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January 1967, Vol 113, No. 1 >
Article | January 1967

Treatment of Hyperglycinemia FREE

WILLIAM L. NYHAN, MD, PhD
[+] Author Affiliations

Received for publication Sept 15, 1966.

Read before the Symposium on Treatment of Amino Acid Disorders, Children's Memorial Hospital, Chicago, Sept 3-4, 1966.

Reprint requests to PO Box 875, Biscayne Annex, Miami, Fla 33136 (Dr. Nyhan).


Am J Dis Child. 1967;113(1):129-133. doi:10.1001/archpedi.1967.02090160179028.
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References
Comments

HYPERGLYCINEMIA is a disorder of amino-acid metabolism in which elevated concentrations of glycine are found in the blood, urine, and cerebrospinal fluid. It is now clear that there are at least two forms of hyperglycinemia which probably represent distinct diseases. The first of these to be recognized1 is characterized by recurrent episodes of ketosis leading to coma. More recently, a patient has been described by Gerritsen et al2 who lacked these and other clinical aspects of the syndrome, but had convulsions and diminished excretion of oxalic acid in the urine. Two siblings reported by Mabry and Karam3 may represent the same syndrome, and others have been observed. This hypo-oxaluric form of hyperglycinemia will be considered by Dr. Gerritsen. I shall consider the ketotic type of hyperglycinemia.

Manifestations and Characteristics  Prominent manifestations of the disease are indicated in Table 1. The most striking feature is that of ketosis,

REFERENCES

1 +
Childs, B., et al:  Idiopathic Hyperglycinemia and Hyperglycinuria: A New Disorder of Amino Acid Metabolism , Pediatrics 27:522, 1961;.
2 +
Gerritsen, T.; Kaveggia, E.; and Waisman, H.A.:  A New Type of Idiopathic Hyperglycinemia With Hypo-Oxaluria , Pediatrics 36:882, 1965;.
3 +
Mabry, C.C., and Karam, A.:  Idiopathic Hyper-Glycinemia and Hyperglycinuria , Southern Med J 56:1444, 1963;.
4 +
Childs, B., and Nyhan, W.L.:  Further Observations of a Patient With Hyperglycinemia , Pediatrics 33:403, 1964;.
5 +
Nyhan, W.L.; Chisolm, J.J., Jr.; and Edwards, R.O., Jr.:  Idiopathic Hyperglycinuria: III. Report of a Second Case , J Pediat 62:540, 1963;.
6 +
Tada, K., et al:  Idiopathic Hyperglycinemia (the First Case in Japan) , Tohoku J Exp Med 80: 218, 1963;.
7 +
Schreier, K., and Muller, W.:  Idiopathische Hyperglycinamie (Glycinose) , Deutsch Med Wschr 89:1739, 1964;.
8 +
Visser, H.K.A.; Veenstra. H.W.; and Pik, C.:  Hyperglycinaemia and Hyperglycinuria in a Newborn Infant , Arch Dis Child 39:397, 1964;.
9 +
Sass-Kortsak, A., et al:  Neonatal Hyperglycinemia , Proc Soc Pediat Res 35:32, 1965;.
10 +
DeVries, A., et al: Glycinuria:  A Hereditary Disorder Associated With Nephrolithiasis , Amer J Med 23:408, 1957;.
11 +
Käser, H.; Cottier, P.; and Antener, I.:  Glucoglycinuria: A New Familial Syndrome , J Pediat 61: 386, 1962;.
12 +
Cleveland, W.W., et al:  Acquired Fanconi Syndrome Following Degraded Tetracycline , J Pediat 66:333, 1965;.
13 +
Nyhan, W.L., and Childs, B.:  Hyperglycinemia: V. The Miscible Pool and Turnover Rate of Glycine and the Formation of Serine , J Clin Invest 43:2404, 1964;.
14 +
Nyhan, W.L.; Borden, M.; and Childs, B.:  Idiopathic hyperglycinemia, a new disorder of amino acid metabolism. II. The concentrations of other amino acids in the plasma and their modification by the administration of leucine , Pediatrics 27:539, 1961;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Childs, B., et al:  Idiopathic Hyperglycinemia and Hyperglycinuria: A New Disorder of Amino Acid Metabolism , Pediatrics 27:522, 1961;.
2 +
Gerritsen, T.; Kaveggia, E.; and Waisman, H.A.:  A New Type of Idiopathic Hyperglycinemia With Hypo-Oxaluria , Pediatrics 36:882, 1965;.
3 +
Mabry, C.C., and Karam, A.:  Idiopathic Hyper-Glycinemia and Hyperglycinuria , Southern Med J 56:1444, 1963;.
4 +
Childs, B., and Nyhan, W.L.:  Further Observations of a Patient With Hyperglycinemia , Pediatrics 33:403, 1964;.
5 +
Nyhan, W.L.; Chisolm, J.J., Jr.; and Edwards, R.O., Jr.:  Idiopathic Hyperglycinuria: III. Report of a Second Case , J Pediat 62:540, 1963;.
6 +
Tada, K., et al:  Idiopathic Hyperglycinemia (the First Case in Japan) , Tohoku J Exp Med 80: 218, 1963;.
7 +
Schreier, K., and Muller, W.:  Idiopathische Hyperglycinamie (Glycinose) , Deutsch Med Wschr 89:1739, 1964;.
8 +
Visser, H.K.A.; Veenstra. H.W.; and Pik, C.:  Hyperglycinaemia and Hyperglycinuria in a Newborn Infant , Arch Dis Child 39:397, 1964;.
9 +
Sass-Kortsak, A., et al:  Neonatal Hyperglycinemia , Proc Soc Pediat Res 35:32, 1965;.
10 +
DeVries, A., et al: Glycinuria:  A Hereditary Disorder Associated With Nephrolithiasis , Amer J Med 23:408, 1957;.
11 +
Käser, H.; Cottier, P.; and Antener, I.:  Glucoglycinuria: A New Familial Syndrome , J Pediat 61: 386, 1962;.
12 +
Cleveland, W.W., et al:  Acquired Fanconi Syndrome Following Degraded Tetracycline , J Pediat 66:333, 1965;.
13 +
Nyhan, W.L., and Childs, B.:  Hyperglycinemia: V. The Miscible Pool and Turnover Rate of Glycine and the Formation of Serine , J Clin Invest 43:2404, 1964;.
14 +
Nyhan, W.L.; Borden, M.; and Childs, B.:  Idiopathic hyperglycinemia, a new disorder of amino acid metabolism. II. The concentrations of other amino acids in the plasma and their modification by the administration of leucine , Pediatrics 27:539, 1961;.

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