Pathogen Safety Data Sheets: Infectious Substances – Creutzfeldt-Jakob agent, Kuru agent

PATHOGEN SAFETY DATA SHEET - INFECTIOUS SUBSTANCES

SECTION I - INFECTIOUS AGENT

NAME: Creutzfeldt-Jakob agent, Kuru agent

SYNONYM OR CROSS REFERENCE: Subacute spongiform encephalopathy, Creutzfeldt-Jakob disease (CJD), Kuru, Chronic infectious neuropathic agents (CHINA's)

CHARACTERISTICS: Filterable, self-replicating agent, slow infectious pathogen, prion

SECTION II - HEALTH HAZARD

PATHOGENICITY: CJD - insidious onset of confusion, progressive dementia, myoclonic jerks with spasticity, wasting and coma; slight elevation of CSF proteins; death usually occurs in less than 1 year; 10% cases have family history of presenile dementia; amorphous amyloid plaques in cerebellum of 15% of cases; Kuru - CNS disease with cerebellar ataxia, incoordination, tremors, rigidity, progressive wasting and death within 3-9 months

EPIDEMIOLOGY: CJD - Reported from 50 countries with highest incidence found among Libyan Jews in Israel; Kuru - occured in Fore tribe of Papua New Guinea

HOST RANGE: Humans, transmissible to chimpanzees, monkeys, guinea pigs, mice

INFECTIOUS DOSE: Unknown

MODE OF TRANSMISSION: The mode of transmission of most cases is unknown; iatrogenic cases of CJD reported (corneal transplant, from cortical electrodes previously used on known patients, brain or eye surgery, human growth hormone therapy, exposure to infected brain tissues by pathologists), no evidence of transmission of CJD from one person to another: Kuru-handling and eating kuru infected brain during ritualistic cannibalism

INCUBATION PERIOD: Fifteen months to 2 years to CJD iatrogenic cases; 4 to over 20 years for Kuru

COMMUNICABILITY: CNS and other tissues are infectious throughout symptomatic illness; lymphoid and other organs probably infectious before signs of illness appear

SECTION III - DISSEMINATION

RESERVOIR: Human cases constitute the only known reservoir

ZOONOSIS: No documented human infections acquired from animals although this has been hypothesized (consumption of scrapie-infected sheep might result in CJD; in 1996 consumption of BSE-infected beef in UK has been associated with development of CJ-like disease)

VECTORS: None

SECTION IV - VIABILITY

DRUG SUSCEPTIBILITY: N/A

SUSCEPTIBILITY TO DISINFECTANTS: Resistance to commonly used disinfectants is well recognized: formaldehyde, glutaraldehyde, ethanol, and iodine. Immersion in undiluted bleach (60,000 ppm available chlorine) for 1 hour is only partially effective. Disinfection should be carried out using 1N sodium hydroxide at room temperature for 1 hour (shorter treatments have occasionally not inactivated the pathogen)

PHYSICAL INACTIVATION: Resistant to ultraviolet and ionizing radiation, ultrasonication, nucleases, boiling, heat; autoclaving - 15 to 30 min at 121·C or 132·C will not effectively inactivate pathogen, 1 hour at 132·C is recommended)

SURVIVAL OUTSIDE HOST: Contaminated electrodes stored in ethanol-formalin for several years were found to cause CJD in chimpanzee

SECTION V - MEDICAL

SURVEILLANCE: Monitor for clinical signs - diagnosis based on EEG, histopathological findings, transmission to animals from biopsy specimens

FIRST AID/TREATMENT: Any skin contact with infectious materials should be followed by washing with sodium hydroxide; no specific treatment

IMMUNIZATION: None

PROPHYLAXIS: None

SECTION VI - LABORATORY HAZARDS

LABORATORY-ACQUIRED INFECTIONS: No documented laboratory-associated infections with spongiform encephalopathies however, consequences of infection are grave and there are cases of infection from contaminated EEG electrodes and corneal transplants

SOURCES/SPECIMENS: High titres in brain and CNS of persons with Kuru; CJD brain, spleen, liver, lymph nodes, lungs, spinal cord, kidneys, cornea and lens, blood, urine; includes formalin-fixed specimens

PRIMARY HAZARDS: Accidental parenteral inoculation; risk of infection from aerosols, droplets, and exposure.of intact skin, gastric and mucous membranes is not known

SPECIAL HAZARDS: Laboratory animals that have been infected and their tissues should be considered potentially hazardous

SECTION VII - RECOMMENDED PRECAUTIONS

CONTAINMENT REQUIREMENTS: Biosafety level 3 facilities, practices and containment equipment for activities involving these agents; also listed under biosafety level 2 with special precautions; level of containment will depend on the nature of the manipulations and the amount of sera, bio/necropsy materials handled

PROTECTIVE CLOTHING: Gown and gloves when handling potentially infectious materials; eye protection may also be indicated

OTHER PRECAUTIONS: Extreme care must be taken to avoid accidental autoinoculation or other parenteral inoculations of infectious tissues and fluids

SECTION VIII - HANDLING INFORMATION

SPILLS: Allow any potential aerosols to settle; wearing protective clothing, gently cover spill with paper towel and apply 1N sodium hydroxide, starting at perimeter and working towards the centre; allow sufficient contact time (1 hour) before clean up

DISPOSAL: Decontaminate before disposal; steam sterilization (132·C for 1 hour), disinfection with 1N sodium hydroxide for 1 hour, incineration

STORAGE: In sealed containers that are appropriately labelled

SECTION IX - MISCELLANEOUS INFORMATION

Date prepared: September 1996 Prepared by: Office of Biosafety

LCDC

Although the information, opinions and recommendations contained in this Material Safety Data Sheet are compiled from sources believed to be reliable, we accept no responsibility for the accuracy, sufficiency, or reliability or for any loss or injury resulting from the use of the information. Newly discovered hazards are frequent and this information may not be completely up to date.

 

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