[Fulminant coma: think hyperammonemia and urea cycle disorders].
The authors report the case of 14-year-old boy admitted for acute coma without neurological focal symptom. The only relevant finding was the death of one uncle after a coma in the year 1992. This coma was associated with an ammonia blood level of 344 mumol l-1 and it rapidly lead to cerebral death despite a symptomatic treatment. The diagnosis of hereditary ornithine transcarbamylase deficiency was confirmed by liver biopsy in the immediate post-mortem period. The authors recommend the measurement of blood ammonia in every coma without diagnosis, whatever patient's age.
Augris
C
C
Service d'anesthésie, centre hospitalier de Nevers, avenue Colbert, 58000 Nevers, France.
Jouvet
P
P
Benabdelmalek
F
F
Vauquelin
P
P
Caramella
J P
JP
D000293:Adolescent; D000641:Ammonia / Q000097:blood; D003128:Coma / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D005190:Family; D017809:Fatal Outcome; D006801:Humans; D022124:Hyperammonemia / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D014508:Urea / Q000378:metabolism*
D000641:Ammonia; D014508:Urea