Francois B, Cornu G, de Meyer R
Arch Dis Child. 1976 Mar;51(3):228-31. doi: 10.1136/adc.51.3.228.
Peritoneal dialysis rapidly reduced blood ammonia concentration in this child with arginino-succinic acid-lyase deficiency, whereas exchange transfusion did not. Yet this reduction in plasma ammonia level did not produce clinical improvement. We speculate that the effects of ammonia intoxication on the highly susceptible neonatal metabolism are due to an accumulation of toxic products and to an altered energy metabolism. Both aspects must be considered in any attempt to treat congenital hyperammonaemia.
腹膜透析迅速降低了这名患有精氨酸琥珀酸裂解酶缺乏症儿童的血氨浓度，而换血疗法却未能达到这一效果。然而，血浆氨水平的这种降低并未带来临床症状的改善。我们推测，氨中毒对高度易损的新生儿代谢产生影响是由于有毒产物的蓄积以及能量代谢的改变。在任何治疗先天性高氨血症的尝试中，都必须考虑到这两个方面。
