LIVER-DISEASE IN CHILDREN WITH ALPHA1-ANTITRYPSIN DEFICIENCY WITHOUT NEONATAL CHOLESTASIS
LIVER-DISEASE IN CHILDREN WITH ALPHA1-ANTITRYPSIN DEFICIENCY WITHOUT NEONATAL CHOLESTASIS
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DOI：
10.1111/j.1651-2227.1982.tb09563.x
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发表时间：
1982-01-01
期刊：
影响因子：
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通讯作者：
STRANDVIK, B
中科院分区：
文献类型：
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作者：
NEMETH, A;STRANDVIK, B
有。alpha的13个孩子。1-抗胰蛋白酶缺乏症（8 - PiSZ和5 - PiSZ）发生在4-6岁。没有新生儿胆汁淤积症。9例患者血清转氨酶浓度升高，主要为PiZ个体。7例血清转氨酶升高的患者行肝活检。其中1例有肝硬化，4例有中度至重度纤维化。1-抗胰蛋白酶缺乏的个体，也没有新生儿胆汁淤积综合征，在儿童时期就有发展成严重肝脏疾病的高风险。肝硬化患者是唯一一个尿中胆汁酸排泄量增加的患者。
Thirteen children with .alpha.1-antitrypsin deficiency (8 PiZ and 5 PiSZ) were investigated at ages ranging from 4-6. None had had neonatal cholestasis. Nine, mainly the PiZ individuals, had increased serum concentration of transaminases. Liver biopsy was performed in 7 patients with increased serum levels of transaminases. One of these patients had cirrhosis and 4 had moderate to severe fibrosis. .alpha.1-Antitrypsin deficient individuals, also without neonatal cholestasis syndrome run a high risk of developing serious liver disease, already in childhood. The cirrhotic patient was the only one who had increased excretion of bile acids in urine.