Skip to main content
NIH Clinical Center
  Home | Contact Us | Site Map | Search
About the Clinical Center
For Researchers and Physicians
Participate in Clinical Studies

This file is provided for reference purposes only. It was current when it was produced, but it is no longer maintained and may now be out of date. Persons with disabilities having difficulty accessing information may contact us for assistance. For reliable, current information on this and other health topics, we recommend consulting the NIH Clinical Center at http://www.cc.nih.gov/
 
Clinical Center News

Published monthly for CC employees by Office of Communications, Patient Recruitment, and Public Liaison.

past issues

February 2009


‘Take the training, Hope to never use it’: Instruction could help save a life

Informatics lecture series kicks off with data repositories

Tanzanian medical facility conducts NIH-funded clinical research

How to navigate the Mark O. Hatfield Clinical Research Center

Genetic disorder affecting sleep pattern explored

NIH School helps students stay on track

Study of genetic syndrome may reveal pathway to aneurysm formation

Winter’s first snowstorm covers the CC in a sheet of white

NCCAM announces Straus lecture

Patient Safety Champion Award nominations open

News Briefs

New clinical research protocols

Upcoming Events


       

'Take the training, Hope to never use it’
Instruction could help save a life

Evette Barranta models an AED in Bldg 10

As a member of the CC code team, ICU nurse Evette Barranta is one of the first responders to a medical emergency such as cardiac arrest—a “code blue.” The rest of the team consists of a another critical care nurse, two respiratory therapists, a critical care fellow , a surgery fellow, an escort service representative, security, and ambulance response. Barranta’s code cart brings a defibrillator, cardiac medications, an intubation kit and other emergency supplies. The CC installed automated external defibrillators around the building, including near each elevator, so that any staff member or visitor can address a cardiac arrest incident immediately.


By Maggie McGuire

For one December patron of the Rockledge Fitness Center the choice to pursue a healthy, longer life delivered more immediate results than expected. Hit by a heart attack during a workout, the man was saved by the quick thinking of NIH staff and the close proximity of an automated external defibrillator (AED).

“Thanks for saving my life! ... I was very lucky to have this happen in the gym with trained people around,” the survivor wrote in an e-mail to Jewell Webb of NIDA and Peggy Fitzgibbon and Heather Bridge, both of NIAID.

With AEDs available in many public arenas, such as airports and shopping malls, familiarity with the machine and training in cardiopulmonary resuscitation (CPR) are tools of responsible citizenship. The Rockledge incident and another recently at the NIH Executive Boulevard location remind that preparation in cardiac arrest response can mean the difference between life and death.

The NIH Office of Research Services, Division of Occupational Health and Safety’s Occupational Medical Service offers an instructional course multiple times a week, teaching how to properly perform compressions and use the defibrillators. The Clinical Center installed more than 60 AEDS in the Hatfield building in 2006, joining the 33 in the Magnuson building. They are on each level of the new structure at each elevator bank.

Webb had CPR training where she practiced using the AEDs as part of her aerobic instructor certification. On December 15, when a gym-goer collapsed, she, Fitzgibbon, and Bridge performed compressions and gave breaths to the victim while waiting for paramedics to arrive. When their efforts failed to maintain a consistent pulse, the women deployed the defibrillator to shock his heart.

“It was a life-altering experience,” reflected Fitzgibbon.

NIAAA's Julie Usala practices CPR

Julie Usala of NIAAA practices compressions on Annie the Manne-quin at an Occupational Medical Service basic life training course.


For the crew at the Verizon office in the NIH Executive Boulevard location cardiopulmonary resuscitation training is a biyearly requirement. When a co-worker dropped to the ground on December 3, Steve Elmore and Wesley Reynolds knew what to do and sprang into action. Their fellow employee will return to work February 1, Elmore said.

“I felt pretty proud that I could help,” said Elmore, an NIH contractor.

Webb addressed the concern of some who think they would be too afraid to take action in a crisis, “If the situation presents itself, people will be surprised at what they can do. Take the training and hope you never have to use it.”
Juli Egebrecht, director and coordinator of basic life training in the Occupational Medical Service, has taught resuscitation training at NIH for 24 years and estimates about 1,400 people take her course each year. Performing deep, fast compressions in the middle of the chest at the rate of 100 per minute is the most effective technique, Egebrecht said. She advises taking turns with another responder to maintain quality compressions.

automated external febrillator

The latest automated external defibrillators come with detailed visual and audio instructions.




While CPR can be effective, in severe cases of cardiac arrest the shock of an AED is necessary to restart a victim’s heart. The defibrillators in the CC are advanced models that show and tell the user how to properly use the machine, said Michael Dunn, automated external defibrillator program manager in the NIH Office of Research Services, Division of Occupational Health and Safety. A visual on the AED instructs users where to place the pads on the chest of the person in cardiac arrest. The defibrillator reads the heart rhythm of the victim; orally reports if a shock is necessary; and, if so, delivers an electric current. After another heart rhythm reading, the machine administers a second shock, if necessary, and tells the user to continue with resuscitation attempts, said Dunn.

Despite efforts to make the process user-friendly, people should still familiarize themselves with the machine before it needs to be used in a crisis, Dunn said.

Practice and learn more in Egebrecht’s course—sign up at http://dohs.ors.od.nih.gov/cpr_training.htm. Dunn’s office trains on-site as requested; call 301-451-3294 for more information.

 

Back to Top

 

 


   

Informatics lecture series kicks off with data repositories

The Biomedical Translational Research Information System (BTRIS) team hosted the first in a series of lectures on informatics in biomedical and translational research on Jan. 21. Speaker was Michael Kamerick, director of academic research systems and co-director of biomedical informatics at the Clinical and Translational Sciences Institute, University of California, San Francisco. His talk focused on integrated data repositories, large-scale databases containing information from the full array of information systems in a biomedical enterprise. The repositories can be valuable tools for meta-analysis, delivering refined results quickly and securely. 

Data contained in the repositories come from clinical and clinical trials systems, life sciences (genomics, proteomics), research, billing, and registries, clinical trial systems and more. Integrated data repositories support a variety of functions, including hypothesis testing, cohort development, genome/phenome matching, genome-wide association studies, development of quality measures, and general population-based studies.

Gathering such data today takes one of two painful approaches, Kamerick said. Review of paper charts can take years. Manual “screen scraping,” transcribing records from an electronic medical record to a spreadsheet, is time consuming and error prone “Along the way, everything private about every individual is exposed to those investigators: name, address, phone number, social security, medical record number,” he said.

 “The average time it takes from lab discovery to dissemination in general medical practice of a valuable medical discovery is 17 years,” Kamerick said. “This is just too much time. My immediate focus in building these systems is to take time out of the research cycle.”

The repository he uses, i2b2 (Informatics for Integrating Biology and the Bedside), is an open-source platform developed at Partners HealthCare System through NIH funding. At the January 21 lecture, Kamerick demoed the i2b2 workbench, showing how a researcher’s information can be collected and categorized. Categories include demographics, diagnoses, and lab results, depending on the initial query. This proxy chart review reveals only what the user requests and protects patient privacy, Kamerick said.

“There’s this line from privacy to utility in data, and usually more privacy means less utility and vice versa,” said Kamerick. “So how to get to the point where this is sufficient privacy and sufficient utility so science advances and people still feel protected is the great challenge, and I think that this kind of technology offers the possibility.”


Still, integrated data repositories are not the perfect solution to current hurdles in cross-research analysis. There is concern that narrative text explaining the subtle nuances and ambiguities of the human condition will be lost in the entry of forms and fields.  Natural language processing software, which translates computer data to readable human language, reports a 70 percent accuracy rate, with efforts to see that number climb in development, Kamerick said.

Data quality, stakeholder interests, patient rights to opt out of participation, data ownership by either clinician or institution, and security requirements are other issues that are part of ongoing discussions about integrated data repositories. Each academic institution addresses such issues in its own regard, said Kamerick, and is learning from other’s successes and failures.

Upcoming lectures are—Dr. Adam Wilcox of New York Presbyterian Hospital on March 24; Dr. Henry Lowe of the Stanford University School of Medicine on April 21; Dr. Umberto Tachinardi of the University of Chicago on May 19; Dr. Shawn Murphy of Massachusetts General Hospital on June 16; and Dr. Jim Cimino, chief of the CC's Laboratory for Informatics Development, on September 15.

View Kamerick’s lecture in its entirety at http://btris.nih.gov.

Back to Top


   

Tanzanian medical facility conducts NIH-funded clinical research

By Jenny Haliski

KCMC in Tanzania

A typical hospital room at KCMC includes about a dozen beds, often with several patients and their visitors sharing one.


A wheelbarrow used for an ambulance. Dusty, rut-ridden roads that make seeing a doctor a rare event reserved for the seriously ill. Patients with limbs suspended by strings for skeletal traction.

These are a few scenes from Kilimanjaro Christian Medical Centre (KCMC), a hospital in the small town of Moshi in northern Tanzania near the foot of Mt. Kilimanjaro. KCMC, together with Duke University Medical Center and KIWAKKUKI, a community organization fighting HIV/AIDS, receives NIH funding to conduct clinical research on HIV/AIDS, malaria, tuberculosis, and other infectious diseases.

KCMC, which opened in 1971 through the Good Samaritan Foundation, is a consultant, referral hospital, and patients must pay to create a medical record there. Patients are referred to KCMC from the nearby Mawenzi Regional Hospital, where care is free. Clinical officers come to Mawenzi to recruit patients who qualify for NIH-funded studies. The 450-bed KCMC offers inpatient and outpatient services and often operates at 130 percent capacity, with a daily patient census closer to 600.

The seeds of KCMC’s research collaboration were sown in the 1980s, when Duke partnered with Tanzanian colleagues in Dar es Salaam, where Professor John Shao worked before he moved to Moshi to become KCMC executive director in the mid-1990s. The KCMC/Duke relationship moved with Shao and in 2002 expanded its focus on HIV/AIDS prevention, treatment and care. NIH funding followed and snowballed as grants to develop the site’s infrastructure and capacity, launch an initial HIV voluntary counseling and testing service, and establish training opportunities built on each other.
KCMC advisory board meeting
The KCMC community advisory board shapes the research done at the centre based on the needs of local Tanzanian residents.

Dr. John Crump, Duke’s principal investigator at the site, says that although their research focus has been HIV/AIDS and other infectious diseases, it is expanding into other health issues: cervical cancer, cardiovascular disease, and car crash injuries. “I take a long time—decades—to measure progress,” said Crump. “Over that length of time, what staff can we train, what infrastructure can we establish, and what papers can we publish with data that will influence health policies? Our goal is to deliver all aspects of the program into well-equipped Tanzanian hands.”

Currently, there aren’t enough Tanzanian hands ready to take on that challenge. According to Shao, the country’s ratio of physicians to patients is one physician to 23,000 people. Increasing that ratio is one of Tanzania’s, and KCMC’s, major goals. In addition to the hospital, KCMC operates 14 allied health science teaching schools and the Tumaini University Kilimanjaro Christian Medical College. The new medical school building, opened in late 2008, will increase class sizes from 30 to 133 students.

Editor’s note: Haliski, former CCNews editor, was in Moshi last fall and wrote about NIH-funded research there. She’s currently a public affairs specialist in the NIH Office of Communications and Public Liaison.

Back to Top


   

How to navigate the Mark O. Hatfield Clinical Research Center

Getting Around the Mark O. Hatfield Clinical Research Center
There are five major corridors labeled 1000 to 5000 running north to south. Each room is numbered from 100 to 900 running east to west.

Floors 1, 3, 5, and 7 are for patient care. Floors 2, 4, and 6 house offices and interstitial space (infrastructure) space.

Finding a Room
Each room has a five-digit room number. The first digit of the room number is the floor; the second number is the corridor along with each room number.

Map of the NIH CRC NIH Clinical Center February 2009
Skip to main content
NIH Clinical Center
  Home | Contact Us | Site Map | Search
About the Clinical Center
For Researchers and Physicians
Participate in Clinical Studies

This file is provided for reference purposes only. It was current when it was produced, but it is no longer maintained and may now be out of date. Persons with disabilities having difficulty accessing information may contact us for assistance. For reliable, current information on this and other health topics, we recommend consulting the NIH Clinical Center at http://www.cc.nih.gov/
 
Clinical Center News

Published monthly for CC employees by Office of Communications, Patient Recruitment, and Public Liaison.

past issues

February 2009


‘Take the training, Hope to never use it’: Instruction could help save a life

Informatics lecture series kicks off with data repositories

Tanzanian medical facility conducts NIH-funded clinical research

How to navigate the Mark O. Hatfield Clinical Research Center

Genetic disorder affecting sleep pattern explored

NIH School helps students stay on track

Study of genetic syndrome may reveal pathway to aneurysm formation

Winter’s first snowstorm covers the CC in a sheet of white

NCCAM announces Straus lecture

Patient Safety Champion Award nominations open

News Briefs

New clinical research protocols

Upcoming Events


       

'Take the training, Hope to never use it’
Instruction could help save a life

Evette Barranta models an AED in Bldg 10

As a member of the CC code team, ICU nurse Evette Barranta is one of the first responders to a medical emergency such as cardiac arrest—a “code blue.” The rest of the team consists of a another critical care nurse, two respiratory therapists, a critical care fellow , a surgery fellow, an escort service representative, security, and ambulance response. Barranta’s code cart brings a defibrillator, cardiac medications, an intubation kit and other emergency supplies. The CC installed automated external defibrillators around the building, including near each elevator, so that any staff member or visitor can address a cardiac arrest incident immediately.


By Maggie McGuire

For one December patron of the Rockledge Fitness Center the choice to pursue a healthy, longer life delivered more immediate results than expected. Hit by a heart attack during a workout, the man was saved by the quick thinking of NIH staff and the close proximity of an automated external defibrillator (AED).

“Thanks for saving my life! ... I was very lucky to have this happen in the gym with trained people around,” the survivor wrote in an e-mail to Jewell Webb of NIDA and Peggy Fitzgibbon and Heather Bridge, both of NIAID.

With AEDs available in many public arenas, such as airports and shopping malls, familiarity with the machine and training in cardiopulmonary resuscitation (CPR) are tools of responsible citizenship. The Rockledge incident and another recently at the NIH Executive Boulevard location remind that preparation in cardiac arrest response can mean the difference between life and death.

The NIH Office of Research Services, Division of Occupational Health and Safety’s Occupational Medical Service offers an instructional course multiple times a week, teaching how to properly perform compressions and use the defibrillators. The Clinical Center installed more than 60 AEDS in the Hatfield building in 2006, joining the 33 in the Magnuson building. They are on each level of the new structure at each elevator bank.

Webb had CPR training where she practiced using the AEDs as part of her aerobic instructor certification. On December 15, when a gym-goer collapsed, she, Fitzgibbon, and Bridge performed compressions and gave breaths to the victim while waiting for paramedics to arrive. When their efforts failed to maintain a consistent pulse, the women deployed the defibrillator to shock his heart.

“It was a life-altering experience,” reflected Fitzgibbon.

NIAAA's Julie Usala practices CPR

Julie Usala of NIAAA practices compressions on Annie the Manne-quin at an Occupational Medical Service basic life training course.


For the crew at the Verizon office in the NIH Executive Boulevard location cardiopulmonary resuscitation training is a biyearly requirement. When a co-worker dropped to the ground on December 3, Steve Elmore and Wesley Reynolds knew what to do and sprang into action. Their fellow employee will return to work February 1, Elmore said.

“I felt pretty proud that I could help,” said Elmore, an NIH contractor.

Webb addressed the concern of some who think they would be too afraid to take action in a crisis, “If the situation presents itself, people will be surprised at what they can do. Take the training and hope you never have to use it.”
Juli Egebrecht, director and coordinator of basic life training in the Occupational Medical Service, has taught resuscitation training at NIH for 24 years and estimates about 1,400 people take her course each year. Performing deep, fast compressions in the middle of the chest at the rate of 100 per minute is the most effective technique, Egebrecht said. She advises taking turns with another responder to maintain quality compressions.

automated external febrillator

The latest automated external defibrillators come with detailed visual and audio instructions.




While CPR can be effective, in severe cases of cardiac arrest the shock of an AED is necessary to restart a victim’s heart. The defibrillators in the CC are advanced models that show and tell the user how to properly use the machine, said Michael Dunn, automated external defibrillator program manager in the NIH Office of Research Services, Division of Occupational Health and Safety. A visual on the AED instructs users where to place the pads on the chest of the person in cardiac arrest. The defibrillator reads the heart rhythm of the victim; orally reports if a shock is necessary; and, if so, delivers an electric current. After another heart rhythm reading, the machine administers a second shock, if necessary, and tells the user to continue with resuscitation attempts, said Dunn.

Despite efforts to make the process user-friendly, people should still familiarize themselves with the machine before it needs to be used in a crisis, Dunn said.

Practice and learn more in Egebrecht’s course—sign up at http://dohs.ors.od.nih.gov/cpr_training.htm. Dunn’s office trains on-site as requested; call 301-451-3294 for more information.

 

Back to Top

 

 


   

Informatics lecture series kicks off with data repositories

The Biomedical Translational Research Information System (BTRIS) team hosted the first in a series of lectures on informatics in biomedical and translational research on Jan. 21. Speaker was Michael Kamerick, director of academic research systems and co-director of biomedical informatics at the Clinical and Translational Sciences Institute, University of California, San Francisco. His talk focused on integrated data repositories, large-scale databases containing information from the full array of information systems in a biomedical enterprise. The repositories can be valuable tools for meta-analysis, delivering refined results quickly and securely. 

Data contained in the repositories come from clinical and clinical trials systems, life sciences (genomics, proteomics), research, billing, and registries, clinical trial systems and more. Integrated data repositories support a variety of functions, including hypothesis testing, cohort development, genome/phenome matching, genome-wide association studies, development of quality measures, and general population-based studies.

Gathering such data today takes one of two painful approaches, Kamerick said. Review of paper charts can take years. Manual “screen scraping,” transcribing records from an electronic medical record to a spreadsheet, is time consuming and error prone “Along the way, everything private about every individual is exposed to those investigators: name, address, phone number, social security, medical record number,” he said.

 “The average time it takes from lab discovery to dissemination in general medical practice of a valuable medical discovery is 17 years,” Kamerick said. “This is just too much time. My immediate focus in building these systems is to take time out of the research cycle.”

The repository he uses, i2b2 (Informatics for Integrating Biology and the Bedside), is an open-source platform developed at Partners HealthCare System through NIH funding. At the January 21 lecture, Kamerick demoed the i2b2 workbench, showing how a researcher’s information can be collected and categorized. Categories include demographics, diagnoses, and lab results, depending on the initial query. This proxy chart review reveals only what the user requests and protects patient privacy, Kamerick said.

“There’s this line from privacy to utility in data, and usually more privacy means less utility and vice versa,” said Kamerick. “So how to get to the point where this is sufficient privacy and sufficient utility so science advances and people still feel protected is the great challenge, and I think that this kind of technology offers the possibility.”


Still, integrated data repositories are not the perfect solution to current hurdles in cross-research analysis. There is concern that narrative text explaining the subtle nuances and ambiguities of the human condition will be lost in the entry of forms and fields.  Natural language processing software, which translates computer data to readable human language, reports a 70 percent accuracy rate, with efforts to see that number climb in development, Kamerick said.

Data quality, stakeholder interests, patient rights to opt out of participation, data ownership by either clinician or institution, and security requirements are other issues that are part of ongoing discussions about integrated data repositories. Each academic institution addresses such issues in its own regard, said Kamerick, and is learning from other’s successes and failures.

Upcoming lectures are—Dr. Adam Wilcox of New York Presbyterian Hospital on March 24; Dr. Henry Lowe of the Stanford University School of Medicine on April 21; Dr. Umberto Tachinardi of the University of Chicago on May 19; Dr. Shawn Murphy of Massachusetts General Hospital on June 16; and Dr. Jim Cimino, chief of the CC's Laboratory for Informatics Development, on September 15.

View Kamerick’s lecture in its entirety at http://btris.nih.gov.

Back to Top


   

Tanzanian medical facility conducts NIH-funded clinical research

By Jenny Haliski

KCMC in Tanzania

A typical hospital room at KCMC includes about a dozen beds, often with several patients and their visitors sharing one.


A wheelbarrow used for an ambulance. Dusty, rut-ridden roads that make seeing a doctor a rare event reserved for the seriously ill. Patients with limbs suspended by strings for skeletal traction.

These are a few scenes from Kilimanjaro Christian Medical Centre (KCMC), a hospital in the small town of Moshi in northern Tanzania near the foot of Mt. Kilimanjaro. KCMC, together with Duke University Medical Center and KIWAKKUKI, a community organization fighting HIV/AIDS, receives NIH funding to conduct clinical research on HIV/AIDS, malaria, tuberculosis, and other infectious diseases.

KCMC, which opened in 1971 through the Good Samaritan Foundation, is a consultant, referral hospital, and patients must pay to create a medical record there. Patients are referred to KCMC from the nearby Mawenzi Regional Hospital, where care is free. Clinical officers come to Mawenzi to recruit patients who qualify for NIH-funded studies. The 450-bed KCMC offers inpatient and outpatient services and often operates at 130 percent capacity, with a daily patient census closer to 600.

The seeds of KCMC’s research collaboration were sown in the 1980s, when Duke partnered with Tanzanian colleagues in Dar es Salaam, where Professor John Shao worked before he moved to Moshi to become KCMC executive director in the mid-1990s. The KCMC/Duke relationship moved with Shao and in 2002 expanded its focus on HIV/AIDS prevention, treatment and care. NIH funding followed and snowballed as grants to develop the site’s infrastructure and capacity, launch an initial HIV voluntary counseling and testing service, and establish training opportunities built on each other.
KCMC advisory board meeting
The KCMC community advisory board shapes the research done at the centre based on the needs of local Tanzanian residents.

Dr. John Crump, Duke’s principal investigator at the site, says that although their research focus has been HIV/AIDS and other infectious diseases, it is expanding into other health issues: cervical cancer, cardiovascular disease, and car crash injuries. “I take a long time—decades—to measure progress,” said Crump. “Over that length of time, what staff can we train, what infrastructure can we establish, and what papers can we publish with data that will influence health policies? Our goal is to deliver all aspects of the program into well-equipped Tanzanian hands.”

Currently, there aren’t enough Tanzanian hands ready to take on that challenge. According to Shao, the country’s ratio of physicians to patients is one physician to 23,000 people. Increasing that ratio is one of Tanzania’s, and KCMC’s, major goals. In addition to the hospital, KCMC operates 14 allied health science teaching schools and the Tumaini University Kilimanjaro Christian Medical College. The new medical school building, opened in late 2008, will increase class sizes from 30 to 133 students.

Editor’s note: Haliski, former CCNews editor, was in Moshi last fall and wrote about NIH-funded research there. She’s currently a public affairs specialist in the NIH Office of Communications and Public Liaison.

Back to Top


   

How to navigate the Mark O. Hatfield Clinical Research Center

Getting Around the Mark O. Hatfield Clinical Research Center
There are five major corridors labeled 1000 to 5000 running north to south. Each room is numbered from 100 to 900 running east to west.

Floors 1, 3, 5, and 7 are for patient care. Floors 2, 4, and 6 house offices and interstitial space (infrastructure) space.

Finding a Room
Each room has a five-digit room number. The first digit of the room number is the floor; the second number is the corridor along with each room number.

Map of the NIH CRC

Back to Top


   

Genetic disorder affecting sleep pattern explored

By Bill Schmalfeldt

Smith-Magenis syndrome is many times mistaken for something else like Down syndrome. Other times, it is missed completely.
Thanks to the CC’s Bedside-to-Bench Awards program a team of dedicated researchers is working to increase awareness of the syndrome and find new and better ways to treat it.

Leading the team is Dr. Ann C. M. Smith, the Smith in Smith-Magenis syndrome. As a genetic counselor at Children’s Hospital in Denver in 1981, she saw two infants with failure to thrive and defects of the heart and palate, developmental delay, a typical facial appearance, short stature with small hands and feet, low muscle tone, and early expressive speech and motor delays. They were identified to have a piece missing from the short arm of chromosome 17, called deletion 17p11.2. With Dr. Ellen Magenis, a cytogeneticist at Oregon Health & Sciences University, Smith published a paper in 1986 identifying the first nine individuals with deletion 17p11.2., expanding the now-trademark characteristics to include middle ear problems, hoarse voice, ocular abnormalities, sleep disturbances, and neurobehavioral features, such as hyperactivity and aggressive behaviors. The syndrome was officially named in 1989.

Smith, who is a senior genetic counselor with NHGRI and recently retired from the medical faculty at Georgetown University Medical School, leads a team that has received two Bedside-to-Bench Awards, the first in 1999.

That award allowed for a natural history protocol (01-HG-0109) and a research roundtable with experts, researchers, and the parents’ support group PRISMS. These efforts resulted in new findings, including the validation of early expressive speech delays caused by underlying physiological issues related to low muscle tone, decreased tongue strength and movement, laryngeal and palatal abnormalities, poor suck reflex, difficulties transitioning to solid foods, and open mouth posture with drooling.

team working on Smith-Magenis syndrome

Those working on Smith-Magenis Syndrome came from different ICs: (front row, from left) Hanna Hildenbrand, CC Rehabilitation Medicine; Ann Smith, NHGRI; CAPT Michaele Smith, CC Rehabilitation Medicine; (back row) Rebecca Morse, NHGRI; Dr. Maryland Pao, NIMH; Beth Solomon, CC Rehabilitation Medicine; Dr. Wallace Duncan, NIMH; and Dr. Marjan Huizing, NHGRI.





The initial protocol also showed that many with SMS have short stature.  “We’re collecting growth data to generate specific growth curves,” Smith said. Other findings of the initial natural history protocol showed that patients tend toward obesity and elevated total cholesterol during adolescence. “One of genes in this region (of the chromosome deletion) is involved in cholesterol management,” Smith explained.

The natural history protocol also defined an earlier onset of sensory neural hearing loss, occurring shortly after age 10, and an increased frequency of hyperacusis, an over-sensitivity to certain sound frequencies. A person with severe hyperacusis has difficulty tolerating everyday sounds, which may seem unpleasantly loud.

The research team got a second award in 2004, used to assemble an approach to what Smith called “the novel inverted melatonin circadian rhythm” found in Smith-Magenis syndrome patients. Where most produce the sleep hormone melatonin at night (light usually suppresses the formation of the hormone), people with Smith-Magenis syndrome produce it during the day. This causes chronic sleep disturbances with frequent nighttime and early morning awakenings compounded by daytime sleepiness.

Smith’s Phase I treatment trial, begun last year, was designed with information derived from Actiwatches, wristwatch-like accelerometers used to measure a patient’s activity-rest cycle in the home setting and report on the patient’s sleep pattern. Actigraphy is less invasive than standard sleep studies and provides a continuous estimate of sleep based on wrist motion for a prolonged period.

The second Bedside-to-Bench Award funded the purchase of bright light boxes and the creation of a melatonin tablet used for the treatment study.

“Melatonin isn’t regulated by the FDA. You can buy it at health food stores,” Smith said. “But for NIH, we wanted a purer form of melatonin with a time delayed release. So the NIH Pharmacy developed a coated tablet.”

Patients with Smith-Magenis syndrome and healthy volunteers began enrolling in the protocol in April to test the safety and efficacy of the melatonin tablet.

Official statistics on Smith-Magenis syndrome suggest that one out of every 25,000 children is born with the disease, but Smith thinks the number is more like one out of every 15,000. With improved molecular genetic techniques for examining chromosomes, she said, doctors will diagnose more children who might have gone unrecognized.

Smith looks at herself as something of a team captain. “I’ve got some stellar researchers who, before they met this syndrome, didn’t know a thing about it,” she said.  “Now, probably, they have more experience than anyone else in the world.”

She is excited by the prospect of new discovery in the Phase I trial. “I think this population can actually offer us a better understanding of something that is so unusual—this inverted circadian rhythm of melatonin,” she said.  “It will open a new understanding of this syndrome.”

Back to Top


   

NIH School helps students stay on track

Anne Wasson teaches at the NIH School
Teachers in the NIH Children’s School, located across from the main playroom on the Hatfield Building’s first floor, tutor patients in grades kindergarten through 12 and patients studying for the General Educational Development Test. A 2008 Clinical Center Director’s Award honored school staff, including Anne Wasson (above), for their dedication to patient care. She is going over a math assignment with Erik Santa, 14.

Back to Top

 

 

 


   

Study of genetic syndrome may reveal pathway to aneurysm formation

By Maggie McGuire

Dr. Harry Dietz covered experimental therapies for a genetic disorder and how those treatments translate to other afflictions in the Astute Clinician Lecture given January 14.

“Marfan Syndrome and Related Disorders: From Molecules to Medicines” was part of the NIH Director’s Wednesday Afternoon Lecture Series.

Marfan syndrome, a disorder of the connective tissue, affects the ocular, skeletal, and cardiovascular systems. Approximately one in 5,000 Americans has the disorder, which commonly presents with overgrowth of bones, dislocation of the ocular lens, and progressive dilatation of the aortic root. Even with state-of-the-art medical management, Dietz said, Marfan syndrome is still associated with early mortality.

“Can we do better?” Dietz asked the crowd gathered in Masur Auditorium.

Dietz is professor of pediatrics, medicine, and molecular biology and genetics at Johns Hopkins University School of Medicine, and investigator at Howard Hughes Medical Institute. He earned his medical degree from SUNY Upstate Schools of Medicine in Syracuse and completed residencies in pediatrics and anesthesia and in critical care medicine at The Johns Hopkins Hospital.

In his Astute Clinician Lecture Dietz presented that Marfan syndrome results from a deficiency of the fibrillin-1 protein—responsible for maintaining elastic fiber integrity in connective tissue. A slide showed a non-affected patient’s elastic fibers densely packed and well organized and a Marfan syndrome patient’s fibers fragmented and in disarray.

Fibrillin is a component of microfibrils. “With a deficiency of microfibrils, as seen in Marfan syndrome, we observed promiscuous activation of transforming growth factor beta (TGFB),” said Dietz. This activation is thought to speed dilatation of the aorta. Marfan mouse models showed significant improvement in aortic wall architecture and thickness when injected with a TGFB-neutralizing antibody or when treated by losartan, an angiotensin II type-1 receptor blocker that both lowers blood pressure and antagonizes TGFB.

A Phase III trial sponsored by NHLBI’s Pediatric Heart Network and The National Marfan Foundation is testing the efficacy of losartan in slowing aortic root growth in Marfan syndrome patients. A preliminary clinical experience in children with severe Marfan syndrome showed a pronounced decline in aortic root growth after starting losartan.

The speaker and his fellow researchers identified a connection between TGFB and Loeys-Dietz syndrome, too. This vascular condition shares many characteristics with Marfan syndrome, including aortic and arterial aneurysms, leading scientists to explore a similar genetic basis. “The emerging view is that transforming growth factor beta may be a final common pathway to aneurysm formation,” Dietz said.

Remarkably, losartan showed the ability to attenuate or prevent manifestations of Marfan syndrome outside of the cardiovascular system in mouse models including developmental emphysema and skeletal muscle myopathy. Dietz and colleagues went on to show that TGFB also contributes to failed muscle regeneration in a mouse model of Duchenne muscular dystrophy and that losartan could reverse this defect, preserving muscle architecture, size, and function.

The Astute Clinician Lecture was established through a gift from the late Dr. Robert W. Miller and his wife, Haruko. It honors a US scientist who has observed an unusual clinical occurrence, and by investigating it, has opened an important new avenue of research.

To view Dietz’s lecture, visit http://videocast.nih.gov/.

Back to Top


   

Winter’s first snowstorm covers the CC in a sheet of white

snow near the CC south entrance

The west courtyard’s Healing Waters statue and the Magnuson Building’s B1-level patio saw little action on January 27, as the first real snow of the year left its mark.

The Office of Research Facilities (ORF) and the Office of Research Services released recommendations for avoiding an accident or injury in inclement weather. Drive slowly, and do not be intimidated by other drivers into going faster than you are comfortable with. Maintain a safe distance between your vehicle and the one in front of you.

snow near the CC

Note that elevated surfaces (bridges, ramps, the top level of a parking garage) freeze first. If your car begins sliding: take your foot off the gas, steer your vehicle the same direction you are sliding, and gently pump the brake pedal. If your car has anti-lock brakes, use a steady, firm pressure without pumping.

When walking outside in the snow and ice, wear proper footwear. Refrain from walking between parked cars in icy lots, as the shade slows melting. ORF handles pathway and road treatments.

If a sidewalk, roadway, or other surface on the NIH main campus remains hazardous after a crew has visited the area, please report the problem to ORF at 301-435-8000.

Back to Top

 


   

NCCAM announces Straus lecture

Dr. Nuland
Dr. Sherwin B. Nuland of Yale University will deliver the first Stephen E. Straus Distinguished Lecture on March 10 in Masur Auditorium.
On March 10, NCCAM will hold the inaugural Stephen E. Straus Distinguished Lecture in the Science of Complementary and Alternative Medicine. Dr. Sherwin B. Nuland, clinical professor of surgery at Yale University and distinguished author, will present the first lecture “Chinese Medicine, Western Science, and Acupuncture” at 2:30 pm in Masur Auditorium.

During visits to China, Nuland observed major surgery done with no anesthesia, aided by acupuncture. In the Straus lecture, he will describe these operations and tell of his experience. In his recent book, The Uncertain Art, Nuland wrote “the basis of acupuncture’s practical usefulness, even in the operating room, has still not been explained in terms acceptable to most orthodox Western scientists using orthodox Western investigative methods.”

Dr. Stephen E. Straus was the first director of NCCAM. “Steve created a legacy we are continuing to build upon,” said Dr. Josephine P. Briggs, NCCAM’s current director. “He advocated for the most rigorous scientific methods to preserve NCCAM’s credibility. A lecture series in his name is only fitting to honor the work of such a brilliant and extraordinary scientist.”

In addition to serving as NCCAM director, Straus was the senior investigator in the Laboratory of Clinical Investigation at NIAID and was also part of the nationwide research team that showed a vaccine was effective in preventing shingles in older adults. He died in May 2007.

 

Back to Top


   

Patient Safety Champion Award nominations open

Nominations for the 2009 Patient Safety Champion Award are being accepted through Feb. 23, 2009. To underscore the Clinical Center’s commitment to creating a safe environment for patients and staff, the award is given annually to an individual or team who:

  • has initiated a significant improvement in patient safety,
  • is always on the look out for opportunities for improvement in patient safety,
  • exhibits leadership related to patient safety in the CC,
  • has participated in patient safety-focused collaborations within or outside the CC,
  • and actively promotes a safe patient environment.

The awardee will be recognized by CC leadership at a luncheon and at the CC Director’s Annual Award Ceremony and will be featured in the CC News. A nomination form was e-mailed to all CC staff. To request another copy or to submit a form, e-mail Laura Lee at llee@nih.gov. Nominations can also be turned in to Deputy Director for Clinical Care Dr. David Henderson’s office (Room 6-1480) c/o Patient Safety Champion Award.

Back to Top


   

News Briefs

Recycle magazines for patient enjoyment

sports magazine

Don’t pitch your gently read magazines. Recycle them to help brighten the day for Clinical Center patients. Magazines covering a variety of interests are a great resource to help patients pass the time while at the CC for appointments. Drop them off in the red-roofed collection bins located in the elevator lobbies of P2 and P3 parking garages and outside the patient library on the 7th floor of the Hatfield Building. Patient library and Red Cross volunteers collect and sort the magazines, make sure address labels are removed, and distribute them at least weekly to waiting areas in clinics, radiology, and phlebotomy. If you have questions about the Recycled Reads program, call the Patient Library at 301-451-7603.  


Save a life, give blood
The NIH Blood Bank current needs A+, B-, and O- blood donations. Typically, the following blood products are needed each month: 600 units of red blood cells to treat patients with chronic anemias; 2,500 units of platelets to control bleeding in patients with leukemia, cancer, or who have had surgery; 100 units of plasma for surgical patients, patients with cancer, and patients with immunologic deficiencies; 20 units of cryoprecipitate for patients with a variety of bleeding disorders; and 10 units of granulocytes (white blood cells) for patients with serious infections associated with hereditary or acquired abnormalities of white blood cells.

Monday appointment hours were recently extended to 6:30 pm; regular hours to donate are 7:30 am to 5:00 pm. The blood bank is located on the CC’s first floor with convenient, reserved parking in MLP-9. To schedule a donation, visit http://www.cc.nih.gov/blooddonor/appointment.html or call 301-496-1048.

Long-time staffer dies

Rhode Romero
Rohde Romero, a food service worked in the Nutrition Department, died in December. She is remembered by her colleagues as an inspired cook who enjoyed sharing her cooking and food knowledge with others and who took great pride in her work. Nutrition Department Chief David Folio said Romero “always put our patients first and provided excellent service to both internal and external customers. Rohde Romero was a person the staff looked up to for her high standards for cleanliness and food quality, and she was a true advocate for our patients in getting them quality food.”



NIH Library services expand

The NIH Library, located near Building 10’s south entrance, offers a variety of services and resources, including:

  • DXplain, an intelligent, interactive textbook on medical diagnosis from Massachusetts General Hospital that is now available at no charge to NIH staff. Enter a patient description and DXplain generates a list of diagnoses.
  • More than 9,300 titles—99 percent of the NIH Library journal subscriptions—now accessible online 24 hours a day, from anywhere in the world.
  • Access to EndNote Web (free to NIH Staff); PubMed (keep up to date with content changes and search features); WorldCat (search for books and journals in other libraries); Scopus (set up RSS feeds for authors); Web of Science (use citation maps to visualize citation connections); Who's Who on the Web (find biographical information); and IEEE Conference Proceedings.
  • Classes in how to use resources. Try a few new search tools in the “Web Search: Thinking Beyond Google” class. On March 19, a class in searching PubMed for evidence-based medicine is scheduled.

To keep up on the library’s services, subscribe to its e-mail list. Find that information and details on offerings on the library Web site http://nihlibrary.nih.gov. Direct comments or questions to nihlibrary@nih.gov or 301-496-1080.

Back to Top


   

New clinical research protocols 

The following clinical research protocols were approved in December:

A Phase I Study of ABT-888 in Combination with Metronomic Cyclophosphamide in Adults with Refractory Solid Tumors and Lymphomas, 09-C-0048, Shivaani Kummar, MD, NCI

A Group Wide Biology and Banking Study for Phase II Study of R1507, 09-C-0054, Lee Helman, MD, NCI

Autologous and Related Allogeneic Hematopoietic Stem Cell Transplant Data Submission to the Center for International Blood and Marrow Transplant Research (CIBMTR) and the National Marrow Donor Program (NMDP), 09-C-0053, Ronald Gress, MD, NCI

Informed Consent in Pediatric Phase I Cancer Trials, 09-C-0052, Elizabeth Fox, MD, NCI

Clinical, Cellular, and Molecular Investigation into Oculocutaneous Albinism, 09-HG-0035, David Adams, MD, PhD, NHGRI

A Phase I Study of the Intrathecal Administration of Resiniferatoxin for Treating Severe Refractory Pain Associated with Advanced Cancer, 09-D-0039, Andrew Mannes, MD, CC

Phase II Study of Metastatic Cancer that Expresses Carcinoembryonic Antigen (CEA) using Lymphodepleting Conditioning Followed by Infusion of Anti-CEA TCR-Gene Engineered Lymphocytes, 09-C-0047, Steven Rosenberg, MD, PhD, NCI

Normal Blood, Bone Marrow, and Buccal Mucosa Protocol, 09-I-0049, Todd Wilson, DO, NIAID

Phase II Study of Metastatic Melanoma Using a Chemoradiation Lymphodepleting Conditioning Regimen Followed by Infusion of Anti-Mart-1 and Anti-gp100 TCR-Gene Engineered Lymphocytes and Peptide Vaccines, 09-C-0051, Steven Rosenberg, MD, PhD, NCI

Pharmacogenomic Response to Thyrotropin-Releasing Hormone Stimulation in Healthy Volunteers: The Influence of a Common Type 2 Deiodinase Genetic Polymorphism on Serum T3, 09-DK-0058, Francesco Celi, MD, NIDDK

A Phase II Study of Bevacizumab in Combination with Ixabepilone in Subjects with Advanced Renal Cell Carcinoma, 09-C-0057, Olivier Rixe, MD, PhD, NCI

Assessment of the Effects of a DPP-4 Inhibitor (Sitagliptin) Januvia on Immune Function in Healthy Individuals, 09-DK-0055, Kristin Tarbell, PhD, NIDDK

Molecular Bases of Response to Copper Treatment in Menkes Disease, Related Phenotypes, and Unexplained Copper Deficiency, 09-CH-0059, Stephen Kaler, MD, NICHD

Collection of Blood and Bone Marrow Samples from Select Patients with CML to Measure Minimal Residual Disease, 09-H-0062, Agnes Yong, MD, PhD, NHLBI

Imaging Cannabinoid CB1 Receptors in Alcohol Dependence, 09-M-0040, Robert Innis, MD, NIMH

Back to Top


   

Upcoming Events 

February 4, 2009
Ethics Rounds
Are Risks to Family Members Grounds for Exclusion?
David Magnus, PhD
Director, Stanford Center for Biomedical Ethics
Associate Professor of Pediatrics
Stanford University
Lecture will be videocast, http://videocast.nih.gov

February 11, 2009
Contemporary Clinical Medicine: Great Teachers
The Art of Failure in Medicine
Atul Gawande, MD, MPH
Surgeon, Brigham and Women’s Hospital and Dana Farber Cancer Institute
Associate Professor, Department of Surgery, Harvard Medical School and
Department of Health Policy and Management, Harvard School of Public Health
Lecture will be videocast, http://videocast.nih.gov

February 18, 2009
The Winner’s Curse: The Market for Exchange of Science

Neal Young, MD
Chief, Hematology Branch, NHLBI
Ethical Challenges for Medical Journals

Christine Laine, MD, MPH
Senior Deputy Editor, Annals of Internal Medicine
Lecture will be videocast, http://videocast.nih.gov

February 25, 2009
John Doppman Memorial Lecture for Imaging Sciences
Oncologic Imaging: Endless Horizons
Hedvig Hricak, MD, PhD, Drhc
Chairman, Department of Radiology
Carroll and Milton Petrie Chair
Memorial Sloan-Kettering Cancer Center and
Professor of Radiology, Weill Medical College of Cornell University
Lectures will be videocast, http://videocast.nih.gov

Back to Top

This page last updated on 12/14/2017



Archived Spiderweb The information on this page is archived and provided for reference purposes only.

National Institutes
of Health
  Department of Health
and Human Services
 
NIH Clinical Center
Map_Directional.gif" width="650" vspace="0px" hspace="0" height="506" border="0">

Back to Top


   

Genetic disorder affecting sleep pattern explored

By Bill Schmalfeldt

Smith-Magenis syndrome is many times mistaken for something else like Down syndrome. Other times, it is missed completely.
Thanks to the CC’s Bedside-to-Bench Awards program a team of dedicated researchers is working to increase awareness of the syndrome and find new and better ways to treat it.

Leading the team is Dr. Ann C. M. Smith, the Smith in Smith-Magenis syndrome. As a genetic counselor at Children’s Hospital in Denver in 1981, she saw two infants with failure to thrive and defects of the heart and palate, developmental delay, a typical facial appearance, short stature with small hands and feet, low muscle tone, and early expressive speech and motor delays. They were identified to have a piece missing from the short arm of chromosome 17, called deletion 17p11.2. With Dr. Ellen Magenis, a cytogeneticist at Oregon Health & Sciences University, Smith published a paper in 1986 identifying the first nine individuals with deletion 17p11.2., expanding the now-trademark characteristics to include middle ear problems, hoarse voice, ocular abnormalities, sleep disturbances, and neurobehavioral features, such as hyperactivity and aggressive behaviors. The syndrome was officially named in 1989.

Smith, who is a senior genetic counselor with NHGRI and recently retired from the medical faculty at Georgetown University Medical School, leads a team that has received two Bedside-to-Bench Awards, the first in 1999.

That award allowed for a natural history protocol (01-HG-0109) and a research roundtable with experts, researchers, and the parents’ support group PRISMS. These efforts resulted in new findings, including the validation of early expressive speech delays caused by underlying physiological issues related to low muscle tone, decreased tongue strength and movement, laryngeal and palatal abnormalities, poor suck reflex, difficulties transitioning to solid foods, and open mouth posture with drooling.

team working on Smith-Magenis syndrome

Those working on Smith-Magenis Syndrome came from different ICs: (front row, from left) Hanna Hildenbrand, CC Rehabilitation Medicine; Ann Smith, NHGRI; CAPT Michaele Smith, CC Rehabilitation Medicine; (back row) Rebecca Morse, NHGRI; Dr. Maryland Pao, NIMH; Beth Solomon, CC Rehabilitation Medicine; Dr. Wallace Duncan, NIMH; and Dr. Marjan Huizing, NHGRI.





The initial protocol also showed that many with SMS have short stature.  “We’re collecting growth data to generate specific growth curves,” Smith said. Other findings of the initial natural history protocol showed that patients tend toward obesity and elevated total cholesterol during adolescence. “One of genes in this region (of the chromosome deletion) is involved in cholesterol management,” Smith explained.

The natural history protocol also defined an earlier onset of sensory neural hearing loss, occurring shortly after age 10, and an increased frequency of hyperacusis, an over-sensitivity to certain sound frequencies. A person with severe hyperacusis has difficulty tolerating everyday sounds, which may seem unpleasantly loud.

The research team got a second award in 2004, used to assemble an approach to what Smith called “the novel inverted melatonin circadian rhythm” found in Smith-Magenis syndrome patients. Where most produce the sleep hormone melatonin at night (light usually suppresses the formation of the hormone), people with Smith-Magenis syndrome produce it during the day. This causes chronic sleep disturbances with frequent nighttime and early morning awakenings compounded by daytime sleepiness.

Smith’s Phase I treatment trial, begun last year, was designed with information derived from Actiwatches, wristwatch-like accelerometers used to measure a patient’s activity-rest cycle in the home setting and report on the patient’s sleep pattern. Actigraphy is less invasive than standard sleep studies and provides a continuous estimate of sleep based on wrist motion for a prolonged period.

The second Bedside-to-Bench Award funded the purchase of bright light boxes and the creation of a melatonin tablet used for the treatment study.

“Melatonin isn’t regulated by the FDA. You can buy it at health food stores,” Smith said. “But for NIH, we wanted a purer form of melatonin with a time delayed release. So the NIH Pharmacy developed a coated tablet.”

Patients with Smith-Magenis syndrome and healthy volunteers began enrolling in the protocol in April to test the safety and efficacy of the melatonin tablet.

Official statistics on Smith-Magenis syndrome suggest that one out of every 25,000 children is born with the disease, but Smith thinks the number is more like one out of every 15,000. With improved molecular genetic techniques for examining chromosomes, she said, doctors will diagnose more children who might have gone unrecognized.

Smith looks at herself as something of a team captain. “I’ve got some stellar researchers who, before they met this syndrome, didn’t know a thing about it,” she said.  “Now, probably, they have more experience than anyone else in the world.”

She is excited by the prospect of new discovery in the Phase I trial. “I think this population can actually offer us a better understanding of something that is so unusual—this inverted circadian rhythm of melatonin,” she said.  “It will open a new understanding of this syndrome.”

Back to Top


   

NIH School helps students stay on track

Anne Wasson teaches at the NIH School
Teachers in the NIH Children’s School, located across from the main playroom on the Hatfield Building’s first floor, tutor patients in grades kindergarten through 12 and patients studying for the General Educational Development Test. A 2008 Clinical Center Director’s Award honored school staff, including Anne Wasson (above), for their dedication to patient care. She is going over a math assignment with Erik Santa, 14.

Back to Top

 

 

 


   

Study of genetic syndrome may reveal pathway to aneurysm formation

By Maggie McGuire

Dr. Harry Dietz covered experimental therapies for a genetic disorder and how those treatments translate to other afflictions in the Astute Clinician Lecture given January 14.

“Marfan Syndrome and Related Disorders: From Molecules to Medicines” was part of the NIH Director’s Wednesday Afternoon Lecture Series.

Marfan syndrome, a disorder of the connective tissue, affects the ocular, skeletal, and cardiovascular systems. Approximately one in 5,000 Americans has the disorder, which commonly presents with overgrowth of bones, dislocation of the ocular lens, and progressive dilatation of the aortic root. Even with state-of-the-art medical management, Dietz said, Marfan syndrome is still associated with early mortality.

“Can we do better?” Dietz asked the crowd gathered in Masur Auditorium.

Dietz is professor of pediatrics, medicine, and molecular biology and genetics at Johns Hopkins University School of Medicine, and investigator at Howard Hughes Medical Institute. He earned his medical degree from SUNY Upstate Schools of Medicine in Syracuse and completed residencies in pediatrics and anesthesia and in critical care medicine at The Johns Hopkins Hospital.

In his Astute Clinician Lecture Dietz presented that Marfan syndrome results from a deficiency of the fibrillin-1 protein—responsible for maintaining elastic fiber integrity in connective tissue. A slide showed a non-affected patient’s elastic fibers densely packed and well organized and a Marfan syndrome patient’s fibers fragmented and in disarray.

Fibrillin is a component of microfibrils. “With a deficiency of microfibrils, as seen in Marfan syndrome, we observed promiscuous activation of transforming growth factor beta (TGFB),” said Dietz. This activation is thought to speed dilatation of the aorta. Marfan mouse models showed significant improvement in aortic wall architecture and thickness when injected with a TGFB-neutralizing antibody or when treated by losartan, an angiotensin II type-1 receptor blocker that both lowers blood pressure and antagonizes TGFB.

A Phase III trial sponsored by NHLBI’s Pediatric Heart Network and The National Marfan Foundation is testing the efficacy of losartan in slowing aortic root growth in Marfan syndrome patients. A preliminary clinical experience in children with severe Marfan syndrome showed a pronounced decline in aortic root growth after starting losartan.

The speaker and his fellow researchers identified a connection between TGFB and Loeys-Dietz syndrome, too. This vascular condition shares many characteristics with Marfan syndrome, including aortic and arterial aneurysms, leading scientists to explore a similar genetic basis. “The emerging view is that transforming growth factor beta may be a final common pathway to aneurysm formation,” Dietz said.

Remarkably, losartan showed the ability to attenuate or prevent manifestations of Marfan syndrome outside of the cardiovascular system in mouse models including developmental emphysema and skeletal muscle myopathy. Dietz and colleagues went on to show that TGFB also contributes to failed muscle regeneration in a mouse model of Duchenne muscular dystrophy and that losartan could reverse this defect, preserving muscle architecture, size, and function.

The Astute Clinician Lecture was established through a gift from the late Dr. Robert W. Miller and his wife, Haruko. It honors a US scientist who has observed an unusual clinical occurrence, and by investigating it, has opened an important new avenue of research.

To view Dietz’s lecture, visit http://videocast.nih.gov/.

Back to Top


   

Winter’s first snowstorm covers the CC in a sheet of white

snow near the CC south entrance

The west courtyard’s Healing Waters statue and the Magnuson Building’s B1-level patio saw little action on January 27, as the first real snow of the year left its mark.

The Office of Research Facilities (ORF) and the Office of Research Services released recommendations for avoiding an accident or injury in inclement weather. Drive slowly, and do not be intimidated by other drivers into going faster than you are comfortable with. Maintain a safe distance between your vehicle and the one in front of you.

snow near the CC

Note that elevated surfaces (bridges, ramps, the top level of a parking garage) freeze first. If your car begins sliding: take your foot off the gas, steer your vehicle the same direction you are sliding, and gently pump the brake pedal. If your car has anti-lock brakes, use a steady, firm pressure without pumping.

When walking outside in the snow and ice, wear proper footwear. Refrain from walking between parked cars in icy lots, as the shade slows melting. ORF handles pathway and road treatments.

If a sidewalk, roadway, or other surface on the NIH main campus remains hazardous after a crew has visited the area, please report the problem to ORF at 301-435-8000.

Back to Top

 


   

NCCAM announces Straus lecture

Dr. Nuland
Dr. Sherwin B. Nuland of Yale University will deliver the first Stephen E. Straus Distinguished Lecture on March 10 in Masur Auditorium.
On March 10, NCCAM will hold the inaugural Stephen E. Straus Distinguished Lecture in the Science of Complementary and Alternative Medicine. Dr. Sherwin B. Nuland, clinical professor of surgery at Yale University and distinguished author, will present the first lecture “Chinese Medicine, Western Science, and Acupuncture” at 2:30 pm in Masur Auditorium.

During visits to China, Nuland observed major surgery done with no anesthesia, aided by acupuncture. In the Straus lecture, he will describe these operations and tell of his experience. In his recent book, The Uncertain Art, Nuland wrote “the basis of acupuncture’s practical usefulness, even in the operating room, has still not been explained in terms acceptable to most orthodox Western scientists using orthodox Western investigative methods.”

Dr. Stephen E. Straus was the first director of NCCAM. “Steve created a legacy we are continuing to build upon,” said Dr. Josephine P. Briggs, NCCAM’s current director. “He advocated for the most rigorous scientific methods to preserve NCCAM’s credibility. A lecture series in his name is only fitting to honor the work of such a brilliant and extraordinary scientist.”

In addition to serving as NCCAM director, Straus was the senior investigator in the Laboratory of Clinical Investigation at NIAID and was also part of the nationwide research team that showed a vaccine was effective in preventing shingles in older adults. He died in May 2007.

 

Back to Top


   

Patient Safety Champion Award nominations open

Nominations for the 2009 Patient Safety Champion Award are being accepted through Feb. 23, 2009. To underscore the Clinical Center’s commitment to creating a safe environment for patients and staff, the award is given annually to an individual or team who:

  • has initiated a significant improvement in patient safety,
  • is always on the look out for opportunities for improvement in patient safety,
  • exhibits leadership related to patient safety in the CC,
  • has participated in patient safety-focused collaborations within or outside the CC,
  • and actively promotes a safe patient environment.

The awardee will be recognized by CC leadership at a luncheon and at the CC Director’s Annual Award Ceremony and will be featured in the CC News. A nomination form was e-mailed to all CC staff. To request another copy or to submit a form, e-mail Laura Lee at llee@nih.gov. Nominations can also be turned in to Deputy Director for Clinical Care Dr. David Henderson’s office (Room 6-1480) c/o Patient Safety Champion Award.

Back to Top


   

News Briefs

Recycle magazines for patient enjoyment

sports magazine

Don’t pitch your gently read magazines. Recycle them to help brighten the day for Clinical Center patients. Magazines covering a variety of interests are a great resource to help patients pass the time while at the CC for appointments. Drop them off in the red-roofed collection bins located in the elevator lobbies of P2 and P3 parking garages and outside the patient library on the 7th floor of the Hatfield Building. Patient library and Red Cross volunteers collect and sort the magazines, make sure address labels are removed, and distribute them at least weekly to waiting areas in clinics, radiology, and phlebotomy. If you have questions about the Recycled Reads program, call the Patient Library at 301-451-7603.  


Save a life, give blood
The NIH Blood Bank current needs A+, B-, and O- blood donations. Typically, the following blood products are needed each month: 600 units of red blood cells to treat patients with chronic anemias; 2,500 units of platelets to control bleeding in patients with leukemia, cancer, or who have had surgery; 100 units of plasma for surgical patients, patients with cancer, and patients with immunologic deficiencies; 20 units of cryoprecipitate for patients with a variety of bleeding disorders; and 10 units of granulocytes (white blood cells) for patients with serious infections associated with hereditary or acquired abnormalities of white blood cells.

Monday appointment hours were recently extended to 6:30 pm; regular hours to donate are 7:30 am to 5:00 pm. The blood bank is located on the CC’s first floor with convenient, reserved parking in MLP-9. To schedule a donation, visit http://www.cc.nih.gov/blooddonor/appointment.html or call 301-496-1048.

Long-time staffer dies

Rhode Romero
Rohde Romero, a food service worked in the Nutrition Department, died in December. She is remembered by her colleagues as an inspired cook who enjoyed sharing her cooking and food knowledge with others and who took great pride in her work. Nutrition Department Chief David Folio said Romero “always put our patients first and provided excellent service to both internal and external customers. Rohde Romero was a person the staff looked up to for her high standards for cleanliness and food quality, and she was a true advocate for our patients in getting them quality food.”



NIH Library services expand

The NIH Library, located near Building 10’s south entrance, offers a variety of services and resources, including:

  • DXplain, an intelligent, interactive textbook on medical diagnosis from Massachusetts General Hospital that is now available at no charge to NIH staff. Enter a patient description and DXplain generates a list of diagnoses.
  • More than 9,300 titles—99 percent of the NIH Library journal subscriptions—now accessible online 24 hours a day, from anywhere in the world.
  • Access to EndNote Web (free to NIH Staff); PubMed (keep up to date with content changes and search features); WorldCat (search for books and journals in other libraries); Scopus (set up RSS feeds for authors); Web of Science (use citation maps to visualize citation connections); Who's Who on the Web (find biographical information); and IEEE Conference Proceedings.
  • Classes in how to use resources. Try a few new search tools in the “Web Search: Thinking Beyond Google” class. On March 19, a class in searching PubMed for evidence-based medicine is scheduled.

To keep up on the library’s services, subscribe to its e-mail list. Find that information and details on offerings on the library Web site http://nihlibrary.nih.gov. Direct comments or questions to nihlibrary@nih.gov or 301-496-1080.

Back to Top


   

New clinical research protocols 

The following clinical research protocols were approved in December:

A Phase I Study of ABT-888 in Combination with Metronomic Cyclophosphamide in Adults with Refractory Solid Tumors and Lymphomas, 09-C-0048, Shivaani Kummar, MD, NCI

A Group Wide Biology and Banking Study for Phase II Study of R1507, 09-C-0054, Lee Helman, MD, NCI

Autologous and Related Allogeneic Hematopoietic Stem Cell Transplant Data Submission to the Center for International Blood and Marrow Transplant Research (CIBMTR) and the National Marrow Donor Program (NMDP), 09-C-0053, Ronald Gress, MD, NCI

Informed Consent in Pediatric Phase I Cancer Trials, 09-C-0052, Elizabeth Fox, MD, NCI

Clinical, Cellular, and Molecular Investigation into Oculocutaneous Albinism, 09-HG-0035, David Adams, MD, PhD, NHGRI

A Phase I Study of the Intrathecal Administration of Resiniferatoxin for Treating Severe Refractory Pain Associated with Advanced Cancer, 09-D-0039, Andrew Mannes, MD, CC

Phase II Study of Metastatic Cancer that Expresses Carcinoembryonic Antigen (CEA) using Lymphodepleting Conditioning Followed by Infusion of Anti-CEA TCR-Gene Engineered Lymphocytes, 09-C-0047, Steven Rosenberg, MD, PhD, NCI

Normal Blood, Bone Marrow, and Buccal Mucosa Protocol, 09-I-0049, Todd Wilson, DO, NIAID

Phase II Study of Metastatic Melanoma Using a Chemoradiation Lymphodepleting Conditioning Regimen Followed by Infusion of Anti-Mart-1 and Anti-gp100 TCR-Gene Engineered Lymphocytes and Peptide Vaccines, 09-C-0051, Steven Rosenberg, MD, PhD, NCI

Pharmacogenomic Response to Thyrotropin-Releasing Hormone Stimulation in Healthy Volunteers: The Influence of a Common Type 2 Deiodinase Genetic Polymorphism on Serum T3, 09-DK-0058, Francesco Celi, MD, NIDDK

A Phase II Study of Bevacizumab in Combination with Ixabepilone in Subjects with Advanced Renal Cell Carcinoma, 09-C-0057, Olivier Rixe, MD, PhD, NCI

Assessment of the Effects of a DPP-4 Inhibitor (Sitagliptin) Januvia on Immune Function in Healthy Individuals, 09-DK-0055, Kristin Tarbell, PhD, NIDDK

Molecular Bases of Response to Copper Treatment in Menkes Disease, Related Phenotypes, and Unexplained Copper Deficiency, 09-CH-0059, Stephen Kaler, MD, NICHD

Collection of Blood and Bone Marrow Samples from Select Patients with CML to Measure Minimal Residual Disease, 09-H-0062, Agnes Yong, MD, PhD, NHLBI

Imaging Cannabinoid CB1 Receptors in Alcohol Dependence, 09-M-0040, Robert Innis, MD, NIMH

Back to Top


   

Upcoming Events 

February 4, 2009
Ethics Rounds
Are Risks to Family Members Grounds for Exclusion?
David Magnus, PhD
Director, Stanford Center for Biomedical Ethics
Associate Professor of Pediatrics
Stanford University
Lecture will be videocast, http://videocast.nih.gov

February 11, 2009
Contemporary Clinical Medicine: Great Teachers
The Art of Failure in Medicine
Atul Gawande, MD, MPH
Surgeon, Brigham and Women’s Hospital and Dana Farber Cancer Institute
Associate Professor, Department of Surgery, Harvard Medical School and
Department of Health Policy and Management, Harvard School of Public Health
Lecture will be videocast, http://videocast.nih.gov

February 18, 2009
The Winner’s Curse: The Market for Exchange of Science

Neal Young, MD
Chief, Hematology Branch, NHLBI
Ethical Challenges for Medical Journals

Christine Laine, MD, MPH
Senior Deputy Editor, Annals of Internal Medicine
Lecture will be videocast, http://videocast.nih.gov

February 25, 2009
John Doppman Memorial Lecture for Imaging Sciences
Oncologic Imaging: Endless Horizons
Hedvig Hricak, MD, PhD, Drhc
Chairman, Department of Radiology
Carroll and Milton Petrie Chair
Memorial Sloan-Kettering Cancer Center and
Professor of Radiology, Weill Medical College of Cornell University
Lectures will be videocast, http://videocast.nih.gov

Back to Top

This page last updated on 12/14/2017



Archived Spiderweb The information on this page is archived and provided for reference purposes only.

National Institutes
of Health
  Department of Health
and Human Services
 
NIH Clinical Center