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Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017.

Abstract Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017; these cases account for >60% of dCJD cases reported worldwide (1,2). The unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In 2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen, Germany]), frequently used as a patch in neurosurgical procedures, was identified as the probable vehicle of transmission (4). No international recall of the implicated Lyodura occurred, the product had a relatively long shelf life, and the grafts were used frequently in Japanese patients with non-life-threatening conditions (4,5). Since 2008, additional cases have been ascertained, reflecting the identification of previously missed cases and the occurrence of new cases with longer latency periods (interval from exposure to symptom onset) for dCJD (up to 30 years), underscoring the importance of maintaining surveillance for dCJD.
PMID
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Authors

Mayor MeshTerms

Collagen

Keywords
Journal Title mmwr. morbidity and mortality weekly report
Publication Year Start




PMID- 29518068
OWN - NLM
STAT- MEDLINE
DCOM- 20180312
LR  - 20180312
IS  - 1545-861X (Electronic)
IS  - 0149-2195 (Linking)
VI  - 67
IP  - 9
DP  - 2018 Mar 9
TI  - Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017.
PG  - 274-278
LID - 10.15585/mmwr.mm6709a3 [doi]
AB  - Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that,
      according to the most well accepted hypothesis (1), is caused by replicating,
      transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD 
      cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD).
      Iatrogenic CJD can occur after exposure to prion-contaminated instruments or
      products in medical/surgical settings. Cadaveric dura mater graft-associated CJD 
      (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the
      epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017;
      these cases account for >60% of dCJD cases reported worldwide (1,2). The
      unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In
      2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen,
      Germany]), frequently used as a patch in neurosurgical procedures, was identified
      as the probable vehicle of transmission (4). No international recall of the
      implicated Lyodura occurred, the product had a relatively long shelf life, and
      the grafts were used frequently in Japanese patients with non-life-threatening
      conditions (4,5). Since 2008, additional cases have been ascertained, reflecting 
      the identification of previously missed cases and the occurrence of new cases
      with longer latency periods (interval from exposure to symptom onset) for dCJD
      (up to 30 years), underscoring the importance of maintaining surveillance for
      dCJD.
FAU - Ae, Ryusuke
AU  - Ae R
FAU - Hamaguchi, Tsuyoshi
AU  - Hamaguchi T
FAU - Nakamura, Yosikazu
AU  - Nakamura Y
FAU - Yamada, Masahito
AU  - Yamada M
FAU - Tsukamoto, Tadashi
AU  - Tsukamoto T
FAU - Mizusawa, Hidehiro
AU  - Mizusawa H
FAU - Belay, Ermias D
AU  - Belay ED
FAU - Schonberger, Lawrence B
AU  - Schonberger LB
LA  - eng
PT  - Journal Article
DEP - 20180309
PL  - United States
TA  - MMWR Morb Mortal Wkly Rep
JT  - MMWR. Morbidity and mortality weekly report
JID - 7802429
RN  - 0 (Lyodura)
RN  - 9007-34-5 (Collagen)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - *Collagen
MH  - Creutzfeldt-Jakob Syndrome/*epidemiology/*transmission
MH  - Dura Mater/*transplantation
MH  - Female
MH  - Humans
MH  - Japan/epidemiology
MH  - Male
MH  - Middle Aged
MH  - Prevalence
MH  - Tissue Transplantation/*adverse effects
MH  - Young Adult
COIS- No conflicts of interest were reported.
EDAT- 2018/03/09 06:00
MHDA- 2018/03/13 06:00
CRDT- 2018/03/09 06:00
PHST- 2018/03/09 06:00 [entrez]
PHST- 2018/03/09 06:00 [pubmed]
PHST- 2018/03/13 06:00 [medline]
AID - 10.15585/mmwr.mm6709a3 [doi]
PST - epublish
SO  - MMWR Morb Mortal Wkly Rep. 2018 Mar 9;67(9):274-278. doi: 10.15585/mmwr.mm6709a3.