α-1抗胰蛋白酶遗传性缺陷与儿童慢性肝脏和呼吸道疾病相关。
Inherited defect of alpha-1-antitrypsin associated with chronic liver and respiratory tract diseases in children.
作者信息
Rujner J, Madaliński K, Socha J, Piłacik B
机构信息
Department of Gastroenterology, Children's Memorial Hospital, Warsaw.
出版信息
Arch Immunol Ther Exp (Warsz). 1989;37(5-6):665-7.
The frequency of occurrence of alpha-1-antitrypsin (A1AT) deficiency among total of 3228 Polish children with chronic liver diseases and chronic disease of respiratory tract was determined. It was observed that among children with chronic liver diseases which disclosed more frequent defect (concentration of A1AT below 150 mg/dl was found in 10.3% of children), the highest occurrence of deficiency was in children with neonatal hepatitis (23.1%). The deficiency was connected with the presence of ZZ and MZ phenotypes of A1AT.
在3228名患有慢性肝病和慢性呼吸道疾病的波兰儿童中，测定了α-1抗胰蛋白酶（A1AT）缺乏症的发生率。观察到，在慢性肝病患儿中（更频繁出现缺陷，10.3%的儿童A1AT浓度低于150mg/dl），新生儿肝炎患儿中缺乏症的发生率最高（23.1%）。这种缺乏症与A1AT的ZZ和MZ表型的存在有关。
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