| Literature DB >> 1764145 |
Abstract
A patient with Lesch-Nyhan syndrome has had 3 recurrent episodes of coma, each associated with an acute illness. Extensive investigation for known causes of coma has failed to yield a diagnosis. Although coma is not generally recognized as a feature of Lesch-Nyhan syndrome, similar patients have been reported previously. This and other episodic phenomena observed in Lesch-Nyhan syndrome may be explained by the disruption of cellular energy metabolism due to purine depletion, consequent to lack of the purine salvage pathway normally provided by the hypoxanthine-guanine-phosphoribosyl-transferase enzyme.Entities:
Mesh:
Year: 1991 PMID: 1764145 DOI: 10.1016/0887-8994(91)90073-t
Source DB: PubMed Journal: Pediatr Neurol ISSN: 0887-8994 Impact factor: 3.372