Acute treatment
Introduction to acute treatment
An acute hyperammonaemic episode can occur at any
age, even after diagnosis. The goal of treatment for all
patients during an episode is to reduce concentrations
of plasma ammonia. This is best achieved by quickly
providing the patient with adequate energy to reduce
catabolism and promote anabolism, by temporarily
restricting protein intake, and through the use of
N-scavenging drugs (Summar 2001).
The following five patients exemplify common
presentations of a hyperammonaemic episode: a
4-day-old healthy boy who becomes acutely lethargic
and develops seizures and coma; a 5-year-old boy who
presents with sleepiness; a 10-year-old girl who arrives
at the emergency department with ataxia and disorientation;
a 23-year-old woman who becomes comatose
during the delivery of her first child; and a 30-year-old
woman who becomes comatose after eating a highprotein
diet following gastric bypass surgery. Given the
broad range of these episodes, it is essential to target
nutritional management individually. This section will
focus on the treatment of infants suffering an acute
episode, because these cases are the ones most frequently
encountered during initial diagnosis. The principles
for both children and adults are similar, however.