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囊性纤维化杂合子和纯合子中与年龄相关的循环多胺和二胺氧化酶活性异常。

Age-related abnormalities of circulating polyamines and diamine oxidase activity in cystic fibrosis heterozygotes and homozygotes.

作者信息

Baylin S B, Rosenstein B J, Marton L J, Lockwood D H

出版信息

Pediatr Res. 1980 Aug;14(8):921-5. doi: 10.1203/00006450-198008000-00005.

DOI:10.1203/00006450-198008000-00005
PMID:6775274
Abstract

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.

摘要

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。

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Age-related abnormalities of circulating polyamines and diamine oxidase activity in cystic fibrosis heterozygotes and homozygotes.囊性纤维化杂合子和纯合子中与年龄相关的循环多胺和二胺氧化酶活性异常。
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