China
Africa
Explore chapters and articles related to this topic
A Clinical Incident Linked to Prion-Associated Disease
Published in Meera Chand, John Holton, Case Studies in Infection Control, 2018
Undertake a root-cause analysis to determine the details of the incident and whether or not the transmissible spongiform encephalopathy (TSE) guidelines were followed. Inform the local Health Protection Team (HPU) of the details of the incident. The local HPU will then manage the public health response. Inform the National CJD Research and Surveillance Unit.
Caenorhabditis elegans Aging is Associated with a Decline in Proteostasis
Published in Shamim I. Ahmad, Aging: Exploring a Complex Phenomenon, 2017
In certain rare instances, neurodegenerative disease can be caused by infectious proteins known as prion proteins (PrP). For example, Creutzfeldt–Jacob disease is a prion disorder also known as a transmissible spongiform encephalopathy (TSE). When PrP adopts an alternative, self-propagating conformation, PrPSc, disease symptoms may eventually appear [58–60]. Interestingly, like the neurodegenerative diseases described above, aging is a significant risk factor for TSEs, meaning that exposure to PrPSc may not lead to symptoms until late in life, usually after 60 years of age. Most cases of TSE are sporadic, meaning that a normal PrP-encoding gene mutates into a form that encodes the PrPSc variant during aging. Other cases are familial, such that a faulty PrPSc gene is inherited. Other cases of TSE in people occur following the ingestion of beef from cattle exposed to bovine spongiform encephalopathy, also known as mad cow disease. Once the PrPSc prion conformation forms, PrPSc itself is able to seed the misfolding of normal PrP protein into the PrPSc conformation and nucleate protein aggregation.
Dry-Fill Formulation and Filling Technology
Published in Larry L. Augsburger, Stephen W. Hoag, Pharmaceutical Dosage Forms, 2017
Pavan Heda, Vikas Agarwal, Shailesh K. Singh
Issues of cross-linking can be avoided by using hypromellose (HPMC) instead of gelatin capsules. Use of HPMC capsules may also be advantageous from a bovine spongiform encephalopathy/transmissible spongiform encephalopathy (BSE/TSE) perspective, being of vegetable origin. HPMC capsules contain less water compared to gelatin and are also more suitable for hygroscopic drugs.
Infection Prevention: 2020 Review and Update for Neurodiagnostic Technologists
Published in The Neurodiagnostic Journal, 2020
Anna M. Bonner, Petra Davidson
One rare disorder frequently requiring successive EEG studies is Creutzfeldt-Jakob Disease. Creutzfeldt-Jakob Disease (CJD) is neither bacterial nor viral; it is a rare, but transmissible spongiform encephalopathy that is prion-based (prion: derived from “protein” and “infectious”) and known to be fatal. CJD affects approximately one person per million annually worldwide, 350 cases of which occur in the US (NIH 2019). There are three modes by which CJD is transmitted: Sporadic CJD is the most common form, accounting for more than 80% of cases, and as the name implies, this form of CJD appears without known cause or risk factors for the disease.Hereditary CJD accounts for approximately 10–15% of cases in the US and transmission is genetically based.Acquired CJD accounts for fewer than 1% of all cases (approximately 250 patients worldwide) and occurs when the disease is transmitted by exposure to the brain or nervous system tissue, such as during a medical procedure or surgery (NIH 2019; CDC 2019a).