Khurana Anand, Runge Val M, Narayanan Mohanram, Greene John F, Nickel Allan E
Department of Nephrology, Scott and White Clinic and Hospital, Texas A&M University Health Science Center, Temple, Texas 76508, USA.
Invest Radiol. 2007 Feb;42(2):139-45. doi: 10.1097/01.rli.0000253505.88945.d5.
Nephrogenic systemic fibrosis (NSF) is a rare acquired disorder that was first recognized in 1997. Presented is a retrospective review of 6 cases of NSF diagnosed by skin biopsy in our institution during the past 4 years and their relationship to gadodiamide exposure.
Patient age ranged from 23 to 71 years. The onset of symptoms consistent with NSF was between 19 days and 2 months after gadodiamide exposure. Five patients had severe renal impairment and started dialysis around the period of gadodiamide exposure (1 day before the 37 days after contrast administration). The sixth patient had a clotted access at the time of a contrast-enhanced magnetic resonance venogram and was hence not being adequately dialyzed. The dose of gadodiamide ranged from 16 to 40 mL (0.11 to 0.36 mmol/kg). Despite having normal serum bicarbonate, 5 of the 6 patients had an elevated anion gap metabolic acidosis.
In our 6 patients, all had either failing native or transplant kidneys at the time of gadodiamide exposure. The development of NSF was temporally related to gadodiamide injection, suggesting as the etiology dechelation of the agent and thus emphasizing the need for change in clinical practice.
肾源性系统性纤维化(NSF)是一种罕见的后天性疾病,于1997年首次被认识。本文对我院在过去4年中经皮肤活检确诊的6例NSF病例及其与钆双胺暴露的关系进行了回顾性研究。
患者年龄在23至71岁之间。与NSF一致的症状发作于钆双胺暴露后19天至2个月之间。5例患者有严重肾功能损害,并在钆双胺暴露期间(造影剂给药后1天至37天)开始透析。第6例患者在进行对比增强磁共振静脉造影时血管通路堵塞,因此透析不充分。钆双胺剂量为16至40毫升(0.11至0.36毫摩尔/千克)。尽管血清碳酸氢盐正常,但6例患者中有5例存在阴离子间隙代谢性酸中毒。
在我们的6例患者中,所有患者在钆双胺暴露时均有自身肾脏功能衰竭或移植肾失败。NSF的发生与钆双胺注射在时间上相关,提示该药物的去螯合作用是病因,从而强调临床实践需要改变。