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Poland syndrome

Named after Sir Alfred Poland, Poland anomaly (PA) is described as an underdevelopment or absence of the chest muscle (pectoralis) on one side of the body and webbing of the fingers (cutaneous syndactyly) of the hand on the same side (ipsilateral hand). Sometimes referred to as "Poland syndrome," it is an uncommon condition present at birth (congenital). For people born with PA, the breastbone portion (sternal) of the pectoralis is also missing. more...

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Since the severity of Poland anomaly differs from person to person, it is not often diagnosed or reported. Sometimes, a person does not realize they have the condition until puberty, when lopsided (asymmetrical) growth makes it more obvious. The incidence, therefore, is difficult to determine. Current estimates are between one in 10,000 to one in 100,000 births. Poland anomaly is more common in boys than girls, and the right side is affected twice as often as the left. The reasons for these differences are not known.

Clinical Signs & Symptoms:

abnormal gastrointestinal tract (Very frequent sign)
absent pectoral muscles (Very frequent sign)
brachydactyly (Very frequent sign)
dextrocardia (Very frequent sign)
diaphragmatic hernia/defect (Very frequent sign)
humerus absent/abnormal (Very frequent sign)
liver/biliary tract anomalies (Very frequent sign)
maternal diabetes (Very frequent sign)
oligodactyly/missing fingers (Very frequent sign)
radius absent/abnormal (Very frequent sign)
rhizomelic micromelia (Very frequent sign)
syndactyly of fingers (Very frequent sign)
ulnar absent/abnormal (Very frequent sign)
upper limb asymmetry (Very frequent sign)
abnormal rib (Frequent sign)
hypoplastic/absent nipples (Frequent sign)
scapula anomaly (Frequent sign)
agenesis/hypoplasia of kidneys (Occasional sign)
encephalocele/exencephaly (Occasional sign)
hypothal.-hypoph. axis abn. morphol. (Occasional)
hypothal.hypoph. axis abn. function (Occasional)
microcephaly (Occasional sign)
preaxial polydactyly (Occasional sign)
ureteric anomalies(reflux/duplex syst.) (Occasional)
vertebral segmentation anomaly (Occasional sign)

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Yes, he has lived
From National Review, 11/24/03 by Jay Nordlinger

Vixi: Memoirs of a Non-Belonger, by Richard Pipes (Yale, 264 pp., $30)

FOR half a century, Richard Pipes has been one of the world's foremost scholars of Russia, and a man of political and policy influence as well: In the 1970s, he headed "Team B," the group directed to challenge the CIA's assumptions about the Soviet Union (which were wrong). And in the early 1980s, he served on the National Security Council staff of President Reagan. From the time of his birth in Cieszyn, Poland, to now, when he has assumed emeritus status at Harvard, Pipes has lived a rich, meaningful life. Fortunately, he has the ability to recount it, richly and meaningfully.

He calls his memoirs Vixi, which is Latin for "I have lived." In his preface, Pipes says, "It may sound strange coming from a professional historian, but I have always had trouble dealing with the past"--meaning his own past. It is of a piece with the author's character that he overcame any such trouble. When the Nazis and Soviets signed their pact, sealing Poland's doom, Pipes had just turned 16. He and his parents would get out--barely--but "much of my family and nearly all my school friends ... perished without a trace in the Holocaust." One of the reasons for writing this book was that "their memory not be entirely lost."

Pipes's account of the weeks between September 1, when the Nazis began to bomb Warsaw, and late October, when the family escaped, is gripping. (The Pipeses had moved to the capital when the author was four.) These pages are even cinematic, if that is not too cheap or insulting a word. Pipes includes items large and small--the radio kept playing Chopin's "Military" Polonaise, "to keep our spirits up." He quotes from his own diary of the time (powerfully written, and perceived): "Houses collapsed, burying thousands of people or else spreading fire along the streets. Mobs of nearly crazed people, carrying children and bundles, ran along streets that were covered with rubble. German pilots, the worst beasts in the world, deliberately flew low to rake the streets with machine-gun fire."

On October 1, the Germans arrived by truck, and "I noticed with surprise that the soldiers were not the blond supermen of Nazi propaganda: many were short and swarthy and quite unheroic in appearance." Five days later, young Pipes watched Hitler--come to take a victory lap--from a fourth-floor window: "He rode in an open Mercedes, standing up in the familiar pose, giving the Nazi salute. I thought how easy it would be to assassinate him."

Pipes learned indelibly the lesson of appeasement, which he would apply over and over again, not least to the Soviet Union. His hatred of tyranny, lies, and accommodation is almost physical. He also saw, in those horrible weeks, "how quickly the everyday overwhelms the 'historic'"--Polish life returned to something like normal "with surprising rapidity." This experience left Pipes with "the abiding conviction that the population at large plays only a marginal role in history, or at any rate in political and military history, which is the preserve of small elites: people do not make history--they make a living."

In time, the family reached America, and Pipes began a life bursting with opportunity, for so talented and determined a person. In one of the most arresting passages of his book, he writes:

In addition, "I admit to having little patience with the psychological problems of free people, especially if they involve a 'search for identity' or some other form of self-seeking." Pipes is a hard nut, but his hardness--if it is that--is well earned.

An eager immigrant, Pipes went off to wholesome Muskingum College in Ohio, and then into the Army--where he did a lot of reading (among other things). From boyhood, Pipes read like a demon. In fact, one of the pleasures of this book is that the author records his intellectual development, through books, chiefly, but also through art and music. In the Army, he was assigned to learn Russian, and he learned it at Cornell--from which he was eventually graduated. Then it was on to Harvard, where he earned his doctoral degree, and where he would soon gain tenure. "When it was granted to me," he writes, "I gained lasting happiness." This is the voice of a natural-born scholar speaking.

Pipes tells any number of charming, or tart, academic anecdotes. He also makes clear the extreme cowardice and vanity of the modern faculty (and administration). Along the way, he draws sketch after sketch of famous personages: Isaiah Berlin, George Kennan, Richard Nixon. The sketch of Berlin is priceless, describing in a cinching way what I have sometimes called Isaiah Berlin Syndrome (knowing what is right but refusing to stick your neck out, or to open your mouth). Perhaps the saddest thing about Pipes's book is one of the saddest things about his career: the enmity that exists between him and Alexander Solzhenitsyn. They say jarringly harsh things about each other. For years, they were about the only two anti-Communists east of the Hudson River: and to be at war. It is like a cruel joke of history. What divides them is the question of whether Soviet Communism has its roots in Russia generally. Pipes says yes; Solzhenitsyn says it is an insult to Russians, and unsupported by facts or logic. Admirers of them both can only wince.

In Washington, Pipes made waves, disturbing the CIA, the State Department, the detenteniks at large. We revisit the claims of Sovietology, and we remember just how bad they were, those Sovietologists. How can they stand to re-read what they wrote? Maybe they avoid it--Pipes does not.

Pipes made waves in the Reagan White House as well, up against what might be called the "peace camp," headed by Nancy Reagan and Michael Deaver. The author draws some more of his sketches--of Alexander Haig, Richard Allen, Henry Kissinger. Not all of these portraits are fond, to put it mildly. And he is especially interesting on the president himself: "[Reagan] possessed to a high degree the imponderable quality of political judgment. He instinctively understood, as all great statesmen do, what matters and what does not, what is right and what wrong for his country. This quality cannot be taught: like perfect pitch, one is born with it." Yet Reagan "was altogether incapable of thinking abstractly: his mind worked either emotionally or in reaction to individuals whom he could visualize." Even those who disagree with Pipes should have to ponder his arguments.

In whatever he has done, he has attracted controversy. Why? Pipes himself quotes Samuel Butler, who explained in a letter: "I never write on any subject unless I believe the opinion of those who have the ear of the public to be mistaken, and this involves, as a necessary consequence, that every book I write runs counter to the men who are in possession of the field; hence I am always in hot water ..."

It is stirring to be in the company of this mind--Pipes's, not Butler's--for 250 pages. They are filled with immense learning and insight. They are leavened, too, by humor and idiosyncratic asides. The story of his marriage to Irene, a tall and warm beauty, is touching. And I happen to find touching Pipes's notorious stubbornness--often a kind of righteous stubbornness. He tells a funny tale about having to visit the Soviet Embassy after Brezhnev died, and being asked to sign the condolence book. Trapped, he thought fast: and did sign his name, but completely illegibly.

And I have a story from my own experience, and NATIONAL REVIEW's. At the dawn of 2000, we published our "millennium issue," consisting of big-think pieces by big thinkers, including Pipes. In his essay, Pipes cited a book by Henri Frankfort, Kingship and the Gods (on ancient Near Eastern religion and society). He had the g in "gods" down--in the lower case--but, as it was in the title, I, of course, as editor, put it up. He insisted on its being put back down. "I am a Jew," he said, "and there is one God, and I will not have the plural word capitalized." "But Professor Pipes," I pleaded, "I am as monotheistic as anyone, but this is a matter of style, and to have the word up doesn't imply any idolatry: It's just a word in a title, like 'table' or 'chair.'" No, no, said Pipes, it could not be up, title or not.

So, that's how it appeared in the magazine: Kings and gods. It looked weird, and wrong (because it was weird, and wrong)--but, in the Pipesian world, it was really wonderful, and right.

COPYRIGHT 2003 National Review, Inc.
COPYRIGHT 2005 Gale Group

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