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[원전] 울진원전 주변지역 女갑상선암 2.5배 발생 역학조사 보고회

건강과대안 — Wed, 05 Dec 2012 10:57:15 +0000

울진원전 주변지역 女갑상선암 2.5배 발생 역학조사 보고회

울진군의회 앞마당에서 지난 3일 주영수 한림대 의과대학교수 발표

백두산 기자2012.09.05 08:43:07

http://www.thetimes.kr/news/article.html?no=20105

주영수 한림대교수 “핵발전소와 갑상선암의 관련성이 입증확인, 추가조사연구 필요하다”

[더타임스 백두산 기자] 경상북도 울진군의회 앞마당에서 지난 9월 3일 오후 7시에 핵발전소 주변지역 여성 갑상선 암 2.5배 발생 역학조사 보고회가 열렸다. 장시원 울진군의회 의원과 반핵단체인 ‘핵으로부터 안전하게 살고 싶은 울진사람들’이 주최하는 이날 보고회에서는 지난 5월 대한직업환경의학회 춘계학술대회에서 주영수 한림대 의과대학 교수 등이 발표한 ‘원전 종사자 및 주변 지역 주민 역학조사 연구’내용을 주영수 교수가 울진에서 다시 발표하게 되었다.

주영수 교수는 울진 보고회 내용은 원전주변지역 주민 역학조사 연구와 관련, 정식 학회에서 논문으로 발표된 내용으로 학술적 공식인정을 받은 내용으로서 ‘여성의 갑상선 암 발생이 원전 주변지역 주민에 더 많이 발생하며 이는 통계적인 결론이 도출됐다’고 밝혔다.

특히 연구결과에서는 5km 이내에 사는 사람들은 5~30km 거리에 사는 사람보다 더 많은 갑상선 암이 발생하고 또한 5~30km에 사는 사람들은 30km 이상에 사는 사람들보다 더 많은 갑상선 암이 발생한 것을 입증했다. 예를 들면 원자력발전소에 가까이 살수록 갑상선암 발생이 증가한다는 사실을 입증한 것이다.

주영수 교수는 핵발전소와 갑상선암 발생의 관련성이 입증된 만큼 다른 암과의 관련성에 대한 보다 정밀한 추가조사가 필요하다는 결론을 도출했으며, 핵발전소 주변 지역 주민들 중 거주기간 등을 이용한 세분화된 분석과 4개 핵발전소 지역간 비교분석, 국가 암 등록자료를 이용한 핵발전소 소재지역과 전국간의 암발생률 비교 등의 다양한 연구방법들을 동원해서 핵발전소로 인한 건강피해를 철저히 확인할 수 있으므로 이를 위해서 추가적인 연구가 필요하다는 결론을 내렸다.

주 교수연구팀은 앞으로도 직접 지역주민을 방문해 해당 지역에서 원하는 시간과 장소에서 설명회를 열 방침이다.

또, 행사를 주최한 장시원 군의원은 “이같은 중대한 연구결과들은 정부에서도 건강과 직접적인 영향이 있는 중요한 연구인만큼 적극적으로 원전주변에 거주하는 주민들에게 공개적으로 설명해야 한다” 면서 “앞으로도 객관성 있는 연구진들이 머리를 맞대어 체계적인 연구를 해야한다”고 말했다.

이번 보고회는 당초 울진군 대회의 실에서 열릴 예정이었으나 군의 비협조로 당일 울진군의회 앞마당으로 장소가 변경되었다. 또 장시원 군의원은 울진군에 거주하는 주민들의 가족, 친척, 친구, 이웃, 직장 동료 중에 갑상선 암 등으로 치료를 받는 분들이 증가하고 있다. 그러나 울진군 집행부가 적극적으로 주최해야 할 정도로 중요한 사안임에도 불구하고 오히려 군청대회의실 이용마저 불허하는 울진군의 형태를 이해 할 수가 없다고, 울진군은 울진군민의 생명과 건강에는 전혀 관심이 없다는 것을 보여주는 행위라고 말했다.

※다음은 주영수 한림대 의과대학 교수가 발표한 주요 연구조사결과입니다.

‘원전 종사자 및 주변지역 주민 역학조사 연구’에 대한 전문가 검토연구 결과
-연구진: 주영수(한림대의대 교수, 의사 ), 하미나(단국대 교수,의사), 황승식(인하대 교수), 백도명(서울대 보건대학원 교수), 김익중(동국대 교수,의사),김정민(청주의료원 산업의학과장), 김명희(시민건강증진연구소 연구원)

- 일시 및 장소; 2012년 9월3일(월) 오후 7시, 울진군의회 앞마당

1. 2011년도에 발표된, ‘[교육과학기술부 연구용역사업] 원전 종사자 및 주변지역 주민 역학조사 연구(연구책임자, 서울의대 안윤옥 교수)’ 중에서 ‘원전 종사자’ 대상 연구결과

1) 연구에 포함된 원전종사자의 수가 적고 추적기간이 충분하지 않아, 암 발생위험도 분석과 선량-반응관계 분석은 하지 않음. 따라서 2007-2010년까지 4년간, 고리, 영광, 월성, 울진 등 4개 원자력 발전소 방사선 작업 종사자 중, 누적 방사선 피폭량이 100mSv 이상이거나 최근 방사선 피폭량이 높은 종사자 201명과 방사선 노출이 없는 건강한 성인 59명을 선정하여 염색체 이상 검사를 수행함.

2) 염색체형 교환의 빈도는 원전 종사자에서 500개 세포당 0.94로 대조군의 0.14보다 유의하게 증가하였으며, 염색체형 결실의 빈도도 원전 종사자에서 500개 세포당 2.02로 대조군의 0.41보다 높았음. 또한 총 염색체이상 빈도에서도 원전 종사자에서 500개 세포당 8.42 로 대조군의 4.22보다 더 높게 나타남.

3) 최근 1.5년간 원전 종사자 중에서 방사선에 노출된 사람들의 선량을, 5mSv이하, 5mSv~, 10mSv~, 15mSv초과로 구분하였을 때, 염색체형 이상이 각각 2.65±0.25, 2.64±0.25, 3.51±0.46 그리고 3.46±0.34로 대체로 증가하는 경향을 보임.

4) 원전 종사자들이 일반인들에 비하여 ‘염색체이상’에서 차이가 있다는 얘기는, 원전 근무경력으로 인하여 향후 이들 종사자들의 ‘암발생’ 가능성이 일반인에 비하여 더 높을 수 있음을 얘기해 주는 소견임. 따라서 원전 종사자들에 대해서는 단기적으로는 ‘방사선 노출을 정확히 평가하고 저감할 수 있는 방안마련’과, 중장기적으로는 ‘유전자 손상에 따른 발암가능성에 주목하여, 2차 예방(조기발견 및 치료)을 위한 정책적, 제도적 접근전략 모색’이 필요함.

2. 2011년도에 발표된, ‘[교육과학기술부 연구용역사업] 원전 종사자 및 주변지역 주민 역학조사 연구(연구책임자, 서울의대 안윤옥 교수)’ 중에서 ‘원전 주변지역 주민’ 대상 연구결과

1) 원전 가동으로 인한 원전 주변지역 주민의 암 발병위험도를 역학적으로 평가할 목적으로, 1991년 12월부터 2011년 2월까지 전향적 코호트 연구를 수행함. 최종적으로 구축된 연구 코호트는 원전 주변지역 11,367명(남: 4,491명, 여: 6,876명)과 대조지역 24,809명(남: 10,503명, 여: 14,306명)으로, 총 36,176명임. 코호트 입적부터 2008년까지 발생한 암을 확인하는 추구관찰 조사를 통하여 원전 주변지역 코호트에서 705명(남: 393명, 여: 312명)과 대조지역 코호트에서 1,593명(남: 941명, 여: 652명), 총 2,298명의 암 발생을 확인함.

2) 연구결과, 여성에 있어서 원전 주변지역 주민이 대조지역 주민에 비하여 ‘갑상선암’ 발생률이 2.5배(95%신뢰구간, 1.43~4.38배) 높음이 관찰됨. 그럼에도 불구하고, 해당 연구진은 “원전 주변지역의 ’모든 부위 암‘ 발병 위험도와 ’방사선 관련 암‘ 발병위험도가 대조지역에 비하여 남, 녀 모두에서 통계적으로 유의한 차이가 없었다”고 사실과 다르게 결과를 발표하였고, “원전 방사선과 주변지역 주민의 암 발병 위험도간에 인과적인 관련이 있음을 시사하는 증거는 찾을 수 없었다”는 섣부른 결론을 내리기까지 하였음.

3) 이에, 해당 연구를 검증하기 위한 같은 분야 전문가들로 구성된 연구진들이 원자료를 받아서 검토하고 재분석한 결과, 원전 주변지역에서 여성 갑상선암이 통계적으로 유의하게 높게 발생했음을 재현할 수 있었고, 이런 결과를 야기할 수 있는 검출오류(detection bias)의 가능성은 없으며, 오히려 20년의 기간 동안 연구대상자들이 기존 암환자들을 제외하면서 계속 모집되어왔기 때문에 실제로는 보다 건강한 사람들 위주의 연구가 수행되었음에도 불구하고 그러한 의미 있는 차이를 확인할 수 있었다는데 더 주목해야 한다고 결론을 내림. 또한 추가적으로 연도별 전체 갑상선암 발생률을 분석해 본 결과, 갑상선암이 오래 전부터 원전 주변지역에서 높게 발생해왔을 가능성에 대해서도 의심해 볼 수 있는 결과를 확인할 수 있었음.

4) 따라서, 지금부터는 ‘원전 주변지역 여성의 높은 갑상선암 발생원인’의 ‘원전’관련성에 대한 보다 정밀한 추가조사와, 원전 주변지역 주민들만을 한정하되 거주기간이나 혹은 원전과 거주지간의 거리 등을 이용한 세분화된 분석, 그리고 4개 원전지역을 구분하여 각 지역 내 혹은 지역들 간의 비교분석, 국가암등록자료를 이용한 원전소재지역과 전국간의 암발생률 비교 등, 보다 다양한 연구방법들을 동원하여 원전으로 인한 가능한 건강피해를 철저히 확인하고 모니터링할 수 있는 추가적인 연구가 필요하다고 판단됨.

5) 특히, 최근 10년 사이에 연구대상자의 약 60~70%가 모집되었고 그 과정에서 기존의 암환자들이 모두 배제됨으로써 발생할 수 있는 ‘역(逆) 선택오류(selection bias)’의 가능성과 ‘짧은 관찰기간’ 문제를 극복하기 위하여, 보다 중장기적인 추구조사가 기획되고 시행되어야 할 것이며 이를 위해서 정부와 한수원은 보다 책임 있는 자세를 보여주어야 한다고 판단됨. 또한 2011년도의 최종보고서의 결론은, 연구결과를 제대로 반영하지 않았을 뿐만 아니라 근거 없는 예단을 통해 원전의 위험성에 대한 면죄부를 주고자 한 것으로 보이므로, 과학적인 사실에 입각하여 정확하게 재정리 되어야 할 것임

[광우병] 미국 캘리포니아 광우병 최종 역학보고서

건강과대안 — Fri, 17 Aug 2012 19:44:39 +0000

2012년 8월 3일 미 농무부(USDA)가 캘리포니아 광우병 최종역학조사보고서를 발표했습니다.
예상대로 충분한 과학적 근거도 제시하지 못하고 광우병에 걸린 홀스타인 젖소는 오염된 사료와 무관하다고 발표했습니다. (비정형 광우병의 원인이 사료와 관련이 있는지, 없는지는 아직까지 과학적으로 충분히 규명되지 못한 상황입니다)

캘리포니아 광우병 소를 안락사시켜 렌더링 공장에 팔아넘겼고… 렌더링 공장에서 그야말로 우연하게 무작위추출(렌덤 샘플링)에 의해 광우병 검사가 실시되었음에도… 미국의 광우병 위험관리체계는 전혀 문제가 없다고 발표했습니다.

미국 정부의 발표는 캘리포니아 광우병 소가 도축되어 인간의 식품체계로 유입되지 않았다는 점을 강조하고 있습니다. 이것은 당연한 내용입니다. 세상에 어떤 미친 정부가 광우병 검사를 통해 양성이 확인된 소를 도축하여 인간의 식품체계로 유입되도록 그냥 손 놓고 있겠습니까?

문제는 미국에서 1년에 4천만 마리가 넘는 소를 도축하고 있고… 그 중에서 광우병 검사를 4만 마리만 하고 있는데… 광우병에 걸렸으나 광우병 검사를 받지 않고 인간의 식품체계로 유입된 사례가 발생할 가능성이 중요한 것입니다. 왜냐하면 광우병은 사후 부검을 통한 검사를 통해서만 진단을 할 수 있기 때문입니다. 또한 비정형 광우병, 무증상 광우병의 경우는 정밀한 광우병 검사를 실시하지 않으면 감염여부를 전혀 확인할 수 없습니다.

미국 정부는 캘리포니아 광우병 소가 낳은 새끼 중 생존해 있는 1마리만을 안락사시켜 광우병 검사를 실시했으며… 광우병 소의 birth cohorts는 생존해 있는 소가 없어서 광우병 검사를 실시하지 못했습니다.

해당 농장에 사료를 공급했던 회사는 12곳인데… 그 중 1곳은 폐업을 한 관계로 조사를 하지 못했으며 11곳을 조사했습니다.

미국은 광우병 위험물질(SRM) 중에서 30개월 이상 소의 뇌와 척수만을 육골분 사료의 원료로 사용하는 것을 금지하고 있을 뿐입니다. 나머지 광우병 위험물질 부위는 사료의 원료로 여전히 사용되고 있으며… 30개월 미만인 경우엔 뇌와 척수도 사료의 원료로 사용할 수 있습니다. 문제는 렌더링업자들이 소의 연령이 30개월 미만인지 그 이상인지를 구분할 수 있는 객관적 지표를 갖추지 못했다는 점입니다.

이런 상황에 눈 감은 미국 정부의 최종 역학조사보고서는 “눈 가리고 아웅”하는 요식행위에 불과할 뿐입니다.

==================

인도적 가축취급 및 도축관련 규정, 폐기가축 처분규정, 도축 검사 기립불능소 발견시 처분요건, 미농무부 인도적 가축도축 규정, 인도적 취급 및 도축요건 등 관련규정

- Humane Methods of Livestock Slaughter Act, 7 USC, 1901-1907

- Humane Methods of Livestock Regulations (9 CFR 313)

- Humane Handling and Slaughter of Livestock (FSIS Directive 6900.2, Revision 2)

- Disposition of Non-Ambulatory Disabled Cattle (FSIS Notice 76-11)

- Requirements for the Disposition of Cattle that Become Non-Ambulatory Disabled Following Ante-Mortem Inspection (9 CFR Part 309)

- Humane Handling and Slaughter Requirements and the Merits of a Systematic Approach to Meet Such Requirements (FSIS Notice)

- Disposition of condemned livestock(9 CFR 309.13)

========================================

Contact:
Lyndsay Cole (970) 494-7410

Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation

출처 : http://www.aphis.usda.gov/newsroom/2012/08/bse_update.shtml

Today, USDA is releasing its final report on the epidemiological investigation of a dairy cow from California that tested positive for bovine spongiform encephalopathy (BSE) in April 2012. This epidemiological report is the result of months of close coordination with the U.S. Food and Drug Administration (FDA), the California Department of Food and Agriculture (CDFA), local officials, and the associated dairy and rendering facility.

In accordance with World Organization for Animal Health guidance, USDA conducted a thorough epidemiological investigation following the BSE detection. This included on-the-ground investigations and records review from the rendering facility, the index farm, and associated premises, as well as traceback for progeny and birth cohorts of the index cow.
The results of this thorough investigation confirmed that at no time was the U.S. food supply or human health at risk, and that the United States’ longstanding system of interlocking safeguards against BSE continues to be effective.

This case was found in an animal that was sampled for the disease at a rendering facility in central California. This animal was never presented for slaughter for human consumption, so at no time presented a risk to the food supply, or to human health in the United States.

The index animal was a 10 year 7 month-old Holstein cow from a central California dairy. The animal was humanely euthanized after it developed lameness and became recumbent, and was sampled by a renderer contracted to collect samples as part of USDA’s ongoing BSE surveillance. Results from immunohistochemistry and Western blot tests at USDA’s National Veterinary Services Laboratories (NVSL) confirmed the animal positive for atypical BSE. Samples were also sent to the World Organization for Animal Health (OIE) reference laboratories in Canada and England. The laboratories confirmed that the index cow was positive for atypical (L-type) BSE.

As a result of on-the-ground investigation and records review, USDA and CDFA identified only one live offspring of the cow, which was humanely euthanized and found to be negative for BSE. No birth cohorts of the index animal were found alive.

The carcass of the index animal (along with approximately 90 other carcasses being held at the renderer’s transfer station), were disposed of in a landfill in accordance with all Federal, State and local regulations. The carcass of the index animal did not enter the human or animal food chain.

In conjunction with USDA’s investigation, FDA and CDFA conducted an extensive feed investigation. Twelve feed suppliers were identified to the index premises; one of which was no longer in business. The remaining 11 were found to be in compliance with FDA and CDFA regulations and requirements. FDA has released a full report on the feed investigation. It is available at www.fda.gov.

The United States has a longstanding system of three interlocking safeguards against BSE that protects human and animal health, the most important of which is the removal of specified risk materials – or the parts of an animal that would contain the BSE agent should an animal have the disease – from all animals presented for slaughter in the United States. The second safeguard is a strong feed ban that protects cattle from the disease. The third safeguard—which led to this detection— is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working.

View Final Report

Note to Reporters: USDA news releases, program announcements and media advisories are available on the Internet and through Really Simple Syndication (RSS) feeds.

USDA is an equal opportunity provider and employer. To file a complaint of discrimination, write: USDA, Office of the Assistant Secretary for Civil Rights, Office of Adjudication, 1400 Independence Ave., SW, Washington, DC 20250-9410 or call (866) 632-9992 (Toll-free Customer Service), (800) 877-8339 (Local or Federal relay), (866) 377-8642 (Relay voice users).

[광우병] 비전형 미 광우병, 사료가 원인일 수도 있다(미 방송)

건강과대안 — Sat, 05 May 2012 18:32:38 +0000

“변종 광우병, 사료가 원인일 수도” 미 방송 보도

“더 큰 규모로 발생했을 가능성 배제 못해”

출처 : KorMedi 2012.05.04 19:10
http://www.kormedi.com/news/article/1203569_2892.html

지난 주 미국에서 확인된 변종 광우병의 원인이 사료일 가능성을 배제할 수 없다는 전문가들의 주장이 나왔다. 미국 MSNBC 방송은 지난 2일 “만일 사료가 원인이라면 광우병이 좀 더 큰 규모로 발생했을 가능성도 있다”고 지적했다. 다음은 보도 내용 요약이다.

지금 단계에서 경보를 울릴 필요는 없다. 하지만 이것이 공중보건에 미치는 영향은 미국 농무성이 국민들에게 믿기를 바라는 것만큼 분명하지 않을 수 있다고 정상급 과학자들은 지적하고 있다.

문제의 젖소가 걸린 광우병은 비전형적인 L형으로 확인됐다. 일부 실험에 따르면 이 희귀병은 실험실의 생쥐 뿐 아니라 사람이 아닌 영장류에게까지 종간 장벽을 뛰어넘어 전염될 수 있는 것으로 나타났다. 연구에 따르면 문제의 감염원은 전형적 광우병보다 더욱 전염성이 강할 수 있으며 살코기에 들어있을 가능성이 더 크며(전형적 광우병의 감염원은 뇌와 신경조직에 주로 들어있다) 우유에 들어있을 가능성도 있다. 물론, 기존의 모든 연구는 결론을 내리기에는 규모가 너무 작은 것들이라고 많은 과학자들은 지적한다.

미국 정부는 이번 광우병이 사람의 건강에 위험하지 않다고 강조하지만 추가 연구가 시급한 실정이다. 4월 25일 미국 농무성은 이번의 비전형 광우병이 “감염된 사료를 먹는 동물과 일반적으로 관련돼 있지 않다”는 성명을 발표했다. 하지만 이 같은 결론은 “심하게 과장된 단순화”라고 폴 브라운 박사는 말한다. 최근 미국 국립보건원에서 퇴직한 그는 이 분야의 세계적 전문가다. “(농무성은) 그 같이 주장할 근거를 전혀 가지고 있지 않다.” 또한 클린턴 행정부의 농무성 관료였던 린다 디튈러 박사 역시 “사료와 관련이 없다고는 말할 수 없다”는 입장을 밝혔다.

농무성 동식물 건강검사국의 코울은 지난 1일 “이번 비전형 광우병의 기원은 아무도 모른다”며 한발 물러섰다. 사료 문제는 특히 중요하다. 만일 저절로 일어난 돌연변이가 아니라 사료가 원인이라면 문제의 사례는 더 큰 규모로 발발한 광우병의 일부일 수 있기 때문이다.

문제의 젖소가 걸린 광우병이 오염된 사료 때문일 가능성이 있을까? 영국의 광우병 사태 이후 미국을 비롯해 세계 거의 모든 나라의 농장주들은 소와 양을 비롯한 포유동물의 잔해를 소에게 먹이는 행태를 중단했다. 하지만 농장의 사료는 다른 방식으로 오염됐을 수도 있다. 미국 농무성은 소의 잔해를 닭에게 먹이는 것을 여전히 허용하고 있다. 닭 사육장에 깔렸던 짚은 대소변이 포함된 채로 소의 사료로 사용된다. 이론상으로는 이를 통해 소가 광우병에 전염될 가능성을 배제할 수 없다.

만일 사료가 원인이라면 문제의 젖소가 포함됐던 무리에서 광우병이 발발할 가능성은 없을까. 문제의 젖소를 키웠던 낙농 지대에선 농장 한 곳당 3000마리 정도를 키우는 것이 보통이며 1만마리를 키우는 곳도 일부 있다. 해당 지역에 거주하는 캘리포니아 대학교 데이비스 캠퍼스의 부학장인 짐 컬러 박사가 전한 내용이다.

미국 소비자 연맹의 마이클 한센 박사를 비롯한 많은 과학자들은 미국도 유럽과 동일한 조치를 취해야 한다고 주장한다. 병든 것처럼 보이는 모든 동물과 6살 이상의 동물은 식품 공급 계통에 편입되기 전에 전수 조사를 받아야 한다는 말이다. 건강한 동물이라도 6살이 넘으면 모두 조사해야 하는 근거는 광우병이 생기려면 보통 그 정도 기간이 걸리기 때문이다. 또한 자생적이고 비전형적 광우병이 발생하는 진정한 근원과 실제적인 영향 범위를 우리는 알 필요가 있다.

하지만 미국은 극히 적은 수만 검사하고 있다. 연간 도축되는 3500만 마리 중에서 검사를 받는 것은 약 4만 마리에 불과하다. 이와 관련해선 검사비용 논란이 있다. 마리 당 25~35달러가 소요된다는 것이다. 하지만 검사를 대규모로 시행하면 고기 1 파운드 당 몇 센트 밖에 들지 않을 것이다. 영국과 유럽, 일본을 비롯한 많은 나라가 검사에는 그만한 가치가 있다는 결정을 내린 바 있다.

미국의 식량 공급 계통에 편입되지 않은 젖소 한 마리가 국민 건강에 위협이 된다고 생각하는 과학자는 거의 없을 것이다. 하지만 현재의 감시 시스템이 충분하지 않다고 주장하는 학자들은 많다. 한편, 문제의 광우병 젖소가 낳은 새끼 한 마리를 추적해 검사한 결과 광우병이 발견되지 않았다고 미국 농무성은 3일 발표했다.

======================

Are USDA assurances on mad cow case ‘gross oversimplification’?

By Robert Bazell, Chief science and medical correspondent, NBC News

http://vitals.msnbc.msn.com/_news/2012/05/02/11501754-are-usda-assurances-on-mad-cow-case-gross-oversimplification?lite

The mad cow discovered in California last week was not really a mad cow. It suffered from a closely related disease. There is no cause for alarm at this point, but several top scientists say the public health implications may not be as clear the U.S. Department of Agriculture would have us believe.

The diseased dairy cow from a rendering (or carcass recycling) plant in Hanford, Calif., near Fresno, was infected with a condition variously known as BASE (bovine amyloidotic spongiform encephalopathy), atypical BSE and L-type BSE, which has so far been found in about 70 animals in the world. Lyndsay Cole, a spokeswoman for USDA, confirmed the diagnosis in an email Tuesday.

This condition, first reported in two Italian cows in 2004, causes the same rapid crippling and death as the classic bovine spongiform encephalopathy (BSE) that swept through Britain and much of Europe in the 1980s and ’90s. But the brains of the animals look very different after their demise.

Some experiments have shown that this rare disease can jump from species to species, infecting lab mice and even non-human primates. The research also suggests that the infectious agent for the rare disease could be more virulent than BSE, more likely to appear in meat (classical BSE is mostly in brain and nervous tissue) and might be carried in milk. Many scientists are quick to point out that all this research consists of studies too small to be conclusive.

However, there is an urgent need for further study, they say.

What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”

The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. “(The agency) has no foundation on which to base that statement.”

“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.

In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said

The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak.

The British and European outbreaks of BSE ignited because the industry turned cattle — natural vegetarians – into cannibals, feeding them the remains of cattle and other animals. U.S. farmers did the same, but Britain had a huge incidence of a related disease in sheep called scrapie, and many scientists believe that was the source of the massive cattle outbreak. Although experiments showed that BSE could infect monkeys and other animals, it was not until the first human infections that anyone realized the threat it poses to people. The human form of the disease, first discovered in Britain in the 1980s, has been blamed for the deaths of at least 280 people worldwide, with 175 in the UK alone.

How could the California cow have been infected with feed? Following the British outbreak, ranchers in the U.S. and most of the rest of the world stopped feeding cattle the remains of cattle, sheep and other mammals. But a farmer’s feed still could get contaminated by other means. The USDA still allows chickens to consume the remains of cattle. Chicken litter, containing urine and feces, is fed to cows. That could theoretically transmit the infection to cattle.

And if it is feed, what does that say about the potential of an outbreak in the rest of this cow’s heard? It appears the USDA and the California Department of Food and Agriculture are investigating. Dr. Jim Cullor, associate dean of the University of California, Davis, School of Veterinary Medicine and an expert on many animal illnesses, spoke to me from his office, which is close to the dairy farm that housed the sick cow. He would not identify the farm (nor will any government agency) but he did say dairy farms in the area usually have about 3,000 animals (about half of them milk producers). But some farms in the area have as many as 10,000 head, Cullor explains. Typically, the inspectors would visit the farm’s “hospital,” where sick animals are treated. They would also go over the hospital’s records as well as the farmer’s feed and records of past feed purchases.

“That farmer will feel like he’s had a visit from the IRS,” Cullor quipped.

But does such an inspection guarantee safety? Dr. Michael Hansen of the Consumers Union, along with many scientists, argues that, like Europe, the U.S. should test all animals that look sick or are over 6-years-old before they enter the food supply. The rationale behind testing healthy animals 6 years old or older is that BSE usually takes that long to develop.

“With thorough testing we would know the food supply is safe,” Hansen said. “We wouldn’t be guessing.”

We would also learn the true incidence and origin of spontaneous and atypical cases.

But the U.S. tests far fewer animals – about 40,000 of the 35 million cattle slaughtered annually. The argument is about cost, an estimated $25 to $30 per animal. Widespread testing would add a few cents to the cost of a pound of beef. Britain, Europe, Japan and several other nations have decided it is worth it. The USDA says it is not and declares: “The surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population.”

Few scientists would argue that the one California cow which never was headed to the U.S. food supply represents a health hazard. But many maintain that the current surveillance is insufficient. Dr. Kurt Giles, an expert in neurogenerative diseases now at the University of California, San Francisco, was at Oxford during the British outbreak. He told me USDA’s assurances about safety today remind him of British statements during the 1980s.

“It is so reminiscent of that absolute certainty,” he said.

Robert Bazell is NBC’s chief science and medical correspondent. Follow him on Facebook and on Twitter @RobertBazellNBC

[광우병] 한국 현지조사단과 AP통신 뉴스, 서로 상반된 내용

건강과대안 — Sat, 05 May 2012 17:27:59 +0000

미 농무부의 역학조사 결과 캘리포니아 광우병 감염소가 2년 이내에 출산하였던 새끼가
광우병에 걸리지 않은 것으로 확인되었다는 AP 통신의 보도입니다.

AP통신 보도에서 중요한 내용이 들어 있습니다.

미 농무부 역학조사관들은 캘리포니아 광우병 소와 함께 사육된 소들을 검사하기 위한
그들의 소재파악이 불가능할 것이라고 말했다고 합니다.

Investigators said they have been unable to locate for testing the cattle that were raised with the one who developed mad cow disease.

이 얘기는 결국 미국의 이력추적시스템이 제대로 작동하고 있지 않다는 얘기입니다.
그러므로 광우병 역학조사가 실패할 것이라는 점을 시사합니다.

미국 정부 역학조사 규정에 따르면 위험 상태의 소(At-risk cattle)는 “광우병 확정진단된 소,
출생 코호트(birth cohort), 사료 코호트(feed cohort), 양성 판정이 나오기 2년전 이내에
광우병 양성 판정 소의 자손”이라고 정의하고 있습니다.

그 중 출생 코호트(birth cohort)와 사료 코호트(feed cohort) 조사를 제대로 하지 못할
것이라는 점을 예상할 수 있습니다.

사료 코호트( Feed Cohort)는 광우병 양성소가 생애 첫 일년동안 소비한 것과 같은 사료를
공급받은 소들이라고 정의하고 있습니다. 출생년도를 특정할 수 없는 경우 그 범위를 약간
넓게 잡기도 합니다.

출생 코호트(Birth Cohort)는 “대부분의 사례에서 동일한 사료원에 노출된 소들을 확실하게
결정하는 것은 불가능함에 따라 위험 상태라고 간주되는 소를 결정하기 위해 출생 코호트
(Birth Cohort)라는 개념을 사용한다”고 밝히면서 “출생 코호트(Birth Cohort)는 광우병 양성
판정 소가 태어나기 1년전 혹은 1년 후 동안의 기간에 같은 목장에서 출생한 모든 소를
포함한다”고 정의하고 있습니다.

미국에서는 광우병이 확인될 때마다 역학조사에 실패하고 있습니다. 2004년 미국 텍사스주
광우병 최종 역학조사보고서(http://www.aphis.usda.gov/newsroom/hot_issues/bse/downloads/bse_final_epi_report8-05.pdf) 를 보면…

According to the owner, the index cow produced her last calf either in Fall 2003 or Spring 2004, and the calf prior to that was born either in Fall 2002 or Spring 2003.

“가축 소유주에 따르면, 광우병 감염소(the index cow )는 지난 2003년 가을 혹은 2004년 봄에
마지막으로 송아지를 낳았다고 하며, 그에 앞서 송아지를 낳은 것은 2002년 가을이나 2003년
봄이라고 한다.”

이력추적제가 제대로 실시되지 않고 있다보니 출생기록이 남아 있을 리 없고… 그 소가
어디로 팔려갔는지, 누구에게 팔려갔는지, 어떤 병에 걸려 어떤 치료를 받았으며, 어떤
사료를 먹었는지도… 모두 농장주의 기억에 의존할 수 밖에 없습니다.

농장주가 2년 이내의 기억도 제대로 하지 못해서 광우병에 걸린 소가 2003년 가을에
송아지를 출산했는지, 2004년 봄에 송아지를 출산했는지 명확히 기억하지 못하고
있다는 사실을 확인할 수 있습니다.

또한 AP통신의 기사에서는 미국이 1년에 3천5백만두의 소를 도축하지만 광우병 검사를 4만두
실시하고 있다고 밝히고 있으며, 렌더링 회사 베이커 커모디티즈에서는 광우병 검사 프로그램에
자발적으로 참여하고 있다고 밝히고 있습니다.

The USDA tests 40,000 of the approximately 35 million cattle slaughtered annually for BSE. Baker Commodities is a voluntary participant in the testing program.

그렇다면 미국에서는 광우병 검사 프로그램에 자발적으로 참여하지 않은 렌더링 공장도 상당수
있다는 이야기입니다. 한국정부의 현지조사단은 이런 사실을 알고나 있는지 모르겠습니다.

또한 한국 현지조사단이 무작위 추출로 우연히 광우병 검사를 받게 된 것이 아니라는
사실을 확인했다고 했는데… 5월 2일자 AP통신은 아래와 같이 여전히 랜덤 샘플링에
의한 광우병 검사를 실시한 것이라고 보도하고 있습니다. 한국의 현지조사단과 AP통신
둘 중 하나는 거짓말을 하고 있는 셈입니다.

It had been euthanized at a Tulare County dairy a week earlier and sent to the Baker Commodities rendering plant near the Central California town of Hanford, where random testing happened to be taking place that day.

툴레어 카운티의 젖소를 1주일 전에 안락사시켰으며, 중부 캘리포니니아 핸포드 지역에
소재한 렌더링 공장인 베이커 커모디티즈로 보내졌다. 이곳에서 그날 무작위 추출에 의한
검사가 일어났다.

미국 정부도 실패한 역학조사를 한국의 현지조사단이 단 1주일도 안 되는 시간에 어떻게
확인할 수 있다는 말인지… 실제로 현장에 가 볼 수 있는 시간이 부족한 상황에서
동부지역 워싱턴DC의 미 농무부, 중부지역 아이오와주의 미 국립수의연구소, 동부지역
캘리포니아 주립대학 데이비스 캠퍼스를 비행기로 돌면서 3일씩이나 허비했습니다.

=========================

USDA: Offspring of mad cow did not have disease

By TRACIE CONE

updated 5/2/2012 7:14:56 PM ET

FRESNO, Calif. — Investigators looking into California’s first case of mad cow disease say they have tracked down at least one of her offspring in another state.

Since there is no live test for the disease also known as bovine spongiform encephalopathy, it was euthanized and brain samples were sent to the national laboratory. The test was negative, officials said Wednesday.

The USDA announced April 24 that the nation’s fourth case of mad cow disease was discovered in the 10-year-old cow. It had been euthanized at a Tulare County dairy a week earlier and sent to the Baker Commodities rendering plant near the Central California town of Hanford, where random testing happened to be taking place that day.

That dairy and another associated with it are under quarantine, which is standard procedure. The USDA has declined to name the dairies or the state where the offspring was found.

USDA officials also said on Wednesday that within the last two years, the diseased cow gave birth to a stillborn calf. They did not say how that carcass was disposed.

Officials also are investigating the calf ranch where the diseased cow was raised before she was sold into dairy productions. Investigators said they have been unable to locate for testing the cattle that were raised with the one who developed mad cow disease.

Mad cow disease is a deadly affliction of the central nervous system that can be transmitted to humans who eat meat from infected cows. The incubation period is two to eight years.

Cows can contract the disease by eating rendered remains from other sick cattle, which are processed into protein supplements. It’s no longer legal to feed cattle to cattle, but rendered cattle are fed to chickens, and chicken droppings and spilled feed are rendered back into cattle feed.

The FDA and the California Department of Food and Agriculture have been examining feed records at the affected dairy and have identified at least 10 suppliers.

The USDA tests 40,000 of the approximately 35 million cattle slaughtered annually for BSE. Baker Commodities is a voluntary participant in the testing program.

[광우병] 미 농무부 광우병 역학조사 중간 발표와 한국 현지조사단의 엉터리 조사

건강과대안 — Sat, 05 May 2012 16:57:44 +0000

미 농무부 동식물검역청에서 캘리포니아 광우병 확인 소 역학조사 중간결과를 5월 2일자로 발표했습니다.

한국 정부가 파견한 민관합동조사단은 미 농무부의 역학조사에 실질적으로 참여하지도 못하고
엉뚱한 곳에서 삽질을 하고 있는 것은 아닌지 염려됩니다.

워싱턴DC 미농무부, 아이오와주 에임스의 미 국립수의연구소, 캘리포니나 주립대학 데이비스 캠퍼스 등 3곳은 민관합동조사단이 굳이 방문할 필요가 없는 곳이었는데… 공무원 친목회에서
관광을 떠난 것도 아니고 왜 이런 곳에서 허송세월을 보냈는지 모르겠습니다.

미국이 광우병 발생사실을 통보해주면서 비전형 광우병 L-타입이라는 사실을 알려주지
않았나요? 검사 데이터만 제공받으면 한국에서도 확인이 가능한 것을 굳이 검사기관을
방문하여 확인한 이유가 무엇인지 알 수 없습니다.

만일 현지조사단이 미국으로 떠나기 전까지 미국정부가 비전형 광우병 L-타입이라는 사실을
알려주지 않았다면 그것은 더 큰 문제라고 봅니다. 미국 시민단체도 광우병 발생 소식을
들은 직후 비전형 광우병 L-타입이라는 사실을 알고 있었는데… 한국 정부가 그것을 몰랐다면
아주 심각한 문제라고 생각합니다.

미국 정부의 역학조사 중간 발표에 따르면…

1. 목장 2개가 qurantine 상황이고

2. 캘리포니아 광우병 소가 지난 2년간 새끼 2마리를 낳았는데 한 마리는 사산(stillborn), 다른 한 마리는 다른 주의 농장에서 사육 중 안락사시켜 검사를 했는데 광우병 음성.

3. 광우병 소의 출생 코호트 소(birth cohort catlle) 조사를 해야 하는데 그들의 소재파악이
불가능한 상황.

4. 캘리포니아 광우병 소가 10년 전에 사육되었던 송아지 사육장에 대한 조사가
진행 중임.

5. 미 식약청과 캘리포니아주 식약청은 광우병 소를 사육하였던 농장의 사료
기록, 렌더링 시설, 캘리포니아 목장을 조사하고 있음. 현재까지 10개의 사료
회사가 광우병 소가 발생한 농장에 사료를 공급했던 것으로 밝혀짐.

6. 렌러링 시설에서는 사료 조사관이 모든 육골분 사료원료의 미국내 유통이
연방 표시(라벨링) 요건을 충족시키고 있는 사실을 확인하였음.

그런데 한국정부의 현지조사단은 좀 생뚱맞고 엉터리 같아 보이는 얘기들을 언론에
흘리고 있네요.

베이커 커모디티즈의 데니스 러키(Dennis Luckey) 부사장은 AP통신과 인터뷰에서 “이 젖소는 국가예찰프로그램에 따라 무작위로 선발되어 광우병 검사를 받게 되었다”고 밝혔고, 마이클 마쉬(Michael Marsh) 미국 서부지역 낙농협회(Western United Dairymen) 회장도 AP 통신과의 인터뷰에서 “이번 소는 30개월령 이상이며, 보행이 불가능하거나 병든 소가 아니었다. 최종 확인 시 정상적인 상태였다”고 밝혔습니다. (근거자료 : Lauran Neergraard & Sam Hananel(2012), 「New case of mad cow disease in California」, 《AP》, April 24, 2012)

미국 정부가 그렇게 예찰시스템이 잘 되어 있는데… 광우병 발표 시 왜 발생농장을 특정하지 못하고, 광우병 소의 연령에 대해서도 밝히지 못했을까요? 캘리포니아 주가 속해있는 미국 서부지역 낙농협회(Western United Dairymen) 회장은 왜 언론에 광우병 소에 대한 거짓 정보를 얘기했을까요?

미국의 광우병 예찰체계가 잘 작동하고 있는데… 왜 1년에 13만두~19만두나 발생하는 다우너 소들에 대해서 광우병 검사를 제대로 하지 않고 있을까요?

만일 예찰체계가 제대로 작동했다면 왜 다우너 증상으로 안락사시킨 소를 렌더링 회사에 팔아넘겼을까요?

현지조사단이 처리장 현장 방문에서 가축 사체에서 추출한 원료나 골분은 일절 식용으로 반출되지 않는다는 점도 분명히 확인했다는 말은 또 무슨 뚱단지 같은 얘기일까요?

미국 캘리포니아 광우병 젖소 사체를 처리했던 렌더링 공장 Baker Commodities, Inc
의 누리집에 들어가서 확인해보았더니…

———————–

http://bakercommodities.com/product-detail.php?pid=16 (Baker Commodities, Inc
의 누리집)

Products : Protein Meal

Meat and bone meal produced from the rendering process is used as a
protein and energy supplement in poultry and swine feed and may also
be utilized as an ingredient in the manufacture of pet food. Also,
meat and bone meal contains high nitrogen and phosphorus levels which
allows for its inclusion in the production of some fertilizers.

[번역] (베이커 커모디티즈의) 제품 : 단백질 사료원료

렌더링 과정을 통해 생산되는 육골분 사료원료는 가금류(참고-닭, 칠면조, 오리 등 사육하는 조류를 가금류라고 함.) 및 돼지 사료의 단백질과 에너지 보충제로 사용되고 있으며, 또한 애완동물 사료를 제조하는데 구성성분으로 사용되고 있다. 아울러 육골분 사료원료는 비료 생산에 투입될 수 있는 고함량의 질소와 인을 포함하고 있다.

—————-

Baker Commodities, Inc가 자사 제품 소개에서 육골분 사료원료를 제조하고 있다고 홍보하고 있습니다…정부 민관현지조사단이 주장은 무슨 소리이며… 그 회사의 부사장이 AP 통신 등과 인터뷰에서 해당 소가 무작위 추출(랜덤 샘플링)으로 광우병 검사를 받지 않았다면 사료 원료 사용되었을 것이라는 얘기도 했었는데… 정부 현지조사단은 미국의 예찰시스템이 잘 작동하고 있다고 엉뚱한 얘기를 하고 있습니다.

미국 정부의 역학조사 중간 발표를 전하고 있는 미국 언론(http://www.msnbc.msn.com/id/47270265)은 렌더링 시설이 미국 예찰 프로그램에 자발적 참가업체라고 되어 있습니다 (Baker Commodities is a voluntary participant in the testing program)

그렇다면 렌더링 회사의 미국정부 예찰 프로그램에 대한 참가 여부는 의무가 아니라 렌더링 회사의 자율적 결정에 의한 것이라는 이야기인데… 미국 현지조사단은 미국의 렌더링 회사가 몇 개이며… 그 중 미국정부 예찰 프로그램에 대해 자발적으로 참가하고 있는 업체가 몇 개인지를 확인해야 마땅하잖아요. 도대체 현지조사단은 무엇을 조사하고 있는지 알 수가 없습니다.

그리고 광우병 소를 처리한 렌더링 업체인 베이커 커모디티즈에서 1년 간 처리하고
있는 소의 사체가 몇 두나 되며, 그 중 몇 마리를 대상으로 광우병 검사를 실시했는지도
현지조사단이 확인해야 합니다.

정말 궁금한 것은 한국의 현지 조사단이 미국으로 현지 조사를 떠나기 전에 “현지조사
체크 리스트”를 미리 준비해갔는지 의문입니다.

일본 정부의 경우 농림수산성과 후생노동성 누리집에 현지조사 체크 리스트를 아래처럼
모두 공개하고 있습니다.

米国産牛肉（大腸）の混載事案に関する米国農務省の調査報告書の提出について
(미국산 쇠고기(대장)의 혼입된 사안에 관한 미국 농무성 조사보고서 제출에 대해)
http://www.mhlw.go.jp/stf/houdou/2r98520000027h4e.html

심지어 미국에서 보내온 영문 원본과 그것을 일본어로 번역한 번역문까지 모두 아래와 같이
국민들에게 공개하고 있습니다.

米国農務省からの調査報告書（英文・仮訳）（PDF:273KB） (미국 농무성으로부터 온 조사보고서, 영문 원본 및 번역문)
http://www.mhlw.go.jp/stf/houdou/2r98520000027h4e-att/2r98520000027h8l.pdf

그런데 한국 정부는 현지조사단을 파견하기 전에 가축방역협의회나 전문가위원회 조차도
개최하지 않고 농식품부가 밀실에서 조사단을 급조하여 출발했습니다.

그리고 미국 현지로 떠난 민관합동조사단의 조사활동은 미국 농무부가 아래와 같이
중간 역학조사 결과를 누리집에 발표한 내용조차도 담지 못하고 있습니다.

5월 9일 민관합동현지조사단이 귀국하여 또 어떻게 국민의 눈과 귀를 속일 지
두눈 부릅뜨고 감시해야 하겠습니다.

=====================================

Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States

출처 : 미 농무부 동식물검역청 보도자료
http://www.aphis.usda.gov/newsroom/2012/05/bse_update_050212.shtml

Contact:
Lyndsay Cole (970) 494-7410
Lawrence Hawkins (916) 930-5509

On April 24, USDA’s Animal and Plant Health Inspection Service confirmed the nation’s 4th case of Bovine Spongiform Encephalopathy (BSE) in an animal that was sampled for the disease at a rendering facility in central California. This animal was never presented for slaughter for human consumption, so at no time presented a risk to the food supply, or to human health in the United States.

Through its continuing epidemiological investigation, APHIS–in collaboration with the California Department of Food and Agriculture (CDFA)–has identified that one progeny born to the positive cow in the last 2 years was stillborn, and another has been located on a site in another state. That animal has been appraised, humanely euthanized, and sampled for BSE at the National Veterinary Services Laboratories in Ames, Iowa. Test results for that animal are negative for BSE. No birth cohort cattle have been located through the investigation.

A hold order has been placed on all cattle at a second dairy (dairy 2) that is associated with the dairy of the initial positive cow (also called the index dairy). Both dairies remain under quarantine. Inventories of both the index dairy and dairy 2 have been completed by CDFA. Records are still being matched and validated to determine if any at-risk cattle may be present.

In addition, a calf ranch where the initial positive cow was raised 10 years ago is being investigated.

The Food and Drug Administration and CDFA continue the investigation of feed records at the index dairy, rendering facility and calf ranch. To date, 10 feed firms have been identified as suppliers for the index dairy during the time period of interest. At the rendering facility, feed investigators confirmed that all domestic distribution of meat and bone meal meets federal labeling requirements.

USDA will continue to work closely with CDFA and FDA to provide additional information as it is available.

The United States has a longstanding system of three interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials – or the parts of an animal that would contain BSE should an animal have the disease – from all animals presented for slaughter in the United States. The second safeguard is a strong feed ban that protects cattle from the disease. The third safeguard – which led to this detection – is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population.

Note to Reporters: USDA news releases, program announcements and media advisories are available on the Internet and through Really Simple Syndication (RSS) feeds. Go to the APHIS news release page at www.aphis.usda.gov/newsroom and click on the RSS feed link.
USDA is an equal opportunity provider, employer and lender. To file a complaint of discrimination, write: USDA, Director, Office of Civil Rights, 1400 Independence Ave., SW., Washington, DC 20250-9410 or call (800) 795-3272 (voice) or (202) 720-6382 (TDD).

[프리온질병] 뇌경막 이식 수술 관련 CJD 한국 최초 사례(김윤중 교수팀)

건강과대안 — Wed, 30 Nov 2011 10:47:55 +0000

한림대 김윤중 교수팀이 대한의학회에서 발행하는 영문학술지 [J Korean Med Sci] 2011년
11월호에 뇌경막 이식 수술 후 23년이 지난 다음 iCJD로 사망한 한국 최초의 보고 사례인
54세 여성 사망자에 관한 case report입니다. 전문은 첨부파일을 보세요.

J Korean Med Sci. 2011 Nov;26(11):1515-1517. Published online 2011 October 30. http://dx.doi.org/10.3346/jkms.2011.26.11.1515
The Korean Academy of Medical Sciences. http://www.jkms.org/DOIx.php?id=10.3346/jkms.2011.26.11.1515


Dura Mater Graft-Associated Creutzfeldt-Jakob Disease: The First Case in Korea
Hye Lim Kim,¹ Ju Young Do,¹ Han Jeong Cho,² Yong-Chul Jeon,² Seok Joo Park,² Hyeo Il Ma,¹ Jun Ho Song,³ Yul Lee,⁴ Hyun Choi,⁵ Kyung Chan Choi,⁶ Yong Sun Kim,²^,⁷ Inga Zerr,⁸ Kai Kallenberg,⁹ and Yun Joong Kim¹^,²
¹Department of Neurology, Hallym University College of Medicine, Hallym University, Anyang, Korea.
²ILSONG Institute of Life Science, Hallym University, Anyang, Korea.
³Department of Neurosurgery, Hallym University College of Medicine, Hallym University, Anyang, Korea.
⁴Department of Radiology, Hallym University College of Medicine, Hallym University, Anyang, Korea.
⁵Department of Anesthesiology and Pain Medicine, Hallym University College of Medicine, Hallym University, Anyang, Korea.
⁶Department of Pathology, Hallym University College of Medicine, Hallym University, Chuncheon, Korea.
⁷Department of Microbiology, Hallym University College of Medicine, Hallym University, Chuncheon, Korea.
⁸Department of Neurology, National Reference Center for TSE, Goettingen, Germany.
⁹Department of Neuroradiology, University Medicine, Georg-August-University, Goettingen, Germany.
Address for Correspondence: Yun Joong Kim, MD. ILSONG Institute of Life Science, Hallym University, Department of Neurology, Hallym University Sacred Heart Hospital, Rm 607, ILSONG Bldg, 170 Gwanpyong-ro, Dongan-gu, Anyang 431-060, Korea. Tel: +82.31-380-1666, Fax: +82.31-381-8820, Email: yunkim@hallym.ac.kr

Received April 19, 2011; Accepted September 05, 2011.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract
Since 1987, dura mater graft-associated iatrogenic Creutzfeldt-Jakob disease (dCJD) has been reported in many countries. We report the first case of dCJD in Korea. A 54-yr-old woman, who underwent resection of the meningioma in the left frontal region and received a dura mater graft 23 yr ago presented with dysesthesia followed by psychiatric symptoms and ataxia. Her neurological symptoms rapidly progressed to such an extent that she exhibited myoclonus, dementia, and pyramidal and extrapyramidal signs within 8 weeks. The 14-3-3 protein was detected in her cerebrospinal fluid; however, an electroencephalogram did not reveal characteristic positive sharp wave complexes. Diffusion-weighted magnetic resonance images, obtained serially over 64 days, revealed the rapid progression of areas of high signal intensity in the caudate nucleus and cingulate gyrus to widespread areas of high signal intensity in the cortex and basal ganglia. Pathological examination of brain biopsy specimens confirmed the presence of spongiform changes and deposition of prion protein in the neurons and neuropils.

Keywords: Creutzfeldt-Jakob Syndrome, Dura Mater Graft, Iatrogenic Disease.

[프리온질병] 일본의 1979~2008 뇌 경막 이식수술과 관련된 iCJD

건강과대안 — Tue, 29 Nov 2011 13:10:49 +0000

MMWR Morb Mortal Wkly Rep. 2008 Oct 24;57(42):1152-4.

Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts–Japan, 1978-2008.

Centers for Disease Control and Prevention (CDC).

Erratum in

MMWR Morb Mortal Wkly Rep. 2008 Oct 31;57(43):1181..

출처 : http://www.ncbi.nlm.nih.gov/pubmed/18946463

Abstract

Creutzfeldt-Jakob disease (CJD) is the most common of the human prion diseases (also known as transmissible spongiform encephalopathies), which, according to the leading hypothesis, are caused by an abnormal protein (i.e., prion) that is able to induce abnormal folding of normal cellular prion proteins. Annual worldwide incidence of these always fatal neurodegenerative diseases is estimated at 0.5-2.0 cases per million population. CJD can occur sporadically, or as a genetic disease, or can be transmitted iatrogenically. In 1996, a new human prion disease, variant CJD (vCJD), was first described in the United Kingdom. This disease was believed to have resulted from human consumption of cattle products contaminated with the prions responsible for bovine spongiform encephalopathy (BSE, commonly known as mad cow disease). That year, in part to check for possible vCJD cases, a national survey was conducted in Japan; 821 CJD cases were identified, including 43 cases associated with receipt of cadaveric dura mater grafts. A single brand of dural graft (Lyodura) produced by a German manufacturer before May 1987 was identified as the most likely vehicle of transmission in all but one case. By 2003, continued surveillance in Japan had identified a total of 97 such cases. Since then, an additional 35 cases have been identified. This report updates previous reports and summarizes the investigation of all 132 cases to date linked to dural grafts. The results suggest that, because of the long incubation period between graft receipt and symptom onset (possibly >24.8 years), continued surveillance in Japan might identify additional CJD cases associated with dural grafts.

[프리온질병] 일본의 1979~2003 뇌 경막 이식수술과 관련된 iCJD

건강과대안 — Tue, 29 Nov 2011 13:07:14 +0000

Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts — Japan, 1979–2003

출처 : http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5248a3.htm

In 1997, a nongovernment surveillance group for Creutzfeldt-Jakob disease (CJD) in Japan supported financially by the Ministry of Health and Welfare* (MHW) reported 43 cases of CJD associated with receipt of cadaveric dura mater grafts (1). In all but one case, the most probable vehicle of transmission was a single brand of dural graft (LYODURA^® [B. Braun Melsungen AG, Melsungen, Germany]) produced before May 1987. As of March 2003, ongoing surveillance in Japan had identified an additional 54 dura mater graft–associated cases. This report summarizes the investigation of the 97 cases, which indicated that during 1983–1987, the estimated minimum risk for CJD within 17 years of receipt of the implicated product in Japan was approximately one case per 1,250 grafts. No cases have been reported among patients who received their first dural graft after 1991; however, because of the long latency period between graft placement and symptom onset, additional cases of graft-associated CJD are likely to be reported.

During 1996–2003, cases of CJD were identified in Japan by using 1) a mail survey of neurologic, psychiatric, and neuropathologic institutions (overall response rate: 74%) (1) and 2) subsequent reporting of CJD patients by clinicians to MHW. During this period, 97 cadaveric dura mater graft–associated CJD cases were identified. A case of dura mater–associated CJD was defined as a case in which a patient received a cadaveric dura mater graft and subsequently had CJD diagnosed by a physician and reviewed and accepted as CJD by a surveillance panel of neurologists.

The 97 CJD patients had illness onset during September 1985–April 2002 (Figure 1). Median age at onset was 58 years (range: 15–80 years); mean age was 55 years. Mean age at onset was younger than that reported for sporadic CJD in Japan (66 years). A total of 58 (60%) patients were female. Neuropathologic confirmation of CJD diagnosis was obtained for 20 (21%) patients; 65 (84%) of the other 77 patients with physician-diagnosed CJD had an electroencephalogram with a periodic synchronous discharge pattern consistent with CJD.

All 97 patients received dura mater grafts during 1978–1991 (Figure 2). Three patients received more than one dural graft during this period, including one patient reported previously (1). In all three cases, the first graft was considered to be the source of infection. Medical conditions leading to the use of dural grafts in these patients included tumor (n = 46), brain hemorrhage (n = 14), Jannetta procedure for facial palsy (n = 13) and for trigeminal neuralgia (n = six), intracranial aneurysm (n = eight), unspecified anomalies (n = five), hematoma (n = three), injury (n = one), and ossification of the spinal posterior longitudinal ligament (n = one).

Latency periods ranged from 14 months (receipt in 1987 and onset in 1989) to 275 months (receipt in 1978 and onset in 2001). The median and mean latency periods were 122 and 125 months, respectively. A total of 93 patients received dural grafts during 1978–1987. In 1987, the manufacturer revised collection and processing procedures for the implicated product to reduce the risk for CJD transmission. Four patients received grafts during 1988–1991. No cases have been reported among patients who received their first dural graft after 1991. A total of 86 (89%) patients were documented to have received LYODURA^®; the brand name of dural graft was unknown for 11 patients. A total of 81 (84%) of the 97 patients received their dural grafts during 1983–1987, during which time an estimated 100,000 patients received LYODURA^® grafts in Japan. All 81 patients died from CJD within 17 years after receipt of the grafts. Lot numbers of the dura mater grafts used for the 97 patients could not be identified. As of September 2003, five additional cases were under investigation in Japan for suspected dural graft–associated CJD.

Reported by: Y Nakamura, MD, M Watanabe, MD, K Nagoshi, MD, Dept of Public Health, Jichi Medical School, Minamikawachi; T Kitamoto, MD, Dept of Neuropathology, Tohuku Univ School of Medicine, Sendai; T Sato, MD, Kohnodai Hospital, National Center for Neurology and Psychiatry, Ichikawa; M Yamada, MD, Dept of Neurology, Kanazawa Univ Graduate School of Medical Science, Kanazawa; H Mizusawa, MD, Dept of Neurology, Tokyo Medical and Dental Univ School of Medicine, Tokyo, Japan. R Maddox, MPH, J Sejvar, MD, E Belay, MD, LB Schonberger, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

Editorial Note:

Dural graft–associated CJD cases continue to be identified in Japan. The estimated minimum risk within 17 years after receipt of LYODURA^® is approximately one case per 1,250 recipients. The precise number of dura mater grafts used in Japan is unknown, but an estimated 20,000 grafts per year might have been used during 1983–1987. The widespread use of LYODURA^® during neurosurgical procedures in Japan is the most probable source of the unusually high number of dural graft–associated CJD cases in Japan (2). Dural graft recipients have symptom onset at a younger age compared with age at onset in sporadic cases of CJD in Japan. The identification of additional cases over time has resulted in an expected increase in the latency period between dural graft placement and symptom onset. The mean and range for this latency of CJD from contaminated grafts is unknown, but the upper limit now exceeds 22 years. The occurrence of new cases, the increase in the mean and range of the latency period, and the identification of suspected cases under investigation all suggest that this outbreak is ongoing.

No cases in Japan were reported to be related to receipt of a dural graft other than LYODURA^®. For 11 cases, the manufacturer brand name was unknown. Although LYODURA^®, or in one case either LYODURA^® or a dural graft from another manufacturer (Tutoplast^® [Pfrimmer-Viggo GmbH & Co., Erlangen, Germany]), was suspected in these cases, documentation of a specific source was unavailable. Four patients received dural grafts after collection and processing procedures were revised by the manufacturer in 1987, but whether the implicated dural grafts were LYODURA^® produced before 1987 is unknown. That all LYODURA^®-associated CJD cases to date occurred among patients who received grafts before 1992 suggests that all implicated grafts likely were processed before 1987; the implicated product’s expiration date is 5 years after processing.

LYODURA^® never was produced by the manufacturer for distribution in the United States, and relatively few LYODURA^® grafts were used in this country. In May 1987, after identification of the first dural graft–associated CJD case in a U.S. patient who had received the implicated product, the manufacturer revised its procedures for collecting and processing dura mater grafts to reduce the risk for CJD transmission (e.g., by discontinuing the commingling of dura and disinfecting them with sodium hydroxide) (3,4). Subsequently, numerous other dura mater graft–associated cases were identified worldwide; nearly all patients had received the implicated product, including one additional U.S. patient. In 1997, the report of 43 cases of dura mater graft–associated CJD in Japan represented the largest cluster of such cases in any one country (1).

In one of the CJD cases reported in Japan, the implicated graft was used in a spinal (not an intracranial) procedure. This case suggests that transmission from contaminated dura might occur in areas of the neuraxis outside of the cranial vault.

In 1997, the Food and Drug Administration’s Transmissible Spongiform Encephalopathy Advisory Committee (TSEAC) recognized that the use of human dura mater in the United States carries an inherent risk for transmitting CJD. However, the committee recommended that the use of such grafts be left to the discretion of the treating neurosurgeon, provided that the human dura mater is procured and processed according to appropriate safety measures (5). In 1997, an estimated 4,500 dural grafts were distributed for use in the United States (6). After the TSEAC recommendations were issued, the number of dural grafts distributed for use in the United States declined to an estimated 900 grafts in 2002 (B.E. Buck, M.D., Miami Tissue Bank, personal communication, 2003).

The cases described in this report indicate that recipients of contaminated dura mater grafts might remain at risk for CJD for >22 years after receiving grafts. CDC continues to conduct surveillance for cases of CJD in the United States. Patients with a rapidly progressive dementia consistent with CJD and a history of dural graft implantation should be reported through local or state health departments to CDC, telephone 404-639-3091.

References

CDC. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts—Japan, January 1979–May 1996. MMWR 1997;46:1066–9.
Brown P, Preece M, Brandel JP, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000;55:1075–81.
Thadani V, Penar PL, Partington J, et al. Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft. J Neurosurg 1988;69:766–9.
CDC. Epidemiologic notes and reports update: Creutzfeldt-Jakob disease in a patient receiving a cadaveric dura mater graft. MMWR 1987;36:324–5.
U.S. Food and Drug Administration. Class II Special Controls Guidance Document: Human Dura Mater; Draft Guidance for Industry and FDA. Rockville, Maryland: U.S. Food and Drug Administration, 2002.
Solomon R. Methods used in human cells, tissues, and cellular and tissue-based product (HCT/P) establishments. Available at http://www.fda.gov/ohrms/dockets/ac/03/transcripts/3969t2.pdf.

* Subsequently named the Ministry of Health, Labor, and Welfare.

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About CJD and CJD Support Network in Japan February of 2006

CJD Support Network in Japan

http://www.cjd-net.jp/EnglishPage.htm

Creutzfeldt-Jakob Disease (CJD) is one of intractable and fatal diseases caused by an infectious agent called prion. There are four types of CJD: Sporadic, Familial, Variant and Iatrogenic. Sporadic CJD is said to occur in one out of one million people. Variant CJD is thought to occur in relation to Bovine Spongiform Encephalopathy(BSE). Outbreak of BSE and Variant CJD is reported from many countries, especially the UK.

Japanese government researched whether there was Variant CJD in Japan in 1996. Consequently, in Japan, however, none of variant CJD has yet been reported from 1996 until 2004. Instead, until February 2006, 117 iatrogenic CJD patients due to human dura mater grafts have been reported. This figure of dura mater victims is abnormally high in comparison with other countries.

The main reason for this is thought to be the results of using many prion contaminated human dura mater named Lyodura produced by B.Braun in Germany between 1973 and 1996. B.Braun had been adopting gamma radiation for the sterilisation of Lyodura since 1973. This method, however, was found to be ineffective for the sterilisation of CJD agent by Gibbs, Gajdusek and others in 1978. In spite of this fact, this company continued to produce Lyodura. In addition to adopting ineffective sterilisation method and not identifying CJD donor, B.Braun produced Lyodura by “pooling”. This was primarily due to the packaging technique by B. Braun. In this process, 600 sheets of duras were packaged together in one plastic bag regardless of the duras condition. As the results, healthy duras were thought to be contaminated with prion-infected duras.

In 1987, the FDA in the US prohibited the use of Lyodura because the first CJD patient was reported after using Lyodura. However the Japanese government did not follow this measure and did not take any step to prohibit selling Lyodura.

In Japan, CJD victims filed suits against the Japanese government, B.Braun and BSS, the importer in Ohtsu and Tokyo since 1996. In March 2002, after 6 years of litigations, an historical out- of-court settlement was reached. The defendants apologised to CJD victims. By 2006, 71% of plaintiffs have received compensation from the settlement. The litigation is not yet finished as of February 2006.

The CJD Support Network(CS Net) of Japan was established on June 2002. Volunteers and victims themselves, medical workers, researchers, lawyers support this Network. CS Net provides information, support and assistance for any forms of CJD to patients and their families in Japan. CS Net also promotes research, education and awareness concerning CJD problems. CS Net has been getting financial support by the Japanese Government from the fiscal year of 2003 as one of the results of the settlement. In Japan, there has been 22 cases of BSE reported as of January 2006, and regrettably, one Variant CJD case has been reported in February 2005.

[프리온질병] 호주의 라이오듀라(Lyodura) 사용 및 iCJD 위험

건강과대안 — Tue, 29 Nov 2011 13:05:13 +0000

Public Health

Lyodura use and the risk of iatrogenic Creutzfeldt–Jakob disease in Australia

Fiona J Brooke, Alison Boyd, Genevieve M Klug, Colin L Masters and Steven J Collins

MJA 2004; 180 (4): 177-181

출처 : http://www.mja.com.au/public/issues/180_04_160204/bro10566_fm.html

Abstract

Although infectiousness is a feature of Creutzfeldt–Jakob disease (CJD), only a small proportion of cases are linked to transmission through healthcare provision.

As of January 2003, over 120 cases of CJD associated with use of human cadaveric dura mater had been recognised worldwide; almost all were associated with the commercial product Lyodura.

Most cases (97) have occurred in Japan, giving an overall risk estimate of around 1 per 2268 patients treated with Lyodura (0.04%) in that country.

In Australia, five cases of CJD have so far been linked to Lyodura, but, given the protracted tails of previous epidemics of transmissible spongiform encephalopathies, further cases are possible.

Results of surveys of Lyodura use in Australia are incomplete, but information from the manufacturer suggests that 2208–2478 sheets of Lyodura may have been used here.

This use translates to a relatively high incidence of Lyodura-associated CJD, with current overall rates appearing around five times higher than those reported in Japan; reasons for this difference are unclear.

Creutzfeldt–Jakob disease (CJD) is a fatal, transmissible, neurodegenerative disorder belonging to the group known as the transmissible spongiform encephalopathies (TSEs). CJD can occur without explanation (sporadic), secondary to mutations in the prion protein gene (PRNP), or as a complication of medical treatment using contaminated therapeutic agents or equipment (iatrogenic). Although corneal grafts and neurosurgical equipment have been associated with disease transmission, the most common causes of iatrogenic CJD have been treatments involving human-derived cadaveric pituitary hormones or dura mater.1

Creutzfeldt–Jakob disease and Lyodura

The first identified case of CJD in a dura mater recipient was reported in the United States in early 1987.2 In response, the US Food and Drug Authority issued a safety alert in April 1987, seeking immediate discontinuation of use of the identified dura mater batch (Lyodura batch #2105).3 A second patient with CJD linked to Lyodura was detected in New Zealand in 1988,4 but the specific batch could not be identified. This has remained a frequent difficulty when tracing contamination sources.

As of January 2003, over 120 CJD cases related to dura mater use had been detected globally, with 97 in Japan.5 These cases were predominantly associated with Lyodura, a commercial product produced since 1969 by B Braun Melsungen AG (based in Germany). Only a few reports suggest the possibility of CJD after use of dura mater from other commercial or non-commercial sources.6-8

Lyodura consists of lyophilised, irradiated human dura mater sourced post mortem. Additional processing with immersion in a solution of sodium hydroxide (1 M) was instituted in 1987, with a noticeable reduction in Lyodura-related cases thereafter.9 Lyodura has been used in a number of countries, including Australia, Japan, Canada, the United States and the United Kingdom, mainly in neurosurgery, but also in orthopaedic, otological, dental, urological, gynaecological and cardiac procedures (Box 1) (Dr L Schonberger, Assistant Director, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Ga, USA, personal communication).

Most cases of CJD associated with dura mater have occurred in Japan. In 1996, in response to the growing incidence, a survey was undertaken of Lyodura use in almost 3000 Japanese healthcare institutions. This estimated that up to 100 000 people received Lyodura grafts between 1983 and 1987,9,10 and up to 220 000 between 1979 and 1991 (out of a total of 260 000 who received dura mater grafts). Use of these grafts greatly declined after 1991 but may have continued until 1997.11,12 Assuming that all cases of CJD associated with dura mater were a consequence of Lyodura use, the overall risk of Lyodura-associated CJD in Japan is approximately 0.04%.5

Estimating Lyodura use and risk in Australia

Lyodura was approved by the Australian Therapeutic Goods Administration for importation and use in Australia in 1972. The product licence was withdrawn in early May 1987, shortly after recognition of the first case of CJD linked to Lyodura use.

To date, five cases of CJD have been epidemiologically linked to neurosurgical use of Lyodura in Australia. Their clinical features have already been described13-15 and are summarised in Box 2. Patient 1 presented in 1987, about 5 years after implantation of Lyodura. The longest incubation period was in Patient 4, who presented in 1999 after an incubation period of almost 17 years.14 The most recent (fifth) patient died in 2000. Patients 1, 2 and 4 were exposed in 1982, while Patients 3 and 5 were exposed in 1985 and 1986, respectively.

A number of studies have attempted to determine the number of people exposed to Lyodura in Australia. We collated the available information and undertook further enquiries, as a basis for estimating the risk of Lyodura-associated CJD in this country (Box 3).

Implications for Australia

Quantifying past use of Lyodura in Australia relies on data that cannot be fully confirmed. Initial estimates by the Commonwealth Department of Health and Ageing in discussion with the Therapeutic Goods Administration placed an upper limit of between 5000 and 10 000 individuals potentially exposed to Lyodura. However, from the results reported here, the true number is likely to be much smaller — probably fewer than 2500. If so, the risk of Lyodura-associated CJD is higher in Australia (0.20%–0.23%) than in other countries that have undertaken similar investigations, such as Japan.

Although the risk of Lyodura-associated CJD in Japan has appeared to fluctuate over time, two relatively stable features are a peak in contaminated grafts between 1983 and 1987, and the paucity of Lyodura-associated cases after 1987, when the manufacturer instituted effective decontamination of the tissue with a 1 M solution of sodium hydroxide.1,5,9 Ninety-seven cases associated with dura mater had been recognised to 2003,5 with predictions that further cases are likely until 2020, and that final numbers may reach 160.12 Acknowledging a total of 220 000 people exposed to Lyodura, and assuming all CJD associated with dura mater was associated with Lyodura (which is reasonable based on evidence published to date),11 then the cumulative overall risk of CJD from Lyodura in Japan is currently around 0.04% (95% CI, 0.03%–0.05%) (Box 4). Estimated risk in Australia is much higher — 0.20% to 0.23% (95% CIs, 0.06%–0.47% and 0.07%–0.53%, respectively). With the same assumptions, the risk of CJD in the higher-risk period in Japan (1983–1987) is about 0.08% (95% CI, 0.06%–0.10%), while that in Australia (1982–1986) is 0.43% (95% CI, 0.14%–0.99%). Notwithstanding the need for certain assumptions to facilitate these comparisons, the risk in Australia appears about five times greater than the analogous point estimates of risk in Japan. The reality of the differences is further supported by the lack of overlap between the confidence intervals of the calculated estimates.

Nevertheless, the risk of CJD associated with Lyodura in Australia is well below the risk associated with exposure to human-derived pituitary growth hormone (hGH) in France (Box 4). As of 1999, 55 cases of CJD had been linked to French-derived hGH, from a cohort of 1361 patients, representing an attack rate of about 1 in 25 recipients (4.0%).17 A more recent report suggested the risk could be much higher, at 81 per 1361 (about 6.0%).18

The reason for the higher estimated risk of Lyodura-associated CJD in Australia compared with Japan is not known. Perhaps most likely is chance receipt of a relatively high percentage of contaminated batches of Lyodura and use of multiple pieces of Lyodura per patient in Australia, although the latter is contrary to anecdotal recollections of Australian neurosurgeons. Alternatively, the difference may reflect better case ascertainment in Australia, which, in contrast to Japan, had an established comprehensive, prospective, national surveillance program for CJD, with international comparisons of incidence rates for sporadic CJD attesting to the adequacy of case ascertainment.14

Another potential explanation is genetic difference between the populations of the two countries. For example, homozygosity for methionine at codon 129 of the PRNP gene appears to predispose to iatrogenic CJD.1 However, contrary to expectations, this homozygosity appears more common in the Japanese population (about 92%) than in occidental populations (about 37%).19

Finally, the differences in risk may reflect greater use of Lyodura in Japanese patients with malignancies, whose survival was shorter than the lengthy incubation periods typical of TSEs, or in non-neurosurgical applications, with attendant lower transmission efficiency. Japanese studies suggest that Lyodura-related transmissions have been essentially restricted to neurosurgical procedures,10,11 and our comparative risk analysis was predicated on the assumption that most Lyodura was used in these procedures in both countries. Although some Lyodura was most likely used in non-neurological procedures, the proportion is impossible to quantify in different countries, leaving uncertainty about our risk comparisons.

In addition to neurosurgery and the non-neurological applications identified by Australian surveys, additional uses of Lyodura have been reported in the US (Box 1). Cases of CJD have been linked epidemiologically to Lyodura used in the embolisation of the external carotid artery for treating a nasopharyngeal angiofibroma,20 as well as dura mater used to embolise intercostal arteries before thoracic surgery.7 This reinforces the likelihood that non-neurosurgical use of Lyodura can result in transmission.10 Therefore, risk of Lyodura-associated CJD linked to non-neurosurgical uses appears a genuine possibility, which may be very difficult to identify epidemiologically, given the often deficient state of medical records.

The inability to clearly identify surgical uses of Lyodura, especially non-neurosurgical uses, also raises the possibility of secondary iatrogenic transmissions from Lyodura-treated patients during an extended preclinical or incubation phase. Animal models clearly support the possibility of lengthy presymptomatic periods (which may even exceed the lifespan of hosts) during which transmission is possible.21-24 Generally, infection control and other guidelines focus on the need to limit transmission risk through identifying patients who received Lyodura during neurosurgery, and do not include guidance on risk associated with other surgical applications. This approach may need to be reconsidered based on studies such as ours.

1: Non-neurosurgical uses of Lyodura in the United States*

Development of ligaments to stabilise shoulder joints

Replacement of the tracheal wall

Covering pleura defects

Securing bronchial stumps

Repair of pericardium

Repair of diaphragm defects (traumatic or congenital)

Arthroplasties of the elbow

Reinforcement of fascia in abdominal hernias

Reinforcement of tendons or ligaments

Plastic enlargement of the urinary bladder

Other miscellaneous surgical uses

* Based on information from the US National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Ga.

2: Features of the five Australian cases of Creutzfeldt–Jakob disease associated with Lyodura

3: Studies of Lyodura use in Australia

Survey of hospitals in Victoria

In 1987, after notification of the first patient with Lyodura-associated Creutzfeldt–Jakob disease (CJD), the Victorian Department of Community Services and Health surveyed use of Lyodura in all private and public hospitals in Victoria.

Five hospitals responded, out of an uncertain total number. They reported use of 40 Lyodura grafts in 1985–1986 and 36 in 1986–1987, all in neurosurgical procedures (unpublished data).

Survey of neurosurgeons

In 1995, the Neurosurgical Society of Australasia surveyed 100 neurosurgeons (comprising all practising neurosurgeons and retired surgeons still on their register) about use of Lyodura or other dura mater during their practice lifetimes.13 This survey was also used to assess the feasibility of tracing recipients.

Sixty-five neurosurgeons responded (response rate, 65%); 35 of these (54%) had used dura mater grafts in cranial or spinal procedures at some time, with 34 having used Lyodura. Eleven of the 34 (32%) believed that they could identify over 90% of recipients from their records, nine (27%) that they could identify 50%–90%, and 14 that they could identify fewer than 50%. The survey could not determine the precise amount of Lyodura or specific batches used, nor the number of procedures involving Lyodura. However, all three cases of Lyodura-related CJD recognised before the survey were in patients of respondents who reported using Lyodura. The remaining two confirmed cases presented after this survey.

Survey of non-neurosurgical use

In 2001, the Department of Health and Aged Care (DHAC) undertook a survey through the Royal Australasian College of Surgeons (RACS) to assess non-neurosurgical use of Lyodura. Over 5000 practising and retired surgeons across 15 surgical specialties were asked about use of dura mater grafts (and specifically Lyodura) over their practice lifetimes.

Responses were received from 172 surgeons (response rate, 3.4%). This very poor response rate precluded meaningful analysis. However, the survey confirmed that Lyodura had been used in otorhinolaryngological procedures, such as tympanoplasty, myringoplasty and mastoidectomy, with respondents reporting use of about 100 grafts in such applications. Given the poor response rate, time elapsed since the product was withdrawn, recall bias and retirement of surgeons during this time, this may be a significant underestimate of use in non-neurosurgical applications. This possibility is supported by the US Centers for Disease Control and Prevention report that up to 20% of Lyodura use in the US was in non-neurosurgical applications (Dr L Schonberger, personal communication).

Quantification study

In 2002, the Department of Health and Ageing, in conjunction with the Australian National Creutzfeldt–Jakob Disease Registry, undertook a study to quantify Lyodura use in Australia and to determine the types of procedures in which it was used, as a basis for estimating the risk of Lyodura-associated CJD.

Methods: The Registry has ethical approval for its surveillance methods and activities from the University of Melbourne Human Research Ethics Committee.

As well as collating results of previous surveys, we asked the manufacturer of Lyodura, B Braun Melsungen AG, about supplies of Lyodura to Australia, including batch numbers and the total quantity of Lyodura imported into and distributed within the country. We also contacted other potential sources of information on Lyodura use, including the Australian Therapeutic Goods Administration and the Health Insurance Commission.

Results: Braun Melsungen indicated that:

Before 1978, about two to five packs of Lyodura per month were distributed nationally.

Between 1978 and 1982, 600 packs were distributed in mainland Australia.

Between 1983 and 1987, 1278 sheets were distributed for use in mainland Australia.

According to the 1985 Braun catalogue for Australia, Lyodura was available in a range of sizes, with some packs containing up to 6 pieces. The manufacturer indicated that packs sold in Australia contained either one large sheet or two smaller sheets. Its information suggests that generally the larger sheets were used for neurosurgery, and smaller sheets for other surgical applications. The single-sheet packs outsold the double-sheet packs by about three to one. Braun did not supply documentary verification of this information.

Neither the Australian Therapeutic Goods Administration nor the Health Insurance Commission could furnish further information on Lyodura use.

Based on the manufacturer’s information, and assuming that all distributed product was used, then a maximum of 750 sheets may have been used in Australia between 1978 and 1982. Before 1978, assuming the same distribution of pack sizes, then 180–450 sheets may have been distributed.

These data suggest a total use in Australia of 2208 to 2478 Lyodura grafts. Assuming maximum and equal annual use, then about 1172 Lyodura grafts were used in the higher-risk period 1982–1986.

Risk estimation: Risk estimates for Lyodura-associated CJD were based on data from the manufacturer alone, as survey results were very incomplete, and Lyodura use reported in the surveys was likely to be encompassed by the manufacturer’s information.

Risk estimates were calculated from the total number of individuals exposed and the number of CJD cases detected over defined periods, with 95% confidence intervals calculated using the Poisson distribution.

Based on the five Lyodura-associated CJD cases detected to 2003, the attack rate ranged from 1 in 496 patients who received Lyodura (0.20%) to 1 in 442 (0.23%) (Box 4), depending on whether a higher or lower estimate of pre-1978 use was used. In the higher-risk period (1982–1986), the attack rate may have been as high as 1 in 234 (0.43%). These calculations were based on exposure through any surgical application of Lyodura, assuming that only one sheet was used per patient and that all distributed product was used. The latter fact cannot be verified, and previous reports suggest that not all distributed Lyodura was necessarily used.16 These attack rates are therefore likely to be underestimates.

The attack rates after neurosurgery in Australia may be even higher, depending on the proportion of Lyodura that was used in this type of surgery. Should the proportion be close to 60% of the total product used (as suggested by the manufacturer) or 80% (as found in the US), the overall neurosurgical attack rate may be as high as 1 in 397 (ie, 80% of 496) to 1 in 265 (ie, 60% of 442), or 0.25% to 0.38%.

The CJD Registry also recently reviewed all records to determine whether any patients on the CJD register might have been exposed to Lyodura through non-neurosurgical applications. No potential cases were identified.

4: Risks of Creutzfeldt–Jakob disease (CJD) related to iatrogenic exposures in different countries

Country	Period	Deaths from CJD*	Recipients†	Risk (95% CI)

Lyodura
Japan	Overall (1979–2003)	97	220 000	0.04% (0.03%–0.05%)
	Higher-risk (1983–1987)	81	100 000	0.08% (0.06%–0.10%)
Australia	Overall (pre-1978–2003)	5	2 208‡	0.23% (0.07%–0.53%)‡
			2 478‡	0.20% (0.06%–0.47%)‡
	Higher-risk (1982–1986)	5	1 172	0.43% (0.14%–0.99%)
Human growth hormone
France	To 1999	55	1 361	4.04% (3.04%–5.26%)

* Based on cases of CJD with a history of recognised iatrogenic exposure during the defined period. † Based on estimated total national use of Lyodura or human cadaveric pituitary growth hormone during the defined period. ‡ A range exists for the number of Australian Lyodura recipients because of uncertainty about use before 1978.

Competing interests

None identified.

Acknowledgements

The Australian National Creutzfeldt–Jakob Disease Registry is funded by the Australian Department of Health and Ageing. The authors thank Associate Professor Peter Reilly (University of Adelaide) for many details of the Neurosurgical Society of Australasia survey of neurosurgeons, Dr L Schonberger (Centers for Disease Control and Prevention, USA) for estimated percentages for non-neurosurgical use of Lyodura, and Professor Emeritus Donald Simpson, Department of Neurosurgery, Royal Adelaide Hospital, SA, for assistance in reporting and evaluating patients with Lyodura-related CJD.

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(Received 26 Aug 2003, accepted 11 Dec 2003)