Baylin S B, Rosenstein B J, Marton L J, Lockwood D H
Pediatr Res. 1980 Aug;14(8):921-5. doi: 10.1203/00006450-198008000-00005.
Serum putrescine (Pu), spermidine (Sd), spermine (Sp), and plasma diamine oxidase ativity (DAO) were determined in cystic fibrosis (CF) homozygotes and heterozygotes plus age-matched controls. Mean (+/- S.E.) levels of Pu (0.51 +/- 0.06 nmoles/ml), Sd (0.63 +/- 0.07 nmoles/ml), and Sp (0.33 +/- 0.08 nmoles/ml) were higher (P < 0.001) in 17 control children versus 16 healthy adults (Pu = 0.15 +/- 0.04; Sd = 0.14 +/- 0.03; Sp = 0 in all). All 4 CF adults tested, but only 3 of 24 CF children, had increased polyamines relative to age-matched controls. Pu (0.33 +/- 0.04), Sd (0.33 +/- 0.05) and Sp (0.16 +/- 0.12) were increased (P < 0.001) in 11 adult heterozygotes; in turn, Pu (0.56 +/- 0.11; P < 0.025) and Sd (0.86 +/- 0.22; P < 0.005) were higher in the adult CF patients than in the heterozygotes. Plasma DAO was higher (P < 0.001) in 45 control children (9.3 +/- 0.7 units/ml) versus 61 healthy adults (5.7 +/- 0.07). Levels greater than or equal to +2 S.D. for controls occurred in 8 (21%) of 39 CF patients. Plasma DAO (19 +/- 4.8) was increased (P < 0.001) in 27 adult heterozygotes; 10 (37%) had values greater than or equal to +2 S.D. for normal adults. One or more polyamines and/or DAO were increased in each of 11 heterozygotes in whom all parameters were measured. These composite abnormalities might be considered as a potential means for identification of asymptomatic CF heterozygotes.
在囊性纤维化(CF)纯合子、杂合子以及年龄匹配的对照组中测定了血清腐胺(Pu)、亚精胺(Sd)、精胺(Sp)和血浆二胺氧化酶活性(DAO)。17名对照儿童的Pu(0.51±0.06纳摩尔/毫升)、Sd(0.63±0.07纳摩尔/毫升)和Sp(0.33±0.08纳摩尔/毫升)的平均(±标准误)水平高于16名健康成年人(Pu = 0.15±0.04;Sd = 0.14±0.03;Sp在所有成年人中均为0)(P < 0.001)。所有4名接受检测的CF成年患者,但24名CF儿童中只有3名,相对于年龄匹配的对照组,其多胺水平升高。11名成年杂合子的Pu(0.33±0.04)、Sd(0.33±0.05)和Sp(0.16±0.12)升高(P < 0.001);反过来,成年CF患者的Pu(0.56±0.11;P < 0.025)和Sd(0.86±0.22;P < 0.005)高于杂合子。45名对照儿童的血浆DAO(9.3±0.7单位/毫升)高于61名健康成年人(5.7±0.07)(P < 0.001)。39名CF患者中有8名(21%)的水平大于或等于对照组的+2标准差。27名成年杂合子的血浆DAO(19±4.8)升高(P < 0.001);10名(37%)的值大于或等于正常成年人的+2标准差。在11名测量了所有参数的杂合子中,每一名的一种或多种多胺和/或DAO都升高了。这些综合异常情况可被视为识别无症状CF杂合子的一种潜在手段。