Clinical features and outcome of eight infants with intrahepatic porto‐venous shunts detected in neonatal screening for galactosaemia
Clinical features and outcome of eight infants with intrahepatic porto‐venous shunts detected in neonatal screening for galactosaemia
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新生儿半乳糖血症筛查中发现 8 例肝内门静脉分流婴儿的临床特征和结果
DOI：
10.1111/j.1651-2227.1998.tb01521.x
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
N. Sakura
中科院分区：
文献类型：
--
作者：
H. Ono;H. Mawatari;N. Mizoguchi;T. Eguchi;N. Sakura
在18例发现持续性半乳糖血症且无酶缺乏的新生儿中，肝内门-静脉（P - V）分流是8例的病因。我们回顾性分析了8例患儿的临床及生化特征。4例患者接受泼尼松龙治疗，其中1例因动静脉分流导致心力衰竭，同时接受肝动脉栓塞治疗。其余4例患者仅进行观察，未接受药物治疗。无论采用何种治疗方法，5名1岁前的婴儿P - V分流消失，另外3名持续存在。所有婴儿在肝功能检查中均显示轻度或中度异常。没有人表现出与分流相关的高氨血症或神经精神症状。数据表明，婴儿肝内P - V分流的预后通常是良好的。在没有P - V分流相关并发症的情况下，除了半乳糖消除饮食外，没有其他治疗方法。
Of 18 newborn infants found to have persistent galactosaemia and without enzyme deficiencies, intrahepatic porto‐venous (P‐V) shunts were the cause in 8 cases. We retrospectively analysed the clinical and biochemical features of the eight infants. Four patients received prednisolone, one of whom with heart failure owing to arteriovenous shunts also underwent hepatic arterial embolization. The other four patients were merely observed without receiving drug therapy. Regardless of treatment, the P‐V shunts disappeared in five infants before the age of 1 y and persisted in three others. All infants showed mild or moderate abnormalities in liver function tests. None exhibited hyperammonemia or neuropsychiatric symptoms related to the shunts. The data indicated that the prognosis of infants with intrahepatic P‐V shunts is generally good. In the absence of complications related to the P‐V shunts, no treatment other than galactose elimination diet is indicated.