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CYSTINE DEFICIENCY DURING DIETOTHERAPY OF HOMOCYSTINEMIA
Author(s) -
SANSARICQ CLAUDE,
GARG SANTOSH,
PATRICIA M.,
SADASHIV NORTON,
PHANSALKAR V.,
SNYDERMAN SELMA E.
Publication year - 1975
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1975.tb03824.x
Subject(s) - cystine , medicine , homocystinuria , methionine , urine , dietary therapy , endocrinology , biochemistry , amino acid , cysteine , chemistry , enzyme
Sansaricq, C, Garg, S., Norton, P. M., Phansalkar, S. V. and Snyderman, S. E. (Department of Pediatrics, New York University Medical Center, New York, USA) Cystine deficiency during dietotherapy of homocystinemia. Acta Paediatr Scand 64:215, 1975.–Cystine deficiency was inadvertently produced in a boy receiving specific dietary therapy for homocystinuria. This was manifested as a loss in weight, the reappearance of significant amounts of homocystine in the plasma and urine, and the elevation of the plasma methionine level. In addition, there was a significant reduction in the level of cystine in the plasma. This reduction in plasma cystine level differentiates cystine deficiency from loss of biochemical control due to failure to keep the prescribed diet. The addition of cystine to the regime of this child, without any other dietary modification, resulted in a complete remission.