PEDIATRICS Vol. 32 No. 3 September 1963, pp. 347-357
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CITRULLINURIA

W. C. McMurray Ph.D.1, J. C. Rathbun F.R.C.P.(C)2, F. Mohyuddin M.Sc.3, and S. J. Koegler M.D., D.C.H.4

1 Department of Biochemistry, University of Western Ontario
2 Department of Paediatrics, University of Western Ontario
3 The Wards of War Memorial Children's Hospital
4 The Children's Psychiatric Research Institute, London, Canada

A heretofore undescribed disorder manifested by mental retardation, citrullinuria, hypercitrullinemia, and hyperammonemia is reported. Dietary studies show a direct relationship between protein intake and urinary citrulline excretion. Citrulline excretion occurs as an overflow mechanism with plasma citrulline being maintained at about 25 mg/100 ml. Loading studies suggest that the ornithine urea cycle is functioning; high post-absorptive blood ammonia levels indicate that little reserve capacity for urea formation is present.

Submitted on December 24, 1962
Accepted on April 25, 1963