Surgery and Risk of Sporadic Creutzfeldt-Jakob Disease in Denmark and Sweden: Registry-Based Case-Control Studies

Department of Applied Epidemiology, National Center for Epidemiology, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Carlos III Institute of Health, Madrid, Spain.
Neuroepidemiology (Impact Factor: 2.56). 10/2008; 31(4):229-40. DOI: 10.1159/000163097
Source: PubMed


Epidemiologic evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains controversial.
From Danish and Swedish registries we selected 167 definite and probable sCJD cases (with onset between 1987 and 2003) and 3,059 controls (835 age-, sex-, and residence-matched, and 2,224 unmatched). Independent of case/control status, surgical histories were obtained from National Hospital Discharge Registries. Surgical procedures were categorized by body system group and lag time to onset of sCJD. Exposure frequencies were compared using logistic regression.
A history of any major surgery, conducted >/=20 years before sCJD onset, was more common in cases than both matched (OR = 2.44, 95% CI = 1.46-4.07) and unmatched controls (OR = 2.25, 95% CI = 1.48-3.44). This observation was corroborated by a linear increase in risk per surgical discharge (OR = 1.57, 95% CI = 1.13-2.18; OR = 1.50, 95% CI = 1.18-1.91). Surgery of various body systems, including peripheral vessels, digestive system and spleen, and female genital organs, was significantly associated with increased sCJD risk.
A variety of major surgical procedures constitute a risk factor for sCJD following an incubation period of many years. A considerable number of sCJD cases may originate from health care-related accidental transmission.

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    • "The biological plausibility of vCJD transmission by surgery, and by neurosurgery in particular, is still being considered [7]. Insofar as sCJD is concerned, there is increasing, yet limited, epidemiological evidence of significant etiological links to: general surgery [8,9] with long incubation periods [9]; surgery of retina and peripheral nerves after shorter incubation intervals [10]; and recently - with conflicting results – to blood transfusion (BT) connected to surgery after a >10-year lag [11,12]. "
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    ABSTRACT: Background There is increasing epidemiological evidence of etiological links between general surgery and sporadic Creutzfeldt-Jakob disease (sCJD) with long incubation periods. The purpose of this study was to identify specific surgical procedures potentially associated with sCJD to be targeted for preventive presurgical-intervention guidance. Results We propose a three-step clinical guidance outline where surgical procedures associated with sCJD clinical onset – potentially more contaminant - are taken into account. Data on hospital discharges and surgical procedures were obtained from Danish and Swedish national in-patient hospital registries for 167 sCJD cases, onset 1987–2003, and for 835 matched and 2,224 unmatched population controls. Surgery was allocated to different life-time periods as previously reported, and frequencies were compared using logistic regression analysis. In the year preceding clinical onset, persons with sCJD underwent a statistically significant higher number of minor surgical interventions (OR (95% CI): 17.50 (3.64-84.24)), transluminal endoscopies (OR: 2.73 (1.01–7.37)) and gastrointestinal operations (OR: 3.51 (1.21–10.19)) compared to matched controls. Surgical discharges clustered towards clinical onset. These differences increased during the clinical period, with statistically significant higher frequencies for both endoscopies and minor surgery (OR: 13.91 (5.87-32.95), and for main surgical procedures (OR: 2.10 (1.00-4.39)), particularly gastrointestinal surgery (OR: 6.00 (1.83-19.66)), and surgery contacting skeletal muscle. Comparisons with unmatched controls yielded similar results for neurosurgery in the clinical period (OR: 19.40 (2.22-168.34)). Conclusions These results suggest that some types of surgical procedures are associated with sCJD, after clinical onset or particularly just before onset. Selective planning of such surgery to minimize instrument/device contamination or quarantining might be feasible. Conditional to progress in sCJD etiological research, results are relevant for guidance development.
    Full-text · Article · May 2013 · Emerging Themes in Epidemiology
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    • "Hence, the risk of iatrogenic transmission from surgical instruments has previously been considered to be relatively low. However, recent epidemiological studies have suggested an association between surgical treatment and sporadic CJD (Collins et al., 1999; Mahillo-Fernandez et al., 2008; de Pedro-Cuesta et al., 2010). In contrast, in variant CJD (vCJD), PrP Sc and infectivity have been shown to be present in a wide variety of tissues throughout the body in addition to the CNS, these include the lymphoreticular system (spleen, tonsils and lymph nodes), components of the eye and optic nerve, and in the gastrointestinal tract (Wadsworth et al., 2001; Bruce et al., 2001; Head et al., 2004; Joiner et al., 2005; Wadsworth et al., 2007; Notari et al., 2010). "
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    ABSTRACT: Prions are comprised principally of aggregates of a misfolded host protein and cause fatal transmissible neurodegenerative disorders of mammals, such as variant Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in cattle. Prions pose significant public health concerns through contamination of blood products and surgical instruments, and can resist conventional hospital sterilization methods. Prion infectivity binds avidly to surgical steel and can efficiently transfer infectivity to a suitable host, and much research has been performed to achieve effective prion decontamination of metal surfaces. Here, we exploit the highly sensitive Standard Steel-Binding Assay (SSBA) to perform a direct comparison of a variety of commercially available decontamination reagents marketed for the removal of prions, alongside conventional sterilization methods. We demonstrate that the efficacy of marketed prion decontamination reagents is highly variable and that the SSBA is able to rapidly evaluate current and future decontamination reagents.
    Full-text · Article · Nov 2010 · Journal of General Virology
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    No preview · Article · Nov 2008 · Neuroepidemiology
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