Short-term Supplementation Therapy Does Not Affect Elastin Degradation in Severe α1-Antitrypsin Deficiency
Short-term Supplementation Therapy Does Not Affect Elastin Degradation in Severe α1-Antitrypsin Deficiency
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短期补充疗法不会影响严重 α1-抗胰蛋白酶缺乏症中弹性蛋白的降解
DOI：
10.1164/ajrccm.162.6.2002032
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发表时间：
2000
影响因子：
24.7
通讯作者：
G. Snider
中科院分区：
文献类型：
--
作者：
D. Gottlieb;M. Luisetti;P. Stone;L. Allegra;J. CANTEY;C. Grassi;G. Snider
我们对8名男性和4名女性因先天AAT严重不足(17-69 mg/dl)所致的肺气肿患者静脉补充α1-抗胰蛋白酶(AAT)治疗降低尿桥蛋白排泄率(DES)的能力进行了评估。9人以前吸烟，2人现在吸烟，1人报告从不吸烟；他们的平均年龄为54岁(SD 12)，他们的平均FEV1是预测值的41(18%)。采用同位素稀释法和高效液相色谱法测定尿DES。在补充AAT之前，DES的平均排泄量为13.0μg/g肌酐，比健康的非吸烟者高73%。在8wk补充治疗期间，平均尿DES排泄量为13.0(5.9)μg/g肌酐，与基线时期持平(重复测量方差分析p=0.85)。我们的结论是，严重AAT缺乏的肺气肿患者的弹性蛋白降解基线水平异常高，8wk的AAT补充治疗不会对患者产生不良影响。
We evaluated the ability of intravenous supplementation therapy with α1-antitrypsin (AAT) to reduce the rate of urinary excretion of desmosine (DES), a specific marker of elastin degradation, in eight men and four women with emphysema due to severe, congenital deficiency of AAT (range 17–69 mg/dl). Nine were former cigarette smokers, two were current smokers, and one reported never smoking; their mean age was 54 (SD 12) yr and their mean FEV1 was 41 (18%) of predicted. Urinary DES was measured by isotope dilution and HPLC. Prior to the start of AAT supplementation, mean DES excretion was 13.0 (5.0) μ g/g creatinine, 73% higher than in healthy nonsmokers. During 8 wk of supplementation therapy, mean urinary DES excretion was 13.0 (5.9) μ g/g creatinine, unchanged from the baseline period (p = 0.85 by repeated measures ANOVA). We conclude that baseline levels of elastin degradation in emphysematous patients with severe AAT deficiency were abnormally high and that 8 wk of AAT supplementation therapy did not ...
弹性蛋白酶调节其抑制剂（α1-蛋白酶抑制剂）的合成，并夸大纯合子 PiZZ α1 PI 缺陷的缺陷。
DOI：
10.1172/jci113519
发表时间：
1988
期刊：
The Journal of clinical investigation
影响因子：
--
作者：
Perlmutter,DH;Travis,J;Punsal,PI
通讯作者：
Punsal,PI
饮食对锁链素和羟赖氨酰吡啶啉尿排泄的影响。
DOI：
10.1164/ajrccm.149.1.8111578
发表时间：
1994
影响因子：
24.7
作者：
Stone,PJ;Lucey,EC;Snider,GL;Franzblau,C
通讯作者：
Franzblau,C
DOI：
10.1164/ajrccm/144.2.284
发表时间：
1991-08
期刊：
The American review of respiratory disease
影响因子：
--
作者：
P. Stone;J. Bryan-Rhadfi;E. Lucey;D. Ciccolella;George Crombie;B. Faris;G. Snider;C. Franzblau
通讯作者：
P. Stone;J. Bryan-Rhadfi;E. Lucey;D. Ciccolella;George Crombie;B. Faris;G. Snider;C. Franzblau