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Connecticut Medicine 2007While the cause of sporadic Creutzfeldt-Jakob disease (sCJD) is unknown, case clustering could suggest infectious transmission in some instances. (Review)
Review
BACKGROUND
While the cause of sporadic Creutzfeldt-Jakob disease (sCJD) is unknown, case clustering could suggest infectious transmission in some instances.
METHODS
Publications of sCJD clusters were systematically identified and pooled to determine if their clinical, epidemiologic, and genetic features reflected potential infectious clustering mechanisms.
RESULTS
The search yielded five clusters involving 23 cases. Most notably, among 12 cases with reported residential histories, patients lived for a median of 30.0 years (25th percentile: 26.5 years; 75th percentile: 51.0 years) in their respective cluster regions. Of 19 cases that underwent genotyping for polymorphic codon 129, seventeen were methionine homozygotes (89.5%; 95% CI 67.4-98.3%); none were heterozygous.
CONCLUSIONS
Prolonged residency duration and Prnp codon 129 methionine homozygosity may be consistent with underlying disease-clustering mechanisms, although larger, controlled investigations might help to exclude the possibility that these findings reflect the normal distribution of these features among relevant comparison populations.
Topics: Cluster Analysis; Creutzfeldt-Jakob Syndrome; Genotype; Humans; Residence Characteristics; Risk Factors; Time Factors
PubMed: 17619468
DOI: No ID Found -
Brain : a Journal of Neurology Sep 2006Prion diseases are transmissible, invariably fatal, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform... (Review)
Review
Prion diseases are transmissible, invariably fatal, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy and scrapie in animals. A large number of putative treatments have been studied in experimental models over the past 30 years, with at best modest disease-modifying effects. The arrival of variant CJD in the UK in the 1990s has intensified the search for effective therapeutic agents, using an increasing number of animal, cellular and in vitro models with some recent promising proof of principle studies. Here, for the first time, we present a comprehensive systematic, rather than selective, review of published data on experimental approaches to prion therapeutics to provide a scientific resource for informing future therapeutics research, both in laboratory models and in clinical studies.
Topics: Animals; Anti-Bacterial Agents; Anticoagulants; Antimalarials; Antiviral Agents; Cattle; Congo Red; Creutzfeldt-Jakob Syndrome; Disease Models, Animal; Encephalopathy, Bovine Spongiform; Glycosaminoglycans; Humans; Immunotherapy; Polyamines; PrPC Proteins; Prion Diseases; Tetracyclines
PubMed: 16816391
DOI: 10.1093/brain/awl150 -
The Journal of Trauma Jun 2006Both fresh frozen plasma (FFP) and platelets are heavily used in massive transfusion. Although FFP can partially correct abnormal coagulation, a recent systematic review... (Review)
Review
Both fresh frozen plasma (FFP) and platelets are heavily used in massive transfusion. Although FFP can partially correct abnormal coagulation, a recent systematic review revealed no randomized trials showing clinical benefit. Although the overall risks of FFP and platelets are low, they are the least safe blood components, due to immunologic reactions such as allergy/anaphylaxis, transfusion-related acute lung injury (TRALI) and hemolysis due to anti-A or anti-B if transfused across ABO groups. TRALI, an acute syndrome of dyspnoea, hypoxia and pulmonary 'white-out' is now a major cause of transfusion-related death. Since it is usually triggered by donor HLA antibodies, selecting non- immune donors for FFP production may be beneficial. For platelet components, risks may be reduced by platelet additive solution, allowing removal of 70% of plasma. Platelets have the additional hazard of bacterial contamination, with donor skin the predominant source. Improved arm cleansing, divert pouches for the first 30-50 mL blood and bacterial screening have been adopted internationally. Virus risks are now vanishingly low, although new agents e.g. West Nile virus can still appear. Pathogen reduction for FFP is now well established in Europe, with solvent detergent and methylene blue methods licensed, and the psoralen amotosalen in trial. Loss of clotting factors and natural anti-coagulants are recognized side effects. Amotosalen is also licensed for platelet concentrates, with the added benefit of bacterial killing. In the UK, concern regarding vCJD has led to importation of US FFP for children.
Topics: Bacterial Infections; Blood Component Transfusion; Creutzfeldt-Jakob Syndrome; Cytomegalovirus Infections; Hemolysis; Humans; Hypersensitivity; Respiratory Distress Syndrome; Wounds and Injuries
PubMed: 16763481
DOI: 10.1097/01.ta.0000199546.22925.31 -
Surgical Endoscopy Jun 2005Concern has long existed regarding the possible iatrogenic spread of variant Creutzfeldt-Jakob disease (v-CJD) through surgery. This had been fueled by recent reports of... (Review)
Review
BACKGROUND
Concern has long existed regarding the possible iatrogenic spread of variant Creutzfeldt-Jakob disease (v-CJD) through surgery. This had been fueled by recent reports of bovine spongiform encephalopathy in U.S. cattle and the first probable case of blood transmission of v-CJD in the UK.
METHODS
Systematic review of experimental and nonexperimental studies. Studies identified from searches of Medline, Embase, Cochrane Library, Science Citation Index medical databases, searching bibliographies of retrieved papers, and personal communication with international experts in the field.
RESULTS
Six articles satisfied our search criteria. Evidence stems from case reports, case series, and cross-sectional studies. There are no published cases of surgically transmitted v-CJD.
CONCLUSION
We found evidence of v-CJD prion agents in the spleen, appendix, rectum, and adrenal glands of affected patients and evidence of v-CJD prion in the appendix of patients in the preclinical stage of the disease. The risk of transmission of v-CJD prion during abdominal surgery is currently unquantifiable.
Topics: Abdomen; Creutzfeldt-Jakob Syndrome; Endoscopy; Equipment Contamination; Humans; Lymphoid Tissue; Mononuclear Phagocyte System; Risk Factors; Surgical Procedures, Operative
PubMed: 15868249
DOI: 10.1007/s00464-004-9205-2 -
BMJ (Clinical Research Ed.) Jul 2000To determine the strength of association between history of blood transfusion and development of Creutzfeldt-Jakob disease. (Review)
Review
OBJECTIVE
To determine the strength of association between history of blood transfusion and development of Creutzfeldt-Jakob disease.
DATA SOURCES
English and non-English language articles published from January 1966 to January 1999 were retrieved using a keyword search of Medline and Embase. These were supplemented by handsearching key journals and searching bibliographies of reviews.
STUDY SELECTION
Two independent reviewers selected the relevant abstracts and articles. Articles were chosen that reported the results of case-control studies trying to identify rates of prior blood transfusion in patients with Creutzfeldt-Jakob disease and in controls.
DATA EXTRACTION
Odds ratios and information on study quality were extracted from the selected articles by two independent reviewers.
DATA SYNTHESIS
Five studies containing data on 2479 patients were included. Three of the five studies used medical or neurological patients as controls, the other two used population controls. Odds ratios for developing Creutzfeldt-Jakob disease from blood transfusion ranged from 0.54 to 0.89. Four of the five studies had confidence intervals that crossed 1.0. The combined odds ratio was 0.70 (95% confidence interval 0.54 to 0.89).
CONCLUSIONS
Case-control studies do not suggest a risk of developing Creutzfeldt-Jakob disease from blood transfusion. Rather, a trend seems to exist towards a lower frequency of previous blood transfusion in patients with Creutzfeldt-Jakob disease than in controls. However, it is important to be aware of these studies' methodological limitations-primarily the choice of control population and reliability of recall of transfusion status.
Topics: Case-Control Studies; Creutzfeldt-Jakob Syndrome; Humans; Risk Factors; Transfusion Reaction
PubMed: 10875826
DOI: 10.1136/bmj.321.7252.17