Cystic kidney disease in a patient with systemic toxicity from long-term D-penicillamine use.
Cystic kidney disease in a patient with systemic toxicity from long-term D-penicillamine use.
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因长期使用 D-青霉胺而出现全身毒性的囊性肾病患者。
DOI：
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发表时间：
2013
影响因子：
13.2
通讯作者：
N. Dahl
中科院分区：
文献类型：
--
作者：
F. Koraishy;R. Cohen;G. Israel;N. Dahl
用于治疗胱氨酸尿症的D-青霉胺已知会导致胶原蛋白沉积受损和弹性纤维功能障碍。D-青霉胺也与肾小球异常有关，典型的是膜性肾小球肾炎。我们描述了一个严重的双侧囊性肾病患者，长期使用D-青霉胺治疗胱氨酸尿症后发展。在急性肾损伤评价期间观察到肾囊肿。患者在肾功能正常时，既往肾成像没有囊肿证据。同时，他出现了D-青霉胺毒性的多器官表现，包括皮肤拉克萨和穿透性弹性组织变性的皮肤表现。因此，停止D-青霉胺治疗，之后囊性肾病的进展逐渐停止，沿着其他全身毒性表现。据我们所知，这是第一次报告囊性肾病与长期d-青霉胺治疗有关，可能是由长期d-青霉胺治疗引起的。提出的囊肿形成机制是d-青霉胺导致肾细胞外基质功能障碍，导致肾损伤后修复过程受损。
D-penicillamine, used to treat cystinuria, is known to cause impaired collagen deposition and dysfunction in elastic fibers. D-penicillamine also has been associated with glomerular abnormalities, typically membranous glomerulonephritis. We describe a patient with severe bilateral cystic kidney disease that developed after long-term D-penicillamine use for treatment of cystinuria. The cysts in the kidneys were noted during an evaluation for acute kidney injury. The patient had no evidence of cysts on prior renal imaging at a time when his kidney function was normal. Simultaneously, he presented with multiorgan manifestations of D-penicillamine toxicity, including the skin findings of cutix laxa and elastosis perforans serpiginosa. Consequently, D-penicillamine treatment was discontinued, after which the progression of cystic kidney disease gradually ceased, along with the other systemic manifestations of toxicity. To our knowledge, this is the first report of cystic kidney disease associated with and perhaps caused by long-term d-penicillamine therapy. The proposed mechanism of cyst formation is the malfunction of the extracellular matrix of the kidney by d-penicillamine that leads to an impaired repair process after kidney injury.
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进行性系统性硬化症患者青霉胺诱发的快速进展性肾小球肾炎：两名患者的成功治疗和文献综述。
DOI：
10.1016/s0272-6386(86)80019-1
发表时间：
1986
期刊：
American journal of kidney diseases : the official journal of the National Kidney Foundation
影响因子：
--
作者：
Ntoso,KA;Tomaszewski,JE;Jimenez,SA;Neilson,EG
通讯作者：
Neilson,EG
影响因子：
7.5
作者：
Seeger-Nukpezah, Tamina;Golemis, Erica A.
通讯作者：
Golemis, Erica A.
影响因子：
14
作者：
Subramanian, Balajikarthick;Ko, Wei-Che;Yadav, Vikas;DesRochers, Teresa M.;Perrone, Ronald D.;Zhou, Jing;Kaplan, David L.
通讯作者：
Kaplan, David L.
影响因子：
19.6
作者：
Evan, A. P.;Coe, F. L.;Worcester, E. M.
通讯作者：
Worcester, E. M.
影响因子：
3.5
作者：
Takakura, Ayumi;Contrino, Leah;Zhou, Jing
通讯作者：
Zhou, Jing