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Wilson's disease

Wilson's disease or lentigohepatic degeneration is an autosomal recessive hereditary disease, with an incidence of about 1 in 30,000. Its main feature is accumulation of copper in tissues, which manifests itself with neurological symptoms and liver disease. The estimated heterozygous carrier rate is about 1 in 90, meaning that 1 in 90 people are unaffected carriers of this mutation. The disease affects men and women equally and occurs in all races. more...

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Willebrand disease, acquired
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Wilms' tumor
Wilson's disease
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Worster-Drought syndrome
Writer's cramp


The Wilson's disease gene (WND) has been mapped to chromosome 13 (13q14.3) and is expressed primarily in the liver, kidney, and placenta but has also been found in the heart, brain, and lung, albeit at much lower levels. The gene codes for a P-type ATPase that transports copper into bile and incorporates it into ceruloplasmin. Bile is a liquid produced by the liver that helps with digestion.

The mutant form of WND expressed in people with Wilson's disease inhibits the release of copper into bile. As the excretion of copper from the body is thus impaired, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death.

Symptoms and signs

Symptoms usually appear between the ages of 6 and 20 years, but sometimes not until the age of 30, and in rare instances up to age 50. The most classical sign are the Kayser-Fleischer rings (brown rings around the cornea in the eye) that result from copper deposition in Descemet's membrane of the cornea. Other signs depend on whether the damage occurs in the liver, blood, central nervous system, urinary system, or musculoskeletal system. Many signs would be detected only by a doctor, like swelling of the liver and spleen; fluid buildup in the lining of the abdomen; anemia; low platelet and white blood cell count in the blood; high levels of amino acids, protein, uric acid, and carbohydrates in urine; and softening of the bones. Some symptoms are more obvious, like jaundice, which appears as yellowing of the eyes and skin; vomiting blood; speech and language problems; tremors in the arms and hands; and rigid muscles.

Clinical features

Clinical symptoms rarely develop before 5 years of age, despite the biochemical defect being present at birth. The average concentration of hepatic copper may reach 20 times normal levels, whilst plasma ceruloplasmin levels are typically less than 30% of normal.

The age of presentation seems to correlate with the organ system involved. About half (40–50%) of patients first present with hepatic symptoms and half (40–50%) with neurologic symptoms. The average age for hepatic symptoms is 10–14 years, compared with 19–22 years for neurologic symptoms. Patients rarely present after age 40.


  • Chronic active hepatitis, culminating in cirrhosis
  • Fulminant liver failure


  • Cognitive impairment
  • Mood disorder
  • Psychosis


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Wilson's ghost still shadows 21st century - Book Review - Wilson's Ghost
From National Catholic Reporter, 9/19/03 by Maria Falco

WILSON'S GHOST By Robert S. McNamara and James G. Blight Public Affairs Press, 317 pages, $14

Six months following the invasion of Iraq by U.S. and British forces and in the midst of the firestorm of media criticism of the faulty or deliberately exaggerated intelligence used to justify that invasion to the American and British people, one can't help wondering if the prophetic irony of Wilson's Ghost is more a vision of opportunities lost or a nightmare waiting to happen.

In September 1919, Woodrow Wilson made a speech in St. Louis. His speech was an attempt to prevent the rejection by the Senate of U.S. entry into the of League of Nations, and the fiasco of the Treaty of Versailles with all the harsh conditions it imposed upon Germany in direct contradiction of Wilson's promise of "peace without victory." His speech ended with this dire prediction: "You are betrayed. You fought for something you did not get. And ... there will come some time, in the vengeful providence of God, another war, in which not a few hundred thousand ... will have to die, but ... many millions."

Former Secretary of Defense Robert S. McNamara and James G. Blight draw on McNamara's two personal confrontations with this doomsday imagery, which had already been fulfilled once in World War II but which became even more relevant during the Cold War, with McNamara's participation in the alleviation of the Cuban Missile Crisis during the Kennedy administration and in the disastrous escalation of the Vietnam War under Lyndon Johnson. McNamara and Blight not only extract serious lessons from the success of the first and the failure of the second as contradictory approaches to the problem of exorcising the ghost of Woodrow Wilson and his failure to achieve a lasting peace for the 20th century, they also outline what they consider to be the only process by which we in the 21st century can hope to avert a far more devastating catastrophe than either Wilson, Kennedy, Johnson or any major participant in international relations today can begin to imagine.

Indeed, whether intentional or not, their critique of current trends in American foreign policy approaches the seriousness of a jeremiad, a warning to those who lead us of what might happen if we continue in our customary blindly arrogant and unilateral way of muddling through. Their view is a bleak scenario, far beyond the darkness projected by Wilson.

The irony of the situation is that the very behaviors they specifically warn against have in large part already been blunderingly repeated in our current crisis, and it is only a matter of time to see if their predictions now come true or if anything can be done to prevent them. Until just recently it appeared that the only policymaker who fully appreciated the seriousness of the situation was British Prime Minister Tony Blair (see his speech before Congress, July 17, strongly urging a more multilateral approach to the Iraqi undertaking and his almost prayerful promise that the United States will not stand alone in the years to come). His assessment may have been one of the reasons for President George W. Bush's limited calls for United Nations participation in peacekeeping efforts in Iraq.

Listen to some of the caveats proposed in the prologue of Wilson's Ghost: "Do not allow attempts to implement a morality-based foreign policy to be frustrated by moralistic self-righteousness; ... In the absence of a firm commitment to multilateral decision-making, preferably institutionalized in credible international and regional organizations, sustainable peace is illusory; ... Empathize with your adversary or risk the kind of miscalculation, misperception, and misjudgment that, among Great Powers, can lead to catastrophic war."

In the chapter proposing a radical agenda for the 21st century, the authors list two imperatives, one moral and one multilateral. The first, derived from Kantian principles and from the just war doctrine as elaborated by the U.S. Conference of Catholic Bishops in 1993: "Establish as a major goal of U.S. foreign and defense policy, and foreign policies of countries across the globe, the avoidance in the 21st century of the carnage--160 million dead--caused by conflict in the 20th century." The second imperative, deriving from the belief that Woodrow Wilson was inflicted with the "American disease" of insensitive unilateralism shared by a number of administrations since World War I: "Recognize that while the United States must provide leadership to the world to achieve the objective of reducing the risk of conflict, it will not apply its power--economic, political or military--other than in a multilateral context, subject to multilateral decision-making processes."

Wilson's Ghost applies these principles to three alternative scenarios that the authors foresee as looming on the foreign policy horizon of the 21st century: 1. Preventing great power conflict (that is, "Bringing Russia and China in from the cold"); 2. Reducing communal killing ("Intervention in 'dangerous, troubled, failed, murderous states'"); and 3. Avoiding nuclear catastrophe ("Moving steadily and safely to a nuclear weapons-free world"). In their executive summary at the conclusion of the book, McNamara and Blight reiterate their radical agenda: "an unprecedented commitment to avoid killing" and "an equally unprecedented commitment to 'zero-tolerance multilateralism.'"

For the first challenge, their sharply worded message is: "Empathy now!" for, "since the end of the Cold War we have failed to take the necessary steps to prevent Great Power Conflict." In this regard, the authors cite the arbitrary revocation of the ABM Treaty, the admission of former Warsaw Pact Nations into NATO, and the gradual retreat from our recognition of the "One China" policy regarding Taiwan. Whether we agree with the Russians and the Chinese or not, we must recognize (empathize with) their viewpoints and try to reassure these former great powers of our respect and non-aggressive intentions before they cause us more harm than we are willing to contemplate.

On the second challenge, we must ask ourselves the Reinhold Neibuhr question: "How much evil must we do in order to do good?" and establish a framework for multilateral intervention in such killing fields or trouble spots as have erupted all over the world since the end of the Cold War--in sub-Saharan Africa, in Indonesia and the Philippines, in Kashmir as well as Kosovo--in order to stop communal violence in unstable and failed states. And if we are unable to obtain effective action from existing multilateral organizations, we must create a "coalition of the willing" (their term) while never acting unilaterally in such situations, in order to avoid the appearance and unwanted burdens of imperialism. At the same time we must respect cultural differences and let our values speak for themselves without attempting to impose them on others. The authors' use of emphatic language--"Resolve conflict without violence now!"--underscores the urgency they feel about settling differences that could lead to war.

The third challenge presented by the authors--avoiding nuclear catastrophe --is the stuff of future nightmares to come. Here McNamara and Blight argue the United States must move decisively to reduce nuclear danger by calling for radical and immediate reductions--and ultimate elimination--in the nuclear arsenals of current holders of such weapons. At the same time the United States must do all it can to strengthen nuclear nonproliferation treaties with those countries that might want to create nuclear weapons for the purpose of blackmailing their neighbors or the United States into backing down from perceived threats to their own national security. In no event should the United States ever attempt to weaken those treaties by experimenting with new types of tactical nuclear weapons or by proposing to setup a missile shield against them in the misguided belief that Americans can protect themselves while not wishing others to do the same.

Wilson's Ghost was published first in 2001, then reprinted in 2003. Following the Sept. 11 attacks on the Pentagon and the World Trade Center, it became obvious to the authors and to everyone else that we were no longer living in a world where nation states were the sole actors on the international scene, that current international organizations were not particularly competent to prevent regional, much less worldwide disasters, and that a way must be found to eliminate or neutralize the dangers that both state and non-state terrorist organizations pose to us and to the rest of the world.

In the 2003 afterword, McNamara and Blight propose the following adaptation of their original message. To avoid the "mega-terrorism" of weapons of mass destruction, we must "deploy realistic empathy" toward Islamic fundamentalists to learn "what they think they are up to." Empathy is also the principal means for avoiding "narcissistic unilateralism"; and "self-serving 'moral clarity' "must be replaced by "moral accuracy": That is, "How much evil must we do in order to do good?" in dealing with al-Qaeda and the "Axis of Evil." Thus, McNamara and Blight say empathy, not sympathy, must be our third imperative following the events of Sept. 11.

The major difference between the Cold War success of the deterrence policy of "mutually assured destruction" and the current situation is that in the former case nuclear weapons were acquired as a preventative, not for actual use, following Hiroshima and Nagasaki, and other weapons of mass destruction like chemical and biological weapons were systematically destroyed. Today non-state actors are trying to acquire nuclear weapons for use, not for deterrence, against targets in the United States and the West generally, and chemical and biological weapons are rapidly proliferating. In Osama bin Laden's 1998 statement, "The Nuclear Bomb of Islam," the acquisition of such a weapon is a religious duty in order to terrorize the enemies of God. With such a motive, and in the light of the determination of fearless "martyrs" to sacrifice themselves as well as their perceived enemies to their cause, the absolute necessity of empathy must be recognized and implemented to forestall what might otherwise be inevitable.

The "empathy gap," as McNamara and Blight put it, was entirely on our side in the period leading up to Sept. 11. The Sept. 11 "martyrs" were fully aware of our motivations and value systems and the openness of our society, and, practicing the jujitsu tactic of the weak, utilized our own technological and economic strengths against us in launching their attack. Thus they succeeded in destroying our customary confidence in our own invincibility and our sense of security and well being. And since even the weakest of nations and organizations are able to obtain the "great equalizer of the 21st century," nuclear weapons, we have no choice but to practice empathy in order to avoid disaster.

Ironically, our failure to recognize the hazards facing us after the fall of the Berlin Wall and to take appropriate measures to prevent them caused the triumphant proclamation of the "New World Order" by George H.W. Bush to morph into the spontaneous appearance of the "New World Disorder" under his son, George W. Bush. Citing a series of foreign policy blunders in just the first 18 months of the latter Bush's administration--the rejection of the Kyoto Protocol on the global environment, the ABM and land mine treaties, as well as the Comprehensive Test Ban Treaty, the administration's plan to violate the Non-Proliferation Treaty and the assault on the International Criminal Court and its proponents--McNamara and Blight conclude that our European friends have good reason to be concerned about the path we seem to have chosen.

They therefore call for a radical change in the American mindset itself --to be less self-absorbed and convinced of our own invincibility and moral correctness; to think the unthinkable regarding our vulnerability to attacks upon our homeland and to work to completely eliminate weapons of mass destruction now; to empathize with our adversaries as well as with our friends; and to act multilaterally, never unilaterally, where so much is at stake. Anything less will not lead to a Pax Americana, they say, but to America the Hated" and to the condemnation of the United States as the rogue superpower of the 21st century. If we fail, they warn, "the violence and destruction of the 21st century could easily dwarf that of the 20th."

If the hackles on the backs of our necks haven't begun to stand straight up at these words, maybe it is because some of these same conclusions have already occurred to so many other commentators during and as a direct consequence of the war in Iraq that they are coming to sound like cliches. We can hope that the diplomatic fiascos and intelligence scandals surrounding the war will cause our leaders to change course and involve the world community in its aftermath. If so, and if we begin to understand the necessity for empathy as a method of dealing with cultural differences instead of arrogance and a display of moral superiority, then perhaps we will have a chance to exorcise the ghost of Woodrow Wilson and achieve the lasting peace he so much desired.

Maria J. Falco is professor emerita of political science and former academic vice president of DePauw University in Indiana. She is the author and editor of five books.

COPYRIGHT 2003 National Catholic Reporter
COPYRIGHT 2003 Gale Group

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