Marckmann P, Skov L, Rossen K, Thomsen H S
Department of Nephrology, Copenhagen University Hospital Herlev, Denmark.
Clin Nephrol. 2008 Mar;69(3):161-8. doi: 10.5414/cnp69161.
To further characterize the clinical signs and symptoms of nephrogenic systemic fibrosis, a new and serious disease affecting renal failure patients and caused by some Gd-containing contrast agents, including gadodiamide.
22 cases of gadodiamide-related nephrogenic systemic fibrosis followed at the nephrology department of Copenhagen University Hospital Herlev.
Retrospective cohort study based on medical records, personal interviews and physical examinations.
Typical first signs of the disease were skin discoloration, induration and warmth, itching, constant pain and other neuropathic symptoms localized to the lower legs. First sign appeared in a median of 14 days (range 0 â 53 days) after gadodiamide exposure. Associated early symptoms included sleeplessness and transient, diffuse hair loss. The predominant late symptom was symmetrical skin stiffness of extremities with or without restricted joint motion. Ten of 22 patients (45, 95% CI: 27 â 66%) were severely disabled due to contractures on the average of 29 months after being exposed to gadodiamide. Four patients died (18, 95% CI: 6 â 41). Patients perceived that intensive physiotherapy was effective in limiting disabling contractures.
Signs and symptoms of nephrogenic systemic fibrosis vary over time and between patients. The disease leads to severe disability in a significant proportion of affected patients. Intensive physiotherapy may limit the development of contractures.
进一步描述肾源性系统性纤维化的临床体征和症状,这是一种影响肾衰竭患者的新型严重疾病,由包括钆双胺在内的一些含钆造影剂引起。
哥本哈根大学医院赫勒夫肾病科随访的22例与钆双胺相关的肾源性系统性纤维化病例。
基于病历、个人访谈和体格检查的回顾性队列研究。
该病典型的首发体征为皮肤变色、硬结、发热、瘙痒、持续性疼痛及其他局限于小腿的神经病变症状。首发体征出现在接触钆双胺后的中位时间为14天(范围0至53天)。相关的早期症状包括失眠和短暂性弥漫性脱发。主要的晚期症状为四肢对称性皮肤僵硬,伴或不伴关节活动受限。22例患者中有10例(45%,95%可信区间:27%至66%)在接触钆双胺后平均29个月因挛缩而严重致残。4例患者死亡(18%,95%可信区间:6%至41%)。患者认为强化物理治疗对限制致残性挛缩有效。
肾源性系统性纤维化的体征和症状随时间和患者个体而变化。该疾病在相当比例的受影响患者中导致严重残疾。强化物理治疗可能会限制挛缩的发展。