Behandlung des hyperammonamischen komas bei neugeborenen und sauglingen durch hamodialyse oder hamofiltration

Inborn errors of the urea cycle and of the organic acid metabolism can cause acute hyperammonemia in neonates which, if untreated, leads to coma, severe brain damage or death. These complications, however, can be prevented by proper diagnosis and early therapeutic intervention. Rapid decline in ammonia blood levels are essential and can be achieved by the following four therapeutic principles 1, restriction of protein intake (while maintaining essential amino acids), 2. reduction of protein catabolism by a hypercaloric diet, 3. activation of alternative pathways of ammonia elimination, and 4. elimination of ammonia from the circulation by dialysis. We report on 4 cases who were submitted because of acute hyperammonemic coma. All patients were treated immediately after diagnosis with hemodialysis (HD) or hemofiltration (HF). In all 4 patients blood ammonia levels were rapidly decreased to normal values leading to significant improvements of the neurologic state. The long term outcomes were, however, determined by the underlying metabolic disorder. Discussion: Our case reports confirm the effectiveness of HD and HF in the treatment of acute hyperammonemic coma. Since the diagnosis and the prognosis of metabolic disorders are rarely known at the time when the patient becomes symptomatic and needs therapy, any neonate with acute hyperammonemia should be referred to a special care unit where technical facilities for extracorporal dialysis are available.