Successful extracorporeal treatment of a male with hyperammonaemic coma. |
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| Authors: | Maria Haller Angelika Henzler-Le Boulanger J?rn Oliver Sass Matthias Brandis Lothar Bernd Zimmerhackl |
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| Affiliation: | Zentrum für Kinderheilkunde und Jugendmedizin, Albert-Ludwigs-Universit?t, Freiburg, Germany. |
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| Abstract: | | Introduction | Children with urea cycle disorders present with hyperammonaemiaand its non-specific symptoms. Acute hyperammonaemia is a medicalemergency as the developmental and neurological outcome dependson the duration of hyperammonaemic coma [1]. The longer endogenousprotein catabolism continues, the more ammonia will be producedand accumulate and the greater is the risk of coma. To minimizepermanent brain damage, early diagnosis and appropriate therapyis mandatory. With diagnosis of hyperammonaemia, it is essentialto differentiate between urea cycle defects and other causesof encephalopathy. In Figure 1 a practicable flowchart for establishingthe correct diagnosis is depicted [2]. The emergency therapyin children with inborn metabolic disorders presenting
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| Keywords: | citrullinaemia haemodiafiltration inborn errors of metabolism intensive care nutrition peritoneal dialysis |
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