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螯合疗法对威尔逊病氨基酸尿症和肽尿症的影响。

The effect of chelation therapy on the amino aciduria and peptiduria of Wilson's disease.

作者信息

Asatoor A M, Milne M D, Walshe J M

出版信息

J R Coll Physicians Lond. 1983 Apr;17(2):122-5.

PMID:6842414
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5370932/
Abstract
  1. There is an excess urinary output of free amino acids and of urinary peptides in most cases of untreated Wilson's disease. Studies of 11 patients have shown that both these abnormalities are greatly improved by two years of standard chelation therapy. 2. The reduction in excretion of both free amino acids and peptides is purely quantitative, there being no significant change in the percentage composition of amino acids, either free or combined, in relation to their total urinary output. 3. Arguments are advanced that the peptiduria of the disease is usually due to a proximal renal tubular reabsorption defect, but in rare cases it may be due to excess bone breakdown or even to a combination of bone and renal tubular disease in the same patient.
摘要
  1. 在大多数未经治疗的威尔逊氏病病例中,游离氨基酸和尿肽的尿量排出过多。对11名患者的研究表明,经过两年的标准螯合疗法,这两种异常情况都有了很大改善。2. 游离氨基酸和肽类排泄量的减少纯粹是数量上的,游离或结合形式的氨基酸相对于其总尿量的百分比组成没有显著变化。3. 有人提出,该病的肽尿通常是由于近端肾小管重吸收缺陷,但在罕见情况下,可能是由于骨分解过多,甚至是同一患者的骨病和肾小管疾病共同作用所致。
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0c3/5370932/8a493858dcf6/jrcollphyslond90320-0038-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0c3/5370932/8a493858dcf6/jrcollphyslond90320-0038-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0c3/5370932/8a493858dcf6/jrcollphyslond90320-0038-a.jpg

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1
The effect of chelation therapy on the amino aciduria and peptiduria of Wilson's disease.螯合疗法对威尔逊病氨基酸尿症和肽尿症的影响。
J R Coll Physicians Lond. 1983 Apr;17(2):122-5.
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Urinary excretion of peptides and of hydroxyproline in Wilson's disease.威尔逊病患者尿液中肽和羟脯氨酸的排泄情况。
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引用本文的文献

1
Kidney involvement in Wilson's disease: a review of the literature.威尔逊病的肾脏受累:文献综述
Clin Kidney J. 2024 Mar 9;17(4):sfae058. doi: 10.1093/ckj/sfae058. eCollection 2024 Apr.

本文引用的文献

1
The familial nature of the amino-aciduria of Wilson's disease (hepatolenticular degeneration).威尔逊氏病(肝豆状核变性)氨基酸尿症的家族性本质。
Am J Med Sci. 1952 Apr;223(4):392-400. doi: 10.1097/00000441-195204000-00006.
2
Wilson's disease and the Fanconi syndrome.威尔逊病与范科尼综合征。
Q J Med. 1962 Jul;31:361-84.
3
Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride.用二盐酸三乙撑四胺(曲恩汀)治疗威尔逊氏病。
Lancet. 1982 Mar 20;1(8273):643-7. doi: 10.1016/s0140-6736(82)92201-2.
4
The separation of peptides from amino acids in urine by ligand exchange chromatography.通过配体交换色谱法从尿液中的氨基酸中分离肽。
J Chromatogr. 1967 Aug;29(2):398-402. doi: 10.1016/s0021-9673(00)92686-4.
5
Renal function in Wilson's disease: response to penicillamine therapy.威尔逊病的肾功能:对青霉胺治疗的反应
Am J Med Sci. 1970 Dec;260(6):381-98. doi: 10.1097/00000441-197012000-00007.
6
Renal clearances of different amino acids in Wilson's disease before and after treatment with penicillamine.青霉胺治疗前后威尔逊病患者不同氨基酸的肾脏清除率
Acta Med Scand. 1971 Nov;190(5):395-9. doi: 10.1111/j.0954-6820.1971.tb07448.x.
7
Effect of penicillamine on failure of renal acidification in Wilson's disease.青霉胺对威尔逊病肾酸化功能衰竭的影响。
Lancet. 1968 Apr 13;1(7546):775-8. doi: 10.1016/s0140-6736(68)92227-7.
8
Urinary excretion of peptides and of hydroxyproline in Wilson's disease.威尔逊病患者尿液中肽和羟脯氨酸的排泄情况。
Clin Sci Mol Med. 1976 Oct;51(4):369-78. doi: 10.1042/cs0510369.