PHILADELPHIA INTERNATIONAL MEDICINE NEWS BUREAU
Contact: Leonard N. Karp
lkarp@philadelphiamedicine.com
215-735-3989
January 31, 2002

For Immediate Release:

1. Tiny Camera Helps Jefferson Docs Diagnose Intestinal Disorders Non-invasively

2. Fox Chase Cancer Center Clinical Trial Treats Precursor to Cutaneous T-cell Lymphoma

3. University of Pennsylvania Medical Center's Scheie Eye Institute Discovering New Insight to Night Blindness

Tiny Camera Helps Jefferson Docs Diagnose Intestinal Disorders Non-invasively

Philadelphia - A new procedure at Thomas Jefferson University Hospital, a member of Philadelphia International Medicine, allows a patient to effectively swallow a small camera to help diagnose intestinal disorders.

"This new non-invasive procedure is another example of the world-class medical care available at Philadelphia International Medicine's network of hospitals," said Andrew Wigglesworth, PIM President and CEO. Wigglesworth said that appointments and other services at any of the nine hospitals are available with just one call to PIM at 215-735-3595.

The new procedure at Thomas Jefferson University Hospital helps physicians "see" inside the patient through a new, non-invasive approach.

As an example, when a patient with unexplained internal bleeding saw her gastroenterologist recently at Thomas Jefferson University Hospital, she was shocked to learn that the doctor was recommending a diagnostic procedure that she, the patient, thought was nothing short of incredible.

Known as capsule endoscopy, this new device--which is just slightly larger than a vitamin capsule--is swallowed by a patient in the morning along with a drink of water. It takes more than 50,000 color pictures of the digestive system while it travels through the stomach and the small intestine. The capsule is eliminated by the body about 12 hours later after transmitting the photos in the form of radio waves to a data recorder that the patient wears around the waist.

Doctors at Thomas Jefferson University Hospital are among a select group nationally to use this newly approved device to diagnose unexplained gastrointestinal disorders.

"This truly is a Fantastic Voyage," says Mitchell Conn, MD, clinical associate professor of Medicine (Gastroenterology and Hepatology) at Jefferson Medical College of Thomas Jefferson University (TJU), referring to the 1966 futuristic movie. "For many patients, it will replace the long, complicated and potentially painful methods currently used to evaluate the small bowel."

According to Anthony Infantolino, MD, clinical assistant professor of Medicine (Gastroenterology and Hepatology) at Jefferson Medical College of TJU, "investigating disorders of the small intestine presents the biggest challenge because of the way the small intestine is shaped and because it is so long--about 20 feet. The capsule offers the ideal way to visualize this area."

Approximately eight hours after the patient swallows the capsule, the patient returns to the physician's office to have the data equipment removed. The patient wears the equipment in a waist pack throughout the day. In addition to the data recorder, the waist pack includes a battery pack and eight leads that are attached to the patient's abdomen and act as receivers of the radio wave images being sent out by the capsule. The physician can then download the photos into a computer to diagnose the bleeding or other problem as the pictures are viewed. This process can take up to two hours.

Patients who are eligible for this diagnostic procedure are those with unexplained gastrointestinal disorders such as gastrointestinal bleeding, unexplained diarrhea and unexplained abdominal pain. The capsule cannot be used for individuals who have an already identified bowel obstruction and does not replace standard upper endoscopy or colonoscopy.

Fox Chase Cancer Center Clinical Trial Treats Precursor to Cutaneous T-cell Lymphoma

Individuals with parapsoriasis, a red, scaly rash located in the "bathing suit" area, could be at risk for developing a potentially deadly cancer. Researchers at Fox Chase Cancer Center, a member of Philadelphia International Medicine, are conducting an innovative clinical trial using bexarotene (Targretin), a form of vitamin A in a topical gel, to treat parapsoriasis and prevent the disease from progressing into cancer.

"What's unique about this trial is that we are treating what is considered a precursor to cancer," says Stuart Lessin, MD, director of the Dermatology Oncology Program at Fox Chase Cancer Center. A precursor acts as a warning sign alerting that the onset of cancer is imminent. Parapsoriasis is a skin condition considered a precursor to cutaneous T-cell lymphoma, (CTCL) which is a potentially deadly form of cancer that manifests in the skin and targets the body's white blood cells (lymphocytes), impairing the patient's immune system. Despite its name, parapsoriasis differs from psoriasis in its appearance and location on the body. The skin condition starts as a red, scaly rash that appears on the trunk of the body, instead of on the extremities.

"Parapsoriasis has a similar name to psoriasis, but it's a very different disease," says Dr. Lessin. "Parapsoriasis has the potential to progress to CTCL and differentiation between parapsoriasis and early stage CTCL can be difficult and often requires evaluation by an experienced specialist," says Dr. Lessin.

Patients can live with the complications of CTCL long after the initial diagnosis and according to the Mycosis Fungoides Foundation, approximately 16,000 people in the United States are affected with the disease annually. CTCL slowly progresses from scaling skin patches to larger areas of thickened plaques and then to tumor nodules. In the late stages, the cancer grows to involve lymph nodes, blood and internal organs and most patients die from infections due to breakdown of the skin and a crippled immune system.

The FDA has approved Targretin for the treatment of CTCL, but Dr. Lessin and his colleagues are using the gel as a prevention tool. "If we can treat patients who are diagnosed with parapsoriasis, we can potentially stop the cancer before it has a chance to start or progress," says Dr. Lessin.

Bexarotene (Targretin) is a unique type of retinoid, a synthetic form of vitamin A that binds to specific retinoid receptors and regulates cell function. The drug inhibits cancer growth and induces apoptosis, or programmed cell death.

Patients who are newly diagnosed with parapsoriasis or who have skin lesions suggestive of, but not diagnostic of CTCL, may be eligible to participate in this clinical trial, which is only available at Fox Chase Cancer Center. In the trial, the topical gel is initially applied to the skin lesions everyday for two weeks, than twice a day for up to 16 weeks. Tissue from the skin lesions is taken or biopsied before and after treatment to determine response to the gel.

"We're hoping that patients suffering from parapsoriasis will respond to the Targretin therapy, thereby clearing up the uncomfortable condition, and preventing further progression of the disease," Dr. Lessin said.

The trial is sponsored by Ligand Pharmaceuticals Inc., makers of Targretin .

University of Pennsylvania Medical Center's Scheie Eye Institute Discovering New Insight to Night Blindness

As presented in a recent edition of the Proceedings of the National Academy of Sciences, physicians at the University of Pennsylvania Medical Center's Scheie Eye Institute are discovering new insight to a condition that can cause night blindness, loss of peripheral vision and sensitivity to blue light. The University of Pennsylvania Medical Center is a member of PIM.

The posthumous gift of eyes from a patient with a rare retinal disease, enhanced S cone syndrome (ESCS), has taught researchers more about the role of NR2E3, the gene that causes this form of blindness. ESCS is an inherited condition that gradually causes night blindness, loss of peripheral vision, and sensitivity to blue light. Researchers at the Scheie Eye Institute at the University of Pennsylvania Medical Center led the study, which was presented in the January 8 edition of the Proceedings of the National Academy of Sciences.

"This is one of those occasions where a generous eye donor has helped scientists to understand a rare disease," said Ann H. Milam, PhD, lead author and a researcher at the F. M. Kirby Center for Molecular Ophthalmology within the Scheie Eye Institute. The donated retinas came from a person with two copies of the mutated gene, one from each parent. "Here, we can learn what the NR2E3 gene does by seeing what physically happens when it fails to work correctly."

According to the researchers, NR2E3 has a functional role in the development and arrangement of specialized cells in the retina, called rods and cones, which detect light. While rods allow people to distinguish between dark and light, cones allow people to see colors. There are three types of cones, each named after the wavelength (color) of light they detect - short (blue), medium (green), and long (red), or S, M, and L. To obtain an accurate picture of what color the eyes see, the brain combines information from the three types of cones much as a television similarly combines red, blue, and green light.

"People with ESCS have an overabundance of S cones, which accounts for their sensitivity to blue light," said Milam. "The most common mutation in NR2E3 causes this overabundance of S cones, deficit of rods, L and M cones, and the death of rods and cones in the periphery of the retina."

Researchers still do not have a complete understanding of how rods and cones develop, but their death is a key component to many forms of inherited blindness such as retinitis pigmentosa and age-related macular degeneration. Although ESCS is a rare disease, Milam and her colleagues believe that a better understanding of NR2E3 will lead to a better overall appreciation of the molecular basis of vision.

"One thing is for sure: genes, and the proteins they produce, do not work alone," said Milam. "In untangling the series of events that involve the NR2E3 gene, we will certainly uncover genes involved in both normal and abnormal development of the eye."

Given the relative rarity of ESCS, the researchers are grateful to receive the donated retinas, facilitated by the Foundation Fighting Blindness in Owings Mills, MD. According to Milam, the degree of degeneration in ESCS can vary from patient to patient, which has caused a great deal of confusion in the clinical literature. Indeed, several different diagnoses, from stationary night blindness to reitinitis pigmentosa, have been applied to patients that are now known to have ESCS.

Researchers who collaborated with Milam include doctors S. G. Jacobson, L.Rose, A.Cideciyan, M. Barakat, W. Tang, N Gupta, and T. Aleman of Penn, E. M. Stone and V. C. Sheffield of the University of Iowa, and A. F. Wright of Western General Hospital of Edinburgh, Scotland.

The study was supported in part by Public Health Service research grants, the Foundation Fighting Blindness, the F. M. Kirby Foundation, the Macula Vision Research Foundation, the Macular Disease Foundation, the Fight for Sight research division of Prevent Blindness America, and the Mackall Trust.

Philadelphia International Medicine is an organization that provides medical and patient support services to international patients. It also provides continuing medical education and health care training and education to international physicians, administrators and other practitioners. As the international department of several Philadelphia-area hospitals, international patients gain access to physicians and hospitals rated among the best in the world through one telephone call to PIM. You can reach PIM by calling 1-215-735-3575; fax, 1-215-790-1267; or e-mail, physicians@philadelphiamedicine.com. You can find out more about PIM through its Website at www.philadelphiamedicine.com.

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