Erick Gomez-Fuentes was born with "Prune Belly Syndrome," a defect that occurs once in every 40,000 births. Erick has been at UNC Hospitals since his birth in 2008 and has already touched the hearts of the doctors and nurses on his floor. His family's love and determination has sustained Erick as he awaits an intestinal transplant. Though the obstacles to Erick living a normal life are huge, his mother is determined to help him get well.

A rare occurrence anywhere, it was the third EXIT performed at UNC since Nancy Chescheir, M.D., performed the first in 2003. EXIT is a specialized fetal intervention procedure employed most commonly when problems during pregnancy may obstruct the fetus’s airway. In such cases, if the baby were delivered without the intervention, it could  be at risk for a loss of oxygen, says Bill Goodnight, M.D., assistant professor in the Department of Obstetrics and Gynecology who led a team of more than a dozen physicians and nurses in the procedure.

In EXIT, surgeons perform a specialized C-section while the mom is under general anesthesia in which the baby is partially delivered – head first, then a shoulder and an arm – leaving the baby attached to the placenta by the umbilical cord. The placenta continues to provide oxygen to the baby while the newborn’s airway is established.

In the procedure Thursday, “we had the baby on placental circulation for about 15 minutes,” Goodnight says. The team completed intubation and confirmed the airway with endoscopy then finished delivering the baby with no complications. “The baby went off to the nursery and the mom’s doing well.” The family chooses to remain anonymous.

At 28 weeks gestation a mass was detected on the baby’s neck, and Drs. Goodnight and Ashley Hickman, M.D., a senior fellow in obstetrics and gynecology, started thinking about the need  for delivery via EXIT. The mass continued to grow, and about a month ago a fetal MRI showed that the growth had begun to deviate the trachea, and was possibly affecting the fetus’s ability to swallow, so Goodnight began planning the EXIT.

Goodnight and Hickman are part of the Center for Maternal & Infant Health, which meets weekly with colleagues from other disciplines to review and coordinate care for babies with anomalies.

“Our goal is to provide all services available for the sickest babies in North Carolina. The collective expertise of this group allows us to do that, while coordinating care with a woman’s local doctor,” says Goodnight, who graduated from the UNC School of Medicine and completed a fellowship in maternal fetal medicine at the Medical University of South Carolina before returning to UNC last year.

“At UNC, we take our mission to the people of North Carolina very seriously,” says Daniel Clarke-Pearson, M.D., chairman of obstetrics and gynecology. “We are fortunate to have a collection of extremely skilled and caring physicians, in our department and across the hospital, that enables us to meet our mission.”

Kathleen Smith, M.D., and Adriene Ray, M.D., from adult anesthesiology had an especially crucial role, Goodnight says, because the mother had to be under general anesthesia while keeping the uterus relaxed and blood flow to the placenta normal.

Nate Nonoy, M.D., and Michael Danekas, M.D., from pediatric anesthesiology intubated the baby. Others on the team included George Retsch-Bogart, M.D., and Kathy Adobe from pediatric pulmonology; Cyril Engmann, M.D., and Lynne Harrington Johnson, M.D. from neonatal-perinatal medicine; and Amelia Drake in otolaryngology.

Over the next few days doctors will carefully keep watch over the baby and determine the best way to remove the growth.

• An impressive record of scholarly publications, including four textbooks and 39 chapters in other medical books
• An exhaustive atlas he compiled with co-author Dr. John Fenoglio in 1978, titled Tumors of the Cardiovascular System, which has been described as “a recognized classic in medical literature” and “a work of enduring usefulness”
• The Legion of Merit of the United States of America in 1984 and the Distinguished Achievement Award from the Society for Cardiovascular Pathology in 2001 and many, many others.

McAllister is the current president of the UNC Medical Alumni Association, a member of the UNC Board of Visitors and an executive committee member of the Medical Foundation of North Carolina.

     

Juan Reyes-Alonso, who started working as an interpreter at UNC Hospitals in 2003, explains what it was like for the hospitals’ 26 interpreters to work with the pager system that was in place back then.

“All of us carried pagers, and there was just one pager phone number for all of us. So, when a doctor requested an interpreter, all of our pagers would go off at the same time. And all of us who were free at the time would call the doctor back, so the doctor might get calls from 10 different interpreters all at once. It was a mess!” said Reyes-Alonso, UNC Hospitals’ lead interpreter.

About five years ago, the interpreters dropped the pager system and started using cell phones in conjunction with a Web-based dispatch management service called ServiceHub. The service was originally designed to meet the needs of the transportation and field service industries. UNC Hospitals was ServiceHub’s first hospital client, said Hans Wynholds, chairman of the Cupertino, Calif.-based company.

Shane Rogers, director of Interpreter Services at UNC Hospitals, said the ServiceHub system was a big improvement over the pager system.

“The ServiceHub system gave us many advantages that we didn’t have before,” Rogers said. “For example, it gave people requesting interpreters a Web page for submitting their requests, and they could see whether or not an interpreter had been assigned to handle their request, and if so, which one.”

“But the cell phones we were using didn’t work as well as we had hoped. In some parts of the hospital cell phones just can’t get a signal. And sometimes the cell phone service would go down, and then we had to rely on the phone company to get it up and running again.”

A possible solution to that problem presented itself about a year ago, when UNC Hospitals began installing a WiFi system throughout the medical center. “Once the hospitals’ own wireless system was in place, we realized this could be an opportunity for us to use wireless handheld devices instead of pagers or cell phones,” Rogers said.

So, Rogers asked ServiceHub if this was possible. ServiceHub then re-wrote the mobile portion of their customized software for UNC Hospitals that enabled the interpreters to access the ServiceHub system from handheld wireless devices.

	An iPod touch screen shot from the ServiceHub system. (The names and data shown here are fictional.)

At that point, Reyes-Alonso tested the system with several wireless devices, including the iPod Touch and Web-enabled cell phones. Of the half dozen or so devices he tested, he found the iPod Touch to be the easiest to use and the most effective, Reyes-Alonso said.

All 26 interpreters began carrying an iPod Touch in early September 2009. With the iPod Touch, the interpreters are always connected, no matter where they might be inside UNC Hospitals. When an interpreter responds to a request, all the other interpreters can see that response almost instantly, so the requester doesn’t get calls from 10 interpreters all at once. In addition, many tasks that used to require a computer – such as consulting a bilingual medical dictionary – can now be done through the iPod Touch.

“The iPod Touch has greatly reduced a lot of the frustrations we used to have with our pagers and our cell phones,” Reyes-Alonso said. “I love it.”

Rogers cites additional advantages.

“Now that we’re using the hospitals’ own wireless system, if the system goes down, then we’ll be dealing with our own people to get it back up, instead of having to call the phone company and wait for them to take care of it.” Plus, Rogers said, the monthly cost to his department to lease their iPod Touches from ServiceHub is about half what it cost for the same number of cell phones.

Wynholds says UNC Hospitals was the first of its hospital clients to fully implement the mobile version of its system. Several other hospitals are now thinking about following UNC Hospitals’ example, he said.

“UNC has really embraced the technology, and they have worked hard to help us understand how to make it work effectively in the hospital environment,” he said.

“We’ve learned a lot in working with UNC over the years, and we’ve pretty much allowed the product to evolve in line with their specifications.”

CHAPEL HILL -- A study from the University of North Carolina at Chapel Hill has identified a key player in a molecular process essential for DNA replication within cells.

The new findings highlight a protein called FLASH, already shown to play a role in initiating apoptosis, or programmed cell death.  Apoptosis is a normal biochemical response that occurs when a cell is damaged beyond repair after viral infection or accumulation of mutations that could lead to uncontrolled cellular proliferation, or cancer.  Apoptosis is also crucial to the developing embryo through selective cell death, which allows proper differentiation of physical structures, such as fingers and toes.

According to senior study author Zbigniew Dominski, Ph.D., associate professor of biochemistry and biophysics at UNC, the new study demonstrates that FLASH is also required for the proper synthesis of histone messenger RNA, which gives rise to histone proteins.

Histones are the chief protein components of chromatin and act as a scaffold allowing packaging of DNA into a condensed form that fits inside the nucleus of a cell. As the DNA interacts with histones and with metabolic signals from within the cell, these proteins help regulate gene expression.

“Our study suggests for the first time that a potential link exists between the processes of histone messenger RNA formation and apoptosis,” Dominski said. “FLASH is crucial for the production of histone messenger RNA, without which the cell can’t make the histone proteins around which DNA is packaged.”

The research is described in the Oct. 23, 2009 issue of the journal Molecular Cell.

For the study, Dominski adapted a laboratory system that reproduces in the test tube what normally occurs in the cell when FLASH participates in the biochemical cleavage event that results in mature histone messenger RNA. This enabled his team to explore what might occur when FLASH was added or removed.

“We could then figure out exactly what portion of FLASH would restore the protein’s function in generating histone mRNAs and remarkably, only the first 100 or so amino acids are required.  The remaining 2,000 amino acids of this large protein likely control other processes in the cell, including apoptosis and DNA replication,” he explained.

Co-author William F. Marzluff, Ph.D., is distinguished professor of biochemistry and biophysics and executive associate dean for basic research in the UNC School of Medicine. He noted that FLASH is the first component found in this protein complex “that integrates or initiates many cellular functions – DNA replication, apoptosis, histone production.  Having this small piece of the puzzle makes it a lot easier to identify others.” 

Other UNC coauthors include Xiao-cui Yang, laboratory technician, and Yan Yan, undergraduate student, both from the Department of Biochemistry and Biophysics and the UNC Program in Molecular Biology and Biotechnology and Brandon D. Burch, graduate student in in genetics and molecular biology.

Funding for the study came from the National Institute of General and Medical Sciences, a component of the National Institutes of Health.