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innovative therapies, that were unimaginable just a few years ago.
Ongoing NIAMS epidemiological investigation of Native Americans in Alaska and Siberian Eskimos--populations that have a high prevalence of spondylitis--promises to shed light on the roles of genetic factors and environmental triggers, such as bacterial infections.
Systemic lupus erythematosus (lupus) is an immune-mediated inflammatory disease that afflicts numerous Americans, largely young women, especially black women of child bearing age. The disease affects the skin, joints, kidneys, brain, and other organs. Recently, a new antibody was identified that is clearly associated with psychiatric disorders due to lupus; this antibody will make better diagnosis possible.
The Institute has set up a task force to study lupus in high-risk populations.
With advice from the task force, NIAMS is developing instructive kits that will help health care providers and community organizations conduct health promotion programs targeted to young black women. The Institute also plans to sponsor an international workshop on systemic lupus erythematosus to assess current knowledge and develop directions for future research.
Scleroderma is a serious disorder of connective tissue and the fine blood
vessels. Besides damaging the skin, muscles, and joints, the disease may also affect internal organs, such as the kidneys, heart, and lungs, making it lifethreatening in some people. Women are affected three times more often than men. The first symptom of scleroderma is usually Raynaud's phenomenon, wherein blood vessels in the fingers narrow as the result of vasospasm, causing the hands to become pale and cold. There is also pathologic evidence of vascular disease seen under the microscope. Accordingly, there is keen interest in exploring vascular abnormalities that may contribute to the etiology of the disease. NIAMS will issue a program announcement to stimulate more research on
causal mechanisms in scleroderma.
Over 100 different conditions are included among the heritable connective tissue disorders, all of which stem from abnormalities of connective tissue, the scaffolding that girds bone, skin, blood vessels, and the protective coverings of internal organs. Although the disorders are uncommon, they are
In April 1990, NIAMS cosponsored a multidisciplinary
scientific workshop on heritable connective tissue disorders.
participants made it clear that sophisticated research methods are enabling
scientists to unravel the complex biology of connective tissue and to uncover
the several defects that cause the disorders.
The Institute is encouraging
additional research in this area, and is exploring the possibility of a patient
registry to facilitate progress against these diseases.
Reducing the risk of sports Injury is important to millions of Americans
who participate in exercise programs and athletics.
The Institute's mission
includes responsibility for research on exercise physiology and sports
Basic facts about how muscles function at the molecular level are
now being discovered.
Recent research has revealed a mechanism by which a
serious knee injury can destroy a joint.
In April 1991, the Institute,
concert with the National Advisory Board, will hold a workshop on scholastic
sports injuries to monitor the causes of athletic injuries and identify ways to
We are continuing to make progress in research on the numerous skin
diseases that affect so many Americans.
In epidermolysis bullosa, a
devastating inherited blistering disease that affects the skin and mucous
membranes, researchers have found that the anchoring fibrils-- which link the
outer layer of the skin to the inner layer--are conspicuously absent or
Recent investigations have also shown that type VII collagen, the
major structural protein of anchoring fibrils, can be broken down by both
collagenase and gelatinase.
Other research, utilizing patients assembled by
the National Epidermolysis Bullosa Registry, suggests that abnormal synthesis
of type VII collagen be responsible for the reduced number of anchoring
Alopecia areata is a disease of the hair follicle that results in patchy
or total loss of hair.
The psychological impact of the disease can be
debilitating, especially in young people.
In October 1990, NIAMS and the
National Alopecia Areata Foundation sponsored a 2-day research workshop on the
Experts in a wide variety of areas were convened for an open exchange factors controlling hair growth, autoimmune aspects of the disease,
of information on clinical and histopathologic features of alopecia areata,
pharmacologic interventions, and animal models.
A new anatomic site that
controls hair growth has been discovered, and the ability to grow hair in a
test tube has been achieved.
These findings open new avenues of research that
will be pursued.
Psoriasis is a common chronic skin disease characterized by scaling and
redness produced by skin cells that divide rapidly. For many years, psoriasis
was treated with preparations of tars and chemotherapeutic agents, such as
Recently, clinical studies established the efficacy of
cyclosporine A, an immuno-suppressive drug in the treatment of psoriasis.
turn, this has led investigators to explore the role of immune-mediated factors
in causing the disease and to search for alternative drugs that cause fever
side effects than does cyclosporine A.
The NIAMS is encouraging further
exploration of molecular, immunologic, and pharmacologic aspects of psoriasis.
Significant strides have been made in the Institute's Intramural Program.
This past year two new laboratories were established to expand the scope of
research: the Laboratory of Skin Biology and the Laboratory of Structural
Fundamental studies of proteins responsible for naturation
of the epidermis, the skin's outer layer, are the focus of research in the
Laboratory of Skin Biology.
In the Laboratory of Structural Biology Research,
new sophisticated instrumentation is facilitating analysis of extremely small
structures, including membrane-associated components of the AIDS virus.
In the area of orthopaedic research, Intramural scientists have identified
the various phases through which an injured bone must pass to heal properly.
They have detected the presence of a specific natural protein, transforming
growth factor-beta (TGF-beta), between and around the ends of fractured bone;
and determined that TGF-beta not only initiates a repair response, but also
controls the rate of fracture healing.
Another ground-breaking accomplishment achieved by intramural scientists
was the development of an animal model for eosinophilia-myalgia syndrome (EMS),
a new epidemic inflammatory disease associated with ingestion of contaninated
L-tryptophan. Symptoms range from those of the flu, to severe rashes and
permanent nerve and muscle damage.
The new animal model provides the first
direct, experimental evidence linking EMS to contaminated batches of the
dietary supplement L-tryptophan.
Collaborations between NIAMS and the Department of Defense continue in studies of skin manifestations in human immunodeficiency virus (HIV)-infected individuals. Establishment of a new interagency agreement will significantly increase the rate of accrual of HIV-positive subjects into the studies to give us an earlier estimate of the prevalence of skin disorders and accelerate the pace of this research.
Mr. Chairman, the budget request for NIAMS for fiscal year 1992 is $204,797,000. I will be pleased to answer any questions you may have.
BIOGRAPHICAL SKETCH OF DR. LAWRENCE D. SHULMAN
July 25, 1919, Boston, Massachusetts
Education: llarvard University, A.B., 1941; Yale University Graduate
Professional Organizations: American Association for the Advancement of Science, American College of Physicians, American Federation for Clinical Research, American Rheumatism Association (President, 1974-75), American Society for Bone and Mineral Research, The Arthritis Foundation, Brazilian Society of Rheumatology, Carla Curzio Neapolitan Society, Lupus Foundation of America, New York Academy of Science, Pan American League Against Rheumatism (President, 1982-86), Society for Clinical Trials, Sydenham Society, Society for Investigative Dermatology.
Honors Awards: Senior Investigator Award, The Arthritis Foundation, 195762. Pemberton Memorial Lecture, 1963. Fellow, American College of Physicians, 1967. Wallace R. Graham Memorial Lecture: Toronto, 1973. The Heberden Medal for Research in the Rheumatic Diseases, Heberden Society, London, 1975. Distinguished Service Award, The Arthritis Foundation, 1979. Honorary Member, Brazilian Society of Rheumatology, 1980. Honorary Member, Chilean Society of Rheumatology, 1981. Knowles Lecture: San Francisco, 1982. Honorary Member, Costa Rican Association of Rheumatology, 1984. Honorary Member, Australian Rheumatism Association, 1985. The Public Health Service Superior Service Award, 1985. National Lupus Hall of Fame, 1986. Honorary Member, Swiss Society of Rheumatology, 1987. Elected Master, American Rheumatism Association, 1987.
Senator HARKIN. Thank you very much, Dr. Shulman.
Dr. SHULMAN. It is going to be before the next hearings.
Well, I had a question on osteoporosis, but you covered that. And lyme disease-you covered that quite well. And lupus.
Just a moment. [Pause.] My apologies. I have to run over and cast another vote right now. My apologies.
(A brief recess was taken.)
QUESTIONS SUBMITTED BY THE SUBCOMMITTEE Senator HARKIN. Thank you very much. There will be some additional questions which will be submitted for your response in the record.
[The following questions were not asked at the hearing, but were submitted to the Institute for response subsequent to the hearing:)