 1986, occurs in thalamic region. Duration 18months. Can cause autonomic hyperactivity-increased NR, cortisol, hallucinations, delirium.
Pathogenesis of prion disease Aquired infection-eating, growth hormone, organ transplant or Heriditary transmission-Both care infectious and hereditary
Prion characteristics Prion is a small protein substance that lacks nucleic acid and is resistant to modification and hydrolysis. The human PrPc does not enter cells nor reproduces. The foreign PrPc enters brain cells and converts to PrPsc and accumulates to cause amyloid pla
Treatment options for prion disease No known form of effective therapy. Try Acyclovir, INF, Amphotericin B, amatadine, Vriabine and others
Future therapies for treating Prion disease Anthracycline (Doxyrubicin), Glycerol+dimethly sulfoxide, Doxyxyxline or tetracycline, Recombinant vaccination or Anti-PrP monoclonial antibody
Doxyrubicin MOA Inhibits the prion dises in mice
Glycerol +dimethyl sulfoxide Interfere with PrPsc formation
Doxycycline or tetracycline- Prolong the incubation period
Prevention of Prion disease Highest concentration is in Brain, spinal cord, dura mater, eye-avoid these areas, Huters wear gloves, when hadeling, blood, urine,feces Wash hands
Prions are not killed by UV light, alocholo, liquid detergents, iodine, formaldehyde, formalin, ammonia
Prions are killed by Chlorine, guanidine thiocyanate, sodium hydroxide, autoclaving
Created by: liza001
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