PEDIATRICS Vol. 43 No. 1 January 1969, pp. 40-43
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A SIMPLE SCREENING TEST FOR HISTIDINURIA

Marcia G. Gerber M.D.1 and Donald A. Gerber M.D.1

1 Department of Medicine of the State University of New York Downstate Medical Center and Kings County Hospital Center, Brooklyn

Histidinemia, a childhood disease associated with mental retardation, can be identified by the presence of histidinuria. A rapid and simple screening test for histidinuria has been developed by the authors and is based on the inhibition by histidine of the blue color formed by the reaction of cupric ion and bis-cyclohexanone oxaldihydra-zone. The formation of a colorless solution in this test should identify all urine specimens in which the concentration of histidine is greater than 60 mg/l00 ml and most specimens in which the concentration of histidine is greater than 20 mg/100 ml. By appropriate dilution of a urine sample, the screening test may be used to estimate, semi-quantitatively, the concentration of histidine in urine.

Submitted on October 25, 1967
Accepted on July 17, 1968