Examination 4: Part I: Prions, Clinicopathologic Correlation, Anaerobes Flashcards
MadCow, Kuru, and CWD are all examples of transmissible spongiform encephalopathies (TSEs) or _____ disease. The protein involved has two forms _____ and _____.
Prion
PRP-c PRP-Sc (Coded for by the PRNP gene)
The exact physiological role of _____ is not known but it may aid in the formation of _____.
Creutzfeldt-Jakob Disease (CJD) has three forms. They are _____, _____, and _____.
PRPc, Synapses
Sporadic, Hereditary, Acquired
Effective or partially effective heat inactivation of CJD can be done by _____, _____, or _____, because boiling, alcohol, ammonia, formaldehyde, HCl, H2O2, and UV/microwave radiation is _____.
Incineration (1000C)
Autoclaving (134C for 20 min)
Autoclaving (121C for an hour)
Not effective
_____ is linked to cannibalism in indigenous people in the highlands of Papua New Guinea.
Is this transmissible from human to human?
How long is the incubation period of Kuru?
Kuru
Yes
Anywhere from 10-13 years to 50
On average, how many people are infected with CJD per year? How many cases are there per year in the US?
How are TSEs usually transmitted? Are spouses of CJD patients at any increased risk?
1 in 1 million per year (300/year in United States)
Through dural implants, contaminated pituitary growth hormone, cornea transplants. No.
What are a couple of differences between CJD and vCJD?
vCJD predominantly affects younger people
Delayed onset of neurological abnormalities.
Genetic defect in _____ gene coding for PrPc also causes Fatal Familial Insomnia, which is _____. The inheritance pattern of this disease is _____.
PRNP.
Prion disease interfering with sleep that causes severe mental deterioration.
Autosomal dominant
What is the primary difference between Fatal Familial Insomnia and other prion diseases?
When does Fatal Familial Insomnia typically start?
How long is a Fatal Familial Insomnia patient expected to survive following symptom onset?
FFI primarily affects the thalamus which controls sleep.
In someone’s 30’s
7 to 73 months
What are some symptoms of FFI?
How can FFI typically be diagnosed (instrument)?
Sleep disturbances, muscle spasms, stiffness
Metal deterioration
PET scan
What is Gerstmann-Straussler-Scheinker?
What is the expected longevity of a GSS patient?
This disease typically affects people in their _____.
Prion disease exhibiting loss of coordination. Clumsiness. Deterioration of cerebellum responsible for muscle tone and coordination, balance. Followed by slow mental dysfunction.
5 years
40s (35-50 years of age)
The inheritance pattern of Gerstmann-Straussler-Scheinker is _____ but _____.
How is GSS typically diagnosed (one of four answers)?
Autosomal dominant.
Some cases can arise spontaneously.
Genetic testing for mutated gene, family hx, amyloid plaques radiographically (PET scans), H&P
CWD, first identified in the 1960s in Colorado, only affects _____ and _____ and is therefore _____.
There is growing concern over _____.
Deer and Elk. Not found to be transmitted to humans like Madcow with vCJD.
the transmission of CWD from deer to livestock such as cattle and sheep.
Scrapies
Prion disease infecting sheep and goats, seen worldwide. First recognized as a disease 250 years ago.
Is CWD transmissable to humans?
Why is CWD a growing concern?
There are no documented CWD transmission to humans.
Under experimental conditions the disease can be transmitted to Squirrel Monkeys. Possible long-term incubation may be occurring without our awareness.
Anaerobic organisms lack what two enzymes?
Microaerophiles grow under _____ % oxygen, whereas aerotolerant organisms grow under _____ % oxygen.
Superoxide dismutase, catalase
5-10%, 5%
Capnophiles “prefer” _____ % CO2 and _____ % O2
Where are anaerobes most abundant in the body? In this location, what is the most abundant species?
1-10% CO2 15% O2
GI tract, Bacterioides
- Microaerophilic
- Gram-positive (thin) rods with spiral forms
- Catalase negative
- Normal flora of female genital tract
Colony morphology is pinpoint with alpha hemolysis
Lactobacillus
Proprionibacterium and Lactobacillus can both be normal flora of the ____.
Skin
Normal skin flora
Often associated with acne (inflammation serous/ purulent)
Can cause endocarditis, CNS infections, osteomyelitis, and arthritis
Most common anaerobic contaminant of BLOOD cultures
Proprionibacterium
C difficile is _____ to antibiotics such as _____, _____, and _____.
These antibiotics may lead to a condition called _____.
Resistant
Amoxicillin
Ampicillin
Clindamycin
Pseudomembranous colitis
An opening in the colon is a _____.
Anastomosis
Colostomy
Cross-connection between two channels (e.g., in the alimentary canal). Treatment that bypasses toxic megacolon of C. difficile
Complications of C difficile treatment may include _____, _____, or _____.
Perforated colon
Toxic megacolon
Pseudomembranous colitis.
C perfringens is found in _____ contaminated with feces. Some strains are part of _____. Some strains can also be found on _____ (fomite).
Soil and water
Normal flora
Kitchen counters
C perfringens can lead to soft tissue infections such as _____. (4)
Cellulitis
Myonecrosis (Gas gangreen, high mortality rate)
Food poisoning (Meat products)
Enteritis necroticans
Myonecrosis
Pseudomembranous colitis
Gas gangrene (high mortality rate)
Deposition of a pseudomembrane on mucosal surface composed of:
- fibrin
- neutrophils
- dead bacterial cells
A non-antibiotic treatment for clostridium perfingens would be…?
Enteritis necroticans is…?
Debridement of diseased tissue and air exposure
Inflammation (necrosis) of the jejunum and ileum
Often segmental in nature, perforation is possible
Enteritis necroticans from Cp
3 at risk groups for Enteritis Necroticans
People undergoing protein deprivation
People with poor hygiene/food handling habits
People from African/Asian countries
C botulinum spores are _____ and resemble a _____.
Subterminal
Tennis racket
C botulinum consists of _____ distinct genotypic groups. Humans are affected by _____. Animals are affected by _____. _____ is not usually known to cause disease.
C botulinum toxin can (primarily) cause _____.
I and II
III
IV
Flaccid paralysis
There are _____ types of Cb toxin. _____ cause human disease.
7 (A - G)
A B E F