Beddis I R, Hughes E A, Rosser E, Fenton J C
Arch Dis Child. 1980 Jul;55(7):516-20. doi: 10.1136/adc.55.7.516.
A fatal case associated with severe hyperammonaemia is described in which no urea cycle enzyme deficiency could be found. This prompted further investigation of blood ammonia levels in neonates admitted to the premature baby unit at Hammersmith Hospital. 102 specimens were taken from 42 babies within the first 3 weeks of life; the babies had a variety of clinical conditions. The mean ammonia level was 94.5 mumol/l (132.3 micrograms/100 ml) (range 32-255 mumol/l) (44.8-357 micrograms/ml), SD +/- 41.0). These results, although higher than the range for older babies in hospital, were not as high as in the baby with severe hyperammonaemia. Serial levels in 10 babies suggested that the range of blood ammonia levels was greatest in the first 2 weeks of life and narrowed considerably after this period. Great care is needed in collecting blood samples and measuring them if accuate results are to be obtained.
本文描述了一例与严重高氨血症相关的致命病例,其中未发现尿素循环酶缺乏。这促使对哈默史密斯医院早产儿病房收治的新生儿血氨水平进行进一步调查。在出生后的前三周内,从42名婴儿身上采集了102份样本;这些婴儿患有多种临床病症。血氨平均水平为94.5微摩尔/升(132.3微克/100毫升)(范围为32 - 255微摩尔/升)(44.8 - 357微克/毫升),标准差±41.0)。这些结果虽然高于住院较大婴儿的范围,但不如患有严重高氨血症的婴儿那么高。对10名婴儿的连续检测结果表明,血氨水平范围在出生后的前两周最大,在此之后大幅缩小。如果要获得准确结果,采集血样和测量时需要格外小心。