Infectious Precipitants of Acute Hyperammonemia Are Associated with Indicators of Increased Morbidity in Patients with Urea Cycle Disorders - 23/11/13
, Hye-Seung Lee, PhD 2, members of the Urea Cycle Disorders Consoritum∗
Marshall L. Summar, MD, PhD 3Abstract |
Objective |
To prospectively characterize acute hyperammonemic episodes in patients with urea cycle disorders (UCDs) in terms of precipitating factors, treatments, and use of medical resources.
Study design |
This was a prospective, longitudinal observational study of hyperammonemic episodes in patients with UCD enrolled in the National Institutes of Health–sponsored Urea Cycle Disorders Consortium Longitudinal Study. An acute hyperammonemic event was defined as plasma ammonia level >100 μmol/L. Physician-reported data regarding the precipitating event and laboratory and clinical variables were recorded in a central database.
Results |
In our study population, 128 patients with UCD experienced a total of 413 hyperammonemia events. Most patients experienced between 1 and 3 (65%) or between 4 and 6 (23%) hyperammonemia events since study inception, averaging fewer than 1 event/year. The most common identifiable precipitant was infection (33%), 24% of which were upper/lower respiratory tract infections. Indicators of increased morbidity were seen with infection, including increased hospitalization rates (P = .02), longer hospital stays (+2.0 days; P = .003), and increased use of intravenous ammonia scavengers (+45%-52%; P = .003-.03).
Conclusion |
Infection is the most common precipitant of acute hyperammonemia in patients with UCD and is associated with indicators of increased morbidity (ie, hospitalization rate, length of stay, and use of intravenous ammonia scavengers). These findings suggest that the catabolic and immune effects of infection may be a target for clinical intervention in inborn errors of metabolism.
Le texte complet de cet article est disponible en PDF.Keyword : IV, LOS, OTC, RDCRN, UCD, UCDC
Plan
| Funded by the National Institute of Child Health and Human Development (U54RR19454 and U54HD061221) and the National Institutes of Health (NIH) Office of Rare Diseases Research, and the intramural research program of the NIH (to P.M.). The Urea Cycle Disorders Consortium also is supported by the O'Malley Foundation, the Rotenberg Family Fund, the Dietmar-Hopp Foundation, and the Kettering Fund. The views expressed in written materials or publications do not necessarily reflect the official policies of the Department of Health and Human Services; nor does mention by trade names, commercial practices, or organizations imply endorsement by the US Government. The Urea Cycle Disorders Consortium is a part of the NIH Rare Diseases Clinical Research Network. The authors declare no conflicts of interest. |
Vol 163 - N° 6
P. 1705 - décembre 2013 Retour au numéro
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