TRANSIENT HYPER-AMMONEMIA OF PRETERM INFANT
TRANSIENT HYPER-AMMONEMIA OF PRETERM INFANT
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DOI：
10.1056/nejm197810262991704
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发表时间：
1978-01-01
影响因子：
158.5
通讯作者：
NYHAN, WL
中科院分区：
文献类型：
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作者：
BALLARD, RA;VINOCUR, B;NYHAN, WL
描述了 5 名早产儿（妊娠 34-36 周），他们在出生后 48 小时内患上严重疾病。每个人都有轻度呼吸窘迫，并在 48 小时内发展为深度昏迷，需要呼吸机辅助。血浆中的氨浓度范围为844-7640μg/dl。 4人接受换血和腹膜透析；氨值在 72 小时内恢复到正常范围 (< 150 μg/dl)，并且即使在蛋白质攻击后仍保持在正常范围。这 4 只随后正常进食和发育。第 5 个婴儿在未尝试降低血浆氨的情况下死亡。在这名婴儿（以及其他两名婴儿）的肝组织中测量的尿素循环酶处于正常范围。原因不明的暂时性高氨血症可能是新生儿高氨血症中相对常见的一种；它对及时诊断和积极治疗反应良好。
Five preterm infants (34-36 wk gestation) in whom an overwhelming illness developed within the 1st 48 h of life are described. Each had mild respiratory distress that progressed within 48 h to deep coma requiring ventilatory assistance. Ammonia concentrations in the plasma ranged from 844-7640 .mu.g/dl. Four received exchange transfusion and peritoneal dialysis; ammonia values returned to the normal range (< 150 .mu.g/dl) within 72 h and remained there even after protein challenge. These 4 subsequently fed and developed normally. The 5th infant died without an attempt to lower plasma ammonia. In this infant (and 2 of the others) urea-cycle enzymes measured in liver tissue were in the normal range. Transient hyperammonemia of unknown cause may be a relatively common variety of neonatal hyperammonemia; it responds well to prompt diagnosis and aggressive therapy.