Features of Alagille syndrome in 92 patients: Frequency and relation to prognosis
Features of Alagille syndrome in 92 patients: Frequency and relation to prognosis
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DOI：
10.1002/hep.510290331
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发表时间：
1999-03-01
期刊：
影响因子：
13.5
通讯作者：
Piccoli, DA
中科院分区：
文献类型：
--
作者：
Emerick, KM;Rand, EB;Piccoli, DA
我们对92例阿拉杰里综合征（AGS）患者进行了研究，以确定临床表现的频率，并将临床发现与预后相关联。肝活检标本显示85%的患者小叶间胆管稀少。96%的患者出现胆汁淤积，97%有心脏杂音，51%有蝴蝶椎，78%有后胚胎环，96%有特征性面容。40%的患者存在肾脏疾病，14%的患者发生颅内出血或中风。心内先天性心脏病的存在是唯一与死亡率增加有统计学关联的临床特征（P<0.001）。（最后一个P后面似乎内容不完整）
We have studied 92 patients with Alagille syndrome (AGS) to determine the frequency of clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy specimens showed paucity of the interlobular ducts in 85% of patients. Cholestasis was seen in 96%, cardiac murmur in 97%, butterfly vertebrae in 51%, posterior embryotoxon in 78%, and characteristic facies in 96% of patients. Renal disease was present in 40% and intracranial bleeding or stroke occurred in 14% of patients. The presence of intracardiac congenital heart disease was the only clinical feature statistically associated with increased mortality (P