Saethre-Chotzen disease gene
Johns Hopkins Children's Center scientists have identified TWIST as the disease gene causing Saethre-Chotzen syndrome, one of the most common genetic conditions with craniosynostosis, the early closure of the cranial sutures. Their findings, which also include the mapping of TWIST in the human genome, appear in the January issue of Nature Genetics.
Saethre-Chotzen syndrome is an autosomal dominant disorder that affects between one and two in every 50,000 people. The syndrome is characterized by a spectrum of malformations, including early closure of the cranial sutures leading to a misshapen head and facial asymmetry, underdeveloped cheekbones, low-set or abnormal ears with a mild hearing deficit, dental defects and limb abnormalities. In addition, some individuals have mild to moderate mental retardation. Individuals with Saethre-Chotzen syndrome usually require some medical or surgical intervention to manage these problems, said Ethylin Jabs, associate professor of pediatrics, medicine and surgery.
Against all odds: inner city kids demonstrate success
A study aimed at measuring the life success of inner city children born between 1960 and 1965 has found that in this, the fourth decade of their lives, most have done well, and generally better than their parents in terms of educational attainment, life style, health and financial independence.
The study, conducted by the School of Public Health and published in the Jan. 15 issue of Pediatrics, was designed to identify and evaluate characteristics from early childhood--for blacks and whites, males and females--that might predict success or failure in the lives of more than 2,500 children whose mothers, living in low-income neighbohoods in East Baltimore, attended a Johns Hopkins pre-natal clinic between 1960 and 1965.
Some of the predictors of successful adult lives were not surprising. Living with both parents and continuing freedom from poverty predicted good outcomes, said Janet Hardy, professor emeritus, Johns Hopkins Children's Center. Behavior--in and out of school--was an accurate predictor as well.
Children on the honor roll who participated in extracurricular activities at school, had summer jobs, did not smoke regularly before age 18, lived with the family through age 16, stayed away from drugs and alcohol and did not get into difficulty with the law in their teens had the most success in all four categories. Almost 80 percent graduated from high school or had GEDs. More than 75 percent were financially independent, meaning they were not on any form of government assistance. Sixty percent were judged to be in good physical and mental health, and 70 percent were living "a healthy life style."
A less rosy side of the picture emerged as well. Reading and language achievement levels among the study group were well below national norms when they were 7 to 8 years old. Now into their 30s, females generally had higher educational attainment and fewer behavioral problems than males, but were less likely to be healthy or financially independent. Only 55 percent of the males had attained a "healthy lifestyle" by not engaging in "self-destructive" or "anti-social activities."
Clothing with drawstrings poses strangulation risk
Articles of clothing with drawstrings, such as hooded sweatshirts and coats, or jackets with drawstrings about the waist, may lead to serious injury and accidental death.
After studying 47 investigations of drawstring entrapments occurring between 1985 and 1994, in which eight children were killed, researchers at the School of Public Health's Center for Injury Research and Policy outlined two distinct hazard patterns: strangulation caused when drawstrings are snagged by playground equipment and dragging when drawstrings are caught in vehicles. Their results, published in the Jan. 15 issue of the Archives of Pediatrics and Adolescent Medicine, are accompanied by recommendations for reducing the drawstring hazard by eliminating or modifying drawstrings.
"Eliminating drawstrings is an effective way to prevent these accidents. Children would be safer with 'no strings attached'," said lead author Dorothy Drago, who conducted the study while a graduate student at the School of Public Health.
Alternatives to eliminating drawstrings may be shortening drawstrings, sewing them into channels, making drawstrings "break away," or removing toggles and knots at the ends. These measures, however, need scientific testing before their benefits are established.
The U.S. Consumer Product Safety Commission has periodically addressed clothing entrapment and has issued safety recommendations on sliding board construction. The National Highway Transportation and Safety Administration issued a 1993 voluntary recall to modify school bus door apparatus. The potential for drawstring injury persists because the NHTSA has no authority to enforce the recall or modifications.
Great Britain banned drawstrings on children's clothing in 1976.
Durden leaving IAAY
for Sylvan Learning Systems
After 16 years as head of a Hopkins program that promotes the academic abilities of children in the United States and abroad, William G. Durden will leave the Institute for the Academic Advancement of Youth in April to join Sylvan Learning Systems Inc.
Durden began his association with the institute in 1978 as a part-time instructor of German. In 1980 he was named executive director of the Center for Talented Youth, which was restructured as the IAAY last year.
"When I started here I had three tasks," Durden said. "To test the viability of the optimal match concept, to create an administrative system and to draft the financial health of the organization. I think I have accomplished those tasks, and I think I have done well. I hope it is judged that way."
Durden will be president of a business division of Sylvan. His specific duties have not yet been disclosed. Last year, Sylvan formed an alliance with the IAAY to develop curricula for highly able students and a computerized version of the Scholastic Assessment Test.
"Over the past several years, Sylvan has forged strong ties with Johns Hopkins and the IAAY," Sylvan president and CEO Doug Becker said. "During that time we have developed enormous respect for Dr. Durden's vision and leadership."
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