Research news (InTouch Fall 2008)

Adolfo ArizaSeconds for Care: Evaluation of Five Health Supervision Visit Topics Using a New Method
This study was conducted to gain understanding of the time it takes and some of the actions that occur to address key preventive topics during pediatric health supervision visits of children between the ages of two and ten years. The authors developed a new method and a tool (the Pediatric Health Supervision Timer Software— PHSTS© Children’s Memorial Hospital) to help conduct the assessments of five preventive health topics (diet, growth, safety, physical activity and tobacco). This observation method was developed to assess pediatric primary care practice and to later assess the impact of practice-based interventions on health supervision visit time. Said Adolfo Ariza, MD, co-investigator on the study, “We found that during health supervision visits, pediatricians are literally taking seconds on some of these topics, thus the title of the paper: ‘Seconds for care.’” The research was conducted by Lisa A. Martin, MD, MPH; Ariza; J. Scott Thomson, MLIS; and Helen J. Binns, MD, MPH, of the Pediatric Practice Research Group, the Mary Ann & J. Milburn Smith Child Health Research Program of the research center; and the Departments of Pediatrics and Preventive Medicine at the Feinberg School. The paper will be published in an upcoming issue of the Journal of Pediatrics.

GLI1 pathwayDefining a Developmental Pathway for Medulloblastoma

Medulloblastoma, a rapidly growing tumor of the cerebellum, accounts for almost one in five of all childhood brain tumors1. Younger children have poor outcomes, while those who survive are at risk for long-term neurological effects. Understanding the underlying mechanisms of medulloblastoma is necessary in order to develop better therapies for this devastating disease2. Previous research has shown that the GLI1 oncogene, part of the Sonic hedgehog (Shh) pathway, is overactive in a subset of medulloblastomas.

In a forthcoming publication in the International Journal of Cancer, Joon Won Yoon, PhD, David Walterhouse, MD, and colleagues studied changes in gene expression profiles in experimental cells transformed by GLI1. They compared the genes they identified to those found in medulloblastomas with activation of the Shh pathway. Of the 25 resulting genes, several hold possibilities for further study, including CXCR4, which plays a role in cerebellar development, and whose expression may re-initiate developmental programs that contribute to medulloblastoma. Another is p53, a tumor suppressor. The group identified a mutation in the p53 gene in their experimental cells, suggesting that inactivation of p53 may shift the balance toward cell survival and proliferation in this subset of medulloblastomas.

“Understanding mechanisms such as the hedgehog pathway in childhood cancers should one day help us develop better treatments,” Walterhouse said. “There are already inhibitors of these pathways, but to improve on them we need to better understand how they work.”

Walterhouse is associate professor of Pediatrics at the Feinberg School, a member of the Developmental Biology Program of the research center and an attending physician in the Division of Hematology, Oncology and Stem Cell Transplant at Children’s Memorial. Co-authors on the paper are Yoon; Richard Gilbertson, MD, PhD; Stephen Iannaccone; and Philip Iannaccone, MD, PhD.

1. American Brain Tumor Association, “Focusing on tumors: Medulloblastoma.”, accessed October 7, 2008.
2. Crawford JR, MacDonald TJ, Packer RJ. Medulloblastoma in childhood: new biological advances. Lancet Neurol. 2007 Dec;6(12):1073-85. Review.

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