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. Author manuscript; available in PMC: 2015 Aug 1.
Published in final edited form as: J Pediatr. 2014 May 29;165(2):401–403.e3. doi: 10.1016/j.jpeds.2014.04.012

Figure 4.

Figure 4

In subject 3, the 3-day trial of NCG showed an encouraging biochemical response that a longer trial was planned. Following baseline (day 0) measurement of ammonia, urea, and amino acids, she was prescribed NCG 100 mg/kg/d.

When a marked decrease in ammonia and glutamine was again observed, the sodium phenylbutyrate (Buphenyl) daily dose was tapered in 500 mg decrements over the ensuing 6 weeks. In a single instance (day 10), due to parental error, the phenylbutyrate dose was unintentionally reduced by 1500 mg/day, and this error was not identified until the subsequent clinical assessment. She remained clinically well throughout.