Hyperammonemic crises in patients with urea cycle disorders on chronic nitrogen scavenger therapy with either sodium phenylbutyrate or glycerol phenylbutyrate

Objective:
Hyperammonemic crises (HACs) in patients with urea cycle disorders (UCDs) are associated with mortality and intellectual/cognitive decline. Fasting ammonia correlates with daily ammonia exposure and the risk and rate of HACs. UCD patients receiving sodium phenylbutyrate (NaPBA) and switched to glycerol phenylbutyrate (GPB) in three short-term comparative studies were examined over a 12-month period during dosing with GPB to identify characteristics associated with HACs.
Methods:
UCD patients completing long term studies (n=100) and those who had HACs were examined for the following: 1) Demographics, 2) Incidence and rate of HAC, 3) Time to first HAC, and 4) Duration of, and hospitalization for, HACs. Comparisons were reported for the pre-study and the 12 month follow-up study.

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