Metabolism
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An unconscious child with hyperammonaemia and keto-acidosis
At the
age of 28 weeks Angela was admitted to the Emergency Department at her local
hospital in a coma, having suffered a convulsion after feeding. She had a mild
infection and slight fever at the time.
Since birth she had been a sickly child, and she frequently vomited and became drowsy after feeding. She was bottle fed and at one time cows’ milk allergy was suspected, although the problems persisted when she was fed on a soya-milk substitute. She had always shown poor muscle tone, and indeed at times was described as a "floppy baby", unable to hold her head up unsupported.
On admission she was moderately hypoglycaemic (plasma glucose 2.8 mmol /), ketotic and her plasma pH was 7.29. Analysis of a blood sample showed normal levels of insulin, but considerable hyperammonaemia (plasma ammonium ion concentration 500 µmol/L; reference range 40 - 80 µmol/L).