Jerome Cohen (孔傑榮)

再增錄一篇美國的台灣專家關心國內法治觀念逐漸消失的報導。2009-01-28

下面有三篇文章，都該收錄在我的部落格上。

Jerome Alan Cohen教授是紐約大學法律學教授，向來關心中國及台灣的政治、法律，曾經於1970年代從中國牢獄，營救出韓戰後被關閉二十年的飛行員John T. Downey而有名。馬英九就是他在哈佛大學任職時的學生。

Cohen教授最近透過Associated Press的記者PETER ENAV，嚴厲批評台灣法界行徑，在法務部長王清峰領頭的「法律日」，由法官、檢察官、及其他法界高階人士一起聚會中，用諷刺喜劇嘲弄正在被調查、尚未定罪的陳水扁。這種在文明國家不可能見到的、毫無法學修養的行為，他說好像是馬戲團上演，不可思議（unthinkable）；只在七、八個月內，有人意欲將台灣政治過去十五年來的進步一掃而光。他還指出只有馬英九才能糾正這種風氣。(這正指出台灣法治精神的大幅退步，是馬本人的所為！) 台灣的法界人士的守法觀念已經淪落到令外國人關心、投書，法務部、總統府還不願承認錯誤、改進，才是我們該痛心、警戒的重點。

再看金恆偉揭穿偏藍、偏國民黨的新聞媒體如何扭曲這位「孔傑榮」教授幾篇批評目前台灣國民黨政府的文章。當然這只是舉例，其他扭曲事實的、不合記者職業倫理的報導，是「罄竹難書」。台灣民眾能夠依賴這種報紙得到正確的訊息、判斷國家政策是否正確，才怪！

馬英九上任「中華民國總統」大位之後的所作所為，皆以和中國迅速統一為唯一目標，除此之外幾乎樣樣違反三月總統選舉前對台灣選民的承諾。再加上其能力之差，不僅國內藍綠兩派老少都同意，連新加坡李光耀都會批評他的作風[缺少智慧]，似是打牌時掀光底牌。這種沒有誠信又腦筋不夠靈光的人，不要說是政治領導人，連一個學校、公司恐怕都無法讓他主持。

看看目前國家經濟已將陷入窘境，其次將是國防崩潰、主權消失。前幾天還透露：中華民國外交部的日曆竟有中共國慶日、毛澤東生日列為假日，才駭人聽聞。當然這不是無能，這是他們為了試試水溫、看國民反應而下的小手腳。一國的外交部門無恥至此，還能說什麼！

現在很多人因失業、生活陷入困境，小孩失學，有人餓死，有人凍死。作為一國領導者，還會電視前說笑、裝可愛；去購買游泳褲，故意張在電視前，是否把選民當傻瓜？妳們還不會噁心？

國民黨政權逐漸恢復過去控制人民行為的社會，設法繼續其政權，維護自身利益。很多國民黨高官極力傾中，還不是為他們自己家人在中國的商業利益？他們才不理你一般民眾勞工的權益！這些高官權貴的家屬在中國做什麼生意，可以要求他們列舉，馬上就知道。

觀察到其他文明國的政治家極度強調「誠信」、重視「民眾感受」、「負責任」的態度；又看著馬戲團處處奸詐背信，全面向他心中的專制母國傾斜，拆除自家國旗國名、更禁唱台灣歌曲，來迎接中國人，這種無恥的模樣，實在是世界奇聞奇觀。可是民調還有百分之四十的信任度！台灣要自稱是文明國家，確實是還有一大截民眾公民教育的空間。

馬英九過去數十年反台灣、反台灣人、反台灣民主的行為，表示他的性格已是如此，還以為台灣主權不會被他損害，是太過「天真」了；忘記了國民黨貪污多年，還沒有一人被關入牢，各個逃亡中國或其他國家，逍遙法外，是極危險的「健忘」。

如果大多數台灣人寧願放棄民主、人權，向中國專制政權屈膝投降，則另當別論。可能世界上還有一些不管你死活的國家，稱讚台灣人犧牲自己權益的精神，會促進世界和平呢！不過目前不是有高達百分之八十的民眾還認同台灣為主權國，並自認是台灣人，引以為傲？比兩千三百萬人口更小，科技工業發展更落後的國家，都還能維持自立、繁榮。為什麼還要傾中，變得非依賴對台灣主權有強烈敵意的中國不可？

國家認同的原則在於生命理念的一致，而不在於DNA的相似度或文化的同源；不能為了賺更多錢，而犧牲自由與人權；更不應該受心存不軌的政治集團操縱，只盯住眼前一小件問題、逞一時之快，而出賣國家前途。

在台灣的居民，要生活在自由民主、重視人權的國家，或是要成為無權自決的地方二等國民、受人宰割、自取其辱，都是你（妳）自己的決定。

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TAIWAN: US-based Taiwan experts add names to open letter

Among the judicial concerns from international academics and writers is leaks to the media regarding ongoing cases

Taipei Times
Saturday, January 24, 2009

By William Lowther

WASHINGTON, D.C. --- Two important Taiwan experts based in Washington have added their names to the open letter published in the Taipei Times earlier this week expressing concern about what they see as an erosion of justice in Taiwan.

The new signatories are former deputy assistant secretary of state for East Asian and Pacific Affairs Randall Schriver and George Washington University academic Michael Yahuda.

In the original letter a group of international academics and writers urged President Ma Ying-jeou to order an independent inquiry into the way police squashed protests during the visit of Chinese envoy Chen Yunlin.

The letter said: "The establishment of a scrupulously neutral commission is essential if there is to be a fair and objective conclusion on the disturbances that occurred during the Chen Yunlin visit."

Freedom House, Amnesty International and US professor Jerome Cohen have also strongly recommended an independent inquiry.

At the same time, the group has expressed concern about the legal proceedings in the case of former president Chen Shui-bian and political pressure from KMT members of the Legislative Yuan that preceded a switch from a three-judge panel that had released the former president on his own cognizance to a court that subsequently detained him again.

The letter said there had been a "widespread pattern of leaks to the media regarding ongoing cases -- leaks, which because of their content and nature can only have come from the prosecutors' offices."

It mentioned a recent skit in which some prosecutors involved in Chen's case poked fun at the former president.

"This pattern of behavior displays a distinct bias in the judicial system and a disregard for fair and impartial processes," it said.

It concluded by again urging Ma "to ensure that your government and its judiciary and parliamentary institutions safeguard the full democracy, human rights and freedom of expression."

In an article published this week in its Taiwan Communique, the Washington-based Formosan Association for Public Affairs said the last few months had seen a further erosion of human rights and democracy in Taiwan .

It said the downward slide started in mid-October with the arrest and detention of former and present officials of the Democratic Progressive Party administration and worsened with aggressive police behavior during the Chen Yunlin visit in early November.

"Both developments were reminiscent of Taiwan 's police state under the Kuomintang's [KMT] martial law, which lasted from 1947 until 1987," the article said.

It quoted Cohen, who was Ma's law professor at Harvard, as saying that the recent court proceedings against Chen Shui-bian "mocked the promise" of fairness.

"At what point does the presumption of innocence become meaningless and the pre-conviction detention morph into punishment for a crime not finally proved?" Cohen asked.

Date Posted: 1/24/2009

 

Mentor urges Taiwan 's Ma to ensure trial fairness

By PETER ENAV,Associated Press Writer AP - Saturday, January 17

TAIPEI, Taiwan - The Harvard Law School mentor of Taiwan 's President Ma Ying-jeou said Saturday that his former student needs to urgently act to prevent an "increasingly disturbing circus atmosphere" from prejudicing his predecessor's right to a fair trial.

Jerome Cohen's comments to The Associated Press follow last week's biting skit that mocked former President Chen Shui-bian, performed by prosecutors at a Taiwan "Law Day" dinner attended by Justice Minister Wang Ching-feng, judges, and other leading lights of the island's legal community.

The skit made fun of Chen at the time of his arrest on graft charges in November, with a woman depicting him waving his manacled hands in the air and complaining about police brutality and judicial persecution.

At least one of the prosecutors appearing in the skit is involved in the continuing investigation of Chen.

Chen goes on trial Monday on charges of money laundering, bribery and looting a special presidential fund. While admitting his family wired more than $20 million to overseas bank accounts under its control, he says the money was unused campaign contributions that under Taiwanese law are his to deal with freely.

Cohen, now a law professor at New York University , is one of the world's foremost experts on legal systems in Taiwan and China . He mentored Ma three decades ago at Harvard and they remain close. They last met in the presidential office in Taipei on Dec. 16.

In a telephone interview with the AP, Cohen called performing the skit "unthinkable."

"It is as if there are people trying to repudiate all the progress that Taiwan has made over the past 15 years," he said, referring to the island's gradual transition from dictatorship to multiparty democracy.

Cohen said Ma should move swiftly to force Justice Minister Wang to clarify remarks she made in defense of the skit, including her characterization that it was, "just a little performance reflecting on current affairs."

"If he doesn't get this, who in Taiwan can get it?" he said.

Presidential spokesman Wang Yu-chi said Ma would not intervene with the justice minister on Chen's trial.

"President Ma Ying-jeou's stand is that he respects the judiciary and he does not interfere with individual cases," he said. "At the same time he hopes that the judiciary will behave in a way that does not induce improper political reactions on the part of the public."

There is "an increasingly disturbing circus atmosphere" surrounding the Chen trial, Cohen said, that includes not only the mounting of the skit, but also a decision by a Taipei court to reverse itself and order Chen jailed pending his trial.

Chen was originally locked up for 32 days to allow prosecutors to build their case against him, then ordered freed on his own recognizance by the court. However, he was returned to jail on Dec. 29 after a new panel of judges heard the prosecutors' second appeal against him.

Cohen said Ma's handling of the Chen case revealed an apparent choice to placate the extremist wing of his ruling Nationalist Party rather than reaching out to Taiwan 's broad political middle.

"He's acting like (President George W.) Bush in catering to the most right wing conservative elements," he said.

Presidential spokesman Wang rejected that characterization.

"(Ma's) hands-off policy has irritated some party members who hoped he could deal with the Chen case more aggressively," he said. "So it is incorrect to say that President Ma is trying to placate some party members with the way he is treating the Chen case."

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《中時》KUSO孔傑榮

《中國時報》裁員改版，號稱「精英報」。表現在形式上的改變，就是在每一版上打出「編輯」的「名字」，以此向讀者保證，並示負責。

確實，「傳媒詐術」的手法之一，就是在標題上玩把戲。編輯台為記者所寫的報導下標題時，有意作偽來誤導視聽；反正躲在文字之後，不怕被人指責或揭穿。《中時》公開亮出編輯的大名以昭大信；當然表示「童叟不欺」。

然而，《中時》在「時報廣場」上的「孔傑榮專欄」，卻是不折不扣的「黑心貨」；版面上公然掛上「總主筆」及「編輯」的名字，譯文後敢於附上譯者大名。如此陣仗更彰顯是「有意」的「詐欺」；三重的「保證」的目的，不過是鬆懈讀者戒心而吃下文字的「三聚氰胺」。

去年 十一月十三日 ，「孔傑榮的專欄」痛斥馬政府，在北京兩岸首腦陳雲林訪台時，「逾越了自由社會的界限」，並且質疑司法沒有嚴守「政治中立」以及違反「無罪推論」的基本原則，甚而指控「喚醒『戒嚴黑暗時期』不義程序的幽靈」。作者的題目非常明顯〈為了兩岸關係不惜犧牲台灣公民自由〉，譯文卻胡謅成〈陳雲林旋風颳人權問題〉，完全取消強有力的批判性。這種扭曲同樣見於內文，比如作者用描述性文字說：「台灣警方無法保護 陳 先生副手」，譯文卻成為「張銘清的『攻擊』事件」；這與陷人於罪的「官方」說法完全一致！

一月八日，「孔傑榮專欄」極為精采。譯者是「紐約大學法學院亞美法研究員宋名晰」，理應專家譯筆，依然是「黑心貨」。原文題目一針見血：〈扁案法官已經毀了他們的機會〉，譯文卻讓人摸不著頭腦的曖昧：〈對扁案的追訴：第一階段〉，什麼又什麼的！因此內文第一段出現的「毀了」，譯文變成了不知所云的「貽誤」；孔傑榮論及「法院受理本案的程序」，強烈的抨擊「已經嘲弄了社會期待」，譯文卻用輕鬆的字眼「期待落空」取代。

孔傑榮非常注意扁案的發展，他認為羈押庭決定收押陳水扁，是明顯「違反」大法官釋字第六五三號，譯文卻用「矛盾」一筆帶過。至於把「不利證據」改成「犯罪證據」，不只不專業，也暴露了內心的「黑暗」。

譯文的胡掰多不可數，最後再引一例。孔文用問句表達對台灣司法政治化的不信任：「有沒有政治人物秘密的恐嚇法院？」譯文作為「有無政治人物脅迫該法院」，為何「秘密」兩字不見了？

《中時》已脫手給「旺旺集團」；新老闆是坐視還是有意？這才是重點。

（作者金恆煒，當代雜誌總編輯）

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信馬者能怪誰？

民進黨執政時籌設的「二二八國家紀念館」，在馬英九上台後立即停工，預算更遭到國民黨佔絕對多數的立法院刪除，引發二二八受難家屬不滿，批評多次為二二八事件表達歉意的馬英九邪惡，違信背義，說一套做一套。

其實，二二八家屬根本不必嚴厲斥責馬英九，因為他說一套做一套的作風，是「一路走來、始終如一」，何曾變過？言行不一，固然是他的錯，但相信他的人，只能怪自己。

尤其，在主權與認同問題上，馬英九會隨著客觀環境不同，因應情勢所需，拋出各種不同的說法，特別是選舉時，只要能騙到選票，什麼違背自己信念，或者肉麻的話都說得出口。（他還會陪著受害者哽咽呢！）最可怕的是，他說起這些謊言來，還是一副非常誠懇的表情，因此，如果他說太陽從西邊出來，恐怕都會有人相信。

「燒成灰都是台灣人」，這是何等莊嚴的應許，縱使一輩子愛台灣，死生不改其志的人，可能都說不出口，唯恐自己努力不夠，信念不夠堅定，會褻瀆了自我的良知。

匪夷所思的是，身體裡流著終極統一血液，精神上懷抱著大中國情結的馬英九，說起此話來，竟然如此自然、順口、輪轉，絕不會跳針，一點都沒有勉強的感覺。聽到這麼感人的話，選民若不把票投給他，簡直是沒有良心。

 

President Barack Obama's inaugural address

今天是半非裔的Barack Obama就職美國第四十四任總統的日子。他的父親是肯亞人，曾到哈佛攻讀經濟學博士學位，但從小遺棄他，母親也是似嬉皮型的女人，小時在印尼生長、上國小。之後在夏威夷由祖父母養育，進入哈佛大學法學院，終於四十八歲被選為全世界最有權力的人，這段故事很令人感動。好高興我能見證美國、也可能是世界最歷史性的一天

美國是由移民組成的、依據兩百年前先人定下的理念、最尊重民主自由平等人權的國家。不過對非裔的種族起歧視一直持續。六十年前他的父親要去餐廳，會被拒絕座位；我四十三年前到美國，黑人還得坐在巴士後面。但是一旦民智開啟，發覺平等自由與人權不應該只限於白人，由政府立法，種族間的隔閡逐漸被打破。看這個白人佔最多數的國家，能夠如此憑良心，捨棄成見，選賢與能，決定前途，我深信她會繼續成為領導世界的模範國，「民主、平等、自由、人權」終究會擴展到全世界。

他本人的文才也很好，這篇總統就職演說，據說是他自己主筆的。

President Barack Obama's inaugural address on January 20, 2009.

OBAMA: My fellow citizens:

I stand here today humbled by the task before us, grateful for the trust you have bestowed, mindful of the sacrifices borne by our ancestors. I thank President Bush for his service to our nation, as well as the generosity and cooperation he has shown throughout this transition.

Forty-four Americans have now taken the presidential oath. The words have been spoken during rising tides of prosperity and the still waters of peace. Yet, every so often the oath is taken amidst gathering clouds and raging storms. At these moments, America has carried on not simply because of the skill or vision of those in high office, but because we the people have remained faithful to the ideals of our forebears, and true to our founding documents.

So it has been. So it must be with this generation of Americans.

That we are in the midst of crisis is now well understood. Our nation is at war, against a far-reaching network of violence and hatred. Our economy is badly weakened, a consequence of greed and irresponsibility on the part of some, but also our collective failure to make hard choices and prepare the nation for a new age. Homes have been lost; jobs shed; businesses shuttered. Our health care is too costly; our schools fail too many; and each day brings further evidence that the ways we use energy strengthen our adversaries and threaten our planet.

These are the indicators of crisis, subject to data and statistics. Less measurable but no less profound is a sapping of confidence across our land — a nagging fear that America 's decline is inevitable, and that the next generation must lower its sights.

Today I say to you that the challenges we face are real. They are serious and they are many. They will not be met easily or in a short span of time. But know this, America — they will be met.

On this day, we gather because we have chosen hope over fear, unity of purpose over conflict and discord.

On this day, we come to proclaim an end to the petty grievances and false promises, the recriminations and worn out dogmas, that for far too long have strangled our politics.

We remain a young nation, but in the words of Scripture, the time has come to set aside childish things. The time has come to reaffirm our enduring spirit; to choose our better history; to carry forward that precious gift, that noble idea, passed on from generation to generation: the God-given promise that all are equal, all are free and all deserve a chance to pursue their full measure of happiness.

In reaffirming the greatness of our nation, we understand that greatness is never a given. It must be earned. Our journey has never been one of shortcuts or settling for less. It has not been the path for the faint-hearted — for those who prefer leisure over work, or seek only the pleasures of riches and fame. Rather, it has been the risk-takers, the doers, the makers of things — some celebrated but more often men and women obscure in their labor, who have carried us up the long, rugged path towards prosperity and freedom.

For us, they packed up their few worldly possessions and traveled across oceans in search of a new life.

For us, they toiled in sweatshops and settled the West; endured the lash of the whip and plowed the hard earth.

For us, they fought and died, in places like Concord and Gettysburg ; Normandy and Khe Sanh.

Time and again these men and women struggled and sacrificed and worked till their hands were raw so that we might live a better life. They saw America as bigger than the sum of our individual ambitions; greater than all the differences of birth or wealth or faction.

This is the journey we continue today. We remain the most prosperous, powerful nation on Earth. Our workers are no less productive than when this crisis began. Our minds are no less inventive, our goods and services no less needed than they were last week or last month or last year. Our capacity remains undiminished. But our time of standing pat, of protecting narrow interests and putting off unpleasant decisions — that time has surely passed. Starting today, we must pick ourselves up, dust ourselves off, and begin again the work of remaking America .

For everywhere we look, there is work to be done. The state of the economy calls for action, bold and swift, and we will act — not only to create new jobs, but to lay a new foundation for growth. We will build the roads and bridges, the electric grids and digital lines that feed our commerce and bind us together. We will restore science to its rightful place, and wield technology's wonders to raise health care's quality and lower its cost. We will harness the sun and the winds and the soil to fuel our cars and run our factories. And we will transform our schools and colleges and universities to meet the demands of a new age. All this we can do. All this we will do.

Now, there are some who question the scale of our ambitions — who suggest that our system cannot tolerate too many big plans. Their memories are short. For they have forgotten what this country has already done; what free men and women can achieve when imagination is joined to common purpose, and necessity to courage.

What the cynics fail to understand is that the ground has shifted beneath them — that the stale political arguments that have consumed us for so long no longer apply. The question we ask today is not whether our government is too big or too small, but whether it works — whether it helps families find jobs at a decent wage, care they can afford, a retirement that is dignified. Where the answer is yes, we intend to move forward. Where the answer is no, programs will end. Those of us who manage the public's dollars will be held to account — to spend wisely, reform bad habits, and do our business in the light of day — because only then can we restore the vital trust between a people and their government.

Nor is the question before us whether the market is a force for good or ill. Its power to generate wealth and expand freedom is unmatched, but this crisis has reminded us that without a watchful eye, the market can spin out of control — and that a nation cannot prosper long when it favors only the prosperous. The success of our economy has always depended not just on the size of our gross domestic product, but on the reach of our prosperity; on our ability to extend opportunity to every willing heart — not out of charity, but because it is the surest route to our common good.

As for our common defense, we reject as false the choice between our safety and our ideals. Our founding fathers, faced with perils we can scarcely imagine, drafted a charter to assure the rule of law and the rights of man, a charter expanded by the blood of generations. Those ideals still light the world, and we will not give them up for expedience's sake. And so to all the other peoples and governments who are watching today, from the grandest capitals to the small village where my father was born: know that America is a friend of each nation and every man, woman, and child who seeks a future of peace and dignity, and that we are ready to lead once more.

Recall that earlier generations faced down fascism and communism not just with missiles and tanks, but with sturdy alliances and enduring convictions. They understood that our power alone cannot protect us, nor does it entitle us to do as we please. Instead, they knew that our power grows through its prudent use; our security emanates from the justness of our cause, the force of our example, the tempering qualities of humility and restraint.

We are the keepers of this legacy. Guided by these principles once more, we can meet those new threats that demand even greater effort — even greater cooperation and understanding between nations. We will begin to responsibly leave Iraq to its people, and forge a hard-earned peace in Afghanistan . With old friends and former foes, we will work tirelessly to lessen the nuclear threat, and roll back the specter of a warming planet. We will not apologize for our way of life, nor will we waver in its defense, and for those who seek to advance their aims by inducing terror and slaughtering innocents, we say to you now that our spirit is stronger and cannot be broken; you cannot outlast us, and we will defeat you.

For we know that our patchwork heritage is a strength, not a weakness. We are a nation of Christians and Muslims, Jews and Hindus — and non-believers. We are shaped by every language and culture, drawn from every end of this Earth; and because we have tasted the bitter swill of civil war and segregation, and emerged from that dark chapter stronger and more united, we cannot help but believe that the old hatreds shall someday pass; that the lines of tribe shall soon dissolve; that as the world grows smaller, our common humanity shall reveal itself; and that America must play its role in ushering in a new era of peace.

To the Muslim world, we seek a new way forward, based on mutual interest and mutual respect. To those leaders around the globe who seek to sow conflict, or blame their society's ills on the West — know that your people will judge you on what you can build, not what you destroy. To those who cling to power through corruption and deceit and the silencing of dissent, know that you are on the wrong side of history; but that we will extend a hand if you are willing to unclench your fist.

To the people of poor nations, we pledge to work alongside you to make your farms flourish and let clean waters flow; to nourish starved bodies and feed hungry minds. And to those nations like ours that enjoy relative plenty, we say we can no longer afford indifference to the suffering outside our borders; nor can we consume the world's resources without regard to effect. For the world has changed, and we must change with it.

As we consider the road that unfolds before us, we remember with humble gratitude those brave Americans who, at this very hour, patrol far-off deserts and distant mountains. They have something to tell us, just as the fallen heroes who lie in Arlington whisper through the ages. We honor them not only because they are guardians of our liberty, but because they embody the spirit of service; a willingness to find meaning in something greater than themselves. And yet, at this moment — a moment that will define a generation — it is precisely this spirit that must inhabit us all.

For as much as government can do and must do, it is ultimately the faith and determination of the American people upon which this nation relies. It is the kindness to take in a stranger when the levees break, the selflessness of workers who would rather cut their hours than see a friend lose their job which sees us through our darkest hours. It is the firefighter's courage to storm a stairway filled with smoke, but also a parent's willingness to nurture a child, that finally decides our fate.

Our challenges may be new. The instruments with which we meet them may be new. But those values upon which our success depends — hard work and honesty, courage and fair play, tolerance and curiosity, loyalty and patriotism — these things are old. These things are true. They have been the quiet force of progress throughout our history. What is demanded then is a return to these truths. What is required of us now is a new era of responsibility — a recognition, on the part of every American, that we have duties to ourselves, our nation, and the world, duties that we do not grudgingly accept but rather seize gladly, firm in the knowledge that there is nothing so satisfying to the spirit, so defining of our character, than giving our all to a difficult task.

This is the price and the promise of citizenship.

This is the source of our confidence — the knowledge that God calls on us to shape an uncertain destiny.

This is the meaning of our liberty and our creed — why men and women and children of every race and every faith can join in celebration across this magnificent Mall, and why a man whose father less than sixty years ago might not have been served at a local restaurant can now stand before you to take a most sacred oath.

So let us mark this day with remembrance, of who we are and how far we have traveled. In the year of America 's birth, in the coldest of months, a small band of patriots huddled by dying campfires on the shores of an icy river. The capital was abandoned. The enemy was advancing. The snow was stained with blood. At a moment when the outcome of our revolution was most in doubt, the father of our nation ordered these words be read to the people:

"Let it be told to the future world ... that in the depth of winter, when nothing but hope and virtue could survive...that the city and the country, alarmed at one common danger, came forth to meet it."

America, in the face of our common dangers, in this winter of our hardship, let us remember these timeless words. With hope and virtue, let us brave once more the icy currents, and endure what storms may come. Let it be said by our children's children that when we were tested we refused to let this journey end, that we did not turn back nor did we falter; and with eyes fixed on the horizon and God's grace upon us, we carried forth that great gift of freedom and delivered it safely to future generations.

Thank you. God bless you. And God bless the United States of America .

 

霍亂菌Vibrio cholerae及 腸炎弧菌Vibrio parahemolyticus

新聞報導南非洲的辛巴威（Zimbabwe）霍亂流行，到2009年 一月十七日 為止，已經有約五萬人感染，兩千兩百多人死亡。想起部落格上該有霍亂菌Vibrio cholerae，及同類細菌、但病況較不嚴重、傳染性較低的腸炎弧菌Vibrio parahemolyticus簡介。

也想起亞洲1960-1962年 El Tor vibrio strain 的大流行。台灣農產品出口頓減，經濟損失慘重，政府後來似乎也盡量報喜不報憂。當時我是實 習 醫師，曾經到「公共衛生教學示範中心」努力一番，想要做實驗、寫論文。當時這一段故事也是值得懷念。

Vibrio cholerae (霍亂菌)

l       Comma-shaped(弧形) 革蘭陰性桿菌。可在6%NaCl中生長(而異於另一個親水細菌Aeromonas)。

l       只對人致病引起cholera(霍亂)。

l       最重要的血清型為serovar O:1（唸成 “歐one”，不是 “零一”）及serovar O:139。O:1有兩個biotypes：classical (epidemic)及El Tor (endemic也可引起epidemic)；O:1又有3個次血清型：Ogawa(小川)、Inaba(稻葉)及Hikojima(彥島)。O:139於1992年自孟加拉灣地區開始流行﹐已成為目前最重要的霍亂病原。在台灣因為生食甲魚蛋而有一胃切除之病人得病。是三十餘年來手首度本土性霍亂案例。細菌則可能是在飼主由大陸帶入之飼料中。

l       有纖毛，可動，有mucinase(黏液分解酵素)；可附著在腸上皮接受體。

l       此菌之特徵為能在鹼性pH8.5-9.0中繁殖，因此可利用它從其他多種細菌中分離出來。

l       在胃酸中會死亡。因此無胃酸或常抑制胃酸者容易得病。

l       無症狀帶菌者(asyptomatic carriers)為感染原﹔可在鮮水中生長而經水傳染。人對人之感染少見。

l       潛伏期﹕2-3 days。

病變﹕

l       產生enterotoxin(腸毒素)導致極嚴重的水瀉、腹瀉，糞便看似洗米水 (rice water stool)，水份電解質流失、脫水(dehydration)、休克(shock)。不治療時mortality(死亡率) 40 - 60%，早期治療時1%。

診斷﹕

l       培養糞便於enrichment medium (增殖培養劑，alkaline peptone water, pH 9) 35°C中18小時，然後次培養(subculture)在TCBS (thiosulfate citrate bile salt sucrose agar)中35°C、3 - 6小時。

防治﹕治療可用iv fluid 或 oral fluid

l       Oral fluid口服法： glucose 20 g m /L (或sucrose 40 g m /L)+ NaCl 3.5 g m /L + KCl 1.5 g m /L + trisodium citrate 2.9 g m /L or NaHCO₃ 2.5 g m /L(最簡單的泡法為one liter飲用水+ 5 g rams鹽 + 40 g rams糖)(水加糖可以促進鹽份之吸收，因而可以不用靜脈注射水份電解質就可治療)。輕度脫水(體重之5 %)及中度脫水(體重之7 %)可在4-6小時內以口服法補充失去的水份量。以後則每4小時以口服法補充水樣糞便量之1.5倍。

l       有休克時以IV fluid (Na ion 130 m Eq/L + K ion 10 -15 m Eq/L + bicarbonate or acetate or lactate ions 25 -48 m Eq/L)，可用Ringer’s solution或WHO “diarrhea treatment solution” ( 4 gm NaCl + 1 gm KCl + 0.5 gm sodium acetate + 8 gm glucose in one L)或Dacca solution ( 5 gm NaCl + 4 gm NaHCO3 + 1 gm KCl/L)。第一小時給30 mL/Kg for infants； 對一歲以上者要30 分鐘內IV 給予。以後視病人之情況可改為口服。

l       抗生素可以縮短病程、減少水份排出量、及細菌之排放量。可用以下幾種藥，但世界各地區之Vibtio cholerae抗藥性不同，應先瞭解：

Ø       tetracycline 500 mg, qid (or 12.5 mg/kg, qid for children), 3 days;

Ø       TMP/SMZ (320 mg/1600 mg) bid, (or 8 mg/40 mg/kg for children in 2 divided doses), 3 days; (O139 strain對此藥有抗藥性)；

Ø       Furazolidine 100 mg, qid (or 1.25 mg/kg, qid for children), 3 days; 

Ø       Erythromycin 250 mg qid (or 10 mg/kg, tid for children), 3 days。

Ø       Ciprofloxacin 30 mg/kg, one dose, 不超過1 gm.或15 mg/km, bid, 3 days。

l       第一類法定傳染病，應立即報告，並強制隔離。

l       要找出感染原，如有可能進行消毒。

l       加強民眾衛生教育，不需要時少服抑制胃酸的藥物。

l       用killed whole cell vaccine with or without nontoxic cholera toxin可以有69 %(前者) 或41 %保護；live attenuated oral vaccine也正在研發中。以前用的im killed cholera vaccine效果不佳、副作用大。

 

Vibrio parahemolyticus (腸炎弧菌)

l       Comma-shaped(弧形) 革蘭陰性桿菌。可在6%NaCl中生長(而異於另一個親水細菌Aeromonas)。

l       Halophilic (嗜鹽的；salt-loving)。常污染海鮮食品，引起急性胃腸炎群突發感染。

l       產生cytotoxin(細胞毒素)，引起腹瀉。

l       潛伏期：8小時到2天。

l       需特殊培養劑：TCBS，18小時；35°C.。

（採自2002年許清曉著『傳染病防治手冊』，最有用的中英文索引Index佔全書三分之一，可在藝軒圖書公司購得。以上是有關此病原的基本常識。最新資料還是要從網路或圖書館醫學雜誌取得。）

 

 

 

漫談真性多血症

[景福醫訊2009-10, 26卷10期，pp.2-7]

 

自己得了此病，才學到「真性多血症」（polycythemia vera; PV）近四年來有一個突破性發展，不僅診斷方法翻新，以後也可能針對病因標靶治療。

八年前偶而感覺會有極輕微的眩暈，而且持續兩、三個星期。自己驗一下血液CBC（complete blood count；全血球計數），但也忘了看結果。過了約一星期，檢驗科同仁在走廊上趕上我，說，「 許教授，你的血球數都很高哦」。果然，HGB是17.7 gm% (成人男性正常值，12.9-17.9)，HCT 52.5% (38-53)，RBC 626萬/uL(420-620)，血小板61.6萬/uL (12-40)，WBC白血球9,300 /uL(3,600-9,800)。雖然HCT、WBC都還不太令人緊張，但是血小板如此高，確定是有不能小視的問題。

趕緊查閱文獻，徵詢血液專科的同學，似是得了「真性多血症」（polycythemia vera; PV）。馬上找自家的陳主任、和信的褚主任，做骨髓檢查。一星期內放血(phlebotomy)一千三百CC。再經過老友郭承統的強力推薦，到他的長庚醫院見 施麗雲 醫師。 施 醫師是台灣多血症專家，研究 Endogenous erythroid colony (EEC) assay (EPO-independent erythroid colony growth; BFU-E)，她發表的方法，可以在發病之前就準確地診斷真性多血症，受國際重視十餘年。不僅如此，看病之用心、仔細，讓我這個美國訓練、強調仔細看病的醫師非常佩服。

以後接受各項傳統必需的檢查，再加EEC assay，還做 MRA (腦部核磁共振加血管造影術) 排除腦部暫時性缺氧（TIA）的可能，已經確定有「真性多血症」。其後三年只用放血的方法降低紅血球數，同時每天開始服用aspirin。但是血小板逐漸增加到85萬以上。深怕發生合併症，於是開始服用抑制骨髓細胞增生的 hydroxyurea。Hydroxyurea的療效很好，副作用幾乎沒有，比較常見的是高劑量時指甲會變色、皮膚會變黑。不過不會抑制免疫力、身體抵抗力，似乎也不會像是radioactive P32治療，促進PV演變成為白血病。用藥四年多，自己調整劑量，目前血小板、HCT、WBC都在正常範圍之內。

多血症有「真性」的、以及「續發性」的（secondary polycythemia）。續發性的多血症是在多年吸煙、慢性肺疾病、住在高山地區者可以見到，是因為血中氧氣濃度低，刺激erythropoietin (EPO)分泌而引起的。

EPO是主要從腎臟皮質的間質細胞（peritubular fibroblast）釋出的hormone。在血液中有使紅血球半生期延長、促進紅血球的分化、成熟、以及合成血色素的作用，（其實它還可以促進神經損傷恢復以及血管及平滑肌增生、傷口癒合）。它也刺激骨髓製造紅血球。第一步，和骨髓內幹細胞（stem cells）表面的EPO接受器（Epo-receptor; EpoR）結合，再經過細胞內酵素JAK2傳遞訊息到細胞核，促進紅血球的分裂、增多。總效果就是血中紅血球數增加。

EPO的分泌多寡是由血液中氧氣濃度調節。肺部功能差，或住在高山上，血中氧氣濃度低等情況之下，EPO的分泌加快，隨後血中紅血球增加，提升血液輸送更多氧氣的效能。因此，續發性多血症時血中EPO會增加；而真性多血症時EPO不增，卻仍會有紅血球不斷地產生，顯然是細胞內的訊息傳導系統出了毛病。這些有病變的PV骨髓細胞在試管內雖然沒有添加EPO，仍會繁殖(在體外就成EEC assay)。

 

 

酵素被命名為JAK，原來是 “Just Another Kinase” (“只是又一個酵素”) 的簡稱，但是因為這類分子群有兩種相反的作用，一為促進酵素作用，另一為抑制其作用，因此後來改稱為Janus。Janus是希臘神話中的守門神，有一個頭兩個臉，面向相反方向（January是一年之開始，janitor是看門人或學校及公司工友，都源於這個把守第一關的門神名稱）。Janus kinases有幾組，其功用都和在細胞內傳導訊息有關，例如各種hormones、或刺激細胞的cytokines（細胞激素），接觸細胞表面後，就是靠JAKs傳遞訊息到細胞核，才能夠發揮功能。

真性多血症，PV （以前的名稱真多，primary polycythemia; polycythemia rubra vera; myeloproliferative disorder; erythremia; splenomegalic polycythemia; Vaquez's disease; Osler's disease; polycythemia with chronic cyanosis - myelopathic polycythemia; erythrocytosis megalosplenica; cryptogenic polycythemia。似乎每一位專家都想自己給這個病命名，爭當多血症之父！），是骨髓幹細胞有後天性突變而引起的血球異常增生，增生的骨髓細胞是單株性的，因而都呈現一些特定的異常karyotype（核型）。單株性的細胞增生就是癌症細胞的特徵。

PV還有一點和續發性多血症不一樣，不僅血流中紅血球多，連嗜中性白血球、血小板都會增加，骨髓中可以看到異常密集的、產生這三種細胞的前身：erythroid cells、 myeloid cells（產生granulocytes）（granulocytes＝顆粒性白血球，包括: neutrophils=嗜中性白血球, eosinophils＝嗜伊紅性白血球, 及basophils＝嗜鹼性白血球）、 及megakaryocytes(產生血小板)。

PV因為紅血球太多，血液變得濃稠而流通遲緩，而會有很多種徵候：面紅、頭痛、耳鳴、眩暈、視力障礙、呼吸困難、皮癢(40%)、血管梗塞(15-60%)、靜脈炎、出血傾向（15-35%; 可能是血小板功能有異常）、脾腫（75％）、肝腫（40％）。可能發生的合併症是：痛風、胃出血、Budd-Chiari syndrome（肝靜脈梗塞）、心臟衰竭、心肌梗塞、中風、骨髓纖維化（myelofibrosis），白血病等。約有20％病人有和血管梗塞相關的徵候，例如靜脈炎、中風、心肌梗塞、視力模糊、肝靜脈梗塞等問題。追蹤10年、20年後，約有10％到30％再發生骨髓纖維化、骨髓外造血（extramedullary hematopoiesis）。只用放血治療，低於5％的PV會演變成為白血病，不過依照使用的治療法（如P32），這個比例會更高。

有點奇怪，為什麼所有教科書上寫的PV徵候都是如此嚴重，我怎麼只有輕度的眩暈？想來我是很早就注意到極輕的症狀，就做了CBC，又找到 施 醫師做EEC assay，因此在早期就開始追蹤。同時也是有副作用如此少的hydroxyurea，因此可以讓醫師不猶豫地早下藥，將紅血球數及血小板數壓在正常值以內，因而合併症都來不及發生。不過hydroxyurea並不會延緩疾病的過程，因此更可能的原因是，PV的臨床呈現方式多樣，我是屬與較為徵候少的、進行慢的，以後才可能逐漸嚴重。

臨床診斷PV。因為不知道基本的病因，1970年代Polycythemia Vera Study Group (PVSG)設定一套要診斷PV時的條件如下：

主要條件：

       A1 紅血球體積(Red cell mass; RCM)增加, 男性 > 36  mL/kg, 女性 > 32 mL/kg

A2 動脈血氧氣飽和度 (aO2sat) > 92%

A3 脾腫

次要條件：

B1 血小板數目 > 400 x 10⁹/L

B2 白血球數目 (WBC) > 12 x 10⁹/L

B3 Leucocyte alkaline phosphatase (LAP) > 100 (正常：45-105)

     B4 血清中維他命B12 > 900 pg/mL (180-900) 或 維他命B12結合能力(unbound B12 binding capacity) > 220 pg/mL

如果：

一、同時有三項主要條件中A1 + A2 + A3 ， 或者

二、A1 + A2 再加次要徵候中之兩項

則PV診斷成立。

 

2004英國的British Committee for Standard of Haematology、以及隨時更新的聯合國衛生組織（WHO）也各自設立相似的臨床診斷條件，不過增加了EEC assay，EPO，Karyotype (核型)、及骨髓鏡檢等檢查作為主要或次要條件。

其實這些診斷標準是專家根據經驗設定的。都沒有絕對性。只要基本病因不明，用人為的診斷標準，都會有一些病人是在灰色地帶。有太多的限制就會有些病人被排除遺漏，不過嚴格的設限，以後的調查研究報告結果會比較可靠。

到了2005年，有五個研究團隊報告，絕大多數PV病人的骨髓細胞內 JAK2 酵素基因都果然有後天性變異（mutation）。這個基因蛋白的第617位置的正常氨基酸valine，由另一個氨基酸 phenylalanine代替，成為一種不正常基因，被命名為 JAK2 V617F，而骨髓細胞內這個不正常基因可以用PCR（聚合酶連鎖反應）偵測。PCR方法診斷 PV 的陽性率，因為各個研究團隊的PCR技術敏感度及臨床診斷的正確度不一，而各有些不一樣。可是近來報告的 PV 案例 JAK2 V617F 陽性率都接近百分之九十五。

因為已經有變異的 JAK2 V617F 基因陽性骨髓細胞，有能力在沒有EPO刺激時就不停地產生紅血球，因此這個基因分子內一個氨基酸的改變，是導致 PV 的多血症主兇，殆無疑問。從而以後 PV 的診斷，JAK2 V617F 的 PCR陽性結果將是不可缺的，也會是必要的條件。

想來也真神奇，人體細胞內數以萬計的基因當中一個基因，所構成的幾百個氨基酸之中，有一個發生變異，換成另外一種氨基酸，就害得我要定期放血、服藥、作各種檢查、還要擔心是否會因此縮短生命！好像是，天上星星那麼多，有一顆殞落或迷路，整個天體的運行就會出軌！人體的結構是多麼奧妙精緻！比我們所膽敢想像的還要細膩千萬倍。更神奇的是，人類以兩百年來發展的科學方法，居然可以找出這一個肉眼看不出的分子內氨基酸改變!

疾病的致病原因被鎖定到基因分子變化的例子，最早是在1960年Peter C. Nowel發現的Philadelphia chromosome (Ph)(這位在University of Philadelphia的醫師學者也因為發現lymphocyte transformation而得到Lasker prize)，這異常的Ph染色體在1973年由芝加哥大學 Janet Rowley更進一步地找到，是由於第九及第二十二染色體之間，各有一小部分染色體互換位置(這種機制稱為translocation)而發生。Ph陽性(Ph+)是和慢性骨髓性白血病（chronic myelogenous leukemia; CML）及部分急性淋巴球性白血病（acute lymphocytic leukemia; ALL）的發生相關。現在 PV 的發生原因，被確認為在於一個細胞內酵素基因分子的JAK2 發生突變(絕大部分在exon14的V 617F， 小部分在exon12），可說是繼1960年發現Ph染色體之後的又一個重大發現。

正常的骨髓裡面有「多功能幹細胞」（pleuripotential stem cells），可以由不同的cytokines刺激，分化，而演變成為erythroblasts（紅血球芽細胞）、myeloblasts（骨髓芽細胞，產生顆粒性白血球）、以及megakaryocytes（巨核細胞，產生血小板）這些細胞的單株性惡性增生疾病稱為骨髓增生性疾病（myeloproliferative diseases; MPD）。

所謂 MPD 這一類血液疾病，因為發生增殖病變的細胞種類不同，而有：

1.        1. PV （紅血球、血小板、嗜中白血球都增加）、

2.        2. essential thrombocythemia (ET；血小板增多症。只 有血小板增加),

3.        3. primary myelofibrosis (PMF；原發性骨髓纖維化。骨髓內纖維細胞增加，而各種血球反而減少),

4.         4. chronic myelogenous leukemia; （慢性骨髓性白血病； Ph+CML),

5.         5. hyper-eosinophilic syndrome（嗜伊紅白血球增多症；HES。這種嗜伊紅血球增加到超過1.5 x 10⁹/L，超過六個月，又沒有明顯已知會提升這種細胞的原因者。),

6.         6. chronic neutrophilic leukemia（慢性嗜中性白血病； CNL)等。

可以想見MPD是骨髓中「多功能幹細胞」因為對刺激不同細胞分化的各種 cytokines 敏感度不同，而導致的不同系列細胞的惡性增生。每一種尚未成為白血病的MPD疾病，最後轉變成為急性白血病的可能性比正常人高。

JAK2 V617F 在每一PV病人幾乎都可以偵測到。在其他種類骨髓增生症(MPD)也可以檢測陽性，但陽性比例較低，例如 PMF、ET 有30-70%是 JAK2 V617F 陽性。在 PV，除了紅血球，白血球及血小板都會同時增加。顯然這類MPD，還需要更多研究來釐清各種幹細胞（erythroid、myeloid、megakaryocytic系列）對不同cytokines的敏感度，及互相之間的關係。2008年WHO已根據這些基因: JAK2 V617F , exon12及 MPL （myeloproliferative leukemia virus oncogene）突變之有無，來分類這些疾病（看最下面附註）

更令人期待的是，已經有初步phase I/II clinical trial（phase II是對病人所做的有對照組的臨床試驗，目的為決定藥物的效率及短期內的副作用）的經驗以及其他零星的報告(Blood 110:abstract #558,2007)，用可以抑制這個異常基因的藥物治療，會使 PMF 或其他 MPD 病患病情改善。這種治療法，如果順利，可能幾年後就能用到一般 MPD 病人的治療。

再回到我這個個案的檢驗結果。除了血小板數目，我的WBC、RBC、HCT、HGB 各項數據都差一點沒有達到符合主要或次要診斷的條件。不過有異常高的血小板數目、超過正常值25%的RCM、以及EEC assay、JAK2 V617F呈陽性。臨床徵候中，也只有輕微的眩暈，更沒有脾腫或其他合併症。但是最近出現了惱人的皮膚刺癢症狀，不知是否病況有惡化。MRI也發現骨髓有異樣。尤其皮膚刺癢，時有時無，也似乎和飲食有關。和文獻中所稱，浸熱水之後會發生的刺癢，不一樣。目前正在自己仔細觀察中。這可能將來又是一個有趣的故事。

說不定是天上遠處一顆迷路的小星星，顏色要改變了！但願地球上富於創意的醫學小飛俠趕緊追上，恢復它原來晶亮的色澤，回歸銀河燦爛的行列。

附註：

WHO 2008 年對PV有新的classification如下:

主要條件：

A1 ： Hb > 18.5 g /dL (men)，Hb > 16.5 g /dL    (women)， 或者  increased Red Cell Mass

A2 ： presence of JAK2 V 617F  or exon 12 mutations

次要條件：

B1 ：PV bone marrow

B2 ：Low serum EPO level

B3 ：EEC growth

診斷標準為：

1). 2個A +1個B  或者 

2). A1 + 2個B (因為還有很少數PV是JAK2 V 617F陰性的)

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誌謝：感謝 施麗雲醫師提供WHO資料並指正、修改。

 

許清曉 Blog: http://blog.xuite.net/ccshsu2003/ccshsu/