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New approaches to understanding Lesch-Nyhan disease

Article Abstract:

The neurological and behavioral abnormalities seen in people with Lesch-Nyhan disease may be a result of abnormal dopamine processing in the brain. Lesch-Nyhan disease is caused by a loss of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). HPRT recycles purine bases, which are the foundation of DNA and RNA. In the absence of HPRT, uric acid accumulates. This causes symptoms similar to those with gout, including gouty arthritis and stones in the urinary tract. The gout drug allopurinol can reduce these complications of Lesch-Nyhan disease, but it does not alleviate the neurological and behavioral symptoms. A 1996 study using positron-emission tomography and labelled chemicals similar to the precursor of dopamine found that people with Lesch-Nyhan disease have substantially less dopamine activity in their brains. The gene for HPRT has been successfully introduced into cells in the laboratory, but animal trials have so far been unsuccessful.

Author: Wong, Dean F., Nyhan, William L.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1996
Editorial, Physiological aspects, Dopamine, Lesch-Nyhan syndrome

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Understanding geographic variations in health care delivery

Article Abstract:

Additional research is needed to determine why there are geographical variations in the rates of hospitalization. A 1998 study confirmed such variations in the VA system even though VA hospitals have similar administrative policies. Physicians in the VA system are paid a salary, so they have no financial incentive to hospitalize patients. Nevertheless, hospitalization rates were highest in the Northeast and lowest in the West. This indicates that VA physicians in different areas have the same beliefs as other physicians in their area.

Author: Wennberg, John E.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1999
Statistics, Demographic aspects, Medical care, Medical care utilization, Hospital utilization, United States. Department of Veterans Affairs

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