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Holmgren, G., Hamhraeus, L. and de Chateau, P. (Departments of Paediatrics, University Hospitals, Umea and Uppsala and the Department of Nutrition, University of Uppsala, Sweden). Histidinemia and “normohistidinemic histidinuria”. Acta Paediat Scand, 63: 220, 1974.–Three patients with histidinemia and one with “normohistidinemic histidinuria” are presented. The effects of varying protein intakes on urinary histidine excretion were studied in three of the patients and in 14 controls; histidine excretion in the urine was found to be parallel to an increased protein intake. In the “histidinuria” patient, the urinary histidine excretion was much lower than in the histidinemic patients, hut higher than in the controls at different levels of protein intake. The occurrence of different phenotypes of histidinemia and renal histidinuria and the value of early dietary treatment is discussed.