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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...

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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).

Genetics

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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Courting Newlyweds
From Folio: The Magazine for Magazine Management, 4/1/04 by Liz Borod

Byline: Liz Borod

Sure, marriage rates are declining and divorce rates aren't. But, pop-culture-wise, marriage is hot again. Married celebrities such as Gwyneth Paltrow, Kate Hudson and Reese Witherspoon own magazine covers. Even Ben Affleck tried to settle down. And reality TV has made Trista and Ryan meta-celebrities simply on the basis of their wedding plans.

Do magazine publishers smell money in this? You bet. Young married women, once the target readers of the Seven Sisters, are being wooed anew. Nesting women in their 20s and early 30s are the demo that put Budget Living on the map and has breathed new life into Redbook. A recent Canadian launch called 2 is dedicated to young couples.

The reinvented Redbook is the poster child for this phenomenon. After foundering as a mass-appeal women's book, the magazine returned to its roots as the book of young marrieds. "We want to make it crystal clear that this is what sets Redbook apart. No one else is covering the young married life," says Ellen Kunes, editor-in-chief since 2001. Hence the mag's new tagline is "The Married Girl's Guide to Life." A redesign unveiled in February includes new sections such as " Redbook Married Life" and " Redbook Busy Life." Kunes promises a feature on marriage each month, in addition to a story about marriage and sexuality. A few months back, the mag added "My Life as a (New) Wife," a column by actress Kimberly Williams-Paisley, and "Love Lab," where writers test popular romance advice to see what actually works.

"Our readers are in what we call the decade of change," says Kunes. "Once you get married all these things happen in your life: You buy a home, you combine finances, you have a baby." The assumption is that Redbook's newlyweds are either thinking of having a baby or will be in the not-too-distant future. "It's always a question of how to balance the all-consuming joy of having a baby while maintaining a relationship with your husband and your sense of self," says Kunes.

The ad community has welcomed Redbook's repositioning, particularly its unique focus on the "transition in women's lives - single to married, and married to mom," says Mary Morgan, Redbook's vice president and publisher. "We have seen nice increases from Nestle Food Brands, Clinique, and new business like Hyundai, Pfizer, Kohl's and Pepsi. The redesign and new focus editorially have been very key in these moves."

Redbook's approach reflects a changing view of marriage in America, says Barbara Dafoe Whitehead, Ph.D., codirector of the National Marriage Project, a nonpartisan initiative at Rutgers University in New Jersey. "People used to see marriage as the entry into full-fledged adulthood. It was a way to rear children, provide economically for the family household, and gain societal approval," she says. That view began to change in the 1960s and hasn't stopped. "Soul mate-centered marriage is the new ideal - and it's very romantic," says Whitehead. "I think it has contributed to the marriage/wedding mania." Which explains The Bachelor/Bachelorette syndrome.

The difference for today's young marrieds - which magazines that want to reach them must understand - is that they want to keep the romance and their individuality, even after the kids arrive. Alex Bhattacharji executive editor of Budget Living, a hip lifestyle magazine with a rate base of 300,000, says that he's had readers thank him for not treating them any differently just because they're married. (Budget Living doesn't have any data on how many of their readers are married, but they do know that 40 percent have at least one child, according to Bhattacharji.) "We don't want to patronize our married readers and tell them that they don't have as much fun, dress as well, or travel as much as their single counterparts."

At the same time, many of Budget Living's stories are couple-oriented. It counsels young families on how to start joint accounts, plan their finances and make over rooms on a budget. "Nesting and decorating has a lot to do with whom you're sharing the space," Bhattacharji says. While shelter is usually a female category, the emphasis on how couples decorate their nests has given Budget Living a significant male following: Some 30 percent of its readers are male, including 15 percent of subscribers. The magazine regularly includes men's fashion. Plus, "bargain-hunting can be a fun activity for couples," Bhattacharji says.

American Media Inc. editorial director Bonnie Fuller is cooking up her own low-budget shelter book, a quarterly set for a September premiere. While the magazine, which will have a 250,000-copy run, isn't aimed specifically at newlyweds, editor-in-chief Sara Ruffin Costello says it should be a big hit with "a 33-year-old with her first home." Like Budget Living, it will stress "accessible and affordable" decor, she says. "We hope to show all price points." And the magazine will offer advice on staging weddings.

About the only magazine (so far) to address its readers as young marrieds is 2, which bills itself as "The Only Magazine for Couples." Diane Hall, former publisher of WeddingBells, launched this hip and humorous magazine in mid-March. She printed 125,000 copies to be distributed on newsstands, at wedding shows and to young marrieds through a strategic partnership with the national department store chain Hudson Bay Co.'s wedding gift registry. Like Budget Living, 2 doesn't care if you're married and embraces all kinds of couples: cohabiting couples, engaged couples, same-sex couples and even dating couples. "There's a real voyeuristic element to this whole thing as well, peering into couple's lives as we do," says editor-in-chief Neil Morton, who's 32 and married for 10 years. Indeed, there are columns such as "The In-Law Diaries" and "Ask the Smug Marrieds" mixed in with gadget and music reviews. And, since this magazine is for couples, Morton says he has "high hopes that it will be perceived as a gender-neutral publication."

Husbands and wives sharing their magazines - that's almost as romantic as a proposal on The Bachelorette! Even if the young married celebrities stop being hot with pop-culture junkies, publishers shouldn't fret. Real young married women, who're in no mood to put on an apron, will still want to read magazines that speak to them.

COPYRIGHT 2004 Copyright by Media Central Inc., A PRIMEDIA Company. All rights reserved.
COPYRIGHT 2004 Gale Group

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