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February 7, 1966

Self-Destruction Characterizes Metabolic Disease in Children

JAMA. 1966;195(6):40-41. doi:10.1001/jama.1966.03100060016006

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A metabolic disease in male children which is characterized by an "unusual" behavior pattern has been described by a clinician and biochemist at the University of Miami.

The disease may be considered a new form of gout in childhood. The hyperuricemia observed is usually thought to occur only in men or elderly women, William L. Nyhan, MD, told The Journal.

"The syndrome is associated with the most unusual behavior pattern of any disorder I have seen," the investigator reported.

"The children show extreme selfdestructive behavior—most characteristically manifested in chewing their lips and fingers, although the presence of sensation is apparent."

If they are protected from themselves with boxing glove-like coverings, they appear to calm down and seem to be happy.

As with most diseases involving an inborn error in metabolism, children with this uric acid disorder are severely retarded. They are unable to walk, and their intelligence quotients generally range

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