Nishioka J, Mizushima T, Ono K
Clin Orthop Relat Res. 1979 May(140):194-203.
The therapeutic effectiveness of leucocyte transfusion (LT) was compared with that of plasma infusion (PI) clinically by range of motion (ROM) of joints and biochemically from the standpoint of alpha-L-iduronidase activity and urinary excretion of acid mucopolysaccharides (AMPS) in 2 patients with Hurler's and Scheie's syndromes. Both syndromes are considered to be due to the lack of alpha-L-iduronidase activity, a congenital metabolic disorder. As a result, leukocyte transfusion surpasses plasma infusion with respect to enzyme content, the grade and duration of clinical improvement in the stiffness of joints. Clinical improvement in the stiffness of joints was correlated with the degradation of AMPS when the ratio of urinary AMPS fragments to the total large molecule AMPS has become 50% or more after the leucocyte transfusion and plasma infusion.
从关节活动范围（ROM）方面进行临床比较，并从α-L-艾杜糖醛酸酶活性以及酸性粘多糖（AMPS）尿排泄量方面进行生化比较，研究白细胞输注（LT）与血浆输注（PI）对2例Hurler综合征和Scheie综合征患者的治疗效果。这两种综合征均被认为是由于缺乏α-L-艾杜糖醛酸酶活性所致的先天性代谢紊乱。结果表明，在酶含量、关节僵硬临床改善的程度和持续时间方面，白细胞输注优于血浆输注。当白细胞输注和血浆输注后尿AMPS片段与总大分子AMPS的比例达到50%或更高时，关节僵硬的临床改善与AMPS的降解相关。
