PEDIATRICS Vol. 56 No. 1 July 1975, pp. 65-73
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The Therapy of Hyperammonemia Due to Ornithine Transcarbamylase Defiency in a Male Neonate

Selma E. Snyderman M.D.1, Claude Sansaricq M.D.1, Sadashiv V. Phansalkar Ph.D.1, Robert G. Schacht M.D.1, and Patricia M. Norton B.A.1

1 Department of Pediatrics, New York University Medical Center, New York, New York

Ornithine transcarbamylase deficiency in the male neonate has been considered to be invariably fatal because of the severity of the hyperammonemia. An extreme degree of hyperammonemia in a male neonate was brought under control by a series of exchange transfusions, prolonged peritoneal dialysis, adequate caloric intake, and a mixture of essential amino acids with an excess of aspartic acid and arginine. After the initial phase, it was possible to maintain the plasma ammonia level with dietary therapy alone, in spite of a number of complications that might be expected to cause tissue damage and increase the hyperammonemia.

Submitted on May 30, 1974
Accepted on July 15, 1974




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