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March 1981

HistidinemiaBiochemistry and Behavior

Am J Dis Child. 1981;135(3):210-211. doi:10.1001/archpedi.1981.02130270002002

Time was when histidinemia was considered a rare, exotic entity, relegated to specialists and academic researchers. However, there are mounting reasons for revising this attitude. For one thing, we now realize that this inborn error of metabolism is hardly rare. An incidence of one in 14,000 has been reported in the United States,1 and studies in other parts of the world have shown rates ranging from one in 11,0002 to one in 17,000.3 In the article on histidinemia published in this issue (see p 227), Ito and co-workers note that in Japan one person in 10,000 may be histidinemic.

In the United States, screening for histidinemia is now mandatory in at least one state (New York). Moreover, the implications of this law for physicians at large clearly transcend state boundaries, and it behooves all American physicians to be aware of this condition.

To what extent dietary management