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Costello syndrome

Costello syndrome is a genetic disorder that affects many parts of the body. This condition is characterized by delayed development and mental retardation, distinctive facial features, loose folds of extra skin (especially on the hands and feet), and unusually flexible joints. Heart abnormalities are common, including a very fast heartbeat (tachycardia), structural heart defects, and overgrowth of the heart muscle (hypertrophic cardiomyopathy). Infants with Costello syndrome may be large at birth, but have difficulty feeding and grow more slowly than other children. Later in life, people with this condition have relatively short stature and many lack growth hormone. more...

C syndrome
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CDG syndrome type 1A
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Common cold
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Compartment syndrome
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Conn's syndrome
Constitutional growth delay
Conversion disorder
Cor pulmonale
Cor triatriatum
Cornelia de Lange syndrome
Coronary heart disease
Cortical dysplasia
Corticobasal degeneration
Costello syndrome
Craniodiaphyseal dysplasia
Craniofacial dysostosis
CREST syndrome
Creutzfeldt-Jakob disease
Cri du chat
Cri du chat
Crohn's disease
Crouzon syndrome
Crow-Fukase syndrome
Cushing's syndrome
Cutaneous larva migrans
Cutis verticis gyrata
Cyclic neutropenia
Cyclic vomiting syndrome
Cystic fibrosis
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy

Beginning in early childhood, people with Costello syndrome have an increased risk of developing certain cancerous and noncancerous tumors. Small growths called papillomas are the most common noncancerous tumors seen with this condition. They usually develop around the nose and mouth or near the anus. The most frequent cancerous tumor associated with Costello syndrome is a soft tissue tumor called a rhabdomyosarcoma. Other cancers also have been reported in children and adolescents with this disorder, including a tumor that arises in developing nerve cells (neuroblastoma) and a form of bladder cancer (transitional cell carcinoma).


Mutations in the HRAS gene cause Costello syndrome. The HRAS gene provides instructions for making a protein that helps control cell growth and division. Mutations that cause Costello syndrome lead to the production of an HRAS protein that is permanently active. Instead of triggering cell growth in response to particular signals from outside the cell, the overactive protein directs cells to grow and divide constantly. This unchecked cell division can cause cancerous and noncancerous tumors to develop. It remains unclear how mutations in the HRAS gene cause the other features of Costello syndrome, but many of the signs and symptoms probably result from cell overgrowth and abnormal cell division.

This condition is considered to have an autosomal dominant pattern of inheritance, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Almost all cases have resulted from new mutations in the gene, and occur in people with no history of the disorder in their family. This condition is rare; 150 to 200 cases have been reported worldwide.


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Stars' memoirs: center stage: reminiscences by Carol Burnett, Efrem Zimbalist, Jr., and Roy Rogers' daughter elicit nostalgia, tears, and laughter - Book
From Saturday Evening Post, 1/1/04 by Ted Kreiter

Cowboy Princess Life with My Parents Roy Rogers and Dale Evans by Cheryl Rogers-Barnett & Frank Thompson, 224 pages, Taylor Trade Publishing, $24.95

Cheryl Rogers-Barnett learned at an early age how important first impressions can be. She was just six weeks old in a room filled with orphaned infants at a Dallas foundling home when a young movie star named Roy Rogers came through wiggling his fingers and making laces at each baby. All of them cried except for one--Barnett. She reached up, grabbed his finger, and cooed. That's how she came to be the first child of America's most famous Western couple. She tells their intimate, happy, and heartbreaking story in her new book, Cowboy Princess: Life with My Parents Roy Rogers and Dale Evans.

"I was given the most wonderful parents anyone could hope for," Barnett writes. "Millions of other kids even prayed to be part of our family." And in fact, a lot of them did become part of it. Before it was all over, Roy and Dale had adopted four more children of varying ages and national backgrounds, adding to a family that already included Cheryl, her brother, Dustin, and sister, Linda Lou, born to Roy and his second wife, Arlene. There was also another sister, Robin, born to Dale (actually, Roy's third wife). Add to that three horses named Trigger, two Russian boars, a flock of homing pigeons, Roy's hunting dogs, a baby panther, and a yodeling German shepherd named Sam, and you had a household Barnett de scribes as "rough and tumble."

Barnett says that in spite of her parents' celebrity, Roy and Dale were pretty much the same off screen as they were on. They were "extremely unpretentious, down-to-earth people, and they expected us to be the same way," she writes. At school Barnett mingled with other stars' children-the daughters of Duncan Renaldo (the "Cisco Kid") and Bud Abbott of Abbott and Costello--and she had some blind double dates with Ricky Nelson and James Darren, but she never was the "snobby rich kid of movie stars," as many people thought.

"We weren't rich at all," she writes. "Our folks worked for Republic, one of the 'poverty row' studios, not like MGM or Paramount." She recalls having a five-dollar allowance in high school and doing chores to earn it. When she wanted a car, she was told to find a job; when she finally had enough money to buy one, it was Dad who got to choose it: a big, old, unsexy--but very safe--1950 Chevy, which she named Gertrude the Gutless Wonder.

At home, out of his Western duds and settled comfortably in tennis shoes and T-shirt (or no shirt at all), Roy was basically a man's man who loved hunting and deep-sea fishing with friends like Clark Gable.

He also loved music, and his abilities in that area went beyond guitar-playing and singing. "He could play any kind of musical instrument, especially anything with strings," Barnett writes. As a young girl, she was amazed when he borrowed a fiddle at a square dance and proceeded to "play a super hoedown." She'd never known he could play the fiddle. Roy also was a natural actor who never could quite get over the fact that people paid him for having fun.

Dale Evans was a smart, talented Texas girl who graduated from high school at age 13 and eloped with her high-school sweetheart at age 14. She had always been driven to perform, although she saw herself as a leading lady and never thought she would act in Westerns. Somehow she and Roy ended up working for the same movie studio, Republic Pictures.

One advantage to being Roy Rogers' daughter was easy access to a show-business career if one wanted it. At age 10 Barnett had her first (and only) line in a movie, and at age 15 she appeared in a TV episode, "Outlaws of Paradise Valley." In the show, Pat Brady was supposed to teach her how to make pancakes, but things got out of hand when the crew tried putting kerosene in the batter to make the pancakes smoke. Instead, when they hit the griddle, they blew up, singeing off Barnett's eyebrows, eyelashes, and part of her bangs.

By the time Barnett was in high school, her father had become so famous, he couldn't attend public functions without being mobbed by his admirers, and Barnett suffered the consequences. "Dad never took me to a father/daughter event at school. Dad didn't attend my high school graduation, even though I was one of the two valedictorians of my class," Barnett writes. "Looking back, I'm almost surprised that he attended our weddings, but he wouldn't have dared miss one of those--Mom would have killed him."

Dale, in addition to being a talented songwriter and a best-selling author, was a supreme organizer. Before her death, she even planned every detail of her funeral. Her strict Southern Baptist upbringing made an impact on her large family and extended even to the workplace. She once installed a "cuss box" on the set of the Roy Rogers TV show. Offenders had to deposit a quarter for every bad word they let slip. The money went to the church.

"It was amazing how much money she collected for 'good works' the first two weeks," writes Barnett. But soon the set became a "more refined place" for children.

At home, Roy would sometimes allow activities that Dale wouldn't stand for. "Dad didn't like being the bad guy," Barnett writes. On one occasion, Dale had to change into her Queen-of-the-West persona in order to restore order. Barnett's two teenaged brothers were wrestling in the living room as Roy looked on, laughing. But Dale couldn't see the humor in their possibly wrecking the furniture. When the boys ignored her pleas for them to stop, she went down the hall, got her gun, came back and yelled at the top of her lungs, "Boys, stop right now."

"When there still was no response," Barnett writes, "the next thing they heard was BLAM BLAM, BLAM, BLAM." The gun was filled with blanks, and she fired off every round. "The boys fell back, absolutely stunned," she recalls. "Mom said that even Dad stopped laughing, until he realized what she had done."

Most arguments around the Rogers household ended in laughter, as did the major disagreement that arose when Roy announced he wanted his beloved show horse, Trigger, stuffed and mounted for his museum. Dale "did not think much of the idea," Barnett writes. "She quoted the Bible verse that speaks of 'dust to dust and ashes to ashes.' Dad could not be dissuaded, and a rather heated discussion ensued. It ended when Mom, in complete frustration, screamed at Dad, 'Well, how would you like it if I had you stuffed when you died?' Dad replied in an instant, 'That's all right, Honey. Just skin me out and put me up on Trigger, smiling and waving at the people.'"

Not everything dissolved into laughter, however. The "terrible times" occurred with the death at age two of Roy and Dale's only offspring, Robin, who was born with Down syndrome, and the later deaths of Barnett's adopted sister and brother, Debbie and Sandy, within a year of each other.

Out of Robin's life and death came Dale's book, Angel Unaware, a bestseller that inspired a new perception of handicapped children around the world and which is still in print 50 years later.

Barnett's Cowboy Princess is inspiring in a different way, showing as it does two of the greatest movie heroes being even more heroic in their private lives. They were the genuine article.

One More Time: A Memoir by Carol Burnett, 365 pages, Random House Trade Paperbacks. $14.95

In her part-time job as a hat girl at Palmer's Tea Room in hattan, young Broadway wannabe Carol Burnett devised a way to get more money out of the gentlemen customers. While they were enjoying themselves at the oyster bar downstairs, she would pull the threat coats, then sew it back with a different color of thread. When the men retrieved their coats, she would show them how the loop had been broken and how she had fixed it. It was always good for an extra dime, Burnett says.

This was one of the survival tricks Burnett used on her way to stardom, which she reveals in her fascinating memoir, One More Time, recently reprinted with a new final chapter by Random House.

In the mid-1950s, the future television star was scraping together a living at the Rehearsal Club, an old Manhattan brownstone crammed with girls trying to break into show business and not having much success. Desperate after having spent all her money without yet being seen by an agent, she finally sneaked backstage in a Broadway production and collared actor Eddie Foy, Jr., whom she had never met. The meeting got her an introduction to Foy's agent. The agent had no ready part for her, but while waiting for one to come along, he joked that she might put on her own show.

It wasn't a very good joke, but to Burnett it was a great idea. In a fit of melodramatic inspiration, she did just that, cobbling together a revue with girls from the Rehearsal Club (the few who didn't think she was wacky) and sending invitations to all the New York agents, who surprisingly responded in force. Peeking out from the curtain before the performance, Burnett noted with shock that Marlene Dietrich and Celeste Holm were in the audience.

The critics panned the show, but Burnett was a hit. She had made her own break, like her mom had always said she'd have to, and she finally was on her way. Fortunately, for the sake of her memoir, there would still be plenty of false starts and more marvelous anecdotes along the way.

After she had made an early appearance on the Ed Sullivan show, Burnett recalls going to collect her check from a truculent clerk at the New York unemployment office (whom she nicknamed "The Wicked Witch").

"You've written these numbers in the wrong column," the woman snorted at her. "You say you made $750 in one day. That might have been $75, or more likely it was $7.50. You'll have to fill out another form and wait in line again."

"No. I won't," Burnett responded. "I wrote it in the correct columns. I made seven hundred and fifty dollars two weeks ago. May I have my unemployment check now?"

The woman screamed, "In one day?"

"If you'll read further down, you'll see where I was a guest on The Ed Sullivan Show a week ago Sunday," Burnett said. "Sorry you missed it. I'll let you know before the next time! Bye!"

Just as intriguing as Burnett's account of her rise to fame and fortune is her tragicomic description of the netherworld she came from--a family of dysfunctional, but somehow lovable characters who lived on relief in a Depression-racked section of Hollywood. The cast included her beautiful, alcoholic mother; a handsome and sweet alcoholic father who was separated from the family and died young; a hypochondriacal Christian Scientist grandmother she called Nanny, who played piano and sipped brandy from a bottle; a younger half sister, Chris, whom she later "kidnapped" and took to New York; and an alcoholic parakeet named Tweety. All were crammed together in a one-room Hollywood apartment. She had no bedroom and slept on the living-room couch.

"Old sweaters and underwear and dresses hung from the backs of the chairs and sat in piles on top of the radio and the end tables and the couch," she writes. "When I went to sleep at night, I shoved the stuff to the bottom of the couch with my feet."

The family had no money, but plenty of dreams. She and Nanny would walk the few blocks to Grauman's Chinese Theater and trace the hand and foot prints of the stars in the sidewalk out front.

"I'd usually stand in Betty Grable's square," she writes, "or Linda Darnell's. Betty and Linda had tiny feet."

Once she and Nanny spotted Darnell getting out of her car in front of a theater and begged for her autograph. Burnett recalls, "I looked up at her and stared. It was the first time I'd ever noticed that people's nostrils aren't the same shape. The left one is different from the right one. I'll be darned. But hers were the most glorious nostrils I had ever seen."

As the quirky family story unfolds, one gets candid glimpses of the young comedienne Carol Burnett in the making: kids at recess requesting a display of her double-jointed hip or wanting to hear her blood-curdling Tarzan yell. She was the only one in school who could come anywhere close to the Johnny Weissmuller original.

Her first dramatic part, which came in a ninth-grade play, had a bizarre and tragic twist. She tied for the role of Musie, a sassy maid, with a peculiar boy named Gordon, who wanted to play the part dressed as a girl. Gordon used to follow kids around, cornering them with long strings of jokes that always fell flat.

Burnett's performance was a howling success. The next night she came to see Gordon, resentful that she had been cheated out of playing the part twice. Gordon got laughs when he came out dressed as a girl, but as the part progressed, "something started happening," Burnett recalls.

"He wasn't Gordon in girl's clothes. He was Musie. He wasn't 'acting' funny. He didn't have to. He just was. And he was wonderful." She stood with the rest to give the boy a standing ovation.

"Gordon became the campus star," she says. "None of us could get it through our heads why, just weeks before graduation, he shot himself."

Burnett recalls the strange dreams she had about Gordon after that. Dreams that came true, and psychic experiences, recurred throughout her life. There was her mother's dream of Carol's father calling her name. He was dying and calling to her. It was the middle of the night. She tracked him down and found him just in time to hold his hand. Then shue found her little sister Chris at 3:00 a.m. writing an urgent letter to their mother in California. It had to be mailed immediately. Their mother died just after receiving it.

But there were happy prognostications, too. At the party thrown for her just before she headed to New York to seek her fortune, somebody shouted, "Okay, Burnett, what's gonna be your first Broadway show?" A picture flashed into her mind, she writes. She shouted back, "A musical! And George Abbott will be the director." It happened exactly that way, five years later, in Once Upon a Mattress.

One More Time was originally published in 1986. Today, it is as compelling and fresh as it was then, a life story filled with comedy and tragedy in which comedy won out, and recounted with impeccable virtuosity by a talented lady who also is a gifted writer.

My Dinner of Herbs by Efrem Zimbalist, Jr. 209 pages, Limelight Editions, $25.00

Efrem Zimbalist, Jr., might well have been thought destined for a musical career. He was, after all, the son of two world-famous musicians, concert violinist Efrem Zimbalist and singing star Alma Gluck (America's first million-selling recording artist). Unfortunately, by the age of 10, the future star of The FBI and 77 Sunset Strip was showing "a genuine talent," not so much for his playing as for provoking his violin teacher.

One of Zimbalist's early recollections, recounted in his new memoir, My Dinner of Herbs, is of Mr. Heifetz (the father of Jascha Heifetz), a stiff and formally dressed teacher of the Russian school, flying into a "purple rage," knocking over the music stand and delivering "a jeremiad in vigorous, albeit non-Oxfordian English." That was the last that the young Zimbalist saw of his violin teacher, but only one of the first in a lifelong string of hilarious adventures and misadventures which Zimbalist masterfully brings to light for the first time in his lively memoir.

In 1935, after accompanying his father on a concert tour of the Soviet Union, Zimbalist found himself enrolled by his parents in a Stalinistera boarding school in Kiev, while they visited western Europe. He was expected to learn piano (which he had never studied) in a class of superbly gifted children, one of which was a four-year-old wunderkind. The classes were taught in Ukrainian, a language he did not know. He eventually escaped to Moscow, where his elder sister had been lodged with a family, and they attempted to keep warm with only summer clothing through the Moscow winter. The story ends with them secreting their last five-dollar bill in the bottom of a cold-cream jar to prevent confiscation by Russian border officials, finally fleeing by train to Paris.

Sadly, for all of his extraordinary youthful experiences, the young Zimbalist emerged from his privileged upbringing as a rather undisciplined and spoiled juvenile whose outrageous behavior, among other things, got him expelled from Yale University not once, but twice. It was a period in his life, he admits, he "would do almost anything to undo or relive." He includes it only because, he writes, "were I not to, the picture of me which might otherwise emerge would be disingenuous."

The young Zimbalist excelled as one of America's early student credit card offenders, running up bills for high living and an expensive wardrobe that in today's currency would amount to tens of thousands of dollars. Afraid to confront his parents, he went to New York and found a job as a page and guide for NBC at Radio City. "a confraternity," he writes, "that included such unlikely names as Gordon MacRae and Gregory Peek." The job was key to his first small steps into theater.

Zimbalist also tried the publishing field, working for Time one summer as a mail clerk. He recalls being the only one in the room one day and answering the phone, only to hear the thundering voice of the publisher, Roy Larson, say, "Who's this?"

"Efrem Zimbalist, sir," he responded.

"Yeah?" Larson said. "Well this is Arturo Toscanini. Get your ass up here and sharpen some pencils!"

It wasn't until shortly after Zimbalist returned from his five-year stint as an infantry replacement officer in World War II, having received a "million-dollar" wound in the battle of the Huertgen Forest, that his theater career got its real start--thanks partly to his Army friends, a couple of guys by the names of Garson Kanin and Joshua Logan.

Zimbalist's memoir offers an interesting peek into the personalities of famous actors he met or worked with over the years. In his first Broadway role, Zimbalist worked with Spencer Tracy, who was not much loved by the rest of the cast. Tracy's technique was to bully everyone into overacting so that his own performance might appear more natural. In one scene-stealing episode, Zimbalist recalls, Tracy, who was seated opposite him at a table in the scene, abruptly "slid his chair about a foot upstage" after one of his lines. Zimbalist, unwilling to be so blatantly upstaged, moved his chair as well, and Tracy moved again. Soon the chairs had sped all the way around the table, where the two met and Tracy abruptly picked up his chair and moved downstage with his back to the audience, trying to block Zimbalist from view.

In physical stature at least, Zimbalist was exactly the same height as actor Clark Gable, and that proved to be a godsend when Zimbalist was put up for the part of the villain in the 1957 movie Band of Angels. Not just a regular villain, this villain, writes Zimabalist, "was the slimiest, filthiest, most loathsome and contemptible worm that ever crawled out from under a Cajun rock." He didn't want the part, but was in no position to turn it down. Fortunately, after Gable met him briefly, the director of the movie came down to inform Zimbalist that regrettably, they could not use him in the part. Gable, it seems, preferred not to fight anyone in a movie who was not taller than himself.

Then there was Zimbalist's run-in with Rex Harrison about a grammatical faux pas in Alan Jay Lerner's lyrics to My Fair Lady, in which Harrison had played Professor Higgins, "the embodiment of linguistic rectitude."

At a luncheon in Beverly Hills, Zimbalist asked Harrison, "Was it difficult for you to have to sing, 'I'd be equally as willing for a dentist to be drilling/Than to ever let a woman in my life?'" Certainly, Zimbalist believed, the correct grammar should have been "As to ever let a woman in my life."

Harrison's reply was unexpected. "'What in blazes are you talking about?' he snapped. Turning to the person on his right, he cried, 'Who the devil is this fellow?. Some sort of nut?'"

Zimbalist later learned from friends that Harrison, who had grown up in London's West End, had very little schooling, and that naturally, "he wouldn't know a verb from a turnip!"

Zimbalist's movie career paired him with lots of intriguing leading ladies, including Louise Allbritton, Lana Turner, Natalie Wood, and Jane Fonda, but he reserves highest praise and his most extravagant metaphor for the actress whose husband he played in the thriller Wait Until Dark, Audrey Hepburn. "Working with Audrey, the sublimest jewel in Hollywood's tiara," Zimbalist writes, "was like floating inside the Mona Lisa, behind the smile."

To prepare for his most famous role in the TV series The FBI, Zimbalist went to Washington to study the workings of the real federal bureau and to have an interview with its famous director. He found J. Edgar Hoover to be "taller than expected," "courtly yet congenial," and quite an actor himself. Hoover dominated the conversation without leaving leeway even for an "I see" or "Oh, yes" from his visitor. He covered subjects ranging from Mrs. McLean and the Hope diamond, to Martin Luther King, Jr., to Hollywood, Washington society, Khrushchev, and Shirley Temple.

"It was a virtuoso performance, allegro con brio," Zimbalist recalls, "and he never paused for a word." After coming out for a photo op, Zimbalist looked at his watch. To his surprise the interview had lasted two hours and four minutes. Thereafter, Zimbalist writes, there was no major event in his life in which he did not receive a letter, telegram or telephone call from the famous G-man.

There's far more here, including Zimbalist's early work as a Broadway producer, bringing Gian Carlo Menotti's operas to the stage; his humorous and poignant recollections of the caretaker-turned-brilliant writer and director, Montgomery Pittman; and enlightening anecdotes about his famous stepmother, Mary Curtis Bok Zimbalist, benefactress of the arts and founder of The Curtis Institute of Music in Philadelphia, as well as his hilarious, Twilight Zoneesque account of a nightmarish drive to Philadelphia to attend the premier performance of his own composition.

Zimbalist should have become a writer much earlier in his life; the only disappointing thing here is coming to the final page, long before one is ready for it to end.

A Daughter's Legacy

Carol Burnett dedicates the new edition of her classic memoir to her daughter Carrie Louise Hamilton, who died from lung cancer on January 20, 2002, at the age of 38. Carrie (also an actress and entertainer) found her mother's memoir so moving that she urged her to adapt it for the stage. In a new afterword, Burnett poignantly describes their long-distance, four-year collaboration on a script, which culminated in the Broadway premiere of Hollywood Arms at the Cort Theatre in New York City on Oct. 31, 2002. "My baby and I went the distance," she says.

COPYRIGHT 2004 Saturday Evening Post Society
COPYRIGHT 2004 Gale Group

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