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[Porphyria cutanea uraemica: an obligate systemic disease in chronic kidney insufficiency?].

作者信息

Senger E, Bernd A, Kachel H G, Schoeppe W, Holzmann H

机构信息

Zentrum der Dermatologie und Venerologie, Abteilung Dermatologie I, Johann Wolfgang Goethe-Universität Frankfurt/Main.

出版信息

Hautarzt. 1991 Dec;42(12):764-9.

PMID:1765490
Abstract

Patients with chronic renal failure, especially those receiving maintenance haemodialysis, have a number of dermatological alterations. Some of them are similar to those seen in porphyria cutanea tarda (PCT). Whereas early studies showed normal plasma porphyrin levels, a striking elevation of plasma porphyrins, and particularly of uroporphyrin, has recently been found. We measured the concentrations of porphyrins in the plasma and erythrocytes of 55 patients with chronic renal failure and receiving maintenance haemodialysis and also in 7 patients with PCT and 100 healthy volunteers. The mean concentration of porphyrins in plasma was 2.7-fold, and the maximum concentration, 6-fold the highest value measured in plasma of controls. The mean plasma concentration of uroporphyrin was 6-fold higher up to a maximum value of 37-fold the upper limit of the controls. The plasma porphyrin values of 3 of 7 patients with PCT were on the same high level as those measured in patients undergoing haemodialysis. The mean porphyrin concentration in the erythrocytes of haemodialysis patients were 1.5-fold the control values. Because of the known pathophysiological effect of uroporphyrin, especially its stimulation of the collagen synthesis and the activation of the complement system, we suppose that the porphyria cutanea tarda like skin lesions in patients with chronic renal failure are due to the highly increased uroporphyrin concentration.

摘要

相似文献

1
[Porphyria cutanea uraemica: an obligate systemic disease in chronic kidney insufficiency?].
Hautarzt. 1991 Dec;42(12):764-9.
2
Porphyrin studies in chronic renal failure patients on maintenance hemodialysis.维持性血液透析慢性肾衰竭患者的卟啉研究
Photodermatol. 1987 Feb;4(1):14-22.
3
Porphyria cutanea tarda associated with chronic renal disease and hemodialysis.迟发性皮肤卟啉症与慢性肾病及血液透析相关。
Arch Dermatol. 1980 Feb;116(2):191-5.
4
Bullous dermatosis of end-stage renal disease: a possible association between abnormal porphyrin metabolism and aluminium.
Nephrol Dial Transplant. 1996 Sep;11(9):1787-91.
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[Porphyria cutanea tarda in a chronic hemodialysis patient].
Nihon Hifuka Gakkai Zasshi. 1989 Jun;99(7):819-26.
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Porphyria cutanea tarda (PCT) in a patient with chronic renal failure on haemodialysis.
Br J Dermatol. 1981 May;104(5):575-8. doi: 10.1111/j.1365-2133.1981.tb08174.x.
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Correlation between levels of free and protein-bound plasma porphyrin and urinary porphyrins in porphyria cutanea tarda.迟发性皮肤卟啉症中游离和蛋白结合血浆卟啉水平与尿卟啉之间的相关性。
Int J Biochem Cell Biol. 1995 Jun;27(6):585-8. doi: 10.1016/1357-2725(95)00017-J.
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Hemodialysis-related porphyria cutanea tarda--treatment failure with charcoal hemoperfusion.血液透析相关迟发性皮肤卟啉病——血液灌流治疗失败
Photodermatol. 1986 Jun;3(3):169-73.
9
[Porphyria cutanea tarda, pseudoporphyria and hemodialysis of chronic renal failure patients].
Z Gesamte Inn Med. 1986 Jan 15;41(2):50-2.
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Cutaneous abnormalities and metabolic disturbance of porphyrins in patients on maintenance haemodialysis.维持性血液透析患者的皮肤异常与卟啉代谢紊乱
Clin Exp Dermatol. 1997 May;22(3):124-7.

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