Abstract | BACKGROUND: METHODS: RESULTS: Overall survival was 84% (250 of 299 patients). Ninety-six percent of the patients survived episodes of hyperammonemia (1132 of 1181 episodes). Patients over 30 days of age were more likely than neonates to survive an episode (98% vs. 73%, P<0.001). Patients 12 or more years of age (93 patients), who had 437 episodes, were more likely than all younger patients to survive (99%, P<0.001). Eighty-one percent of patients who were comatose at admission survived. Patients less than 30 days of age with a peak ammonium level above 1000 micromol per liter (1804 microg per deciliter) were least likely to survive a hyperammonemic episode (38%, P<0.001). Dialysis was also used in 56 neonates during 60% of episodes and in 80 patients 30 days of age or older during 7% of episodes. CONCLUSIONS:
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Authors | Gregory M Enns, Susan A Berry, Gerard T Berry, William J Rhead, Saul W Brusilow, Ada Hamosh |
Journal | The New England journal of medicine
(N Engl J Med)
Vol. 356
Issue 22
Pg. 2282-92
(May 31 2007)
ISSN: 1533-4406 [Electronic] United States |
PMID | 17538087
(Publication Type: Clinical Trial, Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Copyright | Copyright 2007 Massachusetts Medical Society. |
Chemical References |
- Phenylacetates
- Ammonia
- Urea
- phenylacetic acid
- Sodium Benzoate
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Topics |
- Adolescent
- Adult
- Age Factors
- Age of Onset
- Amino Acid Metabolism, Inborn Errors
(blood, drug therapy, mortality)
- Ammonia
(blood)
- Carbamoyl-Phosphate Synthase I Deficiency Disease
(drug therapy)
- Child
- Child, Preschool
- Citrullinemia
(drug therapy)
- Female
- Humans
- Hyperammonemia
(drug therapy, etiology, mortality, therapy)
- Infant
- Infant, Newborn
- Male
- Ornithine Carbamoyltransferase Deficiency Disease
(drug therapy)
- Phenylacetates
(adverse effects, therapeutic use)
- Sodium Benzoate
(adverse effects, therapeutic use)
- Survival Analysis
- Urea
(metabolism)
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