The National Institutes of Health (NIH) will hold a Consensus Development Conference on Phenylketonuria (PKU): Screening and Management, October 16-18, 2000, in the main auditorium of the William H. Natcher Building on the NIH campus in Bethesda, Maryland. A news conference will conclude the 2 ½ day meeting at 1:00 p.m. ET on Wednesday, October 18, 2000.

PKU is a rare, inherited metabolic disorder that, if untreated, causes mental retardation as well as other medical problems. Approximately one of every 10,000 infants in the United States is born with PKU, which usually results from a deficiency of a liver enzyme known as phenylalanine hydroxylase. This enzyme deficiency leads to elevated levels of the amino acid phenylalanine in the bloodstream.

The current treatment for PKU involves dietary modification and generally excludes all high protein foods, such as meat, milk, eggs, and nuts, since all protein contains phenylalanine. When a very strict diet is begun early and maintained, children with PKU can have normal development and a normal life span.

The dietary restrictions can be difficult to follow, and dietary nonadherence can result in decline of mental and behavioral performance. Women with PKU must also maintain a strictly controlled diet before and during pregnancy to prevent fetal damage. Scientists are actively exploring nondietary treatments for PKU.

Research on PKU continues to broaden the knowledge base from which informed decisions regarding screening and treatment can be made. The first day and a half of the Conference will consist of expert presentations and public discussion of PKU epidemiology and genetics, screening strategies, and treatment regimens. An independent, non-Federal panel will weigh the scientific evidence and draft a statement addressing the following questions:

• What are the incidence and prevalence of PKU and other forms of hyperphenylalaninemias, and what is known about the genetic and clinical variability?

• What newborn screening strategies are available for diagnosis, what is the effectiveness of these strategies, and what cost-savings are generated by screening and treatment?

• What treatment regimens are used to prevent the adverse consequences of PKU? What is known about the effectiveness of these treatment and management strategies overall and with respect to variables such as time of initiation of dietary management, levels of phenylalanine at various ages, methods for enhancing dietary compliance, duration of dietary management, and dietary regimens for women of childbearing age and other adults?

• Based on this information, what are the recommended strategies for optimal newborn screening and diagnosis and lifelong management and followup of PKU?

• What research is needed to gather information that will optimize the outcome for individuals with PKU and their families?

The panel will present its draft statement to the public for comment at 9:00 a.m. on Wednesday, October 18. Following this public comment session, the panel will release its revised consensus statement at a news conference at 1:00 p.m. and take questions from the media.

The consensus statement is the report of an independent panel and is not a policy statement of the NIH or the Federal Government. The NIH Consensus Development Program was established in 1977 to resolve in an unbiased manner controversial topics in medicine. To date, NIH has conducted 112 such conferences addressing a wide range of controversial medical issues important to health care providers, patients, and the general public.

The primary sponsors of this conference are the NIH Office of Medical Applications of Research and the National Institute of Child Health and Human Development. Conference co-sponsors include the National Human Genome Research Institute, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Neurological Disorders and Stroke, National Institute of Nursing Research, and the Maternal and Child Health Bureau of the Health Resources and Services Administration.

Additional information about this conference, including the meeting agenda, local area hotels, and directions to NIH, is available at the NIH Consensus Development Program Web site at http://consensus.nih.gov.

NOTE TO RADIO EDITORS: An audio report of the conference results will be available after 4 p.m. October 18, 2000 from the NIH Radio News Service by calling 1-800-MED-DIAL (1-800-633-3425).

NIH Videocasting will broadcast the conference live on the Internet. Go to the NIH Videocast Web site — http://videocast.nih.gov/ — any day during the conference and look for the link to the conference under "Today's Events." Viewing the videocast requires RealPlayer software, which can be downloaded from the NIH Videocast Web site. If you require any assistance or assistive devices to participate in this conference, please contact Prospect Associates at 301-592-3320, or via e-mail at pku@prospectassoc.com, at least 48 hours before the conference.

The Office of the Director is a component of the National Institutes of Health, U.S. Department of Health and Human Services.