PEDIATRICS Vol. 34 No. 3 September 1964, pp. 357-371
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HYPOPHOSPHATEMIC RICKETS WITH RENAL HYPERGLYCINURIA, RENAL GLUCOSURIA, AND GLYCYL-PROLINURIA

A Syndrome with Evidence for Renal Tubular Secretion of Phosphorus

Charles R. Scriver M.D.1, Richard B. Goldbloom M.D.2, and Claude C. Roy M.D.3

1 The de Belle Laboratory for Biochemical Genetics and the Department of Metabolism, Montreal Children's Hospital, Montreal 25, Canada
2 Department of Pediatrics, Faculty of Medicine, McGill University, Montreal
3 Department of Pediatrics, Faculty of Medicine, Laval University, Quebec City, Canada

A syndrome of hypophosphatemic rickets, hyperglycinuria, and glucosuria has been found in an adolescent boy. No familial or acquired cause was discovered. The condition began in late childhood. Nine years after its onset dramatic alleviation of extreme osteomalacia and muscle weakness was obtained with phosphorus supplementation of the diet. A number of additional observations were made:

1. The hypophosphatemia was the result of diminished net tubular conservation of phosphorus. On several occasions there was also evidence compatible with net tubular secretion of phosphorus. Hyperparathyroidism was not a factor in this phenomenon.

2. Impaired renal tubular absorption of transport of glycine and glucose was also demonstrated. The inhibition of transport was probably non-competitive in nature.

3. A newly described peptide, glycylproline, was excreted in urine before treatment. The bound hydroxyproline content of urine was also elevated. Both findings disappeared progressively as remineralization of bone occurred.

Submitted on January 24, 1962
Accepted on May 5, 1962




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