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A-T Cancer Clinic at St. Jude Children's
Research Hospital
A-T Clinical Center at Johns
Hopkins Hospital
and Kennedy-Kreiger Institute
In 1995, the A-T Children's Project established and
funded a multidisciplinary, international clinical center at Johns Hopkins
Hospital in Baltimore, Maryland to focus solely on the evaluation and treatment
of patients with ataxia-telangiectasia. This center provides a centralized
clearinghouse for information about management strategies so that doctors around
the U.S. and the world do not need to struggle with A-T in a vacuum.
 The team of physicians includes specialists in immunology, neurology, and
respiratory/pulmonary dysfunction, along with experts in swallowing disorders,
physical therapy and adaptive services.
An A-T family may typically visit this center every two or three years for a
two-day protocol to fully evaluate the patient's condition. Of course, whenever
an A-T patient's condition becomes critical, the clinic can immediately use its
experience with A-T to assist the home physician in designing a treatment.
This clinic is important to A-T research as well, as clinical experience
always assists scientists in devising new research strategies. It is always
important to have a strong link between the research lab and the clinic. In
addition, if any potential therapies are developed, it will be helpful to have a
main center with a patient base already in existence, making the implementation
of clinical trials much easier.
Some Accomplishments So Far...
Since being established, the clinical center has made significant progress in
defining the clinical symptoms of A-T. The physicians there have:
• identified dysfunctional swallowing
with aspiration as a critical cause of pulmonary disease,
• developed tools for assessing the
long-term neurological deterioration of A-T,
• described a relatively common problem of
dysgammaglobulinemia that may have important implications for
understanding the immunologic perturbations of A-T,
• defined growth abnormalities in children
with A-T with the aim of developing a hypothesis for their cause,
• looked at the relationship between
vitamin A levels and lymphopenia in children with A-T -- a study
undertaken because vitamin A deficiency is a common factor linking growth
failure and lymphopenia,
• identified a new hazard to older
individuals with A-T: the development of progressive central nervous
system vascular abnormalities, and
• collected and analyzed data on the
difficulties with cognitive performance that A-T patients face as they
age.
The Ataxia-Telangiectasia Clinical
Center
Established and funded by the A-T Children's Project
Room CMSC 1102
The Johns Hopkins Hospital
Baltimore, Maryland 21287
Toll-free: 800-610-5691
Phone: 410-955-5883
Fax: 410-955-0229
Director: Howard J. Lederman, MD, PhD
hlederm1@jhem.jhmi.edu
Nurse Administrator: Karen Rosquist, RN
Krosquis@jhmi.edu
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