Basile Carlo, Montanaro Alessio, Masi Maria, Pati Gina, De Maio Pasquale, Gismondi Antonio
Division of Nephrology, Hospital of Martina Franca, Italy.
J Nephrol. 2002 Nov-Dec;15(6):676-80.
Calcific uremic arteriolopathy (CUA), also referred to as calciphylaxis, is a syndrome of small vessel calcification of unknown etiology causing painful violaceous skin lesions that progress to non-healing ulcers and gangrene. It is observed mainly in patients with end-stage renal disease, is associated with high morbidity and mortality and has no standard treatment at the present time. Although parathyroidectomy (PTX) has been advocated in some cases, other studies have not found this effective. Hyperbaric oxygen therapy (HOT) consists of breathing 100% O2 at higher than ambient pressure, with the patient inside a sealed chamber. HOT has been used with some success in the treatment of selected problem wounds (those that fail to respond to established medical and surgical management). They are often severely hypoxic; restoration of tissue PO2 to normal or above-normal enhances fibroblast proliferation and collagen production as well as angiogenesis. The present is the largest retrospective case series of CUA treated by means of HOT reported so far and comprises 11 chronic uremic patients on dialysis (9 hemo- and 2 peritoneal dialysis, 6 females and 5 males, mean age 56 +/- 7 SD years, time on dialysis 163 +/- 84 SD months). Four patients had biopsy-proven CUA; 3 had diabetic nephropathy as a cause of uremia; 2 were obese and 3 had a consistent increase of serum calcium x phosphorus product; 3 patients had severe secondary hyperparathyroidism (II(nd) HPTH) and two had been submitted to subtotal PTX some years before CUA; two others had already had the limb amputated. Lesions were in the legs, except for one in a hand, and were prevalently ulcers and necrosis. The number of sessions in each HOT cycle ranged from a minimum of 20 to a maximum of 108 (mean 40.6 +/- 29.0). The results of two therapies cannot be evaluated (one was interrupted by the patient after 10 sessions, and one ended with the death of the patient due to ventricular arrhythmia after eight sessions). Eight of the nine remaining had excellent results with healing of the skin ulcers, but the ninth got worse, making it advisable to amputate the foot. In conclusion, CUA appears to result from a multitude of predisposing and/or sensitizing events that are commonly present in the uremic milieu. The specific factors that induce this disorder in an individual patient are not known. The present retrospective study supports a role of HOT in many cases of CUA, especially considering that, in the absence of severe II(nd) HPTH, there are very few therapeutic options.
钙化性尿毒症小动脉病(CUA),也被称为钙化防御,是一种病因不明的小血管钙化综合征,可导致疼痛性紫蓝色皮肤病变,进而发展为不愈合的溃疡和坏疽。它主要在终末期肾病患者中观察到,与高发病率和死亡率相关,目前尚无标准治疗方法。尽管在某些情况下有人主张进行甲状旁腺切除术(PTX),但其他研究并未发现其有效。高压氧疗法(HOT)是让患者在密封舱内呼吸高于常压的100%氧气。HOT在治疗某些特定的疑难伤口(那些对既定的药物和手术治疗无反应的伤口)方面已取得了一定成功。这些伤口往往严重缺氧;将组织氧分压恢复到正常或高于正常水平可促进成纤维细胞增殖、胶原蛋白生成以及血管生成。本文是迄今为止报道的最大规模的关于采用HOT治疗CUA的回顾性病例系列,包括11例接受透析的慢性尿毒症患者(9例血液透析和2例腹膜透析,6例女性和5例男性,平均年龄56±7标准差岁,透析时间163±84标准差月)。4例患者经活检证实为CUA;3例因糖尿病肾病导致尿毒症;2例肥胖,3例血清钙×磷乘积持续升高;3例患者有严重的继发性甲状旁腺功能亢进(II级HPTH),2例在CUA发生前数年已接受次全PTX;另外2例已进行了肢体截肢。病变除1例在手外,其余均在腿部,主要为溃疡和坏死。每个HOT周期的治疗次数最少为20次,最多为108次(平均40.6±29.0)。两种治疗结果无法评估(1例患者在10次治疗后中断,1例患者在8次治疗后因室性心律失常死亡)。其余9例中有8例取得了极佳效果,皮肤溃疡愈合,但第9例病情恶化,建议截肢。总之,CUA似乎是由尿毒症环境中常见的多种易感和/或致敏事件引起的。导致个体患者发生这种疾病的具体因素尚不清楚。本回顾性研究支持HOT在许多CUA病例中的作用,特别是考虑到在没有严重II级HPTH的情况下,治疗选择非常有限。