{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T09:31:10Z","timestamp":1789205470646,"version":"build-2803163510"},"reference-count":14,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,6,11]],"date-time":"2014-06-11T00:00:00Z","timestamp":1402444800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000276","name":"Department of Health","doi-asserted-by":"publisher","award":["007\/0097"],"award-info":[{"award-number":["007\/0097"]}],"id":[{"id":"10.13039\/501100000276","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Vox Sanguinis"],"published-print":{"date-parts":[[2014,10]]},"abstract":"<jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>In this study, we compare variant <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>reutzfeldt\u2010<jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>akob disease (v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>) cases definitely linked to blood transfusion, those with a history of blood transfusion in which no donor has developed v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> and primary cases with no history of blood transfusion. The aim is to determine whether there are any differences in the demographics or clinical phenotype in these groups that might suggest additional cases of transfusion transmission of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods<\/jats:title><jats:p>All cases of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> who are old enough to donate blood (i.e. &gt;17\u00a0years old) are notified to the <jats:styled-content style=\"fixed-case\">UKBTS<\/jats:styled-content> at diagnosis, regardless of whether they are known to have a blood donation history. A search is then made for donor records and, if found, all components produced and issued to hospitals are identified and their fate determined. Recipient details are then checked against the <jats:styled-content style=\"fixed-case\">NCJDRSU<\/jats:styled-content> register to establish whether there is a match between these individuals and patients who have been diagnosed with v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>. In the reverse study, attempts are made to trace the donors to all cases reported to have received a blood transfusion and donors' details are checked against the register to determine if any have developed v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 177 cases of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> diagnosed in the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content> as of 1 February 2014, the <jats:styled-content style=\"fixed-case\">TMER<\/jats:styled-content> study identified 15 cases reported to have received a blood transfusion. Transfusion records were unavailable for 4 of these cases, all pre\u20101980, and in one other case there was no transfusion recorded in the medical notes. Transfusion records were found for 10 cases. One case transfused at symptom onset was excluded from this analysis. The mean age at onset of symptoms of the remaining nine transfusion recipients (four female and five male) was 42\u00b79\u00a0years; 57\u00b76\u00a0years in the three known transfusion\u2010transmitted cases and 35\u00b75\u00a0years in the six not linked cases. In one of these cases, details of components transfused were unavailable, and the remaining five cases received a total of 116 donor exposures with 112 donors identified, none of whom is known to have developed clinical v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>. To date, five of the 112 identified donors have died and none was certified as dying of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> or any other neurological disorder. Two of the transfusion\u2010transmitted cases did not fulfil diagnostic criteria for probable v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> during life but were confirmed at post\u2010mortem. Both cases were in the older age range (68 and 74\u00a0years, respectively), and neither had a positive <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> brain scan. The remaining cases all fulfilled the criteria for the diagnosis of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> in life, but two of these had atypical features and were older than the expected age at onset for v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In conclusion, it is possible that one or more of the v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> cases that received a blood transfusion derived from an individual not known to have v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> were infected by the blood transfusion. However, the evidence for this is weak, and the absence of a past history of transfusion in most cases of v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> excludes a large number of unrecognised transfusion\u2010transmitted cases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/vox.12161","type":"journal-article","created":{"date-parts":[[2014,6,11]],"date-time":"2014-06-11T10:35:16Z","timestamp":1402482916000},"page":"220-225","source":"Crossref","is-referenced-by-count":26,"title":["Variant <scp>CJD<\/scp> and blood transfusion: are there additional cases?"],"prefix":"10.1111","volume":"107","author":[{"given":"L. R. R.","family":"Davidson","sequence":"first","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit Western General Hospital Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. A.","family":"Llewelyn","sequence":"additional","affiliation":[{"name":"NHS Blood and Transplant Cambridge Centre Cambridge UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. M.","family":"Mackenzie","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit Western General Hospital Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. E.","family":"Hewitt","sequence":"additional","affiliation":[{"name":"NHS Blood and Transplant Colindale Centre London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. G.","family":"Will","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit Western General Hospital Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,6,11]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)91412-9"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/39057"},{"key":"e_1_2_6_4_1","unstructured":"National CJD Research and Surveillance Unit website:http:\/\/www.cjd.ed.ac.uk\/documents\/figs.pdf"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1580"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.f5675"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)05411-3"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63105-7"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1423-0410.2006.00833.x"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)16811-6"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awt032"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07376.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0002878"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2010.02614.x"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2013-305590"}],"container-title":["Vox Sanguinis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fvox.12161","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/vox.12161","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T00:05:39Z","timestamp":1696205139000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/vox.12161"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,6,11]]},"references-count":14,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,10]]}},"alternative-id":["10.1111\/vox.12161"],"URL":"https:\/\/doi.org\/10.1111\/vox.12161","archive":["Portico"],"relation":{},"ISSN":["0042-9007","1423-0410"],"issn-type":[{"value":"0042-9007","type":"print"},{"value":"1423-0410","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,6,11]]}}}